KINDERRADIOLOGIE ONLINE - view radiology images and radiology cases
Lesen Sie diese Seite auf Deutsch   Read this page in English
The Peer Reviewed Pediatric Radiology Platform on the Web
ISSN: 1942-955X :: Publisher: EduRad ::Editor-In-Chief: Roland Talanow, MD, PhD  
Browse Browse || Search Search || Online Book Systematically Online Book Systematically || Discussion Forum Discussion Forum || News News || Submit a Case Submit a Case || Rules Rules |
Most Interesting Cases Most Interesting Cases || "Most Diligent" Authors "Most Diligent" Authors || Case of the Day Case of the Day || Teaching files Teaching files || Links Links |
Newsletter Newsletter || Start Start || Survey Survey || Your Opinion Your Opinion || My Profile My Profile || CME-Credits CME-Credits |
    Pediatric Radiology News / Ads / Jobs Get the Pediatric Radiology News as RSS feed !
    Send us your news or ads for free
  • CME Kurs: Paediatric Body MRI

  • CME course: Paediatric Body MRI

  • Universitätsklinikum Jena sucht eine Ärztin/einen Arzt in Weiterbildung in Vollzeit

  • Kinderspital Zürich sucht Oberärztin / Oberarzt (100 %)

  • Kinderradiologische Oberärztin / Oberarzt gesucht

  • 2 Stellen in der Kinderradiologie - 1 Assistenzärztin/Assistenzarzt und 1 Oberärztin/Oberarzt in Voll- oder Teilzeit

  • Senior Paediatric Radiologist in Pediatric Radiology section needed

  • Facharzt als Oberärztin / Oberarzt in Sektion Kinderradiologie gesucht

  • Stellenangebot für Assistenzarzt (m/w) zur Facharztausbildung für RADIOLOGIE und/oder KINDERRADIOLOGIE

  • „Fellow“-Stelle zur Weiterbildung in Pädiatrischer Radiologie am Kinderspital Zürich

  • Fellow-Stelle Pädiatrische Neuroradiologie

  • FFF Kinderradiologie - Muskuloskelettale Radiologie im Kindesalter (Fit für den Facharzt)

  • Assistenzarzt für Kinderradiologe gesucht an Universitätsklinikum Schleswig-Holstein, Campus Kiel

  • Turnusarzt (m/w) in Ausbildung zum Facharzt (m/w) für Radiologie

  • Assistenzarzt (m/w) zur Weiterbildung im Schwerpunkt Kinderradiologie

  • Send us your news or ