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    There are 30 cases available...

    Go to the top of the page   ID: 20110407230957 Original case in english  More links about this topic on Pubmed (PubMed Reader)
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    Discussion
     
    Polysplenia Syndrome with azygous continuation of IVC, intestinal malrotation, short pancreas, and pre-duodenal portal vein
    Lance Dorsey, Adam Patterson. Polysplenia Syndrome with azygous continuation of IVC, intestinal malrotation, short pancreas, and pre-duodenal portal vein. PedRad [serial online] vol 11, no. 4.
    URL: www.PedRad.info/?search=20110407230957


     
     Pediatric Radiology CasesImages to this case: There are CT-images available for this case. [ CT ] View all modalities [ All ]   
     Pediatric Radiology CasesAuthor/s:

    Lance Dorsey (University of Missouri/Columbia/USA), Adam Patterson (University of Missouri/Columbia/USA)  

     
     Pediatric Radiology CasesEmail Address:

    Viewable for logged on visitors (Log on)  

     
     Pediatric Radiology CasesAge:

    15 Years  

     
     Pediatric Radiology CasesGender:

    Female  

     
     Pediatric Radiology CasesRegion-Organ:

    Syndromes  

     
     Pediatric Radiology CasesMost likely etiology:

    congenital  

     
     Pediatric Radiology CasesHistory:

    15 year old female presents to the ER with nausea and mid-epigastric pain with some radiation to the back which began this morning. Patient also reports headache. No other complaints and complete ROS is otherwise negative. There is an uncertain reported past medical history of "colonic malrotation." Physical exam demonstrates some mild generalized tenderness in the mid upper abdomen, but otherwise unremarkable. Labs were sent and were within normal limits, including wbc count and amylase/lipase. An abdominal CT was then performed with oral and IV contrast.  

     
     Pediatric Radiology CasesPathomorphology or Pathophysiology of this disease :

    Polysplenia syndrome is a rare congenital anomaly which which may be sporadic or familial. Polysplenia syndrome is often associated with multiple cardiopulmonary and abdominal anomalies. This disorder is commonly seen/categoried in association with the spectrum of heterotaxy or cardiospenic syndromes.

    The most common cardiac malformations seen with polysplenia are acyanotic L-R shunt lesions including ASD, PAPVC, and AV canal defects. The cardiac defects associated with asplenia are usually more complex with greater morbidity. Abscence of the suprarenal/intrahepatic IVC is also typical with azygous or hemiazygous continuation. Common abdominal anomalies include intestinal malrotation, abdominal heterotaxy, central location of the liver, pre-duodenal portal vein, short pancreas and agenesis of the gallbladder.  

     
     Pediatric Radiology CasesRadiological findings:


    CT 1 <- view CT 1

    CT 1: Axial CT of the upper abdomen demonstrates multiple spleens of different sizes in the posterior LUQ.



    CT 2 <- view CT 2

    CT 2: Axial CT shows enlarged azygous vein adjacent to the aorta in the lower chest, representing azygous continuation of the abdominal IVC.



    CT 3 <- view CT 3

    CT 3: Coronal CT shows the multiple spleens in the LUQ and azygous continuation of the IVC paralleling the aorta along the right.





    CT 4 <- view CT 4

    CT 4: Axial CT of the mid-abdomen demonstrates short pancreas with no visualized distal body or tail of the pancreas. The contrast opacified vessel seen anterior to the shorted pancreas represents a pre-duodenal portal vein. Also, again noted are several spleens in the left abdomen anterior and lateral to the left kidney.




    CT 5 <- view CT 5

    CT 5: Axial CT of the abdomen shows the small bowel entirely within the right abdomen and the colon located only with the left abdomen. There is also abnormal orientation of the the mesenteric vessels visualized with the superior mesenteric vein seen to the left of the superior mesenteric artery.



    CT 6 <- view CT 6

    CT 6: Axial CT of the lower abdomen demonstrates small bowel within the right lower quadrant with abdnormal location of the cecum and appendix identified within the left lower quadrant.

     

     
     Pediatric Radiology CasesDiagnosis confirmation:

    Laboratory diagnostics  

     
     Pediatric Radiology CasesWhich DD would be also possible with the radiological findings:

    N/A  

     
     Pediatric Radiology CasesCourse / Prognosis / Frequency / Other :

    Polysplenia syndrome is more common in females compared with males. Patients without severe cardiac defects often don't present until adolescence or early adulthood and sometimes are only incidentally discovered. These individuals have a better prognosis. However, polysplenia patients that do have significant cardiopulmonary anomalies can present early and often do not survive. Typically patients with asplenia present as neonates/infants due to very severe cardiac malformations. They have a poor prognosis with very high mortality rate in the first few years of life.  

     
     Pediatric Radiology CasesComments of the author about the case:

    This was an incidental finding discovered on the abdominal CT scan and no findings to explain the patient's acute symptoms were found. The patient's pain subsided and she was discharged from the emergency room in good condition. Follow-up evaluation for possible cardiac anomalies was recommended, however the patient has not returned to our institution for any further workup/evaluation.  

     
     Pediatric Radiology CasesFirst description / History:

    N/A  

     
     Pediatric Radiology CasesLiterature:

    1. Medline: Medline
    Joseph C Turbyville, MD
    Pediatric Asplenia
    Emedicine.com

    2. Medline: Medline
    Michael P. Federle, MD, FACR
    Asplenia and Polysplenia
    statdx.com  

     
     Pediatric Radiology CasesKeywords:

    polysplenia, asplenia, malrotation, azygous continuation, heterotaxy  

     
     Pediatric Radiology Cases Cite this article:

    Lance Dorsey, Adam Patterson. Polysplenia Syndrome with azygous continuation of IVC, intestinal malrotation, short pancreas, and pre-duodenal portal vein. PedRad [serial online] vol 11, no. 4.
    URL: www.PedRad.info/?search=20110407230957  

     
     Pediatric Radiology Cases Read similar articles: heterotaxy&type=1-17">corresponding keywords
    in the same field: Syndromes
    or in the region: Syndromes
    or in the tissue/organ:
    or with the etiology: congenital
     
     Pediatric Radiology CasesImages to this case: There are CT-images available for this case. [ CT ] View all modalities [ All ]   
     
    Polysplenia Syndrome with azygous continuation of IVC, intestinal malrotation, short pancreas, and pre-duodenal portal vein
    Lance Dorsey, Adam Patterson. Polysplenia Syndrome with azygous continuation of IVC, intestinal malrotation, short pancreas, and pre-duodenal portal vein. PedRad [serial online] vol 11, no. 4.
    URL: www.PedRad.info/?search=20110407230957


     

    Search similar cases in:
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    Polysplenia Syndrome with azygous continuation of IVC, intestinal malrotation, short pancreas, and pre-duodenal portal vein
    Other cases by these authors:

    Search Lance Dorsey in Medline Lance Dorsey (1)   
    Search Adam Patterson in Medline Adam Patterson (1)   

    Polysplenia Syndrome with azygous continuation of IVC, intestinal malrotation, short pancreas, and pre-duodenal portal vein  
     
    Polysplenia Syndrome with azygous continuation of IVC, intestinal malrotation, short pancreas, and pre-duodenal portal vein
    Lance Dorsey, Adam Patterson. Polysplenia Syndrome with azygous continuation of IVC, intestinal malrotation, short pancreas, and pre-duodenal portal vein. PedRad [serial online] vol 11, no. 4.
    URL: www.PedRad.info/?search=20110407230957


     

    Which diagnosis have other collegues guessed?


    • Polysplenia Syndrome
      Votes: 7 (35 %)


    • Intestinal malrotation
      Votes: 1 (5 %)


    • Pre-duodenal portal vein
      Votes: 0 (0 %)


    • None of the above
      Votes: 0 (0 %)


    • All of the above
      Votes: 12 (60 %)



        Total answers: 20

     
    Polysplenia Syndrome with azygous continuation of IVC, intestinal malrotation, short pancreas, and pre-duodenal portal vein
    Lance Dorsey, Adam Patterson. Polysplenia Syndrome with azygous continuation of IVC, intestinal malrotation, short pancreas, and pre-duodenal portal vein. PedRad [serial online] vol 11, no. 4.
    URL: www.PedRad.info/?search=20110407230957


     

    Medical Dictionary
    Search in medical dictionary for
    Or type in a keyword
    ( Provided by The On-Line Medical Dictionary )




    1 = less interesting)
     
    Polysplenia Syndrome with azygous continuation of IVC, intestinal malrotation, short pancreas, and pre-duodenal portal vein
    Lance Dorsey, Adam Patterson. Polysplenia Syndrome with azygous continuation of IVC, intestinal malrotation, short pancreas, and pre-duodenal portal vein. PedRad [serial online] vol 11, no. 4.
    URL: www.PedRad.info/?search=20110407230957


     


    Go to the top of the page   ID: 20110329123519 Original case in english  More links about this topic on Pubmed (PubMed Reader)
    Add this case to your RSS feeder: Subscribe to RSS feed Add to Yahoo Add to Google Add to AOL Add to Furl Subscribe to Feed Burner feed

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    Discussion
     
    Pneumomediastinum/pneumothorax - iatrogenic
    Kraig J. Pneumomediastinum/pneumothorax - iatrogenic. PedRad [serial online] vol 11, no. 3.
    URL: www.PedRad.info/?search=20110329123519


     
     Pediatric Radiology CasesImages to this case: There are X-Ray-images available for this case. [ X-Ray ] View all modalities [ All ]   
     Pediatric Radiology CasesAuthor/s:

    Kraig J. Lage (University of Missouri Hospital and Clinics / Columbia / MO / United States)  

     
     Pediatric Radiology CasesEmail Address:

    Viewable for logged on visitors (Log on)  

     
     Pediatric Radiology CasesAge:

    N/A  

     
     Pediatric Radiology CasesGender:

    Male  

     
     Pediatric Radiology CasesRegion-Organ:

    N/A  

     
     Pediatric Radiology CasesMost likely etiology:

    N/A  

     
     Pediatric Radiology CasesHistory:

    Newborn male (39 weeks gestation) who had a forceps assisted delivery secondary to nonreassuring fetal heart tones. At delivery, infant was cyanotic, had poor tone, and no cry. Infant was dried, warmed, suctioned, and stimulated without response. Bag/mask ventillation was administered with response of heart rate rising to greater than 100. Bag/mask ventillation was continued for one minute until spontaneous respirations. Infant continued to have increased work of breathing with flaring, retractions, and tachypnea. CPAP was continued with decreased work of breathing.  

     
     Pediatric Radiology CasesPathomorphology or Pathophysiology of this disease :

    Alveolar rupture is caused by a pressure gradient between the alveolus and the surrounding interstitium. The pressure gradient is a product of either hyperinflation of the alveolus or a decrease in the surrounding interstitial pressure.

    The air forced into the interstitial tissues tracts centrally toward the peribronchial and perivascular tissue, and may continue into the mediastinum, neck and subcutaneous tissues.

    Because not all alveoli are ruptured, adjacent normal alveoli cause an equalisation of pressure between the affected and damaged alveoli with the result that the interalveolar walls remain intact and the lungs inflated.  

     
     Pediatric Radiology CasesRadiological findings:


    CT 1 <- view CT 1

    CT 1: Axial CT of the upper abdomen demonstrates multiple spleens of different sizes in the posterior LUQ.



    CT 2 <- view CT 2

    CT 2: Axial CT shows enlarged azygous vein adjacent to the aorta in the lower chest, representing azygous continuation of the abdominal IVC.



    CT 3 <- view CT 3

    CT 3: Coronal CT shows the multiple spleens in the LUQ and azygous continuation of the IVC paralleling the aorta along the right.





    CT 4 <- view CT 4

    CT 4: Axial CT of the mid-abdomen demonstrates short pancreas with no visualized distal body or tail of the pancreas. The contrast opacified vessel seen anterior to the shorted pancreas represents a pre-duodenal portal vein. Also, again noted are several spleens in the left abdomen anterior and lateral to the left kidney.




    CT 5 <- view CT 5

    CT 5: Axial CT of the abdomen shows the small bowel entirely within the right abdomen and the colon located only with the left abdomen. There is also abnormal orientation of the the mesenteric vessels visualized with the superior mesenteric vein seen to the left of the superior mesenteric artery.



    CT 6 <- view CT 6

    CT 6: Axial CT of the lower abdomen demonstrates small bowel within the right lower quadrant with abdnormal location of the cecum and appendix identified within the left lower quadrant.


    X-Ray 1 <- view X-Ray 1

    X-Ray 1: Large lucency surrounds the superior mediastinum with lucency crossing midline. Subtle lucency is also seen inferior and lateral of the right lung base at the costophrenic angle and hemidiaphragm.





    X-Ray 2 <- view X-Ray 2

    X-Ray 2: Large lucency surrounds the superior aspect of the anterior mediastinum with elevation of the thymic shadow.



     

     
     Pediatric Radiology CasesDiagnosis confirmation:

    N/A  

     
     Pediatric Radiology CasesWhich DD would be also possible with the radiological findings:

    N/A  

     
     Pediatric Radiology CasesCourse / Prognosis / Frequency / Other :

    Pneumomediastinum occurs in approximately 0.1% of neonates and carries a good prognosis, typically without complications or long term sequella.

    Factors predisposing to pneumomediastinum include pneumonia or meconium aspiration syndrome.

    Neonatal pneumomediastinum can be attributable to pulmonary infection, immature lungs and ventilatory support. However, a significant portion of cases of spontaneous pneumomediastinum occur without identifiable risk factors.  

     
     Pediatric Radiology CasesComments of the author about the case:

    Diagnosis of pneumomediastinum is confirmed by frontal CXR.

    Typical radiologic signs are:

    1. In infants, the “spinnaker sign” which is upwards and outwards displacement of thymic lobes raised above the heart by pneumomediastinal air that separates it from the underlying cardiac silhouette.

    2. "continuous diaphragm sign", visible as lucency interposed between the pericardium and the diaphragm.

    3. Linear bands of mediastinal air parallelling the left side of the heart and the descending aorta with the pleura seen as a thin line separated from the mediastinum by the air lucency. The air may extend superiorly along the great vessels into the neck.  

     
     Pediatric Radiology CasesFirst description / History:

    Neonate presenting with respiratory distress.  

     
     Pediatric Radiology CasesLiterature:

    3. Medline: Medline
    Doug Hacking, M.D., and Michael Stewart, M.D.
    Neonatal Pneumomediastinum
    N Engl J Med 2001 June 14; 344:1839

    4. Medline: Medline
    Annik Hauri-Hohl, Oskar Baenziger, and Bernhard Frey
    Pneumomediastinum in the neonatal and paediatric intensive care unit
    Eur J Pediatr. 2008 April; 167(4): 415–418

     

     
     Pediatric Radiology CasesKeywords:

    Pneumomediastinum, Pneumothorax, continuous diaphragm sign, spinnaker sign  

     
     Pediatric Radiology Cases Cite this article:

    Kraig J. Pneumomediastinum/pneumothorax - iatrogenic. PedRad [serial online] vol 11, no. 3.
    URL: www.PedRad.info/?search=20110329123519  

     
     Pediatric Radiology Cases Read similar articles: spinnaker sign&type=1-17">corresponding keywords
    in the same field:
    or in the region:
    or in the tissue/organ:
    or with the etiology:
     
     Pediatric Radiology CasesImages to this case: There are X-Ray-images available for this case. [ X-Ray ] View all modalities [ All ]   
     
    Pneumomediastinum/pneumothorax - iatrogenic
    Kraig J. Pneumomediastinum/pneumothorax - iatrogenic. PedRad [serial online] vol 11, no. 3.
    URL: www.PedRad.info/?search=20110329123519


     

    Search similar cases in:
    More links about this topic on Pubmed  More links about this topic on Yahoo  More links about this topic on Altavista  More links about this topic on MSN  More links about this topic on Google  More links about this topic on Fireball

    Peer-reviewed Radiology Search



    Pneumomediastinum/pneumothorax - iatrogenic
    Other cases by these authors:

    Search Lance Dorsey in Medline Lance Dorsey (1)   
    Search Adam Patterson in Medline Adam Patterson (1)   
    Search Kraig J. Lage in Medline Kraig J. Lage (17)   

    Pneumomediastinum/pneumothorax - iatrogenic  
     
    Pneumomediastinum/pneumothorax - iatrogenic
    Kraig J. Pneumomediastinum/pneumothorax - iatrogenic. PedRad [serial online] vol 11, no. 3.
    URL: www.PedRad.info/?search=20110329123519


     

    Which diagnosis have other collegues guessed?


    • Polysplenia Syndrome
      Votes: 7 (35 %)


    • Intestinal malrotation
      Votes: 1 (5 %)


    • Pre-duodenal portal vein
      Votes: 0 (0 %)


    • None of the above
      Votes: 0 (0 %)


    • All of the above
      Votes: 12 (60 %)


    • IRDS
      Votes: 1 (1 %)


    • Pneumothorax with pneumomediastinum
      Votes: 28 (50 %)


    • Pneumonia
      Votes: 1 (1 %)


    • Child abuse
      Votes: 1 (1 %)


    • Ebstein anomaly
      Votes: 4 (7 %)



        Total answers: 55

     
    Pneumomediastinum/pneumothorax - iatrogenic
    Kraig J. Pneumomediastinum/pneumothorax - iatrogenic. PedRad [serial online] vol 11, no. 3.
    URL: www.PedRad.info/?search=20110329123519


     

    Medical Dictionary
    Search in medical dictionary for
    Or type in a keyword
    ( Provided by The On-Line Medical Dictionary )




    1 = less interesting)
     
    Pneumomediastinum/pneumothorax - iatrogenic
    Kraig J. Pneumomediastinum/pneumothorax - iatrogenic. PedRad [serial online] vol 11, no. 3.
    URL: www.PedRad.info/?search=20110329123519


     


    Go to the top of the page   ID: 20091226123802 Original case in english  More links about this topic on Pubmed (PubMed Reader)
    Add this case to your RSS feeder: Subscribe to RSS feed Add to Yahoo Add to Google Add to AOL Add to Furl Subscribe to Feed Burner feed

    Bookmark and Share


    Discussion
     
    Choledochal cyst
    Sanjeeb Kumar. Choledochal cyst. PedRad [serial online] vol 9, no. 12.
    URL: www.PedRad.info/?search=20091226123802


     
     Pediatric Radiology CasesImages to this case: There are MRI-images available for this case. [ MRI ] View all modalities [ All ]   
     Pediatric Radiology CasesAuthor/s:

    Sanjeeb Kumar Sarma (Down Town Hospital / Guwahati / India)  

     
     Pediatric Radiology CasesEmail Address:

    Viewable for logged on visitors (Log on)  

     
     Pediatric Radiology CasesAge:

    10 Years  

     
     Pediatric Radiology CasesGender:

    Male  

     
     Pediatric Radiology CasesRegion-Organ:

    N/A  

     
     Pediatric Radiology CasesMost likely etiology:

    N/A  

     
     Pediatric Radiology CasesHistory:

    10 years old male patient complains of on and off right upper abdominal pain since last 3-4 months.  

     
     Pediatric Radiology CasesPathomorphology or Pathophysiology of this disease :

    Congenital dilatation of the biliary tract, so called choledochal cyst, is classified based on spectrum of morphologic changes in the bile ducts. The most common classification scheme of Alonso-Lej includes type I, which is dilatation of the CBD; type II, which is diverticulum of the CBD; and type III, which is a rare choledochocele. Todani and his colleagues further added to this classification type IV-A, representing multiple cysts of the IHBDs and EHBDs; type IV-B, representing multiple cysts of EHBDs; and type V, representing multiple cysts of the IHBDs , or Caroli disease.
    The choledochal cyst is not a true cyst of the biliary tract but rather some variation of duct dilatation. Etiology is unknown but supposedly multifactorial.
    On CT choledochal cyst can have varying appearance depending on the extent of ductal involvement and the degree of dilatation. There may only be mild EHBD dilatation or a large water density mass in porta hepatis or adjacent to the head of the pancreas.Reportedly, 60% of choledochal cyst will have associated congenital IHBD dilatation..Congenitally dilated IHBD often have a lobulated cystic appearance with an abrupt transition zone at the junction with the normal ducts.Acquired biliary dilatation usually doesn't have the lobulated cystic appearance and the dilated ducts usually taper gradually towards the periphery of the liver.Direct communication of the cystic duct to the dilated EHBDs will aid making the diagnosis of choledochal cyst.Ultrasonography aids to differentiate with other pathology.However, sometimes DISIDA scan may be required. CT and MR cholangiography are diagnostic.
    Here, is a case of type I disease with tiny calculi both within gall bladder and choledochal cyst.  

     
     Pediatric Radiology CasesRadiological findings:


    CT 1 <- view CT 1

    CT 1: Axial CT of the upper abdomen demonstrates multiple spleens of different sizes in the posterior LUQ.



    CT 2 <- view CT 2

    CT 2: Axial CT shows enlarged azygous vein adjacent to the aorta in the lower chest, representing azygous continuation of the abdominal IVC.



    CT 3 <- view CT 3

    CT 3: Coronal CT shows the multiple spleens in the LUQ and azygous continuation of the IVC paralleling the aorta along the right.





    CT 4 <- view CT 4

    CT 4: Axial CT of the mid-abdomen demonstrates short pancreas with no visualized distal body or tail of the pancreas. The contrast opacified vessel seen anterior to the shorted pancreas represents a pre-duodenal portal vein. Also, again noted are several spleens in the left abdomen anterior and lateral to the left kidney.




    CT 5 <- view CT 5

    CT 5: Axial CT of the abdomen shows the small bowel entirely within the right abdomen and the colon located only with the left abdomen. There is also abnormal orientation of the the mesenteric vessels visualized with the superior mesenteric vein seen to the left of the superior mesenteric artery.



    CT 6 <- view CT 6

    CT 6: Axial CT of the lower abdomen demonstrates small bowel within the right lower quadrant with abdnormal location of the cecum and appendix identified within the left lower quadrant.


    X-Ray 1 <- view X-Ray 1

    X-Ray 1: Large lucency surrounds the superior mediastinum with lucency crossing midline. Subtle lucency is also seen inferior and lateral of the right lung base at the costophrenic angle and hemidiaphragm.





    X-Ray 2 <- view X-Ray 2

    X-Ray 2: Large lucency surrounds the superior aspect of the anterior mediastinum with elevation of the thymic shadow.




    MRI 1 <- view MRI 1

    MRI 1: Axial T2 sections showing dilated CBD with intraluminal sludge and calculi.





    MRI 2 <- view MRI 2

    MRI 2: Axial T2 sections showing multiple small calculi in gall bladder neck (arrow).





    MRI 3 <- view MRI 3

    MRI 3: Axial T2 sections showing gall bladder ( short arrow) and a cystic structure (long arrow)





    MRI 4 <- view MRI 4

    MRI 4: Axial T2 sections showing a cystic structure with luminal sludge and the gall bladder.





    MRI 5 <- view MRI 5

    MRI 5: Axial T2 sections showing dilated CBD anterior to IVC.




    MRI 6 <- view MRI 6

    MRI 6: MRCP showing gall bladder with calculi (short arrow) and a cystic structure (long arrow).





    MRI 7 <- view MRI 7

    MRI 7: MRCP showing both gall bladder and a cystic structure.





    MRI 8 <- view MRI 8

    MRI 8: MRCP showing small calculi in a cystic structure (arrow)



     

     
     Pediatric Radiology CasesDiagnosis confirmation:

    N/A  

     
     Pediatric Radiology CasesWhich DD would be also possible with the radiological findings:

    Gastro-intestinal tract duplication cyst, mesenteric cyst, hepatic cysts, pseudocysts, ovarian cysts, and renal cysts.  

     
     Pediatric Radiology CasesCourse / Prognosis / Frequency / Other :

    If untreated bile stasis, stone formation,infection , pancreatitis,biliary cirrhosis, and portal hypertension may occur.Reported increased incidence of hepatobiliary malignancy.  

     
     Pediatric Radiology CasesComments of the author about the case:

    Surgical treatment was done with improvement and follow up is done on regular basis.  

     
     Pediatric Radiology CasesFirst description / History:

    N/A  

     
     Pediatric Radiology CasesLiterature:

    5. Medline: Medline
    1.Computed tomography and magnetic resonence imaging of the whole body by John R Haaga, Charles F Lanzeri--4th Ed.Mosby 3003.  

     
     Pediatric Radiology CasesKeywords:

    Choledochal, choledochocele, IHBD, EHBD, CBD.  

     
     Pediatric Radiology Cases Cite this article:

    Sanjeeb Kumar. Choledochal cyst. PedRad [serial online] vol 9, no. 12.
    URL: www.PedRad.info/?search=20091226123802  

     
     Pediatric Radiology Cases Read similar articles: CBD.&type=1-17">corresponding keywords
    in the same field:
    or in the region:
    or in the tissue/organ:
    or with the etiology:
     
     Pediatric Radiology CasesImages to this case: There are MRI-images available for this case. [ MRI ] View all modalities [ All ]   
     
    Choledochal cyst
    Sanjeeb Kumar. Choledochal cyst. PedRad [serial online] vol 9, no. 12.
    URL: www.PedRad.info/?search=20091226123802


     

    Search similar cases in:
    More links about this topic on Pubmed  More links about this topic on Yahoo  More links about this topic on Altavista  More links about this topic on MSN  More links about this topic on Google  More links about this topic on Fireball

    Peer-reviewed Radiology Search



    Choledochal cyst
    Other cases by these authors:

    Search Lance Dorsey in Medline Lance Dorsey (1)   
    Search Adam Patterson in Medline Adam Patterson (1)   
    Search Kraig J. Lage in Medline Kraig J. Lage (17)   
    Search Sanjeeb Kumar Sarma in Medline Sanjeeb Kumar Sarma (24)   

    Choledochal cyst  
     
    Choledochal cyst
    Sanjeeb Kumar. Choledochal cyst. PedRad [serial online] vol 9, no. 12.
    URL: www.PedRad.info/?search=20091226123802


     

    Which diagnosis have other collegues guessed?


    • Polysplenia Syndrome
      Votes: 7 (35 %)


    • Intestinal malrotation
      Votes: 1 (5 %)


    • Pre-duodenal portal vein
      Votes: 0 (0 %)


    • None of the above
      Votes: 0 (0 %)


    • All of the above
      Votes: 12 (60 %)


    • IRDS
      Votes: 1 (1 %)


    • Pneumothorax with pneumomediastinum
      Votes: 28 (50 %)


    • Pneumonia
      Votes: 1 (1 %)


    • Child abuse
      Votes: 1 (1 %)


    • Ebstein anomaly
      Votes: 4 (7 %)



        Total answers: 55

     
    Choledochal cyst
    Sanjeeb Kumar. Choledochal cyst. PedRad [serial online] vol 9, no. 12.
    URL: www.PedRad.info/?search=20091226123802


     

    Medical Dictionary
    Search in medical dictionary for
    Or type in a keyword
    ( Provided by The On-Line Medical Dictionary )




    1 = less interesting)
     
    Choledochal cyst
    Sanjeeb Kumar. Choledochal cyst. PedRad [serial online] vol 9, no. 12.
    URL: www.PedRad.info/?search=20091226123802


     


    Go to the top of the page   ID: 20090514012315 Original case in german  More links about this topic on Pubmed (PubMed Reader)
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    Discussion
     
    Cerebellopontine Angle Lipoma
    Achint K. Cerebellopontine Angle Lipoma. PedRad [serial online] vol 9, no. 5.
    URL: www.PedRad.info/?search=20090514012315


     
     Pediatric Radiology CasesImages to this case: There are MRI-images available for this case. [ MRI ] View all modalities [ All ]   
     Pediatric Radiology CasesAuthor/s:

    Achint K Singh  

     
     Pediatric Radiology CasesEmail Address:

    Viewable for logged on visitors (Log on)  

     
     Pediatric Radiology CasesAge:

    13 Years  

     
     Pediatric Radiology CasesGender:

    Female  

     
     Pediatric Radiology CasesRegion-Organ:

    N/A  

     
     Pediatric Radiology CasesMost likely etiology:

    N/A  

     
     Pediatric Radiology CasesHistory: *

    13 year old female with history of hearing loss in right ear. Audiogram revealed moderate sensorineural hearing loss on right side.  

     
     Pediatric Radiology CasesPathomorphology or Pathophysiology of this disease : *

    Lipoma in the CPA are maldevelopmental masses arising from abnormal differentiation of the meningeal precursor tissue.  

     
     Pediatric Radiology CasesRadiological findings: *


    CT 1 <- view CT 1

    CT 1: Axial CT of the upper abdomen demonstrates multiple spleens of different sizes in the posterior LUQ.



    CT 2 <- view CT 2

    CT 2: Axial CT shows enlarged azygous vein adjacent to the aorta in the lower chest, representing azygous continuation of the abdominal IVC.



    CT 3 <- view CT 3

    CT 3: Coronal CT shows the multiple spleens in the LUQ and azygous continuation of the IVC paralleling the aorta along the right.





    CT 4 <- view CT 4

    CT 4: Axial CT of the mid-abdomen demonstrates short pancreas with no visualized distal body or tail of the pancreas. The contrast opacified vessel seen anterior to the shorted pancreas represents a pre-duodenal portal vein. Also, again noted are several spleens in the left abdomen anterior and lateral to the left kidney.




    CT 5 <- view CT 5

    CT 5: Axial CT of the abdomen shows the small bowel entirely within the right abdomen and the colon located only with the left abdomen. There is also abnormal orientation of the the mesenteric vessels visualized with the superior mesenteric vein seen to the left of the superior mesenteric artery.



    CT 6 <- view CT 6

    CT 6: Axial CT of the lower abdomen demonstrates small bowel within the right lower quadrant with abdnormal location of the cecum and appendix identified within the left lower quadrant.


    X-Ray 1 <- view X-Ray 1

    X-Ray 1: Large lucency surrounds the superior mediastinum with lucency crossing midline. Subtle lucency is also seen inferior and lateral of the right lung base at the costophrenic angle and hemidiaphragm.





    X-Ray 2 <- view X-Ray 2

    X-Ray 2: Large lucency surrounds the superior aspect of the anterior mediastinum with elevation of the thymic shadow.




    MRI 1 <- view MRI 1

    MRI 1: Axial T2 sections showing dilated CBD with intraluminal sludge and calculi.





    MRI 2 <- view MRI 2

    MRI 2: Axial T2 sections showing multiple small calculi in gall bladder neck (arrow).





    MRI 3 <- view MRI 3

    MRI 3: Axial T2 sections showing gall bladder ( short arrow) and a cystic structure (long arrow)





    MRI 4 <- view MRI 4

    MRI 4: Axial T2 sections showing a cystic structure with luminal sludge and the gall bladder.





    MRI 5 <- view MRI 5

    MRI 5: Axial T2 sections showing dilated CBD anterior to IVC.




    MRI 6 <- view MRI 6

    MRI 6: MRCP showing gall bladder with calculi (short arrow) and a cystic structure (long arrow).





    MRI 7 <- view MRI 7

    MRI 7: MRCP showing both gall bladder and a cystic structure.





    MRI 8 <- view MRI 8

    MRI 8: MRCP showing small calculi in a cystic structure (arrow)




    MRI 1 <- view MRI 1

    MRI 1: T1W MRI showing hyperintense mass in right CP angle cistern extending into the internal auditory canal.




    MRI 2 <- view MRI 2

    MRI 2: T2W MRI showing hyperintense mass with splaying of 7th and 8th cranial nerves.




    MRI 3 <- view MRI 3

    MRI 3: T1W post gadolinium fat suppressed image showing nonenhancing mass with fat suppression.



     

     
     Pediatric Radiology CasesDiagnosis confirmation:

    N/A  

     
     Pediatric Radiology CasesWhich DD would be also possible with the radiological findings: *

    N/A  

     
     Pediatric Radiology CasesCourse / Prognosis / Frequency / Other : *

    It usually does not grow over time and malignant transformation has not been reported.

    CPA Lipoma is a rare tumor with an incidence of 10% of all intracranial lipomas and less than 0.14% of all CPA tumors. It has a tendency to infiltrate with splaying of 7th and 8th cranial nerves. Surgical excision is usually avoided due to intermingled nerve fibers and adherence to neural structures via fibrous elements.

    The most common site for intracranial lipoma is interhemispheric fissure. Other sites are quadrigeminal cistern, pineal region, CPA, suprasellar cistern and sylvian fissure.  

     
     Pediatric Radiology CasesComments of the author about the case:

    N/A  

     
     Pediatric Radiology CasesFirst description / History: *

    First reported by Klob in 1859.  

     
     Pediatric Radiology CasesLiterature:

    6. Medline: Medline
    Saunders JE, Kwartler JA, Wolf HK, et al.
    Lipomas of the internal auditory canal.
    Laryngoscope 1991;101:1031-7.  

     
     Pediatric Radiology CasesKeywords: *

    CPA Tumors, Lipoma, Cerebellopontine angle, IAC, Internal auditory canal  

     
     Pediatric Radiology Cases Cite this article:

    Achint K. Cerebellopontine Angle Lipoma. PedRad [serial online] vol 9, no. 5.
    URL: www.PedRad.info/?search=20090514012315  

     
     Pediatric Radiology Cases Read similar articles: Internal auditory canal&type=1-17">corresponding keywords
    in the same field:
    or in the region:
    or in the tissue/organ:
    or with the etiology:
     
     Pediatric Radiology CasesImages to this case: There are MRI-images available for this case. [ MRI ] View all modalities [ All ]   

    Thanks to Martina Paetzel, M.D. for translating this case!

     
    Cerebellopontine Angle Lipoma
    Achint K. Cerebellopontine Angle Lipoma. PedRad [serial online] vol 9, no. 5.
    URL: www.PedRad.info/?search=20090514012315


     

    Search similar cases in:
    More links about this topic on Pubmed  More links about this topic on Yahoo  More links about this topic on Altavista  More links about this topic on MSN  More links about this topic on Google  More links about this topic on Fireball

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    Cerebellopontine Angle Lipoma
    Other cases by these authors:

    Search Lance Dorsey in Medline Lance Dorsey (1)   
    Search Adam Patterson in Medline Adam Patterson (1)   
    Search Kraig J. Lage in Medline Kraig J. Lage (17)   
    Search Sanjeeb Kumar Sarma in Medline Sanjeeb Kumar Sarma (24)   
    Search Achint K Singh in Medline Achint K Singh (17)   

    Cerebellopontine Angle Lipoma  
     
    Cerebellopontine Angle Lipoma
    Achint K. Cerebellopontine Angle Lipoma. PedRad [serial online] vol 9, no. 5.
    URL: www.PedRad.info/?search=20090514012315


     

    Which diagnosis have other collegues guessed?


    • Polysplenia Syndrome
      Votes: 7 (35 %)


    • Intestinal malrotation
      Votes: 1 (5 %)


    • Pre-duodenal portal vein
      Votes: 0 (0 %)


    • None of the above
      Votes: 0 (0 %)


    • All of the above
      Votes: 12 (60 %)


    • IRDS
      Votes: 1 (1 %)


    • Pneumothorax with pneumomediastinum
      Votes: 28 (50 %)


    • Pneumonia
      Votes: 1 (1 %)


    • Child abuse
      Votes: 1 (1 %)


    • Ebstein anomaly
      Votes: 4 (7 %)



        Total answers: 55

     
    Cerebellopontine Angle Lipoma
    Achint K. Cerebellopontine Angle Lipoma. PedRad [serial online] vol 9, no. 5.
    URL: www.PedRad.info/?search=20090514012315


     

    Medical Dictionary
    Search in medical dictionary for
    Or type in a keyword
    ( Provided by The On-Line Medical Dictionary )




    1 = less interesting)
     
    Cerebellopontine Angle Lipoma
    Achint K. Cerebellopontine Angle Lipoma. PedRad [serial online] vol 9, no. 5.
    URL: www.PedRad.info/?search=20090514012315


     


    Go to the top of the page   ID: 20090420160623 Original case in german  More links about this topic on Pubmed (PubMed Reader)
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    Discussion
     
    Key as unusual esophageal foreign body in neonate
    Sarma Sanjeeb, Prakritish Bora. Key as unusual esophageal foreign body in neonate. PedRad [serial online] vol 9, no. 4.
    URL: www.PedRad.info/?search=20090420160623


     
     Pediatric Radiology CasesImages to this case: There are X-Ray-images available for this case. [ X-Ray ] There are OP-Situs-images available for this case. [ OP-Situs ] View all modalities [ All ]   
     Pediatric Radiology CasesAuthor/s:

    Sarma Sanjeeb Kumar (Down Town Hospital/Guwahati/India), Prakritish Bora (International Hospital/Guwahati/India)  

     
     Pediatric Radiology CasesEmail Address:

    Viewable for logged on visitors (Log on)  

     
     Pediatric Radiology CasesAge:

    19 Days  

     
     Pediatric Radiology CasesGender:

    Female  

     
     Pediatric Radiology CasesRegion-Organ:

    N/A  

     
     Pediatric Radiology CasesMost likely etiology:

    N/A  

     
     Pediatric Radiology CasesHistory: *

    A 29 days old female infant presented with history of drooling of saliva, excessive crying and mild respiratory distress for two days.  

     
     Pediatric Radiology CasesPathomorphology or Pathophysiology of this disease : *

    A 29 days old female infant was brought to pediatric outpatient department with history of excessive drooling of salvia, crying and respiratory distress for 2 days. On examination the infant was having tachycardia and was tachypnaec also. Clinically chest, CVS, CNS and per abdominal examinations were normal. Chest x ray (antero-posterior view) revealed a metallic foreign body (a key) in the cervical region and superior mediastinum area confirmed the diagnosis of foreign body ingestion [Fig1]. General anesthesia was given. Direct laryngoscope revealed the tip (broad end) of the foreign body as silver color projection at the level of cricopharynx. Surrounding mucosa shows edema and oozing of blood. On touching the tip of the foreign body by the Mc Gill forcep, a metallic click feeling was observed by the pediatrician and by holding the tip the foreign body was removed. It measured approximately 3.4 cm x 0.9 cm and weighs 5 grams [Fig2].  

     
     Pediatric Radiology CasesRadiological findings: *


    CT 1 <- view CT 1

    CT 1: Axial CT of the upper abdomen demonstrates multiple spleens of different sizes in the posterior LUQ.



    CT 2 <- view CT 2

    CT 2: Axial CT shows enlarged azygous vein adjacent to the aorta in the lower chest, representing azygous continuation of the abdominal IVC.



    CT 3 <- view CT 3

    CT 3: Coronal CT shows the multiple spleens in the LUQ and azygous continuation of the IVC paralleling the aorta along the right.





    CT 4 <- view CT 4

    CT 4: Axial CT of the mid-abdomen demonstrates short pancreas with no visualized distal body or tail of the pancreas. The contrast opacified vessel seen anterior to the shorted pancreas represents a pre-duodenal portal vein. Also, again noted are several spleens in the left abdomen anterior and lateral to the left kidney.




    CT 5 <- view CT 5

    CT 5: Axial CT of the abdomen shows the small bowel entirely within the right abdomen and the colon located only with the left abdomen. There is also abnormal orientation of the the mesenteric vessels visualized with the superior mesenteric vein seen to the left of the superior mesenteric artery.



    CT 6 <- view CT 6

    CT 6: Axial CT of the lower abdomen demonstrates small bowel within the right lower quadrant with abdnormal location of the cecum and appendix identified within the left lower quadrant.


    X-Ray 1 <- view X-Ray 1

    X-Ray 1: Large lucency surrounds the superior mediastinum with lucency crossing midline. Subtle lucency is also seen inferior and lateral of the right lung base at the costophrenic angle and hemidiaphragm.





    X-Ray 2 <- view X-Ray 2

    X-Ray 2: Large lucency surrounds the superior aspect of the anterior mediastinum with elevation of the thymic shadow.




    MRI 1 <- view MRI 1

    MRI 1: Axial T2 sections showing dilated CBD with intraluminal sludge and calculi.





    MRI 2 <- view MRI 2

    MRI 2: Axial T2 sections showing multiple small calculi in gall bladder neck (arrow).





    MRI 3 <- view MRI 3

    MRI 3: Axial T2 sections showing gall bladder ( short arrow) and a cystic structure (long arrow)





    MRI 4 <- view MRI 4

    MRI 4: Axial T2 sections showing a cystic structure with luminal sludge and the gall bladder.





    MRI 5 <- view MRI 5

    MRI 5: Axial T2 sections showing dilated CBD anterior to IVC.




    MRI 6 <- view MRI 6

    MRI 6: MRCP showing gall bladder with calculi (short arrow) and a cystic structure (long arrow).





    MRI 7 <- view MRI 7

    MRI 7: MRCP showing both gall bladder and a cystic structure.





    MRI 8 <- view MRI 8

    MRI 8: MRCP showing small calculi in a cystic structure (arrow)




    MRI 1 <- view MRI 1

    MRI 1: T1W MRI showing hyperintense mass in right CP angle cistern extending into the internal auditory canal.




    MRI 2 <- view MRI 2

    MRI 2: T2W MRI showing hyperintense mass with splaying of 7th and 8th cranial nerves.




    MRI 3 <- view MRI 3

    MRI 3: T1W post gadolinium fat suppressed image showing nonenhancing mass with fat suppression.




    X-Ray 1 <- view X-Ray 1

    X-Ray 1: Chest x ray (AP view) showing metallic foreign body (a key) in lower cervical and superior mediastinum area.





    OP-Situs 1 <- view OP-Situs 1

    OP-Situs 1: Showing the foreign body (key) after removal.

     

     
     Pediatric Radiology CasesDiagnosis confirmation:

    N/A  

     
     Pediatric Radiology CasesWhich DD would be also possible with the radiological findings:

    N/A  

     
     Pediatric Radiology CasesCourse / Prognosis / Frequency / Other : *

    No complication followed after removal of the foreign body.  

     
     Pediatric Radiology CasesComments of the author about the case: *

    Though children from 5 months to 6 years of age group present commonly in ENT department with foreign body ingestion, however neonatal foreign body ingestion is rare. It can be accidental like from the hands of parents, relatives or elder sibling. In this case the parents doubted the role of relatives for homicidal attempt. No police case was registered till discharge  

     
     Pediatric Radiology CasesFirst description / History:

    N/A  

     
     Pediatric Radiology CasesLiterature:

    1. Panda N. Management of sharp esophageal foreign bodies in young children: a cause for worry. International Journal of Pediatric Otorhinolaryngology, 2002, 64(3):243-246.

    2. Tasneem Z, Khan M.A. M., Uddin N. Esophageal foreign body in Neonates. J Pak Med Assoc 2004 Mar; 54(3):159-61.

    3. Poorey V. K., Dixit S. and Shakya R. Foreign body oesophagus in neonate with unusual presentation. 2008 June (60)2.

    4. Medatwal A., Gupta P. P., Gulati R. K. Multiple Foreign Bodies in a Neonate. Indian pediatrics. 2008 Nov; (45) 17.  

     
     Pediatric Radiology CasesKeywords: *

    Foreign body, neonate, esophageal, key  

     
     Pediatric Radiology Cases Cite this article:

    Sarma Sanjeeb, Prakritish Bora. Key as unusual esophageal foreign body in neonate. PedRad [serial online] vol 9, no. 4.
    URL: www.PedRad.info/?search=20090420160623  

     
     Pediatric Radiology Cases Read similar articles: key&type=1-17">corresponding keywords
    in the same field:
    or in the region:
    or in the tissue/organ:
    or with the etiology:
     
     Pediatric Radiology CasesImages to this case: There are X-Ray-images available for this case. [ X-Ray ] There are OP-Situs-images available for this case. [ OP-Situs ] View all modalities [ All ]   

    Thanks to Martina Paetzel, M.D. for translating this case!

     
    Key as unusual esophageal foreign body in neonate
    Sarma Sanjeeb, Prakritish Bora. Key as unusual esophageal foreign body in neonate. PedRad [serial online] vol 9, no. 4.
    URL: www.PedRad.info/?search=20090420160623


     

    Search similar cases in:
    More links about this topic on Pubmed  More links about this topic on Yahoo  More links about this topic on Altavista  More links about this topic on MSN  More links about this topic on Google  More links about this topic on Fireball

    Peer-reviewed Radiology Search



    Key as unusual esophageal foreign body in neonate
    Other cases by these authors:

    Search Lance Dorsey in Medline Lance Dorsey (1)   
    Search Adam Patterson in Medline Adam Patterson (1)   
    Search Kraig J. Lage in Medline Kraig J. Lage (17)   
    Search Sanjeeb Kumar Sarma in Medline Sanjeeb Kumar Sarma (24)   
    Search Achint K Singh in Medline Achint K Singh (17)   
    Search Sarma Sanjeeb Kumar in Medline Sarma Sanjeeb Kumar (17)   
    Search Prakritish Bora in Medline Prakritish Bora (2)   

    Key as unusual esophageal foreign body in neonate  
     
    Key as unusual esophageal foreign body in neonate
    Sarma Sanjeeb, Prakritish Bora. Key as unusual esophageal foreign body in neonate. PedRad [serial online] vol 9, no. 4.
    URL: www.PedRad.info/?search=20090420160623


     

    Which diagnosis have other collegues guessed?


    • Polysplenia Syndrome
      Votes: 7 (35 %)


    • Intestinal malrotation
      Votes: 1 (5 %)


    • Pre-duodenal portal vein
      Votes: 0 (0 %)


    • None of the above
      Votes: 0 (0 %)


    • All of the above
      Votes: 12 (60 %)


    • IRDS
      Votes: 1 (1 %)


    • Pneumothorax with pneumomediastinum
      Votes: 28 (50 %)


    • Pneumonia
      Votes: 1 (1 %)


    • Child abuse
      Votes: 1 (1 %)


    • Ebstein anomaly
      Votes: 4 (7 %)



        Total answers: 55

     
    Key as unusual esophageal foreign body in neonate
    Sarma Sanjeeb, Prakritish Bora. Key as unusual esophageal foreign body in neonate. PedRad [serial online] vol 9, no. 4.
    URL: www.PedRad.info/?search=20090420160623


     

    Medical Dictionary
    Search in medical dictionary for
    Or type in a keyword
    ( Provided by The On-Line Medical Dictionary )




    1 = less interesting)
     
    Key as unusual esophageal foreign body in neonate
    Sarma Sanjeeb, Prakritish Bora. Key as unusual esophageal foreign body in neonate. PedRad [serial online] vol 9, no. 4.
    URL: www.PedRad.info/?search=20090420160623


     


    Go to the top of the page   ID: 20080522125523 Original case in english  More links about this topic on Pubmed (PubMed Reader)
    Add this case to your RSS feeder: Subscribe to RSS feed Add to Yahoo Add to Google Add to AOL Add to Furl Subscribe to Feed Burner feed

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    Discussion
     
    Lissencephaly Type 1
    Johannes Gossner, J Larsen. Lissencephaly Type 1. PedRad [serial online] vol 8, no. 5.
    URL: www.PedRad.info/?search=20080522125523


     
     Pediatric Radiology CasesImages to this case: There are MRI-images available for this case. [ MRI ] View all modalities [ All ]   
     Pediatric Radiology CasesAuthor/s:

    Johannes Gossner (Institut für Radiologie/ Klinkum Braunschweig), J. Larsen (Institut für Radiologie/ Klinikum Braunschweig)  

     
     Pediatric Radiology CasesEmail Address:

    Viewable for logged on visitors (Log on)  

     
     Pediatric Radiology CasesAge:

    0 Newborn  

     
     Pediatric Radiology CasesGender:

    Female  

     
     Pediatric Radiology CasesRegion-Organ:

    N/A  

     
     Pediatric Radiology CasesMost likely etiology:

    N/A  

     
     Pediatric Radiology CasesHistory:

    Postpartum hypotrophic and respiratory distressed newborn (intubation required) with rapidly evolving seizures.
    Sonographically decreased sulcal pattern.
    Sibling with known lissencephaly type 1.  

     
     Pediatric Radiology CasesPathomorphology or Pathophysiology of this disease :

    Most severe form of neuronal migration disorders (probably in 12th-16th gestational week). Current classification dpendent on when the cortical development was affected. The type 1 lissencephaly belongs to group A. In the setting of a Miller-Diecke syndrome, a monosomy 17p13 can be found, which is mostly a "de Novo" deletion or translocation.
    Without visualized chromosomal abnormality it can be found as an isolated lissencephaly or in the setting of a Norman-Roberts syndrome.
    However, even without visualized chromosomal abnormalities, in up to 40% of cases.a defect of the LIS 1 gene can be found, which regulates the neural migration by forming PAF (platelet activating factor).  

     
     Pediatric Radiology CasesRadiological findings:


    CT 1 <- view CT 1

    CT 1: Axial CT of the upper abdomen demonstrates multiple spleens of different sizes in the posterior LUQ.



    CT 2 <- view CT 2

    CT 2: Axial CT shows enlarged azygous vein adjacent to the aorta in the lower chest, representing azygous continuation of the abdominal IVC.



    CT 3 <- view CT 3

    CT 3: Coronal CT shows the multiple spleens in the LUQ and azygous continuation of the IVC paralleling the aorta along the right.





    CT 4 <- view CT 4

    CT 4: Axial CT of the mid-abdomen demonstrates short pancreas with no visualized distal body or tail of the pancreas. The contrast opacified vessel seen anterior to the shorted pancreas represents a pre-duodenal portal vein. Also, again noted are several spleens in the left abdomen anterior and lateral to the left kidney.




    CT 5 <- view CT 5

    CT 5: Axial CT of the abdomen shows the small bowel entirely within the right abdomen and the colon located only with the left abdomen. There is also abnormal orientation of the the mesenteric vessels visualized with the superior mesenteric vein seen to the left of the superior mesenteric artery.



    CT 6 <- view CT 6

    CT 6: Axial CT of the lower abdomen demonstrates small bowel within the right lower quadrant with abdnormal location of the cecum and appendix identified within the left lower quadrant.


    X-Ray 1 <- view X-Ray 1

    X-Ray 1: Large lucency surrounds the superior mediastinum with lucency crossing midline. Subtle lucency is also seen inferior and lateral of the right lung base at the costophrenic angle and hemidiaphragm.





    X-Ray 2 <- view X-Ray 2

    X-Ray 2: Large lucency surrounds the superior aspect of the anterior mediastinum with elevation of the thymic shadow.




    MRI 1 <- view MRI 1

    MRI 1: Axial T2 sections showing dilated CBD with intraluminal sludge and calculi.





    MRI 2 <- view MRI 2

    MRI 2: Axial T2 sections showing multiple small calculi in gall bladder neck (arrow).





    MRI 3 <- view MRI 3

    MRI 3: Axial T2 sections showing gall bladder ( short arrow) and a cystic structure (long arrow)





    MRI 4 <- view MRI 4

    MRI 4: Axial T2 sections showing a cystic structure with luminal sludge and the gall bladder.





    MRI 5 <- view MRI 5

    MRI 5: Axial T2 sections showing dilated CBD anterior to IVC.




    MRI 6 <- view MRI 6

    MRI 6: MRCP showing gall bladder with calculi (short arrow) and a cystic structure (long arrow).





    MRI 7 <- view MRI 7

    MRI 7: MRCP showing both gall bladder and a cystic structure.





    MRI 8 <- view MRI 8

    MRI 8: MRCP showing small calculi in a cystic structure (arrow)




    MRI 1 <- view MRI 1

    MRI 1: T1W MRI showing hyperintense mass in right CP angle cistern extending into the internal auditory canal.




    MRI 2 <- view MRI 2

    MRI 2: T2W MRI showing hyperintense mass with splaying of 7th and 8th cranial nerves.




    MRI 3 <- view MRI 3

    MRI 3: T1W post gadolinium fat suppressed image showing nonenhancing mass with fat suppression.




    X-Ray 1 <- view X-Ray 1

    X-Ray 1: Chest x ray (AP view) showing metallic foreign body (a key) in lower cervical and superior mediastinum area.





    OP-Situs 1 <- view OP-Situs 1

    OP-Situs 1: Showing the foreign body (key) after removal.


    MRI 1 <- view MRI 1

    MRI 1: T2 weighted axial image with markedly decreased sulcal pattern (= pachygyria) and thin cortex. Furthermore seen is a hemorrhage into the right lateral ventricle (hypointense).



    MRI 2 <- view MRI 2

    MRI 2: T1 weighted axial image demonstrates also significant pachygyria and prominent external CSF spaces. The fluid-fluid level containing hemorrhage in the posterior horns of the right lateral ventricle is better deliniated on the T1 weighted sequence.



    MRI 3 <- view MRI 3

    MRI 3: T2 weighted axial image at the level of the basal ganglia, which appear normal.



    MRI 4 <- view MRI 4

    MRI 4: Coronal CSF suppressed T2 weighted sequence (FLAIR) also demonstrates a markedly decreased sulcal pattern.


     

     
     Pediatric Radiology CasesDiagnosis confirmation:

    N/A  

     
     Pediatric Radiology CasesWhich DD would be also possible with the radiological findings:

    N/A  

     
     Pediatric Radiology CasesCourse / Prognosis / Frequency / Other :

    Overall poor prognosis with marked mental retardation and early, partially therpy refractary seizures. Usually the children die before they reach the second year of life. Associated malformations are frequent.
    Lissencephaly is a rare malformation. A study from the Netherlands reports 11.7 cases in 1 million newborns. Girls are slightly more frequently affected.
    The cortical surface is smooth up to the 2nd trimester. First sulci and fissures can be detected by ultrasound in the 20th gestational week. Widening of the ventricles is described to be an indirect sign of a neuronal migration disorder. If this is evident, a follow up exam and/or MRI is recommended. Image findings by MRI is indicatory.  

     
     Pediatric Radiology CasesComments of the author about the case:

    N/A  

     
     Pediatric Radiology CasesFirst description / History:

    Miller 1963  

     
     Pediatric Radiology CasesLiterature:

    11. Medline: Medline
    Barkovich AJ, Kuzniecky RI, Jackson GD, Guerrinen R, Dobyns WB
    Classification system for malformations of cortical development: update 2001
    Neurology 2001; 57:2168- 2178

    12. Medline: Medline
    Ghai S, Fong KW, Toi A, Chitayat A, Pantazi S, Blaser S
    Prenatal US and MR imaging findings of Lissencephaly: review of fetal cerebral sulcal development
    Radiographics 2006; 26: 389- 405

    13. Medline: Medline
    Gressens P
    Mechanisms and Disturbances of Neuronal Migration
    Pediatric Research 2000; 48: 725-730

    14. Medline: Medline
    de Rijk-van Andel JF, Arts WFM, Hofman A, Staal A, Niermeijer MF
    Epidemiology of Lissencephaly Type I.
    Neuroepidemiology 1991;10:200-204
     

     
     Pediatric Radiology CasesKeywords:

    Lissencephaly, neuronal migration disorder, Pachygyria, Agyria, Lissencephaly type I, Miller-Dieker syndrome, Chromosome 17p13 syndrome, Chromosomal deletion 17p13, Norman-Roberts syndrome  

     
     Pediatric Radiology Cases Cite this article:

    Johannes Gossner, J Larsen. Lissencephaly Type 1. PedRad [serial online] vol 8, no. 5.
    URL: www.PedRad.info/?search=20080522125523  

     
     Pediatric Radiology Cases Read similar articles: Norman-Roberts syndrome&type=1-17">corresponding keywords
    in the same field:
    or in the region:
    or in the tissue/organ:
    or with the etiology:
     
     Pediatric Radiology CasesImages to this case: There are MRI-images available for this case. [ MRI ] View all modalities [ All ]   
     
    Lissencephaly Type 1
    Johannes Gossner, J Larsen. Lissencephaly Type 1. PedRad [serial online] vol 8, no. 5.
    URL: www.PedRad.info/?search=20080522125523


     

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    Lissencephaly Type 1
    Other cases by these authors:

    Search Lance Dorsey in Medline Lance Dorsey (1)   
    Search Adam Patterson in Medline Adam Patterson (1)   
    Search Kraig J. Lage in Medline Kraig J. Lage (17)   
    Search Sanjeeb Kumar Sarma in Medline Sanjeeb Kumar Sarma (24)   
    Search Achint K Singh in Medline Achint K Singh (17)   
    Search Sarma Sanjeeb Kumar in Medline Sarma Sanjeeb Kumar (17)   
    Search Prakritish Bora in Medline Prakritish Bora (2)   
    Search Johannes Gossner in Medline Johannes Gossner (18)   
    Search J. Larsen in Medline J. Larsen (1)   

    Lissencephaly Type 1  
     
    Lissencephaly Type 1
    Johannes Gossner, J Larsen. Lissencephaly Type 1. PedRad [serial online] vol 8, no. 5.
    URL: www.PedRad.info/?search=20080522125523


     

    Which diagnosis have other collegues guessed?


    • Polysplenia Syndrome
      Votes: 7 (35 %)


    • Intestinal malrotation
      Votes: 1 (5 %)


    • Pre-duodenal portal vein
      Votes: 0 (0 %)


    • None of the above
      Votes: 0 (0 %)


    • All of the above
      Votes: 12 (60 %)


    • IRDS
      Votes: 1 (1 %)


    • Pneumothorax with pneumomediastinum
      Votes: 28 (50 %)


    • Pneumonia
      Votes: 1 (1 %)


    • Child abuse
      Votes: 1 (1 %)


    • Ebstein anomaly
      Votes: 4 (7 %)



        Total answers: 55

     
    Lissencephaly Type 1
    Johannes Gossner, J Larsen. Lissencephaly Type 1. PedRad [serial online] vol 8, no. 5.
    URL: www.PedRad.info/?search=20080522125523


     

    Medical Dictionary
    Search in medical dictionary for
    Or type in a keyword
    ( Provided by The On-Line Medical Dictionary )




    1 = less interesting)
     
    Lissencephaly Type 1
    Johannes Gossner, J Larsen. Lissencephaly Type 1. PedRad [serial online] vol 8, no. 5.
    URL: www.PedRad.info/?search=20080522125523


     


    Go to the top of the page   ID: 20080327175230 Original case in english  More links about this topic on Pubmed (PubMed Reader)
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    Discussion
     
    Henoch-Schoenlein Purpura
    K Gerlach, P Göbel. Henoch-Schoenlein Purpura. PedRad [serial online] vol 8, no. 3.
    URL: www.PedRad.info/?search=20080327175230


     
     Pediatric Radiology CasesImages to this case: There are Ultrasound-images available for this case. [ Ultrasound ] There are CT-images available for this case. [ CT ] There are OP-Situs-images available for this case. [ OP-Situs ] There are Histology-images available for this case. [ Histology ] View all modalities [ All ]   
     Pediatric Radiology CasesAuthor/s:

    K. Gerlach, P. Göbel  

     
     Pediatric Radiology CasesEmail Address:

    Viewable for logged on visitors (Log on)  

     
     Pediatric Radiology CasesAge:

    5 Years  

     
     Pediatric Radiology CasesGender:

    Male  

     
     Pediatric Radiology CasesRegion-Organ:

    N/A  

     
     Pediatric Radiology CasesMost likely etiology:

    N/A  

     
     Pediatric Radiology CasesHistory:

    A 5 year old boy presented in the emergency room because of persistent abdominal pain for 5 days with one episode of vomiting.
    Initially, the clinical picture was that of a nonspecific mesenteric lymphadenitis. Labs demonstrated leukocytosis of 24.00 Gpt/l with deviation to the left. Platelet count was 434.00 Gpt/l and CRP was 34 mg/l. ESR was increased (10 mm/h) with mild hypoproteinemia. Urinalysis was normal except for a few epithelial cells and bacteria.
    Ultrasound demonstrated an inflammatory thickened small bowel wall with a cockade like structure in the right mid abdomen. This bowel wall thickening was also visible in the left mid and lower abdomen with increasing amount of free intraabdominal fluid during the hospital stay.
    A CT of the abdomen was performed with the indication of an inflammatory pseudotumor, DD Burkitt lymphoma and a subileus. The boy underwent a diagnostic laparotomy and resection of a purple and thickened jejunal loop with end to end anastomosis.  

     
     Pediatric Radiology CasesPathomorphology or Pathophysiology of this disease :

    Histologically a hemorrhagic small bowel infarction with purulent lymphangitis was found. A second Pathology report described the picture of a submucosal leukocytoclastic vasculitis of the small vessels.

    Henoch-Schoenlein Purpura is a generalized immune complex vasculitis of the small vessels of unknown etiology.After simple infections, an increased production of IgA, which if possibly due to hypersensitivity to bacterial antigenes (e.g. Streptococci), leads to deposition of immune complexes and to aseptic inflammation in different organ systems. These include skin (affected up to 100%) with palpable purpura, gastrointestinal tract (30-75%), joints, kidneys (6-60%), testis and CNS.  

     
     Pediatric Radiology CasesRadiological findings:


    CT 1 <- view CT 1

    CT 1: Axial CT of the upper abdomen demonstrates multiple spleens of different sizes in the posterior LUQ.



    CT 2 <- view CT 2

    CT 2: Axial CT shows enlarged azygous vein adjacent to the aorta in the lower chest, representing azygous continuation of the abdominal IVC.



    CT 3 <- view CT 3

    CT 3: Coronal CT shows the multiple spleens in the LUQ and azygous continuation of the IVC paralleling the aorta along the right.





    CT 4 <- view CT 4

    CT 4: Axial CT of the mid-abdomen demonstrates short pancreas with no visualized distal body or tail of the pancreas. The contrast opacified vessel seen anterior to the shorted pancreas represents a pre-duodenal portal vein. Also, again noted are several spleens in the left abdomen anterior and lateral to the left kidney.




    CT 5 <- view CT 5

    CT 5: Axial CT of the abdomen shows the small bowel entirely within the right abdomen and the colon located only with the left abdomen. There is also abnormal orientation of the the mesenteric vessels visualized with the superior mesenteric vein seen to the left of the superior mesenteric artery.



    CT 6 <- view CT 6

    CT 6: Axial CT of the lower abdomen demonstrates small bowel within the right lower quadrant with abdnormal location of the cecum and appendix identified within the left lower quadrant.


    X-Ray 1 <- view X-Ray 1

    X-Ray 1: Large lucency surrounds the superior mediastinum with lucency crossing midline. Subtle lucency is also seen inferior and lateral of the right lung base at the costophrenic angle and hemidiaphragm.





    X-Ray 2 <- view X-Ray 2

    X-Ray 2: Large lucency surrounds the superior aspect of the anterior mediastinum with elevation of the thymic shadow.




    MRI 1 <- view MRI 1

    MRI 1: Axial T2 sections showing dilated CBD with intraluminal sludge and calculi.





    MRI 2 <- view MRI 2

    MRI 2: Axial T2 sections showing multiple small calculi in gall bladder neck (arrow).





    MRI 3 <- view MRI 3

    MRI 3: Axial T2 sections showing gall bladder ( short arrow) and a cystic structure (long arrow)





    MRI 4 <- view MRI 4

    MRI 4: Axial T2 sections showing a cystic structure with luminal sludge and the gall bladder.





    MRI 5 <- view MRI 5

    MRI 5: Axial T2 sections showing dilated CBD anterior to IVC.




    MRI 6 <- view MRI 6

    MRI 6: MRCP showing gall bladder with calculi (short arrow) and a cystic structure (long arrow).





    MRI 7 <- view MRI 7

    MRI 7: MRCP showing both gall bladder and a cystic structure.





    MRI 8 <- view MRI 8

    MRI 8: MRCP showing small calculi in a cystic structure (arrow)




    MRI 1 <- view MRI 1

    MRI 1: T1W MRI showing hyperintense mass in right CP angle cistern extending into the internal auditory canal.




    MRI 2 <- view MRI 2

    MRI 2: T2W MRI showing hyperintense mass with splaying of 7th and 8th cranial nerves.




    MRI 3 <- view MRI 3

    MRI 3: T1W post gadolinium fat suppressed image showing nonenhancing mass with fat suppression.




    X-Ray 1 <- view X-Ray 1

    X-Ray 1: Chest x ray (AP view) showing metallic foreign body (a key) in lower cervical and superior mediastinum area.





    OP-Situs 1 <- view OP-Situs 1

    OP-Situs 1: Showing the foreign body (key) after removal.


    MRI 1 <- view MRI 1

    MRI 1: T2 weighted axial image with markedly decreased sulcal pattern (= pachygyria) and thin cortex. Furthermore seen is a hemorrhage into the right lateral ventricle (hypointense).



    MRI 2 <- view MRI 2

    MRI 2: T1 weighted axial image demonstrates also significant pachygyria and prominent external CSF spaces. The fluid-fluid level containing hemorrhage in the posterior horns of the right lateral ventricle is better deliniated on the T1 weighted sequence.



    MRI 3 <- view MRI 3

    MRI 3: T2 weighted axial image at the level of the basal ganglia, which appear normal.



    MRI 4 <- view MRI 4

    MRI 4: Coronal CSF suppressed T2 weighted sequence (FLAIR) also demonstrates a markedly decreased sulcal pattern.



    Ultrasound 1 <- view Ultrasound 1

    Ultrasound 1: Wall thickened, hypervascular small bowel loop in the axial plane.





    CT 1 <- view CT 1

    CT 1: Significantly wall thickened segment of jejunum in the left lower and mid abdomen with diffuse surrounding inflammatory reaction, lymphadenopathy and ascites.





    CT 2 <- view CT 2

    CT 2: Significantly wall thickened segment of jejunum in the left lower and mid abdomen with diffuse surrounding inflammatory reaction, lymphadenopathy and ascites.





    OP-Situs 1 <- view OP-Situs 1

    OP-Situs 1: Inflammatory changes and purple coloration of a small bowel loop, presenting as pseudotumor.





    Histology 1 <- view Histology 1

    Histology 1: Hemorrhagic necrotising enteritis with leukocytoclastic vasculitis.

     

     
     Pediatric Radiology CasesDiagnosis confirmation:

    N/A  

     
     Pediatric Radiology CasesWhich DD would be also possible with the radiological findings:

    Invagination, mesenteric lymphadenitis, inflammatory pseudotumor, Burkitt lymphoma  

     
     Pediatric Radiology CasesCourse / Prognosis / Frequency / Other :

    Henoch-Schoenlein Purpura presents preferably in the cold seasons in the the jung childhood (5-7 years of age), with a slight predominance for boys. The diagnosis is made based on clinical symptoms. Henoch-Schoenlein Purpura is diagnosed if two of four of the following criteria (ACR) are present: palpable purpura, age of manifestation < 20 years, angina abdominalis (abdominal pain and blood in stool), presence of granulocytes in the vessel wall. The treatment is targeted to the symptoms, the prognosis is good.

    After initial improvement, the boy developed recurrent swelling in feet and lower legs, testicular pain, massive thrombocytosis as well as position dependent petechia. During the further course a micro hematuria has developed. After administration of Prednisolone a complete cure could be accomplished.  

     
     Pediatric Radiology CasesComments of the author about the case:

    N/A  

     
     Pediatric Radiology CasesFirst description / History:

    N/A  

     
     Pediatric Radiology CasesLiterature:

    15. Medline: Medline
    Chan JC,Li PK; Lai FM, Lai KN.
    Fatal adult Henoch-Schönlein purpura due to small intestinal infarction.
    J Intern Med 232(1992)181-4

    16. Medline: Medline
    Prenzel F, Pfäffle R, Thiele F, Schuster.
    Decreased factor XIII activity during severe Henoch-Schoenlein purpura - Does it play a role?
    V. Klin Padiatr 2006;218:174-76

    17. Medline: Medline
    Lawes D, Wood J.
    Acute abdomen in Henoch-Schönlein purpura.
    J R Soc Med. 2002 Oct;95(10):505- 6

    18. Medline: Medline
    C.Rieger.
    Vaskulitiden, In: Pädiatrie Grundlagen und Praxis. Hrsg. Lentze, Schaub, Schulte, Spanger, S.662ff. 2. Auflage 2003, Springer Verlag, Berlin, Heidelberg, New York,
     

     
     Pediatric Radiology CasesKeywords:

    Henoch-Schoenlein Purpura, small bowel infarct, thrombocytosis, petechia  

     
     Pediatric Radiology Cases Cite this article:

    K Gerlach, P Göbel. Henoch-Schoenlein Purpura. PedRad [serial online] vol 8, no. 3.
    URL: www.PedRad.info/?search=20080327175230  

     
     Pediatric Radiology Cases Read similar articles: petechia&type=1-17">corresponding keywords
    in the same field:
    or in the region:
    or in the tissue/organ:
    or with the etiology:
     
     Pediatric Radiology CasesImages to this case: There are Ultrasound-images available for this case. [ Ultrasound ] There are CT-images available for this case. [ CT ] There are OP-Situs-images available for this case. [ OP-Situs ] There are Histology-images available for this case. [ Histology ] View all modalities [ All ]   
     
    Henoch-Schoenlein Purpura
    K Gerlach, P Göbel. Henoch-Schoenlein Purpura. PedRad [serial online] vol 8, no. 3.
    URL: www.PedRad.info/?search=20080327175230


     

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    Henoch-Schoenlein Purpura
    Other cases by these authors:

    Search Lance Dorsey in Medline Lance Dorsey (1)   
    Search Adam Patterson in Medline Adam Patterson (1)   
    Search Kraig J. Lage in Medline Kraig J. Lage (17)   
    Search Sanjeeb Kumar Sarma in Medline Sanjeeb Kumar Sarma (24)   
    Search Achint K Singh in Medline Achint K Singh (17)   
    Search Sarma Sanjeeb Kumar in Medline Sarma Sanjeeb Kumar (17)   
    Search Prakritish Bora in Medline Prakritish Bora (2)   
    Search Johannes Gossner in Medline Johannes Gossner (18)   
    Search J. Larsen in Medline J. Larsen (1)   
    Search K. Gerlach in Medline K. Gerlach (21)   
    Search P. Göbel in Medline P. Göbel (6)   

    Henoch-Schoenlein Purpura  
     
    Henoch-Schoenlein Purpura
    K Gerlach, P Göbel. Henoch-Schoenlein Purpura. PedRad [serial online] vol 8, no. 3.
    URL: www.PedRad.info/?search=20080327175230


     

    Which diagnosis have other collegues guessed?


    • Polysplenia Syndrome
      Votes: 7 (35 %)


    • Intestinal malrotation
      Votes: 1 (5 %)


    • Pre-duodenal portal vein
      Votes: 0 (0 %)


    • None of the above
      Votes: 0 (0 %)


    • All of the above
      Votes: 12 (60 %)


    • IRDS
      Votes: 1 (1 %)


    • Pneumothorax with pneumomediastinum
      Votes: 28 (50 %)


    • Pneumonia
      Votes: 1 (1 %)


    • Child abuse
      Votes: 1 (1 %)


    • Ebstein anomaly
      Votes: 4 (7 %)



        Total answers: 55

     
    Henoch-Schoenlein Purpura
    K Gerlach, P Göbel. Henoch-Schoenlein Purpura. PedRad [serial online] vol 8, no. 3.
    URL: www.PedRad.info/?search=20080327175230


     

    Medical Dictionary
    Search in medical dictionary for
    Or type in a keyword
    ( Provided by The On-Line Medical Dictionary )




    1 = less interesting)
     
    Henoch-Schoenlein Purpura
    K Gerlach, P Göbel. Henoch-Schoenlein Purpura. PedRad [serial online] vol 8, no. 3.
    URL: www.PedRad.info/?search=20080327175230


     


    Go to the top of the page   ID: 20070611231443 Original case in english  More links about this topic on Pubmed (PubMed Reader)
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    Discussion
     
    Eosinophilic granuloma / monostotic bone lesion of a langerhans cell histiocytosis (LCH)
    Susanne Oechsle, Kurt Vollert. Eosinophilic granuloma / monostotic bone lesion of a langerhans cell histiocytosis (LCH). PedRad [serial online] vol 7, no. 6.
    URL: www.PedRad.info/?search=20070611231443


     
     Pediatric Radiology CasesImages to this case: There are Ultrasound-images available for this case. [ Ultrasound ] There are CT-images available for this case. [ CT ] There are MRI-images available for this case. [ MRI ] View all modalities [ All ]   
     Pediatric Radiology CasesAuthor/s:

    Susanne Oechsle (Klinikum Augsburg/Abteilung Kinderradiologie/Augsburg/Germany), Kurt Vollert (Klinikum Augsburg/Abteilung Kinderradiologie/Augsburg/Germany)  

     
     Pediatric Radiology CasesEmail Address:

    Viewable for logged on visitors (Log on)  

     
     Pediatric Radiology CasesAge:

    5 Years  

     
     Pediatric Radiology CasesGender:

    Female  

     
     Pediatric Radiology CasesRegion-Organ:

    N/A  

     
     Pediatric Radiology CasesMost likely etiology:

    N/A  

     
     Pediatric Radiology CasesHistory:

    History: Fall on the back of the head 11 days ago. According to mother she developed increased swelling in the left occipital area. There are no neurological deficits.
    Physical examination: Painful palpable left occipital swelling.  

     
     Pediatric Radiology CasesPathomorphology or Pathophysiology of this disease :

    Eosinophilic granuloma is the most common and the least severe varient of the Langerhans Cell histiocytosis. Most commonly found in the skull, femur pelvis, ribs and vertebral column. Most monostotic - in 20% of the cases there are multiple masses. Included in the Langerhans Cell Histiocytosis spectrum are the multisystemic forms - Abt-Letterer-Siwe and the Hand-Schüller-Christian illnesses. (I)

    The eitology of the Langerhans Cell Histiocytosis is still unclear. There is a reactice proliferation and/or accumulation of dendritic cells. Assumingly, there is an intercellular communication defect with cytokine-disregulation. As of now, there is no proof of malignancy. (II)

    X-ray morphology is mainly dependent on the stage of the eosinophilic granuloma. Morphology is very diverse, including aggressive as well as latent and active growth patterns possible.
    In the calvarium, there are usually round or oval osteolyses with diameters up to 3 cm. Most are sharply demarcated, as if "punched" through, but in the acute phase there are also unsharp borders possible. In the healing phase, one may find border sclerosis (50%). Bony leftovers in the mid-section of the lesion are seen as button sequesters. In a severe case, there may be a "map-like" picture. (I)  

     
     Pediatric Radiology CasesRadiological findings:


    CT 1 <- view CT 1

    CT 1: Axial CT of the upper abdomen demonstrates multiple spleens of different sizes in the posterior LUQ.



    CT 2 <- view CT 2

    CT 2: Axial CT shows enlarged azygous vein adjacent to the aorta in the lower chest, representing azygous continuation of the abdominal IVC.



    CT 3 <- view CT 3

    CT 3: Coronal CT shows the multiple spleens in the LUQ and azygous continuation of the IVC paralleling the aorta along the right.





    CT 4 <- view CT 4

    CT 4: Axial CT of the mid-abdomen demonstrates short pancreas with no visualized distal body or tail of the pancreas. The contrast opacified vessel seen anterior to the shorted pancreas represents a pre-duodenal portal vein. Also, again noted are several spleens in the left abdomen anterior and lateral to the left kidney.




    CT 5 <- view CT 5

    CT 5: Axial CT of the abdomen shows the small bowel entirely within the right abdomen and the colon located only with the left abdomen. There is also abnormal orientation of the the mesenteric vessels visualized with the superior mesenteric vein seen to the left of the superior mesenteric artery.



    CT 6 <- view CT 6

    CT 6: Axial CT of the lower abdomen demonstrates small bowel within the right lower quadrant with abdnormal location of the cecum and appendix identified within the left lower quadrant.


    X-Ray 1 <- view X-Ray 1

    X-Ray 1: Large lucency surrounds the superior mediastinum with lucency crossing midline. Subtle lucency is also seen inferior and lateral of the right lung base at the costophrenic angle and hemidiaphragm.





    X-Ray 2 <- view X-Ray 2

    X-Ray 2: Large lucency surrounds the superior aspect of the anterior mediastinum with elevation of the thymic shadow.




    MRI 1 <- view MRI 1

    MRI 1: Axial T2 sections showing dilated CBD with intraluminal sludge and calculi.





    MRI 2 <- view MRI 2

    MRI 2: Axial T2 sections showing multiple small calculi in gall bladder neck (arrow).





    MRI 3 <- view MRI 3

    MRI 3: Axial T2 sections showing gall bladder ( short arrow) and a cystic structure (long arrow)





    MRI 4 <- view MRI 4

    MRI 4: Axial T2 sections showing a cystic structure with luminal sludge and the gall bladder.





    MRI 5 <- view MRI 5

    MRI 5: Axial T2 sections showing dilated CBD anterior to IVC.




    MRI 6 <- view MRI 6

    MRI 6: MRCP showing gall bladder with calculi (short arrow) and a cystic structure (long arrow).





    MRI 7 <- view MRI 7

    MRI 7: MRCP showing both gall bladder and a cystic structure.





    MRI 8 <- view MRI 8

    MRI 8: MRCP showing small calculi in a cystic structure (arrow)




    MRI 1 <- view MRI 1

    MRI 1: T1W MRI showing hyperintense mass in right CP angle cistern extending into the internal auditory canal.




    MRI 2 <- view MRI 2

    MRI 2: T2W MRI showing hyperintense mass with splaying of 7th and 8th cranial nerves.




    MRI 3 <- view MRI 3

    MRI 3: T1W post gadolinium fat suppressed image showing nonenhancing mass with fat suppression.




    X-Ray 1 <- view X-Ray 1

    X-Ray 1: Chest x ray (AP view) showing metallic foreign body (a key) in lower cervical and superior mediastinum area.





    OP-Situs 1 <- view OP-Situs 1

    OP-Situs 1: Showing the foreign body (key) after removal.


    MRI 1 <- view MRI 1

    MRI 1: T2 weighted axial image with markedly decreased sulcal pattern (= pachygyria) and thin cortex. Furthermore seen is a hemorrhage into the right lateral ventricle (hypointense).



    MRI 2 <- view MRI 2

    MRI 2: T1 weighted axial image demonstrates also significant pachygyria and prominent external CSF spaces. The fluid-fluid level containing hemorrhage in the posterior horns of the right lateral ventricle is better deliniated on the T1 weighted sequence.



    MRI 3 <- view MRI 3

    MRI 3: T2 weighted axial image at the level of the basal ganglia, which appear normal.



    MRI 4 <- view MRI 4

    MRI 4: Coronal CSF suppressed T2 weighted sequence (FLAIR) also demonstrates a markedly decreased sulcal pattern.



    Ultrasound 1 <- view Ultrasound 1

    Ultrasound 1: Wall thickened, hypervascular small bowel loop in the axial plane.





    CT 1 <- view CT 1

    CT 1: Significantly wall thickened segment of jejunum in the left lower and mid abdomen with diffuse surrounding inflammatory reaction, lymphadenopathy and ascites.





    CT 2 <- view CT 2

    CT 2: Significantly wall thickened segment of jejunum in the left lower and mid abdomen with diffuse surrounding inflammatory reaction, lymphadenopathy and ascites.





    OP-Situs 1 <- view OP-Situs 1

    OP-Situs 1: Inflammatory changes and purple coloration of a small bowel loop, presenting as pseudotumor.





    Histology 1 <- view Histology 1

    Histology 1: Hemorrhagic necrotising enteritis with leukocytoclastic vasculitis.


    Ultrasound 1 <- view Ultrasound 1

    Ultrasound 1: Ultrasound of the back of the head
    Left occipital identified is a 2 cm, subcutaneous, inhomogeneous and hypoechoic mass lesion. In this area, there is about 12 mm cortical discontinuity. Dura appears deviated inferiorly.
    Color coded Duplex sonography: Increased vascularization in the periphery of the lesion. (Images not shown)
    Ultrasound of the abdomen is unremarkable. (Images not shown)




    CT 1 <- view CT 1

    CT 1: CCT Brain window
    Left occipital osteolytic lesion with soft tissue components, no further osteodestructive findings.






    CT 2 <- view CT 2

    CT 2: CCT Bone window
    Left occipital osteolytic lesion with soft tissue components, no further osteodestructive findings.






    MRI 1 <- view MRI 1

    MRI 1: MRI of the head - T1 axial
    Left occipital soft tissue mass with marked marginal contrast enhancement, size ca. 1,8 x 2,8 x 1,5 cm. It causes the known defect in the posterior skull and mild dural displacement to the inside.
    There is surrounding leptomeningeal contrast enhancement. There is increased T2 signal and fluid level in the center of the lesion, which represents a trauma related secondary hemorrhage.





    MRI 2 <- view MRI 2

    MRI 2: MRI of the head - T2 axial






    MRI 3 <- view MRI 3

    MRI 3: MRI of the head - T1 axial with contrast






    MRI 4 <- view MRI 4

    MRI 4: MRI of the head - T1 spir coronal (oblique coronal with surface coil) with contrast



     

     
     Pediatric Radiology CasesDiagnosis confirmation:

    N/A  

     
     Pediatric Radiology CasesWhich DD would be also possible with the radiological findings:

    Lymphoma, solitary bone metastasis of a Neuroblastoma  

     
     Pediatric Radiology CasesCourse / Prognosis / Frequency / Other :

    Treatment options in Langerhans Cell Histiocytosis: Surgical excision in a solitary skeletal mass (II). Intralesional steroid injection have been tried with questionable results (I). In multisystemic masses and multiple bony lesions, a systemic treatment with corticosteroids, cytostatics and immunosuppressives. Radiation only as a last resort (II).

    The course and prognosis of Langerhans Cell Histiocytosis:
    Monosystemic lesions independent of the treatment have a good prognosis. Multisystemic lesions with organ dysfunction in particularly small children is seen as having a poor prognosis, with possible lethal course. (II)

    Eosinophilic granulomas are mostly seen between the ages of 5 and 10 years. Male gender predominates (makes up of about 60-80% of all Langerhans Cell Histiocytoses). (II)  

     
     Pediatric Radiology CasesComments of the author about the case:

    In this case, there was an unnoticed development of a eosinophilic granuloma in the skull. After trauma, there was hemorrhaging and pain, then the noticed swelling on the left, occipital side.
    Final diagnosis and treatment was accomplished through resection of the mass.  

     
     Pediatric Radiology CasesFirst description / History:

    N/A  

     
     Pediatric Radiology CasesLiterature:

    (I) Bohndorf, Imhof, Fischer:
    Radiologische Diagnostik der Knochen und Gelenke.
    Thieme Verlag 2006. S. 208-209.
    (II) Staatz, Honnef, Piroth, Radkow:
    Kinderradiologie. In: Pareto-Reihe Radiologie.
    Thieme Verlag 2007. S. 257-262.
     

     
     Pediatric Radiology CasesKeywords:

    solitary osteolysis of the skull, langerhans cell histiocytosis, LCH, eosinophilic granuloma, lymphoma, neuroblastoma, swelling of head  

     
     Pediatric Radiology Cases Cite this article:

    Susanne Oechsle, Kurt Vollert. Eosinophilic granuloma / monostotic bone lesion of a langerhans cell histiocytosis (LCH). PedRad [serial online] vol 7, no. 6.
    URL: www.PedRad.info/?search=20070611231443  

     
     Pediatric Radiology Cases Read similar articles: swelling of head&type=1-17">corresponding keywords
    in the same field:
    or in the region:
    or in the tissue/organ:
    or with the etiology:
     
     Pediatric Radiology CasesImages to this case: There are Ultrasound-images available for this case. [ Ultrasound ] There are CT-images available for this case. [ CT ] There are MRI-images available for this case. [ MRI ] View all modalities [ All ]   
     
    Eosinophilic granuloma / monostotic bone lesion of a langerhans cell histiocytosis (LCH)
    Susanne Oechsle, Kurt Vollert. Eosinophilic granuloma / monostotic bone lesion of a langerhans cell histiocytosis (LCH). PedRad [serial online] vol 7, no. 6.
    URL: www.PedRad.info/?search=20070611231443


     

    Search similar cases in:
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    Eosinophilic granuloma / monostotic bone lesion of a langerhans cell histiocytosis (LCH)
    Other cases by these authors:

    Search Lance Dorsey in Medline Lance Dorsey (1)   
    Search Adam Patterson in Medline Adam Patterson (1)   
    Search Kraig J. Lage in Medline Kraig J. Lage (17)   
    Search Sanjeeb Kumar Sarma in Medline Sanjeeb Kumar Sarma (24)   
    Search Achint K Singh in Medline Achint K Singh (17)   
    Search Sarma Sanjeeb Kumar in Medline Sarma Sanjeeb Kumar (17)   
    Search Prakritish Bora in Medline Prakritish Bora (2)   
    Search Johannes Gossner in Medline Johannes Gossner (18)   
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    Search Kurt Vollert in Medline Kurt Vollert (2)   

    Eosinophilic granuloma / monostotic bone lesion of a langerhans cell histiocytosis (LCH)  
     
    Eosinophilic granuloma / monostotic bone lesion of a langerhans cell histiocytosis (LCH)
    Susanne Oechsle, Kurt Vollert. Eosinophilic granuloma / monostotic bone lesion of a langerhans cell histiocytosis (LCH). PedRad [serial online] vol 7, no. 6.
    URL: www.PedRad.info/?search=20070611231443


     

    Which diagnosis have other collegues guessed?


    • Polysplenia Syndrome
      Votes: 7 (35 %)


    • Intestinal malrotation
      Votes: 1 (5 %)


    • Pre-duodenal portal vein
      Votes: 0 (0 %)


    • None of the above
      Votes: 0 (0 %)


    • All of the above
      Votes: 12 (60 %)


    • IRDS
      Votes: 1 (1 %)


    • Pneumothorax with pneumomediastinum
      Votes: 28 (50 %)


    • Pneumonia
      Votes: 1 (1 %)


    • Child abuse
      Votes: 1 (1 %)


    • Ebstein anomaly
      Votes: 4 (7 %)



        Total answers: 55

     
    Eosinophilic granuloma / monostotic bone lesion of a langerhans cell histiocytosis (LCH)
    Susanne Oechsle, Kurt Vollert. Eosinophilic granuloma / monostotic bone lesion of a langerhans cell histiocytosis (LCH). PedRad [serial online] vol 7, no. 6.
    URL: www.PedRad.info/?search=20070611231443


     

    Medical Dictionary
    Search in medical dictionary for
    Or type in a keyword
    ( Provided by The On-Line Medical Dictionary )




    1 = less interesting)
     
    Eosinophilic granuloma / monostotic bone lesion of a langerhans cell histiocytosis (LCH)
    Susanne Oechsle, Kurt Vollert. Eosinophilic granuloma / monostotic bone lesion of a langerhans cell histiocytosis (LCH). PedRad [serial online] vol 7, no. 6.
    URL: www.PedRad.info/?search=20070611231443


     


    Go to the top of the page   ID: 20060104123424 Original case in english  More links about this topic on Pubmed (PubMed Reader)
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    Discussion
     
    Rapunzel syndrome grade I° due to trichobezoar
    Dirk Schaper, A Jassoy, W Lässig. Rapunzel syndrome grade I° due to trichobezoar. PedRad [serial online] vol 6, no. 1.
    URL: www.PedRad.info/?search=20060104123424


     
     Pediatric Radiology CasesImages to this case: There are MRI-images available for this case. [ MRI ] There are OP-Situs-images available for this case. [ OP-Situs ] There are Pathology-images available for this case. [ Pathology ] View all modalities [ All ]   
     Pediatric Radiology CasesAuthor/s:

    Dirk Schaper, A. Jassoy, W. Lässig (Klinik für Kinderchirurgie Krankenhaus St. Elisabeth und St. Barbara Halle und Institut für Radiologie und Klinik für Kinder- und Jugendmedizin Städtisches Krankenhaus Martha-Maria Halle-Dölau gGmbH )  

     
     Pediatric Radiology CasesEmail Address:

    Viewable for logged on visitors (Log on)  

     
     Pediatric Radiology CasesAge:

    14 Years  

     
     Pediatric Radiology CasesGender:

    Female  

     
     Pediatric Radiology CasesRegion-Organ:

    N/A  

     
     Pediatric Radiology CasesMost likely etiology:

    N/A  

     
     Pediatric Radiology CasesHistory:

    In-patient admittance because of reoccuring nausea, vomiting and stomach aches for 6 months. In the pediatrician's office, an epigastric tumor was palpated which was 15 cm in size. The 14 year-old girl was never seriously ill before this, and shows no signs of distress in schoolwork. She has been referred to a dermatologist due to loss of hair.  

     
     Pediatric Radiology CasesPathomorphology or Pathophysiology of this disease :

    Bezoars are conglomerates of undigestable materials like hair (i.e. Hairballs), and are usually located in the stomach. Physiologically, one usually observes this in birds (wool) or in cats. The undigestable left-overs are usually vomited. Bezoars in humans are differentiated due to their make-up. Most common are phytobezoars, which consist of undigestable plant material (cellulose, lignine), the second most common form are trichobezoars, which are usually due to trichophagia (eating of hair) combined with a psychological illness (trichotillomania). Also to the classifications of bezoars are the following: Lactobezoars of premature newborns, the pharmakobezoars due to the clumping of medicinal tablets/capsules. Aside from these four main groups, observations of mixed-types have been made.
    The Rapunzel-Syndrome is when the the bezoar has a "pig-tail" which continues though the gut. This may be observed throughout the entire small intestines and can have an increasingly obstructive effect. Furthermore, gastrointestinal symptoms occur and the bezoar itself.  

     
     Pediatric Radiology CasesRadiological findings:


    CT 1 <- view CT 1

    CT 1: Axial CT of the upper abdomen demonstrates multiple spleens of different sizes in the posterior LUQ.



    CT 2 <- view CT 2

    CT 2: Axial CT shows enlarged azygous vein adjacent to the aorta in the lower chest, representing azygous continuation of the abdominal IVC.



    CT 3 <- view CT 3

    CT 3: Coronal CT shows the multiple spleens in the LUQ and azygous continuation of the IVC paralleling the aorta along the right.





    CT 4 <- view CT 4

    CT 4: Axial CT of the mid-abdomen demonstrates short pancreas with no visualized distal body or tail of the pancreas. The contrast opacified vessel seen anterior to the shorted pancreas represents a pre-duodenal portal vein. Also, again noted are several spleens in the left abdomen anterior and lateral to the left kidney.




    CT 5 <- view CT 5

    CT 5: Axial CT of the abdomen shows the small bowel entirely within the right abdomen and the colon located only with the left abdomen. There is also abnormal orientation of the the mesenteric vessels visualized with the superior mesenteric vein seen to the left of the superior mesenteric artery.



    CT 6 <- view CT 6

    CT 6: Axial CT of the lower abdomen demonstrates small bowel within the right lower quadrant with abdnormal location of the cecum and appendix identified within the left lower quadrant.


    X-Ray 1 <- view X-Ray 1

    X-Ray 1: Large lucency surrounds the superior mediastinum with lucency crossing midline. Subtle lucency is also seen inferior and lateral of the right lung base at the costophrenic angle and hemidiaphragm.





    X-Ray 2 <- view X-Ray 2

    X-Ray 2: Large lucency surrounds the superior aspect of the anterior mediastinum with elevation of the thymic shadow.




    MRI 1 <- view MRI 1

    MRI 1: Axial T2 sections showing dilated CBD with intraluminal sludge and calculi.





    MRI 2 <- view MRI 2

    MRI 2: Axial T2 sections showing multiple small calculi in gall bladder neck (arrow).





    MRI 3 <- view MRI 3

    MRI 3: Axial T2 sections showing gall bladder ( short arrow) and a cystic structure (long arrow)





    MRI 4 <- view MRI 4

    MRI 4: Axial T2 sections showing a cystic structure with luminal sludge and the gall bladder.





    MRI 5 <- view MRI 5

    MRI 5: Axial T2 sections showing dilated CBD anterior to IVC.




    MRI 6 <- view MRI 6

    MRI 6: MRCP showing gall bladder with calculi (short arrow) and a cystic structure (long arrow).





    MRI 7 <- view MRI 7

    MRI 7: MRCP showing both gall bladder and a cystic structure.





    MRI 8 <- view MRI 8

    MRI 8: MRCP showing small calculi in a cystic structure (arrow)




    MRI 1 <- view MRI 1

    MRI 1: T1W MRI showing hyperintense mass in right CP angle cistern extending into the internal auditory canal.




    MRI 2 <- view MRI 2

    MRI 2: T2W MRI showing hyperintense mass with splaying of 7th and 8th cranial nerves.




    MRI 3 <- view MRI 3

    MRI 3: T1W post gadolinium fat suppressed image showing nonenhancing mass with fat suppression.




    X-Ray 1 <- view X-Ray 1

    X-Ray 1: Chest x ray (AP view) showing metallic foreign body (a key) in lower cervical and superior mediastinum area.





    OP-Situs 1 <- view OP-Situs 1

    OP-Situs 1: Showing the foreign body (key) after removal.


    MRI 1 <- view MRI 1

    MRI 1: T2 weighted axial image with markedly decreased sulcal pattern (= pachygyria) and thin cortex. Furthermore seen is a hemorrhage into the right lateral ventricle (hypointense).



    MRI 2 <- view MRI 2

    MRI 2: T1 weighted axial image demonstrates also significant pachygyria and prominent external CSF spaces. The fluid-fluid level containing hemorrhage in the posterior horns of the right lateral ventricle is better deliniated on the T1 weighted sequence.



    MRI 3 <- view MRI 3

    MRI 3: T2 weighted axial image at the level of the basal ganglia, which appear normal.



    MRI 4 <- view MRI 4

    MRI 4: Coronal CSF suppressed T2 weighted sequence (FLAIR) also demonstrates a markedly decreased sulcal pattern.



    Ultrasound 1 <- view Ultrasound 1

    Ultrasound 1: Wall thickened, hypervascular small bowel loop in the axial plane.





    CT 1 <- view CT 1

    CT 1: Significantly wall thickened segment of jejunum in the left lower and mid abdomen with diffuse surrounding inflammatory reaction, lymphadenopathy and ascites.





    CT 2 <- view CT 2

    CT 2: Significantly wall thickened segment of jejunum in the left lower and mid abdomen with diffuse surrounding inflammatory reaction, lymphadenopathy and ascites.





    OP-Situs 1 <- view OP-Situs 1

    OP-Situs 1: Inflammatory changes and purple coloration of a small bowel loop, presenting as pseudotumor.





    Histology 1 <- view Histology 1

    Histology 1: Hemorrhagic necrotising enteritis with leukocytoclastic vasculitis.


    Ultrasound 1 <- view Ultrasound 1

    Ultrasound 1: Ultrasound of the back of the head
    Left occipital identified is a 2 cm, subcutaneous, inhomogeneous and hypoechoic mass lesion. In this area, there is about 12 mm cortical discontinuity. Dura appears deviated inferiorly.
    Color coded Duplex sonography: Increased vascularization in the periphery of the lesion. (Images not shown)
    Ultrasound of the abdomen is unremarkable. (Images not shown)




    CT 1 <- view CT 1

    CT 1: CCT Brain window
    Left occipital osteolytic lesion with soft tissue components, no further osteodestructive findings.






    CT 2 <- view CT 2

    CT 2: CCT Bone window
    Left occipital osteolytic lesion with soft tissue components, no further osteodestructive findings.






    MRI 1 <- view MRI 1

    MRI 1: MRI of the head - T1 axial
    Left occipital soft tissue mass with marked marginal contrast enhancement, size ca. 1,8 x 2,8 x 1,5 cm. It causes the known defect in the posterior skull and mild dural displacement to the inside.
    There is surrounding leptomeningeal contrast enhancement. There is increased T2 signal and fluid level in the center of the lesion, which represents a trauma related secondary hemorrhage.





    MRI 2 <- view MRI 2

    MRI 2: MRI of the head - T2 axial






    MRI 3 <- view MRI 3

    MRI 3: MRI of the head - T1 axial with contrast






    MRI 4 <- view MRI 4

    MRI 4: MRI of the head - T1 spir coronal (oblique coronal with surface coil) with contrast




    MRI 1 <- view MRI 1

    MRI 1: T2-weighted HASTE sequence, coronal.
    Large inhomogeneous, hypointense mass in the T2-weighted image, which fills almost the entire stomach, and continues to the duodenum.






    MRI 2 <- view MRI 2

    MRI 2: T2-weighted HASTE sequence, coronal.
    Large inhomogeneous, hypointense mass in the T2-weighted image, which fills almost the whole stomach, and continues to the duodenum.






    MRI 3 <- view MRI 3

    MRI 3: T2-weighted HASTE sequence, transversal: Here, a large, inhomogeneous, hypointense mass (which has sharp margins), which almost completely fills the stomach. There is distal extension through the pylorus into the duodenum.
    There is a small fluid level to the right and lateral to the mass and air ventral to the mass inside the stomach.




    MRI 4 <- view MRI 4

    MRI 4: T1 weighted 3D-GE-Sequence with fat-saturation and before constrast application. Conspicuous fat saturation in the T1-weighted image, which shows inhomogeneous, hypointense mass in the stomach lumen without evidence of contrast uptake after Gd-DTPA application. Some air in the stomach to the right and lateral of the mass.




    MRI 5 <- view MRI 5

    MRI 5: T1 weighted 3D-GE-Sequence with fat-saturation after contrast application, transversal. Conspicuous fat saturation in the T1-weighted image, which shows an inhomogeneous, hypointense mass in the stomach lumen without evidence of contrast uptake after Gd-DTPA application. Some air in the stomach to the right and lateral of the mass. There is intense contrast uptake in the stomach wall.




    OP-Situs 1 <- view OP-Situs 1

    OP-Situs 1: Specimen





    Pathology 1 <- view Pathology 1

    Pathology 1: Specimen

     

     
     Pediatric Radiology CasesDiagnosis confirmation:

    N/A  

     
     Pediatric Radiology CasesWhich DD would be also possible with the radiological findings:

    Phytobezoar, Diosphyrobezoar, Pharmacobezoar  

     
     Pediatric Radiology CasesCourse / Prognosis / Frequency / Other :

    After diagnostic imaging, initial attempt to reduce the size and removal of the bezoar endoscopically. Because of the size and consistency of the bezoar, it was not possible, and a gastrotomy and removal of the bezoar followed. In this case, this was a trichobezoar with a "pigtail" that reached into the duodenum - about 15 cm in size. The bezoar weighed 1700 grams. After wound healing, the patient was transferred to the clinic for child- and adolescent psychiatry.  

     
     Pediatric Radiology CasesComments of the author about the case:

    In our case, the endoscopic treatment was not possible, so that we decided upon a surgical intervention. In accordance to Alik et al., this was a Rapunzel Syndrome grade I°.  

     
     Pediatric Radiology CasesFirst description / History:

    Bezoars are known since the 12th century, where they were described initially in India. Because of their rarity and the uncertain pathogenesis, bezoars were given magical properties. Even the name, bezoar, comes from the arabic word "Bedzehr" - the persian "Padzahr" - or the hebrew "Beluzaar" - all meaning "opposite material." This, of course, increased the mystical meaning. In the mideval times, bezoars were seen as being valuable, and were even plated in gold. Today, bezoars are more or less a potential medical problem, whose complication rates should not be underestimated.
    Next to the displacement related gastrointestinal symptoms, ileus situations may occur. Furthermore, ulcerations, strangulations and hemorrhages may result and have been described. The name, "Rapunzel Syndrome" was coined by Vaughan et al. in 1968, who referred to the Brothers Grimm fairytale.  

     
     Pediatric Radiology CasesLiterature:

    20. Medline: Medline
    Gockel,I., C.Gaedertz,H.-J.Hain,U.Winckelmann,M.Albani,D.Lorenz
    Das Rapunzel-Syndrom
    Chirurg 2003 74:753-756

    21. Medline: Medline
    M.K.Sanders
    Bezoars: From mystical charms to medical and nutritional management
    Practical Gastroenterology 13 2004

    22. Medline: Medline
    Balik,E.,I.Ulman,C.Taneli,M.Demircan
    The Rapunzel syndrome. A case report and review of the literature
    Eur J Pediatr Surg 3 (1993) 171-173

    23. Medline: Medline
    Vaughan,E.D.,J.L.Sawyers,H,W.Scott
    Rapunzel syndrome: An unusual complication of intestinal bezoar
    Surgery 63: 339-343  

     
     Pediatric Radiology CasesKeywords:

    Bezoar, trichobezoar, hairball, foreign object, stomach, trichophagia, trichotillomania, hair eating, Rapunzel syndrome,  

     
     Pediatric Radiology Cases Cite this article:

    Dirk Schaper, A Jassoy, W Lässig. Rapunzel syndrome grade I° due to trichobezoar. PedRad [serial online] vol 6, no. 1.
    URL: www.PedRad.info/?search=20060104123424  

     
     Pediatric Radiology Cases Read similar articles: Rapunzel syndrome&type=1-17">corresponding keywords
    in the same field:
    or in the region:
    or in the tissue/organ:
    or with the etiology:
     
     Pediatric Radiology CasesImages to this case: There are MRI-images available for this case. [ MRI ] There are OP-Situs-images available for this case. [ OP-Situs ] There are Pathology-images available for this case. [ Pathology ] View all modalities [ All ]   
     
    Rapunzel syndrome grade I° due to trichobezoar
    Dirk Schaper, A Jassoy, W Lässig. Rapunzel syndrome grade I° due to trichobezoar. PedRad [serial online] vol 6, no. 1.
    URL: www.PedRad.info/?search=20060104123424


     

    Search similar cases in:
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    Rapunzel syndrome grade I° due to trichobezoar
    Other cases by these authors:

    Search Lance Dorsey in Medline Lance Dorsey (1)   
    Search Adam Patterson in Medline Adam Patterson (1)   
    Search Kraig J. Lage in Medline Kraig J. Lage (17)   
    Search Sanjeeb Kumar Sarma in Medline Sanjeeb Kumar Sarma (24)   
    Search Achint K Singh in Medline Achint K Singh (17)   
    Search Sarma Sanjeeb Kumar in Medline Sarma Sanjeeb Kumar (17)   
    Search Prakritish Bora in Medline Prakritish Bora (2)   
    Search Johannes Gossner in Medline Johannes Gossner (18)   
    Search J. Larsen in Medline J. Larsen (1)   
    Search K. Gerlach in Medline K. Gerlach (21)   
    Search P. Göbel in Medline P. Göbel (6)   
    Search Susanne Oechsle in Medline Susanne Oechsle (17)   
    Search Kurt Vollert in Medline Kurt Vollert (2)   
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    Search A. Jassoy in Medline A. Jassoy (6)   
    Search W. Lässig in Medline W. Lässig (2)   

    Rapunzel syndrome grade I° due to trichobezoar  
     
    Rapunzel syndrome grade I° due to trichobezoar
    Dirk Schaper, A Jassoy, W Lässig. Rapunzel syndrome grade I° due to trichobezoar. PedRad [serial online] vol 6, no. 1.
    URL: www.PedRad.info/?search=20060104123424


     

    Which diagnosis have other collegues guessed?


    • Polysplenia Syndrome
      Votes: 7 (35 %)


    • Intestinal malrotation
      Votes: 1 (5 %)


    • Pre-duodenal portal vein
      Votes: 0 (0 %)


    • None of the above
      Votes: 0 (0 %)


    • All of the above
      Votes: 12 (60 %)


    • IRDS
      Votes: 1 (1 %)


    • Pneumothorax with pneumomediastinum
      Votes: 28 (50 %)


    • Pneumonia
      Votes: 1 (1 %)


    • Child abuse
      Votes: 1 (1 %)


    • Ebstein anomaly
      Votes: 4 (7 %)


    • Gastric carcinoma
      Votes: 0 (0 %)


    • Phytobezoar
      Votes: 0 (0 %)


    • Ascarides
      Votes: 1 (1 %)


    • Trichobezoar
      Votes: 19 (25 %)



        Total answers: 75

     
    Rapunzel syndrome grade I° due to trichobezoar
    Dirk Schaper, A Jassoy, W Lässig. Rapunzel syndrome grade I° due to trichobezoar. PedRad [serial online] vol 6, no. 1.
    URL: www.PedRad.info/?search=20060104123424


     

    Medical Dictionary
    Search in medical dictionary for
    Or type in a keyword
    ( Provided by The On-Line Medical Dictionary )




    1 = less interesting)
     
    Rapunzel syndrome grade I° due to trichobezoar
    Dirk Schaper, A Jassoy, W Lässig. Rapunzel syndrome grade I° due to trichobezoar. PedRad [serial online] vol 6, no. 1.
    URL: www.PedRad.info/?search=20060104123424


     


    Go to the top of the page   ID: 20050923114058 Original case in english  More links about this topic on Pubmed (PubMed Reader)
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    Discussion
     
    Congenital internal Duodenal Stenosis
    Dirk Schaper, C Hess. Congenital internal Duodenal Stenosis. PedRad [serial online] vol 5, no. 9.
    URL: www.PedRad.info/?search=20050923114058


     
     Pediatric Radiology CasesImages to this case: There are X-Ray-images available for this case. [ X-Ray ] There are Ultrasound-images available for this case. [ Ultrasound ] View all modalities [ All ]   
     Pediatric Radiology CasesAuthor/s:

    Dirk Schaper, C. Hess (Klinik für Kinderchirurgie und Klinik für Pädiatrie des Krankenhaus St. Elisabeth und St. Barbara Halle/S.)  

     
     Pediatric Radiology CasesEmail Address:

    Viewable for logged on visitors (Log on)  

     
     Pediatric Radiology CasesAge:

    1 Newborn  

     
     Pediatric Radiology CasesGender:

    Female  

     
     Pediatric Radiology CasesRegion-Organ:

    N/A  

     
     Pediatric Radiology CasesMost likely etiology:

    N/A  

     
     Pediatric Radiology CasesHistory:

    In the prenatal ultrasound, there is evidence of foreshortened extremities (history of hip dysplasia/chondrodystrophy in the family). Prenatal ultrasound with dilated stomach, double-bubble phenonemon, oligohydramnios. Spontaneous delivery in the 37th week of pregnancy in cephalic presentation. Postnatally unremarkable adaptation. Further diagnostics.  

     
     Pediatric Radiology CasesPathomorphology or Pathophysiology of this disease :

    Internal stenoses arise more often through growth retardation than through vascular or obliterating infections. Beside membrane-like barriers, tubular strictures have been also described.  

     
     Pediatric Radiology CasesRadiological findings:


    CT 1 <- view CT 1

    CT 1: Axial CT of the upper abdomen demonstrates multiple spleens of different sizes in the posterior LUQ.



    CT 2 <- view CT 2

    CT 2: Axial CT shows enlarged azygous vein adjacent to the aorta in the lower chest, representing azygous continuation of the abdominal IVC.



    CT 3 <- view CT 3

    CT 3: Coronal CT shows the multiple spleens in the LUQ and azygous continuation of the IVC paralleling the aorta along the right.





    CT 4 <- view CT 4

    CT 4: Axial CT of the mid-abdomen demonstrates short pancreas with no visualized distal body or tail of the pancreas. The contrast opacified vessel seen anterior to the shorted pancreas represents a pre-duodenal portal vein. Also, again noted are several spleens in the left abdomen anterior and lateral to the left kidney.




    CT 5 <- view CT 5

    CT 5: Axial CT of the abdomen shows the small bowel entirely within the right abdomen and the colon located only with the left abdomen. There is also abnormal orientation of the the mesenteric vessels visualized with the superior mesenteric vein seen to the left of the superior mesenteric artery.



    CT 6 <- view CT 6

    CT 6: Axial CT of the lower abdomen demonstrates small bowel within the right lower quadrant with abdnormal location of the cecum and appendix identified within the left lower quadrant.


    X-Ray 1 <- view X-Ray 1

    X-Ray 1: Large lucency surrounds the superior mediastinum with lucency crossing midline. Subtle lucency is also seen inferior and lateral of the right lung base at the costophrenic angle and hemidiaphragm.





    X-Ray 2 <- view X-Ray 2

    X-Ray 2: Large lucency surrounds the superior aspect of the anterior mediastinum with elevation of the thymic shadow.




    MRI 1 <- view MRI 1

    MRI 1: Axial T2 sections showing dilated CBD with intraluminal sludge and calculi.





    MRI 2 <- view MRI 2

    MRI 2: Axial T2 sections showing multiple small calculi in gall bladder neck (arrow).





    MRI 3 <- view MRI 3

    MRI 3: Axial T2 sections showing gall bladder ( short arrow) and a cystic structure (long arrow)





    MRI 4 <- view MRI 4

    MRI 4: Axial T2 sections showing a cystic structure with luminal sludge and the gall bladder.





    MRI 5 <- view MRI 5

    MRI 5: Axial T2 sections showing dilated CBD anterior to IVC.




    MRI 6 <- view MRI 6

    MRI 6: MRCP showing gall bladder with calculi (short arrow) and a cystic structure (long arrow).





    MRI 7 <- view MRI 7

    MRI 7: MRCP showing both gall bladder and a cystic structure.





    MRI 8 <- view MRI 8

    MRI 8: MRCP showing small calculi in a cystic structure (arrow)




    MRI 1 <- view MRI 1

    MRI 1: T1W MRI showing hyperintense mass in right CP angle cistern extending into the internal auditory canal.




    MRI 2 <- view MRI 2

    MRI 2: T2W MRI showing hyperintense mass with splaying of 7th and 8th cranial nerves.




    MRI 3 <- view MRI 3

    MRI 3: T1W post gadolinium fat suppressed image showing nonenhancing mass with fat suppression.




    X-Ray 1 <- view X-Ray 1

    X-Ray 1: Chest x ray (AP view) showing metallic foreign body (a key) in lower cervical and superior mediastinum area.





    OP-Situs 1 <- view OP-Situs 1

    OP-Situs 1: Showing the foreign body (key) after removal.


    MRI 1 <- view MRI 1

    MRI 1: T2 weighted axial image with markedly decreased sulcal pattern (= pachygyria) and thin cortex. Furthermore seen is a hemorrhage into the right lateral ventricle (hypointense).



    MRI 2 <- view MRI 2

    MRI 2: T1 weighted axial image demonstrates also significant pachygyria and prominent external CSF spaces. The fluid-fluid level containing hemorrhage in the posterior horns of the right lateral ventricle is better deliniated on the T1 weighted sequence.



    MRI 3 <- view MRI 3

    MRI 3: T2 weighted axial image at the level of the basal ganglia, which appear normal.



    MRI 4 <- view MRI 4

    MRI 4: Coronal CSF suppressed T2 weighted sequence (FLAIR) also demonstrates a markedly decreased sulcal pattern.



    Ultrasound 1 <- view Ultrasound 1

    Ultrasound 1: Wall thickened, hypervascular small bowel loop in the axial plane.





    CT 1 <- view CT 1

    CT 1: Significantly wall thickened segment of jejunum in the left lower and mid abdomen with diffuse surrounding inflammatory reaction, lymphadenopathy and ascites.





    CT 2 <- view CT 2

    CT 2: Significantly wall thickened segment of jejunum in the left lower and mid abdomen with diffuse surrounding inflammatory reaction, lymphadenopathy and ascites.





    OP-Situs 1 <- view OP-Situs 1

    OP-Situs 1: Inflammatory changes and purple coloration of a small bowel loop, presenting as pseudotumor.





    Histology 1 <- view Histology 1

    Histology 1: Hemorrhagic necrotising enteritis with leukocytoclastic vasculitis.


    Ultrasound 1 <- view Ultrasound 1

    Ultrasound 1: Ultrasound of the back of the head
    Left occipital identified is a 2 cm, subcutaneous, inhomogeneous and hypoechoic mass lesion. In this area, there is about 12 mm cortical discontinuity. Dura appears deviated inferiorly.
    Color coded Duplex sonography: Increased vascularization in the periphery of the lesion. (Images not shown)
    Ultrasound of the abdomen is unremarkable. (Images not shown)




    CT 1 <- view CT 1

    CT 1: CCT Brain window
    Left occipital osteolytic lesion with soft tissue components, no further osteodestructive findings.






    CT 2 <- view CT 2

    CT 2: CCT Bone window
    Left occipital osteolytic lesion with soft tissue components, no further osteodestructive findings.






    MRI 1 <- view MRI 1

    MRI 1: MRI of the head - T1 axial
    Left occipital soft tissue mass with marked marginal contrast enhancement, size ca. 1,8 x 2,8 x 1,5 cm. It causes the known defect in the posterior skull and mild dural displacement to the inside.
    There is surrounding leptomeningeal contrast enhancement. There is increased T2 signal and fluid level in the center of the lesion, which represents a trauma related secondary hemorrhage.





    MRI 2 <- view MRI 2

    MRI 2: MRI of the head - T2 axial






    MRI 3 <- view MRI 3

    MRI 3: MRI of the head - T1 axial with contrast






    MRI 4 <- view MRI 4

    MRI 4: MRI of the head - T1 spir coronal (oblique coronal with surface coil) with contrast




    MRI 1 <- view MRI 1

    MRI 1: T2-weighted HASTE sequence, coronal.
    Large inhomogeneous, hypointense mass in the T2-weighted image, which fills almost the entire stomach, and continues to the duodenum.






    MRI 2 <- view MRI 2

    MRI 2: T2-weighted HASTE sequence, coronal.
    Large inhomogeneous, hypointense mass in the T2-weighted image, which fills almost the whole stomach, and continues to the duodenum.






    MRI 3 <- view MRI 3

    MRI 3: T2-weighted HASTE sequence, transversal: Here, a large, inhomogeneous, hypointense mass (which has sharp margins), which almost completely fills the stomach. There is distal extension through the pylorus into the duodenum.
    There is a small fluid level to the right and lateral to the mass and air ventral to the mass inside the stomach.




    MRI 4 <- view MRI 4

    MRI 4: T1 weighted 3D-GE-Sequence with fat-saturation and before constrast application. Conspicuous fat saturation in the T1-weighted image, which shows inhomogeneous, hypointense mass in the stomach lumen without evidence of contrast uptake after Gd-DTPA application. Some air in the stomach to the right and lateral of the mass.




    MRI 5 <- view MRI 5

    MRI 5: T1 weighted 3D-GE-Sequence with fat-saturation after contrast application, transversal. Conspicuous fat saturation in the T1-weighted image, which shows an inhomogeneous, hypointense mass in the stomach lumen without evidence of contrast uptake after Gd-DTPA application. Some air in the stomach to the right and lateral of the mass. There is intense contrast uptake in the stomach wall.




    OP-Situs 1 <- view OP-Situs 1

    OP-Situs 1: Specimen





    Pathology 1 <- view Pathology 1

    Pathology 1: Specimen


    X-Ray 1 <- view X-Ray 1

    X-Ray 1: Abdominal overview, hanging. Large stomach bubble in the left upper quadrant, duodenal bubble to the right of the spine, little air in the rest of the abdomen.






    X-Ray 2 <- view X-Ray 2

    X-Ray 2: UGI: Contrast media column with abrupt stop at the transition to the pars horizontalis.






    Ultrasound 1 <- view Ultrasound 1

    Ultrasound 1: Hypoechoic structure with internal floating echos seen in the subhepatic region on the transverse abdominal view.





    Ultrasound 2 <- view Ultrasound 2

    Ultrasound 2: Obliterating peristaltic waves traveling from the left to right.





    Ultrasound 3 <- view Ultrasound 3

    Ultrasound 3: Fluid-filled stomach, dilated pylorus, fluid-filled duodenum.


     

     
     Pediatric Radiology CasesDiagnosis confirmation:

    N/A  

     
     Pediatric Radiology CasesWhich DD would be also possible with the radiological findings:

    Duodenal atresia, Ladd syndrome, annular pancreas  

     
     Pediatric Radiology CasesCourse / Prognosis / Frequency / Other :

    Postnatal confirmation of the diagnosis by ultrasound and x-ray. Planned surgical repair using a duodeno-duodenostomy. Intraoperatively, a tubular stenosis was seen, which had a pinpoint sized lumen. A post-stenotic dilation was not seen. A probe could be passed through the lumen. Postoperative pain management with a peridural catheter. Quick oral nutrition through the feeding tube. The tube was removed on the 11th postoperative day and enteral nutrition was unproblematic.  

     
     Pediatric Radiology CasesComments of the author about the case:

    N/A  

     
     Pediatric Radiology CasesFirst description / History:

    N/A  

     
     Pediatric Radiology CasesLiterature:

    24. Medline: Medline
    Jürgen Waldschmidt
    Das akute Abdomen im Kindesalter
    Ed.Medizin, VCH, 1990

    25. Medline: Medline
    Waldschmidt,J., Charissis, G., Berlien, P., Grasemann, S.
    Das Megaduodenum im Neugeborenenalter
    Z Kinderchir 30:197-208  

     
     Pediatric Radiology CasesKeywords:

    Duodenum, internal, duodenal, stenosis, congenital, duodenal stenosis, internal duodenal stenosis  

     
     Pediatric Radiology Cases Cite this article:

    Dirk Schaper, C Hess. Congenital internal Duodenal Stenosis. PedRad [serial online] vol 5, no. 9.
    URL: www.PedRad.info/?search=20050923114058  

     
     Pediatric Radiology Cases Read similar articles: internal duodenal stenosis&type=1-17">corresponding keywords
    in the same field:
    or in the region:
    or in the tissue/organ:
    or with the etiology:
     
     Pediatric Radiology CasesImages to this case: There are X-Ray-images available for this case. [ X-Ray ] There are Ultrasound-images available for this case. [ Ultrasound ] View all modalities [ All ]   
     
    Congenital internal Duodenal Stenosis
    Dirk Schaper, C Hess. Congenital internal Duodenal Stenosis. PedRad [serial online] vol 5, no. 9.
    URL: www.PedRad.info/?search=20050923114058


     

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    Congenital internal Duodenal Stenosis
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    Congenital internal Duodenal Stenosis  
     
    Congenital internal Duodenal Stenosis
    Dirk Schaper, C Hess. Congenital internal Duodenal Stenosis. PedRad [serial online] vol 5, no. 9.
    URL: www.PedRad.info/?search=20050923114058


     

    Which diagnosis have other collegues guessed?


    • Polysplenia Syndrome
      Votes: 7 (35 %)


    • Intestinal malrotation
      Votes: 1 (5 %)


    • Pre-duodenal portal vein
      Votes: 0 (0 %)


    • None of the above
      Votes: 0 (0 %)


    • All of the above
      Votes: 12 (60 %)


    • IRDS
      Votes: 1 (1 %)


    • Pneumothorax with pneumomediastinum
      Votes: 28 (50 %)


    • Pneumonia
      Votes: 1 (1 %)


    • Child abuse
      Votes: 1 (1 %)


    • Ebstein anomaly
      Votes: 4 (7 %)


    • Gastric carcinoma
      Votes: 0 (0 %)


    • Phytobezoar
      Votes: 0 (0 %)


    • Ascarides
      Votes: 1 (1 %)


    • Trichobezoar
      Votes: 19 (25 %)


    • pyloric hypertrophy
      Votes: 0 (0 %)


    • pancreas annulare
      Votes: 5 (5 %)


    • congenital internal duodenal stenosis
      Votes: 6 (6 %)


    • Ladd syndrome
      Votes: 2 (2 %)



        Total answers: 88

     
    Congenital internal Duodenal Stenosis
    Dirk Schaper, C Hess. Congenital internal Duodenal Stenosis. PedRad [serial online] vol 5, no. 9.
    URL: www.PedRad.info/?search=20050923114058


     

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    Congenital internal Duodenal Stenosis
    Dirk Schaper, C Hess. Congenital internal Duodenal Stenosis. PedRad [serial online] vol 5, no. 9.
    URL: www.PedRad.info/?search=20050923114058


     


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    Discussion
     
    Osteogenesis imperfecta - brittle bone disease
    K Glutig, G Hahn. Osteogenesis imperfecta - brittle bone disease. PedRad [serial online] vol 5, no. 2.
    URL: www.PedRad.info/?search=20050222105123


     
     Pediatric Radiology CasesImages to this case: There are X-Ray-images available for this case. [ X-Ray ] View all modalities [ All ]   
     Pediatric Radiology CasesAuthor/s:

    K. Glutig, G. Hahn (Institut für diagnostische Radiologie Abteilung Kinderradiologie Uniklinikum Dresden/Dresden/Deutschland)  

     
     Pediatric Radiology CasesEmail Address:

    Viewable for logged on visitors (Log on)  

     
     Pediatric Radiology CasesAge:

    0 Newborn  

     
     Pediatric Radiology CasesGender:

    Female  

     
     Pediatric Radiology CasesRegion-Organ:

    N/A  

     
     Pediatric Radiology CasesMost likely etiology:

    N/A  

     
     Pediatric Radiology CasesHistory:

    Mature female infant, 3200 g., 49 cm, Sectio,
    short extremities, especially Femur bilateral,
    round Thorax, blue Sklera
     

     
     Pediatric Radiology CasesPathomorphology or Pathophysiology of this disease :

    generalised connective tissue disorder,
    Collagen metabolic disorder of collagen type I: (Bone collagen)
    Manifestation in the bony skeleton

    insufficient production of spongiosa: multiple micro fractures,
    Diminished bone neoformation,
    sekundary reactive fibrosis and excessive callus formation  

     
     Pediatric Radiology CasesRadiological findings:


    CT 1 <- view CT 1

    CT 1: Axial CT of the upper abdomen demonstrates multiple spleens of different sizes in the posterior LUQ.



    CT 2 <- view CT 2

    CT 2: Axial CT shows enlarged azygous vein adjacent to the aorta in the lower chest, representing azygous continuation of the abdominal IVC.



    CT 3 <- view CT 3

    CT 3: Coronal CT shows the multiple spleens in the LUQ and azygous continuation of the IVC paralleling the aorta along the right.





    CT 4 <- view CT 4

    CT 4: Axial CT of the mid-abdomen demonstrates short pancreas with no visualized distal body or tail of the pancreas. The contrast opacified vessel seen anterior to the shorted pancreas represents a pre-duodenal portal vein. Also, again noted are several spleens in the left abdomen anterior and lateral to the left kidney.




    CT 5 <- view CT 5

    CT 5: Axial CT of the abdomen shows the small bowel entirely within the right abdomen and the colon located only with the left abdomen. There is also abnormal orientation of the the mesenteric vessels visualized with the superior mesenteric vein seen to the left of the superior mesenteric artery.



    CT 6 <- view CT 6

    CT 6: Axial CT of the lower abdomen demonstrates small bowel within the right lower quadrant with abdnormal location of the cecum and appendix identified within the left lower quadrant.


    X-Ray 1 <- view X-Ray 1

    X-Ray 1: Large lucency surrounds the superior mediastinum with lucency crossing midline. Subtle lucency is also seen inferior and lateral of the right lung base at the costophrenic angle and hemidiaphragm.





    X-Ray 2 <- view X-Ray 2

    X-Ray 2: Large lucency surrounds the superior aspect of the anterior mediastinum with elevation of the thymic shadow.




    MRI 1 <- view MRI 1

    MRI 1: Axial T2 sections showing dilated CBD with intraluminal sludge and calculi.





    MRI 2 <- view MRI 2

    MRI 2: Axial T2 sections showing multiple small calculi in gall bladder neck (arrow).





    MRI 3 <- view MRI 3

    MRI 3: Axial T2 sections showing gall bladder ( short arrow) and a cystic structure (long arrow)





    MRI 4 <- view MRI 4

    MRI 4: Axial T2 sections showing a cystic structure with luminal sludge and the gall bladder.





    MRI 5 <- view MRI 5

    MRI 5: Axial T2 sections showing dilated CBD anterior to IVC.




    MRI 6 <- view MRI 6

    MRI 6: MRCP showing gall bladder with calculi (short arrow) and a cystic structure (long arrow).





    MRI 7 <- view MRI 7

    MRI 7: MRCP showing both gall bladder and a cystic structure.





    MRI 8 <- view MRI 8

    MRI 8: MRCP showing small calculi in a cystic structure (arrow)




    MRI 1 <- view MRI 1

    MRI 1: T1W MRI showing hyperintense mass in right CP angle cistern extending into the internal auditory canal.




    MRI 2 <- view MRI 2

    MRI 2: T2W MRI showing hyperintense mass with splaying of 7th and 8th cranial nerves.




    MRI 3 <- view MRI 3

    MRI 3: T1W post gadolinium fat suppressed image showing nonenhancing mass with fat suppression.




    X-Ray 1 <- view X-Ray 1

    X-Ray 1: Chest x ray (AP view) showing metallic foreign body (a key) in lower cervical and superior mediastinum area.





    OP-Situs 1 <- view OP-Situs 1

    OP-Situs 1: Showing the foreign body (key) after removal.


    MRI 1 <- view MRI 1

    MRI 1: T2 weighted axial image with markedly decreased sulcal pattern (= pachygyria) and thin cortex. Furthermore seen is a hemorrhage into the right lateral ventricle (hypointense).



    MRI 2 <- view MRI 2

    MRI 2: T1 weighted axial image demonstrates also significant pachygyria and prominent external CSF spaces. The fluid-fluid level containing hemorrhage in the posterior horns of the right lateral ventricle is better deliniated on the T1 weighted sequence.



    MRI 3 <- view MRI 3

    MRI 3: T2 weighted axial image at the level of the basal ganglia, which appear normal.



    MRI 4 <- view MRI 4

    MRI 4: Coronal CSF suppressed T2 weighted sequence (FLAIR) also demonstrates a markedly decreased sulcal pattern.



    Ultrasound 1 <- view Ultrasound 1

    Ultrasound 1: Wall thickened, hypervascular small bowel loop in the axial plane.





    CT 1 <- view CT 1

    CT 1: Significantly wall thickened segment of jejunum in the left lower and mid abdomen with diffuse surrounding inflammatory reaction, lymphadenopathy and ascites.





    CT 2 <- view CT 2

    CT 2: Significantly wall thickened segment of jejunum in the left lower and mid abdomen with diffuse surrounding inflammatory reaction, lymphadenopathy and ascites.





    OP-Situs 1 <- view OP-Situs 1

    OP-Situs 1: Inflammatory changes and purple coloration of a small bowel loop, presenting as pseudotumor.





    Histology 1 <- view Histology 1

    Histology 1: Hemorrhagic necrotising enteritis with leukocytoclastic vasculitis.


    Ultrasound 1 <- view Ultrasound 1

    Ultrasound 1: Ultrasound of the back of the head
    Left occipital identified is a 2 cm, subcutaneous, inhomogeneous and hypoechoic mass lesion. In this area, there is about 12 mm cortical discontinuity. Dura appears deviated inferiorly.
    Color coded Duplex sonography: Increased vascularization in the periphery of the lesion. (Images not shown)
    Ultrasound of the abdomen is unremarkable. (Images not shown)




    CT 1 <- view CT 1

    CT 1: CCT Brain window
    Left occipital osteolytic lesion with soft tissue components, no further osteodestructive findings.






    CT 2 <- view CT 2

    CT 2: CCT Bone window
    Left occipital osteolytic lesion with soft tissue components, no further osteodestructive findings.






    MRI 1 <- view MRI 1

    MRI 1: MRI of the head - T1 axial
    Left occipital soft tissue mass with marked marginal contrast enhancement, size ca. 1,8 x 2,8 x 1,5 cm. It causes the known defect in the posterior skull and mild dural displacement to the inside.
    There is surrounding leptomeningeal contrast enhancement. There is increased T2 signal and fluid level in the center of the lesion, which represents a trauma related secondary hemorrhage.





    MRI 2 <- view MRI 2

    MRI 2: MRI of the head - T2 axial






    MRI 3 <- view MRI 3

    MRI 3: MRI of the head - T1 axial with contrast






    MRI 4 <- view MRI 4

    MRI 4: MRI of the head - T1 spir coronal (oblique coronal with surface coil) with contrast




    MRI 1 <- view MRI 1

    MRI 1: T2-weighted HASTE sequence, coronal.
    Large inhomogeneous, hypointense mass in the T2-weighted image, which fills almost the entire stomach, and continues to the duodenum.






    MRI 2 <- view MRI 2

    MRI 2: T2-weighted HASTE sequence, coronal.
    Large inhomogeneous, hypointense mass in the T2-weighted image, which fills almost the whole stomach, and continues to the duodenum.






    MRI 3 <- view MRI 3

    MRI 3: T2-weighted HASTE sequence, transversal: Here, a large, inhomogeneous, hypointense mass (which has sharp margins), which almost completely fills the stomach. There is distal extension through the pylorus into the duodenum.
    There is a small fluid level to the right and lateral to the mass and air ventral to the mass inside the stomach.




    MRI 4 <- view MRI 4

    MRI 4: T1 weighted 3D-GE-Sequence with fat-saturation and before constrast application. Conspicuous fat saturation in the T1-weighted image, which shows inhomogeneous, hypointense mass in the stomach lumen without evidence of contrast uptake after Gd-DTPA application. Some air in the stomach to the right and lateral of the mass.




    MRI 5 <- view MRI 5

    MRI 5: T1 weighted 3D-GE-Sequence with fat-saturation after contrast application, transversal. Conspicuous fat saturation in the T1-weighted image, which shows an inhomogeneous, hypointense mass in the stomach lumen without evidence of contrast uptake after Gd-DTPA application. Some air in the stomach to the right and lateral of the mass. There is intense contrast uptake in the stomach wall.




    OP-Situs 1 <- view OP-Situs 1

    OP-Situs 1: Specimen





    Pathology 1 <- view Pathology 1

    Pathology 1: Specimen


    X-Ray 1 <- view X-Ray 1

    X-Ray 1: Abdominal overview, hanging. Large stomach bubble in the left upper quadrant, duodenal bubble to the right of the spine, little air in the rest of the abdomen.






    X-Ray 2 <- view X-Ray 2

    X-Ray 2: UGI: Contrast media column with abrupt stop at the transition to the pars horizontalis.






    Ultrasound 1 <- view Ultrasound 1

    Ultrasound 1: Hypoechoic structure with internal floating echos seen in the subhepatic region on the transverse abdominal view.





    Ultrasound 2 <- view Ultrasound 2

    Ultrasound 2: Obliterating peristaltic waves traveling from the left to right.





    Ultrasound 3 <- view Ultrasound 3

    Ultrasound 3: Fluid-filled stomach, dilated pylorus, fluid-filled duodenum.



    X-Ray 1 <- view X-Ray 1

    X-Ray 1: Thorax-Abdomen in lying position:

    Narrow rip pairs without evidence of previous fractures.

    Unremarkable Clavicles. Normal vertebral bodies without evidence of a significant flattening.





    X-Ray 2 <- view X-Ray 2

    X-Ray 2: Left arm in one plane:

    Evidence of subtle metaphysary step formation of the distal Ulna and the distal Radius. Along the diaphyses and Humerus no evidence of a fracture.






    X-Ray 3 <- view X-Ray 3

    X-Ray 3: Right arm in one plane:

    Evidence of subtle metaphysary step formation of the distal Ulna and the distal Radius. Along the diaphyses and Humerus no evidence of a fracture.





    X-Ray 4 <- view X-Ray 4

    X-Ray 4: Right leg ap:

    Clear bowing of the right femur and extensive Callus formation.

    Status post at least two intrauterin happened fractures with secondary shortening of the Femur. Delicate distalwards narrowing and bowed Fibula and Tibia.





    X-Ray 5 <- view X-Ray 5

    X-Ray 5: Right leg lateral:

    Clear bowing of the right femur and extensive Callus formation.

    Status post at least two intrauterin happened fractures with secondary shortening of the Femur. Delicate distalwards narrowing and bowed Fibula and Tibia.





    X-Ray 6 <- view X-Ray 6

    X-Ray 6: Left lower leg ap:

    Clear bowed deformation of the femur with fresh fracture in the middle diaphysis with clear dislocation. Tibia and Fibula of the left leg also extensively deformed.





    X-Ray 7 <- view X-Ray 7

    X-Ray 7: Head ap:

    Hypertransparent vault of the cranium especially parietal.





    X-Ray 8 <- view X-Ray 8

    X-Ray 8: Head lateral:

    Hypertransparent vault of the cranium especially parietal.


     

     
     Pediatric Radiology CasesDiagnosis confirmation:

    N/A  

     
     Pediatric Radiology CasesWhich DD would be also possible with the radiological findings:

    N/A  

     
     Pediatric Radiology CasesCourse / Prognosis / Frequency / Other :

    Therapy: Bisphosphonates
    Symptomatic: Orthopedic-supportive devices
    No physical strain


    Prognosis variable from type.
    Four types:
    Type II a, b, c, d and Typ III: at birth manifest and prognostic unfavorable.
    Type I und IV: better prognosis with decline of frequency of fractures after puberty.

    Frequency dependent of type: 1,6 or 21,8 per 100000 children,
    ca. 5500 afflicted people in Germany  

     
     Pediatric Radiology CasesComments of the author about the case:

    N/A  

     
     Pediatric Radiology CasesFirst description / History:

    N/A  

     
     Pediatric Radiology CasesLiterature:

    26. Medline: Medline
    Rauch F, Glorieux FH
    Osteogenesis imperfecta
    Lancet. 2004 Apr 24 ;363(9418) :1377-85  

     
     Pediatric Radiology CasesKeywords:

    Osteogenesis imperfecta, brittle bones, dysplasia, brittle bone disease, generalised connective tissue disorder, collagen metabolic disorder, collagen type I  

     
     Pediatric Radiology Cases Cite this article:

    K Glutig, G Hahn. Osteogenesis imperfecta - brittle bone disease. PedRad [serial online] vol 5, no. 2.
    URL: www.PedRad.info/?search=20050222105123  

     
     Pediatric Radiology Cases Read similar articles: collagen type I&type=1-17">corresponding keywords
    in the same field:
    or in the region:
    or in the tissue/organ:
    or with the etiology:
     
     Pediatric Radiology CasesImages to this case: There are X-Ray-images available for this case. [ X-Ray ] View all modalities [ All ]   
     
    Osteogenesis imperfecta - brittle bone disease
    K Glutig, G Hahn. Osteogenesis imperfecta - brittle bone disease. PedRad [serial online] vol 5, no. 2.
    URL: www.PedRad.info/?search=20050222105123


     

    Search similar cases in:
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    Osteogenesis imperfecta - brittle bone disease
    Other cases by these authors:

    Search Lance Dorsey in Medline Lance Dorsey (1)   
    Search Adam Patterson in Medline Adam Patterson (1)   
    Search Kraig J. Lage in Medline Kraig J. Lage (17)   
    Search Sanjeeb Kumar Sarma in Medline Sanjeeb Kumar Sarma (24)   
    Search Achint K Singh in Medline Achint K Singh (17)   
    Search Sarma Sanjeeb Kumar in Medline Sarma Sanjeeb Kumar (17)   
    Search Prakritish Bora in Medline Prakritish Bora (2)   
    Search Johannes Gossner in Medline Johannes Gossner (18)   
    Search J. Larsen in Medline J. Larsen (1)   
    Search K. Gerlach in Medline K. Gerlach (21)   
    Search P. Göbel in Medline P. Göbel (6)   
    Search Susanne Oechsle in Medline Susanne Oechsle (17)   
    Search Kurt Vollert in Medline Kurt Vollert (2)   
    Search Dirk Schaper in Medline Dirk Schaper (59)   
    Search A. Jassoy in Medline A. Jassoy (6)   
    Search W. Lässig in Medline W. Lässig (2)   
    Search Dirk Schaper in Medline Dirk Schaper (59)   
    Search C. Hess in Medline C. Hess (1)   
    Search K. Glutig in Medline K. Glutig (17)   
    Search G. Hahn in Medline G. Hahn (22)   

    Osteogenesis imperfecta - brittle bone disease  
     
    Osteogenesis imperfecta - brittle bone disease
    K Glutig, G Hahn. Osteogenesis imperfecta - brittle bone disease. PedRad [serial online] vol 5, no. 2.
    URL: www.PedRad.info/?search=20050222105123


     

    Which diagnosis have other collegues guessed?


    • Polysplenia Syndrome
      Votes: 7 (35 %)


    • Intestinal malrotation
      Votes: 1 (5 %)


    • Pre-duodenal portal vein
      Votes: 0 (0 %)


    • None of the above
      Votes: 0 (0 %)


    • All of the above
      Votes: 12 (60 %)


    • IRDS
      Votes: 1 (1 %)


    • Pneumothorax with pneumomediastinum
      Votes: 28 (50 %)


    • Pneumonia
      Votes: 1 (1 %)


    • Child abuse
      Votes: 1 (1 %)


    • Ebstein anomaly
      Votes: 4 (7 %)


    • Gastric carcinoma
      Votes: 0 (0 %)


    • Phytobezoar
      Votes: 0 (0 %)


    • Ascarides
      Votes: 1 (1 %)


    • Trichobezoar
      Votes: 19 (25 %)


    • pyloric hypertrophy
      Votes: 0 (0 %)


    • pancreas annulare
      Votes: 5 (5 %)


    • congenital internal duodenal stenosis
      Votes: 6 (6 %)


    • Ladd syndrome
      Votes: 2 (2 %)


    • Achondroplasia
      Votes: 0 (0 %)


    • Hypochondroplasia
      Votes: 0 (0 %)


    • Osteogenesis imperfecta
      Votes: 17 (16 %)


    • Diastrophic Dysplasia
      Votes: 0 (0 %)



        Total answers: 105

     
    Osteogenesis imperfecta - brittle bone disease
    K Glutig, G Hahn. Osteogenesis imperfecta - brittle bone disease. PedRad [serial online] vol 5, no. 2.
    URL: www.PedRad.info/?search=20050222105123


     

    Medical Dictionary
    Search in medical dictionary for
    Or type in a keyword
    ( Provided by The On-Line Medical Dictionary )




    1 = less interesting)
     
    Osteogenesis imperfecta - brittle bone disease
    K Glutig, G Hahn. Osteogenesis imperfecta - brittle bone disease. PedRad [serial online] vol 5, no. 2.
    URL: www.PedRad.info/?search=20050222105123


     


    Go to the top of the page   ID: 20040403230840 Original case in english  More links about this topic on Pubmed (PubMed Reader)
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    Discussion
     
    Trigeminal neuralgia due to Superior Cerebellar Artery (SCA) Anomaly
    A Schlüter. Trigeminal neuralgia due to Superior Cerebellar Artery (SCA) Anomaly. PedRad [serial online] vol 4, no. 4.
    URL: www.PedRad.info/?search=20040403230840


     
     Pediatric Radiology CasesImages to this case: There are MRI-images available for this case. [ MRI ] View all modalities [ All ]   
     Pediatric Radiology CasesAuthor/s:

    A. Schlüter (Sangerhausen/Germany)  

     
     Pediatric Radiology CasesEmail Address:

    Viewable for logged on visitors (Log on)  

     
     Pediatric Radiology CasesAge:

    17 Years  

     
     Pediatric Radiology CasesGender:

    Female  

     
     Pediatric Radiology CasesRegion-Organ:

    N/A  

     
     Pediatric Radiology CasesMost likely etiology:

    N/A  

     
     Pediatric Radiology CasesHistory:

    An almost 17 year-old female patient complains about reoccurring, strong pain in the right side of the face, whereas these mostly are manifested in the forehead as well as the maxillary region; in the corresponding trigeminal nerve branches. The first manifestation of the illness showed itself about 4 years before the examination. Of late, the pain has increased in duration and intensity. Medicinal treatments are only difficult to treat. The patient feels a strong sense of suffering and an inhibition of her quality of life. Clinical-neurological examination showed no relevant causalities as of yet.  

     
     Pediatric Radiology CasesPathomorphology or Pathophysiology of this disease :

    The neurovascular compression is a main cause of chronic trigeminal neuralgia. Cause for this is either small vascular intertwining or - as in this case - vessels with large caliber. Only with high-resolution MRI-sequences could causes be shown. It is assumed that a chronic pulsating irritation causes the neuralgias.  

     
     Pediatric Radiology CasesRadiological findings:


    CT 1 <- view CT 1

    CT 1: Axial CT of the upper abdomen demonstrates multiple spleens of different sizes in the posterior LUQ.



    CT 2 <- view CT 2

    CT 2: Axial CT shows enlarged azygous vein adjacent to the aorta in the lower chest, representing azygous continuation of the abdominal IVC.



    CT 3 <- view CT 3

    CT 3: Coronal CT shows the multiple spleens in the LUQ and azygous continuation of the IVC paralleling the aorta along the right.





    CT 4 <- view CT 4

    CT 4: Axial CT of the mid-abdomen demonstrates short pancreas with no visualized distal body or tail of the pancreas. The contrast opacified vessel seen anterior to the shorted pancreas represents a pre-duodenal portal vein. Also, again noted are several spleens in the left abdomen anterior and lateral to the left kidney.




    CT 5 <- view CT 5

    CT 5: Axial CT of the abdomen shows the small bowel entirely within the right abdomen and the colon located only with the left abdomen. There is also abnormal orientation of the the mesenteric vessels visualized with the superior mesenteric vein seen to the left of the superior mesenteric artery.



    CT 6 <- view CT 6

    CT 6: Axial CT of the lower abdomen demonstrates small bowel within the right lower quadrant with abdnormal location of the cecum and appendix identified within the left lower quadrant.


    X-Ray 1 <- view X-Ray 1

    X-Ray 1: Large lucency surrounds the superior mediastinum with lucency crossing midline. Subtle lucency is also seen inferior and lateral of the right lung base at the costophrenic angle and hemidiaphragm.





    X-Ray 2 <- view X-Ray 2

    X-Ray 2: Large lucency surrounds the superior aspect of the anterior mediastinum with elevation of the thymic shadow.




    MRI 1 <- view MRI 1

    MRI 1: Axial T2 sections showing dilated CBD with intraluminal sludge and calculi.





    MRI 2 <- view MRI 2

    MRI 2: Axial T2 sections showing multiple small calculi in gall bladder neck (arrow).





    MRI 3 <- view MRI 3

    MRI 3: Axial T2 sections showing gall bladder ( short arrow) and a cystic structure (long arrow)





    MRI 4 <- view MRI 4

    MRI 4: Axial T2 sections showing a cystic structure with luminal sludge and the gall bladder.





    MRI 5 <- view MRI 5

    MRI 5: Axial T2 sections showing dilated CBD anterior to IVC.




    MRI 6 <- view MRI 6

    MRI 6: MRCP showing gall bladder with calculi (short arrow) and a cystic structure (long arrow).





    MRI 7 <- view MRI 7

    MRI 7: MRCP showing both gall bladder and a cystic structure.





    MRI 8 <- view MRI 8

    MRI 8: MRCP showing small calculi in a cystic structure (arrow)




    MRI 1 <- view MRI 1

    MRI 1: T1W MRI showing hyperintense mass in right CP angle cistern extending into the internal auditory canal.




    MRI 2 <- view MRI 2

    MRI 2: T2W MRI showing hyperintense mass with splaying of 7th and 8th cranial nerves.




    MRI 3 <- view MRI 3

    MRI 3: T1W post gadolinium fat suppressed image showing nonenhancing mass with fat suppression.




    X-Ray 1 <- view X-Ray 1

    X-Ray 1: Chest x ray (AP view) showing metallic foreign body (a key) in lower cervical and superior mediastinum area.





    OP-Situs 1 <- view OP-Situs 1

    OP-Situs 1: Showing the foreign body (key) after removal.


    MRI 1 <- view MRI 1

    MRI 1: T2 weighted axial image with markedly decreased sulcal pattern (= pachygyria) and thin cortex. Furthermore seen is a hemorrhage into the right lateral ventricle (hypointense).



    MRI 2 <- view MRI 2

    MRI 2: T1 weighted axial image demonstrates also significant pachygyria and prominent external CSF spaces. The fluid-fluid level containing hemorrhage in the posterior horns of the right lateral ventricle is better deliniated on the T1 weighted sequence.



    MRI 3 <- view MRI 3

    MRI 3: T2 weighted axial image at the level of the basal ganglia, which appear normal.



    MRI 4 <- view MRI 4

    MRI 4: Coronal CSF suppressed T2 weighted sequence (FLAIR) also demonstrates a markedly decreased sulcal pattern.



    Ultrasound 1 <- view Ultrasound 1

    Ultrasound 1: Wall thickened, hypervascular small bowel loop in the axial plane.





    CT 1 <- view CT 1

    CT 1: Significantly wall thickened segment of jejunum in the left lower and mid abdomen with diffuse surrounding inflammatory reaction, lymphadenopathy and ascites.





    CT 2 <- view CT 2

    CT 2: Significantly wall thickened segment of jejunum in the left lower and mid abdomen with diffuse surrounding inflammatory reaction, lymphadenopathy and ascites.





    OP-Situs 1 <- view OP-Situs 1

    OP-Situs 1: Inflammatory changes and purple coloration of a small bowel loop, presenting as pseudotumor.





    Histology 1 <- view Histology 1

    Histology 1: Hemorrhagic necrotising enteritis with leukocytoclastic vasculitis.


    Ultrasound 1 <- view Ultrasound 1

    Ultrasound 1: Ultrasound of the back of the head
    Left occipital identified is a 2 cm, subcutaneous, inhomogeneous and hypoechoic mass lesion. In this area, there is about 12 mm cortical discontinuity. Dura appears deviated inferiorly.
    Color coded Duplex sonography: Increased vascularization in the periphery of the lesion. (Images not shown)
    Ultrasound of the abdomen is unremarkable. (Images not shown)




    CT 1 <- view CT 1

    CT 1: CCT Brain window
    Left occipital osteolytic lesion with soft tissue components, no further osteodestructive findings.






    CT 2 <- view CT 2

    CT 2: CCT Bone window
    Left occipital osteolytic lesion with soft tissue components, no further osteodestructive findings.






    MRI 1 <- view MRI 1

    MRI 1: MRI of the head - T1 axial
    Left occipital soft tissue mass with marked marginal contrast enhancement, size ca. 1,8 x 2,8 x 1,5 cm. It causes the known defect in the posterior skull and mild dural displacement to the inside.
    There is surrounding leptomeningeal contrast enhancement. There is increased T2 signal and fluid level in the center of the lesion, which represents a trauma related secondary hemorrhage.





    MRI 2 <- view MRI 2

    MRI 2: MRI of the head - T2 axial






    MRI 3 <- view MRI 3

    MRI 3: MRI of the head - T1 axial with contrast






    MRI 4 <- view MRI 4

    MRI 4: MRI of the head - T1 spir coronal (oblique coronal with surface coil) with contrast




    MRI 1 <- view MRI 1

    MRI 1: T2-weighted HASTE sequence, coronal.
    Large inhomogeneous, hypointense mass in the T2-weighted image, which fills almost the entire stomach, and continues to the duodenum.






    MRI 2 <- view MRI 2

    MRI 2: T2-weighted HASTE sequence, coronal.
    Large inhomogeneous, hypointense mass in the T2-weighted image, which fills almost the whole stomach, and continues to the duodenum.






    MRI 3 <- view MRI 3

    MRI 3: T2-weighted HASTE sequence, transversal: Here, a large, inhomogeneous, hypointense mass (which has sharp margins), which almost completely fills the stomach. There is distal extension through the pylorus into the duodenum.
    There is a small fluid level to the right and lateral to the mass and air ventral to the mass inside the stomach.




    MRI 4 <- view MRI 4

    MRI 4: T1 weighted 3D-GE-Sequence with fat-saturation and before constrast application. Conspicuous fat saturation in the T1-weighted image, which shows inhomogeneous, hypointense mass in the stomach lumen without evidence of contrast uptake after Gd-DTPA application. Some air in the stomach to the right and lateral of the mass.




    MRI 5 <- view MRI 5

    MRI 5: T1 weighted 3D-GE-Sequence with fat-saturation after contrast application, transversal. Conspicuous fat saturation in the T1-weighted image, which shows an inhomogeneous, hypointense mass in the stomach lumen without evidence of contrast uptake after Gd-DTPA application. Some air in the stomach to the right and lateral of the mass. There is intense contrast uptake in the stomach wall.




    OP-Situs 1 <- view OP-Situs 1

    OP-Situs 1: Specimen





    Pathology 1 <- view Pathology 1

    Pathology 1: Specimen


    X-Ray 1 <- view X-Ray 1

    X-Ray 1: Abdominal overview, hanging. Large stomach bubble in the left upper quadrant, duodenal bubble to the right of the spine, little air in the rest of the abdomen.






    X-Ray 2 <- view X-Ray 2

    X-Ray 2: UGI: Contrast media column with abrupt stop at the transition to the pars horizontalis.






    Ultrasound 1 <- view Ultrasound 1

    Ultrasound 1: Hypoechoic structure with internal floating echos seen in the subhepatic region on the transverse abdominal view.





    Ultrasound 2 <- view Ultrasound 2

    Ultrasound 2: Obliterating peristaltic waves traveling from the left to right.





    Ultrasound 3 <- view Ultrasound 3

    Ultrasound 3: Fluid-filled stomach, dilated pylorus, fluid-filled duodenum.



    X-Ray 1 <- view X-Ray 1

    X-Ray 1: Thorax-Abdomen in lying position:

    Narrow rip pairs without evidence of previous fractures.

    Unremarkable Clavicles. Normal vertebral bodies without evidence of a significant flattening.





    X-Ray 2 <- view X-Ray 2

    X-Ray 2: Left arm in one plane:

    Evidence of subtle metaphysary step formation of the distal Ulna and the distal Radius. Along the diaphyses and Humerus no evidence of a fracture.






    X-Ray 3 <- view X-Ray 3

    X-Ray 3: Right arm in one plane:

    Evidence of subtle metaphysary step formation of the distal Ulna and the distal Radius. Along the diaphyses and Humerus no evidence of a fracture.





    X-Ray 4 <- view X-Ray 4

    X-Ray 4: Right leg ap:

    Clear bowing of the right femur and extensive Callus formation.

    Status post at least two intrauterin happened fractures with secondary shortening of the Femur. Delicate distalwards narrowing and bowed Fibula and Tibia.





    X-Ray 5 <- view X-Ray 5

    X-Ray 5: Right leg lateral:

    Clear bowing of the right femur and extensive Callus formation.

    Status post at least two intrauterin happened fractures with secondary shortening of the Femur. Delicate distalwards narrowing and bowed Fibula and Tibia.





    X-Ray 6 <- view X-Ray 6

    X-Ray 6: Left lower leg ap:

    Clear bowed deformation of the femur with fresh fracture in the middle diaphysis with clear dislocation. Tibia and Fibula of the left leg also extensively deformed.





    X-Ray 7 <- view X-Ray 7

    X-Ray 7: Head ap:

    Hypertransparent vault of the cranium especially parietal.





    X-Ray 8 <- view X-Ray 8

    X-Ray 8: Head lateral:

    Hypertransparent vault of the cranium especially parietal.



    The examination showed no tumorous or inflammatory changes intracranially and in the facial skull. Noticeable was, however, a difference of course of the superior cerebellar artery (SCA) on both sides, where on the right side the certain structural contact of the vessel to the entrance in the skull of the 5th cranial nerve on the lateral pons was able to be presented. In comparing both sides, the trigeminal nerve was obviously of larger caliber on the right side.



    MRI 1 <- view MRI 1

    MRI 1: The SCA leaving the basilar artery.





    MRI 2 <- view MRI 2

    MRI 2: Parapontine course of the SCA





    MRI 3 <- view MRI 3

    MRI 3: Neurovascular compression of the right trigeminal root through the SCA





    MRI 4 <- view MRI 4

    MRI 4: Region of contact of the SCA/trigeminal nerve (purposely overly contrasted)





    MRI 5 <- view MRI 5

    MRI 5: Trigeminal nerve on the right side is thicker in comparing both sides.





    MRI 6 <- view MRI 6

    MRI 6: No pathological enhancement of the thickened trigeminal nerve of the right side. The vessels that are seen near the right trigeminal nerve are not the SCA in this image.



     

     
     Pediatric Radiology CasesDiagnosis confirmation:

    N/A  

     
     Pediatric Radiology CasesWhich DD would be also possible with the radiological findings:

    The thickening of the right trigeminal nerve could also be a result of an infection or tumorous process. The missing enhancement (T1-W) makes this etiology unlikely. T2-weighted images show no edema (no difference of signal of both sides).

    Clinically, one should remember in children that such suffering could also be caused by fibrotic dysplasia in the region of the skull base.  

     
     Pediatric Radiology CasesCourse / Prognosis / Frequency / Other :

    Since medication did not lessen the symptoms, a surgical decompression can be tried (Jannetta's Technique).

    It remains hypothetical whether a thickening of the right trigeminal nerve is caused by vascular irritation. A surrounding fibrosis, a post-inflammatory state or a hypertrophy as a result of chronic alteration is conceivable -- I would welcome a discussion of this topic.

    The patient has not yet decided on a surgical treatment.  

     
     Pediatric Radiology CasesComments of the author about the case:

    Of course, 3-D reformations of the imaging material are possible (i.e. sagittal MPR). For a better overview, I have limited myself to select serial images.  

     
     Pediatric Radiology CasesFirst description / History:

    N/A  

     
     Pediatric Radiology CasesLiterature:

    27. Medline: Medline
    Yoshino N, Akimoto H, Yamada I, Nagaoka T, Tetsumura A, Kurabayashi T, Honda E, Nakamura S, Sasaki T
    Trigeminal neuralgia: evaluation of neuralgic manifestation and site of neurovascular compression with 3D CISS MR imaging and MR angiography
    Radiology. 2003 Aug;228(2):539-45. Epub 2003 Jun 11  

     
     Pediatric Radiology CasesKeywords:

    Trigeminal neuralgia, Trigeminal, neuralgia, vessel, alteration, vessel alteration, SCA Alteration, Trigeminal nerve  

     
     Pediatric Radiology Cases Cite this article:

    A Schlüter. Trigeminal neuralgia due to Superior Cerebellar Artery (SCA) Anomaly. PedRad [serial online] vol 4, no. 4.
    URL: www.PedRad.info/?search=20040403230840  

     
     Pediatric Radiology Cases Read similar articles: Trigeminal nerve&type=1-17">corresponding keywords
    in the same field:
    or in the region:
    or in the tissue/organ:
    or with the etiology:
     
     Pediatric Radiology CasesImages to this case: There are MRI-images available for this case. [ MRI ] View all modalities [ All ]   
     
    Trigeminal neuralgia due to Superior Cerebellar Artery (SCA) Anomaly
    A Schlüter. Trigeminal neuralgia due to Superior Cerebellar Artery (SCA) Anomaly. PedRad [serial online] vol 4, no. 4.
    URL: www.PedRad.info/?search=20040403230840


     

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    Trigeminal neuralgia due to Superior Cerebellar Artery (SCA) Anomaly
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    Trigeminal neuralgia due to Superior Cerebellar Artery (SCA) Anomaly  
     
    Trigeminal neuralgia due to Superior Cerebellar Artery (SCA) Anomaly
    A Schlüter. Trigeminal neuralgia due to Superior Cerebellar Artery (SCA) Anomaly. PedRad [serial online] vol 4, no. 4.
    URL: www.PedRad.info/?search=20040403230840


     

    Which diagnosis have other collegues guessed?


    • Polysplenia Syndrome
      Votes: 7 (35 %)


    • Intestinal malrotation
      Votes: 1 (5 %)


    • Pre-duodenal portal vein
      Votes: 0 (0 %)


    • None of the above
      Votes: 0 (0 %)


    • All of the above
      Votes: 12 (60 %)


    • IRDS
      Votes: 1 (1 %)


    • Pneumothorax with pneumomediastinum
      Votes: 28 (50 %)


    • Pneumonia
      Votes: 1 (1 %)


    • Child abuse
      Votes: 1 (1 %)


    • Ebstein anomaly
      Votes: 4 (7 %)


    • Gastric carcinoma
      Votes: 0 (0 %)


    • Phytobezoar
      Votes: 0 (0 %)


    • Ascarides
      Votes: 1 (1 %)


    • Trichobezoar
      Votes: 19 (25 %)


    • pyloric hypertrophy
      Votes: 0 (0 %)


    • pancreas annulare
      Votes: 5 (5 %)


    • congenital internal duodenal stenosis
      Votes: 6 (6 %)


    • Ladd syndrome
      Votes: 2 (2 %)


    • Achondroplasia
      Votes: 0 (0 %)


    • Hypochondroplasia
      Votes: 0 (0 %)


    • Osteogenesis imperfecta
      Votes: 17 (16 %)


    • Diastrophic Dysplasia
      Votes: 0 (0 %)


    • Neuritis of the trigeminal nerve
      Votes: 0 (0 %)


    • Trigeminal neuralgia through SCA anomaly
      Votes: 9 (7 %)


    • Neurinoma of the trigeminal nerve
      Votes: 0 (0 %)


    • Fibrotic dysplasia in the skull base region
      Votes: 0 (0 %)



        Total answers: 114

     
    Trigeminal neuralgia due to Superior Cerebellar Artery (SCA) Anomaly
    A Schlüter. Trigeminal neuralgia due to Superior Cerebellar Artery (SCA) Anomaly. PedRad [serial online] vol 4, no. 4.
    URL: www.PedRad.info/?search=20040403230840


     

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    Trigeminal neuralgia due to Superior Cerebellar Artery (SCA) Anomaly
    A Schlüter. Trigeminal neuralgia due to Superior Cerebellar Artery (SCA) Anomaly. PedRad [serial online] vol 4, no. 4.
    URL: www.PedRad.info/?search=20040403230840


     


    Go to the top of the page   ID: 20040207222335 Original case in english  More links about this topic on Pubmed (PubMed Reader)
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    Discussion
     
    Celiac disease
    D Weber. Celiac disease. PedRad [serial online] vol 4, no. 2.
    URL: www.PedRad.info/?search=20040207222335


     
     Pediatric Radiology CasesImages to this case: There are Ultrasound-images available for this case. [ Ultrasound ] View all modalities [ All ]   
     Pediatric Radiology CasesAuthor/s:

    D. Weber (Uniklinik Leipzig)  

     
     Pediatric Radiology CasesEmail Address:

    Viewable for logged on visitors (Log on)  

     
     Pediatric Radiology CasesAge:

    8 Years  

     
     Pediatric Radiology CasesGender:

    Female  

     
     Pediatric Radiology CasesRegion-Organ:

    N/A  

     
     Pediatric Radiology CasesMost likely etiology:

    N/A  

     
     Pediatric Radiology CasesHistory:

    8 year-old girl with a 3 week history of diarrhea, weight loss of about 2 kg and reoccurring stomach aches.  

     
     Pediatric Radiology CasesPathomorphology or Pathophysiology of this disease :

    Immunological with genetic determination.

    Gliadine/Gluten induced morphological changes of the small intestinal mucosal membranes with atrophy of the villi.

    Clinic: malabsorption

    Begin of illness is mostly in the 6th - 18th month of live, but it can also occur later. Which factors contribute to the clinical manifestation is still unclear.

    Increased rate for lymphomas.  

     
     Pediatric Radiology CasesRadiological findings:


    CT 1 <- view CT 1

    CT 1: Axial CT of the upper abdomen demonstrates multiple spleens of different sizes in the posterior LUQ.



    CT 2 <- view CT 2

    CT 2: Axial CT shows enlarged azygous vein adjacent to the aorta in the lower chest, representing azygous continuation of the abdominal IVC.



    CT 3 <- view CT 3

    CT 3: Coronal CT shows the multiple spleens in the LUQ and azygous continuation of the IVC paralleling the aorta along the right.





    CT 4 <- view CT 4

    CT 4: Axial CT of the mid-abdomen demonstrates short pancreas with no visualized distal body or tail of the pancreas. The contrast opacified vessel seen anterior to the shorted pancreas represents a pre-duodenal portal vein. Also, again noted are several spleens in the left abdomen anterior and lateral to the left kidney.




    CT 5 <- view CT 5

    CT 5: Axial CT of the abdomen shows the small bowel entirely within the right abdomen and the colon located only with the left abdomen. There is also abnormal orientation of the the mesenteric vessels visualized with the superior mesenteric vein seen to the left of the superior mesenteric artery.



    CT 6 <- view CT 6

    CT 6: Axial CT of the lower abdomen demonstrates small bowel within the right lower quadrant with abdnormal location of the cecum and appendix identified within the left lower quadrant.


    X-Ray 1 <- view X-Ray 1

    X-Ray 1: Large lucency surrounds the superior mediastinum with lucency crossing midline. Subtle lucency is also seen inferior and lateral of the right lung base at the costophrenic angle and hemidiaphragm.





    X-Ray 2 <- view X-Ray 2

    X-Ray 2: Large lucency surrounds the superior aspect of the anterior mediastinum with elevation of the thymic shadow.




    MRI 1 <- view MRI 1

    MRI 1: Axial T2 sections showing dilated CBD with intraluminal sludge and calculi.





    MRI 2 <- view MRI 2

    MRI 2: Axial T2 sections showing multiple small calculi in gall bladder neck (arrow).





    MRI 3 <- view MRI 3

    MRI 3: Axial T2 sections showing gall bladder ( short arrow) and a cystic structure (long arrow)





    MRI 4 <- view MRI 4

    MRI 4: Axial T2 sections showing a cystic structure with luminal sludge and the gall bladder.





    MRI 5 <- view MRI 5

    MRI 5: Axial T2 sections showing dilated CBD anterior to IVC.




    MRI 6 <- view MRI 6

    MRI 6: MRCP showing gall bladder with calculi (short arrow) and a cystic structure (long arrow).





    MRI 7 <- view MRI 7

    MRI 7: MRCP showing both gall bladder and a cystic structure.





    MRI 8 <- view MRI 8

    MRI 8: MRCP showing small calculi in a cystic structure (arrow)




    MRI 1 <- view MRI 1

    MRI 1: T1W MRI showing hyperintense mass in right CP angle cistern extending into the internal auditory canal.




    MRI 2 <- view MRI 2

    MRI 2: T2W MRI showing hyperintense mass with splaying of 7th and 8th cranial nerves.




    MRI 3 <- view MRI 3

    MRI 3: T1W post gadolinium fat suppressed image showing nonenhancing mass with fat suppression.




    X-Ray 1 <- view X-Ray 1

    X-Ray 1: Chest x ray (AP view) showing metallic foreign body (a key) in lower cervical and superior mediastinum area.





    OP-Situs 1 <- view OP-Situs 1

    OP-Situs 1: Showing the foreign body (key) after removal.


    MRI 1 <- view MRI 1

    MRI 1: T2 weighted axial image with markedly decreased sulcal pattern (= pachygyria) and thin cortex. Furthermore seen is a hemorrhage into the right lateral ventricle (hypointense).



    MRI 2 <- view MRI 2

    MRI 2: T1 weighted axial image demonstrates also significant pachygyria and prominent external CSF spaces. The fluid-fluid level containing hemorrhage in the posterior horns of the right lateral ventricle is better deliniated on the T1 weighted sequence.



    MRI 3 <- view MRI 3

    MRI 3: T2 weighted axial image at the level of the basal ganglia, which appear normal.



    MRI 4 <- view MRI 4

    MRI 4: Coronal CSF suppressed T2 weighted sequence (FLAIR) also demonstrates a markedly decreased sulcal pattern.



    Ultrasound 1 <- view Ultrasound 1

    Ultrasound 1: Wall thickened, hypervascular small bowel loop in the axial plane.





    CT 1 <- view CT 1

    CT 1: Significantly wall thickened segment of jejunum in the left lower and mid abdomen with diffuse surrounding inflammatory reaction, lymphadenopathy and ascites.





    CT 2 <- view CT 2

    CT 2: Significantly wall thickened segment of jejunum in the left lower and mid abdomen with diffuse surrounding inflammatory reaction, lymphadenopathy and ascites.





    OP-Situs 1 <- view OP-Situs 1

    OP-Situs 1: Inflammatory changes and purple coloration of a small bowel loop, presenting as pseudotumor.





    Histology 1 <- view Histology 1

    Histology 1: Hemorrhagic necrotising enteritis with leukocytoclastic vasculitis.


    Ultrasound 1 <- view Ultrasound 1

    Ultrasound 1: Ultrasound of the back of the head
    Left occipital identified is a 2 cm, subcutaneous, inhomogeneous and hypoechoic mass lesion. In this area, there is about 12 mm cortical discontinuity. Dura appears deviated inferiorly.
    Color coded Duplex sonography: Increased vascularization in the periphery of the lesion. (Images not shown)
    Ultrasound of the abdomen is unremarkable. (Images not shown)




    CT 1 <- view CT 1

    CT 1: CCT Brain window
    Left occipital osteolytic lesion with soft tissue components, no further osteodestructive findings.






    CT 2 <- view CT 2

    CT 2: CCT Bone window
    Left occipital osteolytic lesion with soft tissue components, no further osteodestructive findings.






    MRI 1 <- view MRI 1

    MRI 1: MRI of the head - T1 axial
    Left occipital soft tissue mass with marked marginal contrast enhancement, size ca. 1,8 x 2,8 x 1,5 cm. It causes the known defect in the posterior skull and mild dural displacement to the inside.
    There is surrounding leptomeningeal contrast enhancement. There is increased T2 signal and fluid level in the center of the lesion, which represents a trauma related secondary hemorrhage.





    MRI 2 <- view MRI 2

    MRI 2: MRI of the head - T2 axial






    MRI 3 <- view MRI 3

    MRI 3: MRI of the head - T1 axial with contrast






    MRI 4 <- view MRI 4

    MRI 4: MRI of the head - T1 spir coronal (oblique coronal with surface coil) with contrast




    MRI 1 <- view MRI 1

    MRI 1: T2-weighted HASTE sequence, coronal.
    Large inhomogeneous, hypointense mass in the T2-weighted image, which fills almost the entire stomach, and continues to the duodenum.






    MRI 2 <- view MRI 2

    MRI 2: T2-weighted HASTE sequence, coronal.
    Large inhomogeneous, hypointense mass in the T2-weighted image, which fills almost the whole stomach, and continues to the duodenum.






    MRI 3 <- view MRI 3

    MRI 3: T2-weighted HASTE sequence, transversal: Here, a large, inhomogeneous, hypointense mass (which has sharp margins), which almost completely fills the stomach. There is distal extension through the pylorus into the duodenum.
    There is a small fluid level to the right and lateral to the mass and air ventral to the mass inside the stomach.




    MRI 4 <- view MRI 4

    MRI 4: T1 weighted 3D-GE-Sequence with fat-saturation and before constrast application. Conspicuous fat saturation in the T1-weighted image, which shows inhomogeneous, hypointense mass in the stomach lumen without evidence of contrast uptake after Gd-DTPA application. Some air in the stomach to the right and lateral of the mass.




    MRI 5 <- view MRI 5

    MRI 5: T1 weighted 3D-GE-Sequence with fat-saturation after contrast application, transversal. Conspicuous fat saturation in the T1-weighted image, which shows an inhomogeneous, hypointense mass in the stomach lumen without evidence of contrast uptake after Gd-DTPA application. Some air in the stomach to the right and lateral of the mass. There is intense contrast uptake in the stomach wall.




    OP-Situs 1 <- view OP-Situs 1

    OP-Situs 1: Specimen





    Pathology 1 <- view Pathology 1

    Pathology 1: Specimen


    X-Ray 1 <- view X-Ray 1

    X-Ray 1: Abdominal overview, hanging. Large stomach bubble in the left upper quadrant, duodenal bubble to the right of the spine, little air in the rest of the abdomen.






    X-Ray 2 <- view X-Ray 2

    X-Ray 2: UGI: Contrast media column with abrupt stop at the transition to the pars horizontalis.






    Ultrasound 1 <- view Ultrasound 1

    Ultrasound 1: Hypoechoic structure with internal floating echos seen in the subhepatic region on the transverse abdominal view.





    Ultrasound 2 <- view Ultrasound 2

    Ultrasound 2: Obliterating peristaltic waves traveling from the left to right.





    Ultrasound 3 <- view Ultrasound 3

    Ultrasound 3: Fluid-filled stomach, dilated pylorus, fluid-filled duodenum.



    X-Ray 1 <- view X-Ray 1

    X-Ray 1: Thorax-Abdomen in lying position:

    Narrow rip pairs without evidence of previous fractures.

    Unremarkable Clavicles. Normal vertebral bodies without evidence of a significant flattening.





    X-Ray 2 <- view X-Ray 2

    X-Ray 2: Left arm in one plane:

    Evidence of subtle metaphysary step formation of the distal Ulna and the distal Radius. Along the diaphyses and Humerus no evidence of a fracture.






    X-Ray 3 <- view X-Ray 3

    X-Ray 3: Right arm in one plane:

    Evidence of subtle metaphysary step formation of the distal Ulna and the distal Radius. Along the diaphyses and Humerus no evidence of a fracture.





    X-Ray 4 <- view X-Ray 4

    X-Ray 4: Right leg ap:

    Clear bowing of the right femur and extensive Callus formation.

    Status post at least two intrauterin happened fractures with secondary shortening of the Femur. Delicate distalwards narrowing and bowed Fibula and Tibia.





    X-Ray 5 <- view X-Ray 5

    X-Ray 5: Right leg lateral:

    Clear bowing of the right femur and extensive Callus formation.

    Status post at least two intrauterin happened fractures with secondary shortening of the Femur. Delicate distalwards narrowing and bowed Fibula and Tibia.





    X-Ray 6 <- view X-Ray 6

    X-Ray 6: Left lower leg ap:

    Clear bowed deformation of the femur with fresh fracture in the middle diaphysis with clear dislocation. Tibia and Fibula of the left leg also extensively deformed.





    X-Ray 7 <- view X-Ray 7

    X-Ray 7: Head ap:

    Hypertransparent vault of the cranium especially parietal.





    X-Ray 8 <- view X-Ray 8

    X-Ray 8: Head lateral:

    Hypertransparent vault of the cranium especially parietal.



    The examination showed no tumorous or inflammatory changes intracranially and in the facial skull. Noticeable was, however, a difference of course of the superior cerebellar artery (SCA) on both sides, where on the right side the certain structural contact of the vessel to the entrance in the skull of the 5th cranial nerve on the lateral pons was able to be presented. In comparing both sides, the trigeminal nerve was obviously of larger caliber on the right side.



    MRI 1 <- view MRI 1

    MRI 1: The SCA leaving the basilar artery.





    MRI 2 <- view MRI 2

    MRI 2: Parapontine course of the SCA





    MRI 3 <- view MRI 3

    MRI 3: Neurovascular compression of the right trigeminal root through the SCA





    MRI 4 <- view MRI 4

    MRI 4: Region of contact of the SCA/trigeminal nerve (purposely overly contrasted)





    MRI 5 <- view MRI 5

    MRI 5: Trigeminal nerve on the right side is thicker in comparing both sides.





    MRI 6 <- view MRI 6

    MRI 6: No pathological enhancement of the thickened trigeminal nerve of the right side. The vessels that are seen near the right trigeminal nerve are not the SCA in this image.




    Ultrasound 1 <- view Ultrasound 1

    Ultrasound 1: Widened fluid-distended intestinal loops in the left middle abdominal region with increased peristalsis (red arrow), but without wall-thickening (yellow arrow).




    Ultrasound 2 <- view Ultrasound 2

    Ultrasound 2: Widened fluid-distended intestinal loops in the left middle abdominal region with increased peristalsis, but without wall-thickening. Minimal free fluid (red arrow).




    Ultrasound 3 <- view Ultrasound 3

    Ultrasound 3: Multiple mesenterial lymph nodes (green arrows) reaching up to 19x12mm in size without hyperperfusion.




    Ultrasound 4 <- view Ultrasound 4

    Ultrasound 4: In the initial examination, "bulls-eye" is seen in the left middle abdominal region (light blue arrow), without acute clinical signs. Apparently, this is a small intestinal invagination, which is, in the follow-up exam not seen anymore.

     

     
     Pediatric Radiology CasesDiagnosis confirmation:

    N/A  

     
     Pediatric Radiology CasesWhich DD would be also possible with the radiological findings:

    Mesenterial lymphadenitis, enteritis  

     
     Pediatric Radiology CasesCourse / Prognosis / Frequency / Other :

    Although all sonographic findings are unspecific, there are some that are often found in children with celiac disease:

    - slightly dilated small intestinal loops
    - slight, diffuse intestinal wall thickening
    - increased peristalsis
    - slightly enlarged mesenterial lymph nodes
    - slight widened superior mesenteric artery (increased perfusion)
    - slight widened superior mesenteric vein
    - free fluid
    - increased echogenicity of the liver

    Gluten-free diet lessens the clinical symptoms. A life-long diet is needed.  

     
     Pediatric Radiology CasesComments of the author about the case:

    The ultrasound findings are unspecific. It is not uncommon, however, to find unspecific changes in children with celiac disease!

    Do the mesenterial lymph-nodes always belong to the clinical picture?  

     
     Pediatric Radiology CasesFirst description / History:

    N/A  

     
     Pediatric Radiology CasesLiterature:

    28. Medline: Medline
    Rettenbacher T, Hollerweger A, Macheiner P, Huber S, Gritzmann N.
    Adult celiac disease: US signs.
    Radiology. 1999 May;211(2):389-94

    29. Medline: Medline
    Riccabona M, Rossipal E.
    Value of ultrasound in diagnosis of celiac disease
    Ultraschall Med. 1996 Feb;17(1):31-3.

    30. Medline: Medline
    Dietrich CF, Brunner V, Seifert H, Schreiber-Dietrich D, Caspary WF, Lembcke B.
    Intestinal B-mode sonography in patients with endemic sprue. Intestinal sonography in endemic sprue
    Ultraschall Med. 1999 Dec;20(6):242-7.
     

     
     Pediatric Radiology CasesKeywords:

    celiac disease, glutenenteropathy, nontropical sprue, invagination, mesenterial, mesentery, gluten, gliadin, enteritis, celiac, disease  

     
     Pediatric Radiology Cases Cite this article:

    D Weber. Celiac disease. PedRad [serial online] vol 4, no. 2.
    URL: www.PedRad.info/?search=20040207222335  

     
     Pediatric Radiology Cases Read similar articles: disease&type=1-17">corresponding keywords
    in the same field:
    or in the region:
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     Pediatric Radiology CasesImages to this case: There are Ultrasound-images available for this case. [ Ultrasound ] View all modalities [ All ]   
     
    Celiac disease
    D Weber. Celiac disease. PedRad [serial online] vol 4, no. 2.
    URL: www.PedRad.info/?search=20040207222335


     

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    Celiac disease  
     
    Celiac disease
    D Weber. Celiac disease. PedRad [serial online] vol 4, no. 2.
    URL: www.PedRad.info/?search=20040207222335


     

    Which diagnosis have other collegues guessed?


    • Polysplenia Syndrome
      Votes: 7 (35 %)


    • Intestinal malrotation
      Votes: 1 (5 %)


    • Pre-duodenal portal vein
      Votes: 0 (0 %)


    • None of the above
      Votes: 0 (0 %)


    • All of the above
      Votes: 12 (60 %)


    • IRDS
      Votes: 1 (1 %)


    • Pneumothorax with pneumomediastinum
      Votes: 28 (50 %)


    • Pneumonia
      Votes: 1 (1 %)


    • Child abuse
      Votes: 1 (1 %)


    • Ebstein anomaly
      Votes: 4 (7 %)


    • Gastric carcinoma
      Votes: 0 (0 %)


    • Phytobezoar
      Votes: 0 (0 %)


    • Ascarides
      Votes: 1 (1 %)


    • Trichobezoar
      Votes: 19 (25 %)


    • pyloric hypertrophy
      Votes: 0 (0 %)


    • pancreas annulare
      Votes: 5 (5 %)


    • congenital internal duodenal stenosis
      Votes: 6 (6 %)


    • Ladd syndrome
      Votes: 2 (2 %)


    • Achondroplasia
      Votes: 0 (0 %)


    • Hypochondroplasia
      Votes: 0 (0 %)


    • Osteogenesis imperfecta
      Votes: 17 (16 %)


    • Diastrophic Dysplasia
      Votes: 0 (0 %)


    • Neuritis of the trigeminal nerve
      Votes: 0 (0 %)


    • Trigeminal neuralgia through SCA anomaly
      Votes: 9 (7 %)


    • Neurinoma of the trigeminal nerve
      Votes: 0 (0 %)


    • Fibrotic dysplasia in the skull base region
      Votes: 0 (0 %)


    • Mesenterial lymphadenitis
      Votes: 12 (8 %)


    • Celiac disease
      Votes: 8 (5 %)


    • Hodgkin's disease
      Votes: 1 (0 %)


    • Peutz-Jegher's Syndrome
      Votes: 2 (1 %)



        Total answers: 137

     
    Celiac disease
    D Weber. Celiac disease. PedRad [serial online] vol 4, no. 2.
    URL: www.PedRad.info/?search=20040207222335


     

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    Celiac disease
    D Weber. Celiac disease. PedRad [serial online] vol 4, no. 2.
    URL: www.PedRad.info/?search=20040207222335


     


    Go to the top of the page   ID: 20040206232311 Original case in english  More links about this topic on Pubmed (PubMed Reader)
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    Discussion
     
    Kohler's Disease II, Bilateral Freiberg-Kohler Disease
    Ina Sorge. Kohler's Disease II, Bilateral Freiberg-Kohler Disease. PedRad [serial online] vol 4, no. 2.
    URL: www.PedRad.info/?search=20040206232311


     
     Pediatric Radiology CasesImages to this case: There are X-Ray-images available for this case. [ X-Ray ] View all modalities [ All ]   
     Pediatric Radiology CasesAuthor/s:

    Ina Sorge (Kinderradiologie/Uni Leipzig)  

     
     Pediatric Radiology CasesEmail Address:

    Viewable for logged on visitors (Log on)  

     
     Pediatric Radiology CasesAge:

    13 Years  

     
     Pediatric Radiology CasesGender:

    Female  

     
     Pediatric Radiology CasesRegion-Organ:

    N/A  

     
     Pediatric Radiology CasesMost likely etiology:

    N/A  

     
     Pediatric Radiology CasesHistory:

    Pain initially in the left foot; a few months later also in the right foot, particularly on exertion. Slight swelling, pain on pressure.  

     
     Pediatric Radiology CasesPathomorphology or Pathophysiology of this disease :

    Aseptic bone necrosis are seen in children along with cartilage ossification defects throughout growth or throughout increased exertion. A cause may be passing intraossic vascularization disturbances through a mycotic-embolic vessel obstruction, bland infections, trauma, vessel spasms, vegetative dysregulations or vessel trauma. Constitutional and alimentary factors seem to play a role as well. The infestation can be unilateral or bilateral.  

     
     Pediatric Radiology CasesRadiological findings:


    CT 1 <- view CT 1

    CT 1: Axial CT of the upper abdomen demonstrates multiple spleens of different sizes in the posterior LUQ.



    CT 2 <- view CT 2

    CT 2: Axial CT shows enlarged azygous vein adjacent to the aorta in the lower chest, representing azygous continuation of the abdominal IVC.



    CT 3 <- view CT 3

    CT 3: Coronal CT shows the multiple spleens in the LUQ and azygous continuation of the IVC paralleling the aorta along the right.





    CT 4 <- view CT 4

    CT 4: Axial CT of the mid-abdomen demonstrates short pancreas with no visualized distal body or tail of the pancreas. The contrast opacified vessel seen anterior to the shorted pancreas represents a pre-duodenal portal vein. Also, again noted are several spleens in the left abdomen anterior and lateral to the left kidney.




    CT 5 <- view CT 5

    CT 5: Axial CT of the abdomen shows the small bowel entirely within the right abdomen and the colon located only with the left abdomen. There is also abnormal orientation of the the mesenteric vessels visualized with the superior mesenteric vein seen to the left of the superior mesenteric artery.



    CT 6 <- view CT 6

    CT 6: Axial CT of the lower abdomen demonstrates small bowel within the right lower quadrant with abdnormal location of the cecum and appendix identified within the left lower quadrant.


    X-Ray 1 <- view X-Ray 1

    X-Ray 1: Large lucency surrounds the superior mediastinum with lucency crossing midline. Subtle lucency is also seen inferior and lateral of the right lung base at the costophrenic angle and hemidiaphragm.





    X-Ray 2 <- view X-Ray 2

    X-Ray 2: Large lucency surrounds the superior aspect of the anterior mediastinum with elevation of the thymic shadow.




    MRI 1 <- view MRI 1

    MRI 1: Axial T2 sections showing dilated CBD with intraluminal sludge and calculi.





    MRI 2 <- view MRI 2

    MRI 2: Axial T2 sections showing multiple small calculi in gall bladder neck (arrow).





    MRI 3 <- view MRI 3

    MRI 3: Axial T2 sections showing gall bladder ( short arrow) and a cystic structure (long arrow)





    MRI 4 <- view MRI 4

    MRI 4: Axial T2 sections showing a cystic structure with luminal sludge and the gall bladder.





    MRI 5 <- view MRI 5

    MRI 5: Axial T2 sections showing dilated CBD anterior to IVC.




    MRI 6 <- view MRI 6

    MRI 6: MRCP showing gall bladder with calculi (short arrow) and a cystic structure (long arrow).





    MRI 7 <- view MRI 7

    MRI 7: MRCP showing both gall bladder and a cystic structure.





    MRI 8 <- view MRI 8

    MRI 8: MRCP showing small calculi in a cystic structure (arrow)




    MRI 1 <- view MRI 1

    MRI 1: T1W MRI showing hyperintense mass in right CP angle cistern extending into the internal auditory canal.




    MRI 2 <- view MRI 2

    MRI 2: T2W MRI showing hyperintense mass with splaying of 7th and 8th cranial nerves.




    MRI 3 <- view MRI 3

    MRI 3: T1W post gadolinium fat suppressed image showing nonenhancing mass with fat suppression.




    X-Ray 1 <- view X-Ray 1

    X-Ray 1: Chest x ray (AP view) showing metallic foreign body (a key) in lower cervical and superior mediastinum area.





    OP-Situs 1 <- view OP-Situs 1

    OP-Situs 1: Showing the foreign body (key) after removal.


    MRI 1 <- view MRI 1

    MRI 1: T2 weighted axial image with markedly decreased sulcal pattern (= pachygyria) and thin cortex. Furthermore seen is a hemorrhage into the right lateral ventricle (hypointense).



    MRI 2 <- view MRI 2

    MRI 2: T1 weighted axial image demonstrates also significant pachygyria and prominent external CSF spaces. The fluid-fluid level containing hemorrhage in the posterior horns of the right lateral ventricle is better deliniated on the T1 weighted sequence.



    MRI 3 <- view MRI 3

    MRI 3: T2 weighted axial image at the level of the basal ganglia, which appear normal.



    MRI 4 <- view MRI 4

    MRI 4: Coronal CSF suppressed T2 weighted sequence (FLAIR) also demonstrates a markedly decreased sulcal pattern.



    Ultrasound 1 <- view Ultrasound 1

    Ultrasound 1: Wall thickened, hypervascular small bowel loop in the axial plane.





    CT 1 <- view CT 1

    CT 1: Significantly wall thickened segment of jejunum in the left lower and mid abdomen with diffuse surrounding inflammatory reaction, lymphadenopathy and ascites.





    CT 2 <- view CT 2

    CT 2: Significantly wall thickened segment of jejunum in the left lower and mid abdomen with diffuse surrounding inflammatory reaction, lymphadenopathy and ascites.





    OP-Situs 1 <- view OP-Situs 1

    OP-Situs 1: Inflammatory changes and purple coloration of a small bowel loop, presenting as pseudotumor.





    Histology 1 <- view Histology 1

    Histology 1: Hemorrhagic necrotising enteritis with leukocytoclastic vasculitis.


    Ultrasound 1 <- view Ultrasound 1

    Ultrasound 1: Ultrasound of the back of the head
    Left occipital identified is a 2 cm, subcutaneous, inhomogeneous and hypoechoic mass lesion. In this area, there is about 12 mm cortical discontinuity. Dura appears deviated inferiorly.
    Color coded Duplex sonography: Increased vascularization in the periphery of the lesion. (Images not shown)
    Ultrasound of the abdomen is unremarkable. (Images not shown)




    CT 1 <- view CT 1

    CT 1: CCT Brain window
    Left occipital osteolytic lesion with soft tissue components, no further osteodestructive findings.






    CT 2 <- view CT 2

    CT 2: CCT Bone window
    Left occipital osteolytic lesion with soft tissue components, no further osteodestructive findings.






    MRI 1 <- view MRI 1

    MRI 1: MRI of the head - T1 axial
    Left occipital soft tissue mass with marked marginal contrast enhancement, size ca. 1,8 x 2,8 x 1,5 cm. It causes the known defect in the posterior skull and mild dural displacement to the inside.
    There is surrounding leptomeningeal contrast enhancement. There is increased T2 signal and fluid level in the center of the lesion, which represents a trauma related secondary hemorrhage.





    MRI 2 <- view MRI 2

    MRI 2: MRI of the head - T2 axial






    MRI 3 <- view MRI 3

    MRI 3: MRI of the head - T1 axial with contrast






    MRI 4 <- view MRI 4

    MRI 4: MRI of the head - T1 spir coronal (oblique coronal with surface coil) with contrast




    MRI 1 <- view MRI 1

    MRI 1: T2-weighted HASTE sequence, coronal.
    Large inhomogeneous, hypointense mass in the T2-weighted image, which fills almost the entire stomach, and continues to the duodenum.






    MRI 2 <- view MRI 2

    MRI 2: T2-weighted HASTE sequence, coronal.
    Large inhomogeneous, hypointense mass in the T2-weighted image, which fills almost the whole stomach, and continues to the duodenum.






    MRI 3 <- view MRI 3

    MRI 3: T2-weighted HASTE sequence, transversal: Here, a large, inhomogeneous, hypointense mass (which has sharp margins), which almost completely fills the stomach. There is distal extension through the pylorus into the duodenum.
    There is a small fluid level to the right and lateral to the mass and air ventral to the mass inside the stomach.




    MRI 4 <- view MRI 4

    MRI 4: T1 weighted 3D-GE-Sequence with fat-saturation and before constrast application. Conspicuous fat saturation in the T1-weighted image, which shows inhomogeneous, hypointense mass in the stomach lumen without evidence of contrast uptake after Gd-DTPA application. Some air in the stomach to the right and lateral of the mass.




    MRI 5 <- view MRI 5

    MRI 5: T1 weighted 3D-GE-Sequence with fat-saturation after contrast application, transversal. Conspicuous fat saturation in the T1-weighted image, which shows an inhomogeneous, hypointense mass in the stomach lumen without evidence of contrast uptake after Gd-DTPA application. Some air in the stomach to the right and lateral of the mass. There is intense contrast uptake in the stomach wall.




    OP-Situs 1 <- view OP-Situs 1

    OP-Situs 1: Specimen





    Pathology 1 <- view Pathology 1

    Pathology 1: Specimen


    X-Ray 1 <- view X-Ray 1

    X-Ray 1: Abdominal overview, hanging. Large stomach bubble in the left upper quadrant, duodenal bubble to the right of the spine, little air in the rest of the abdomen.






    X-Ray 2 <- view X-Ray 2

    X-Ray 2: UGI: Contrast media column with abrupt stop at the transition to the pars horizontalis.






    Ultrasound 1 <- view Ultrasound 1

    Ultrasound 1: Hypoechoic structure with internal floating echos seen in the subhepatic region on the transverse abdominal view.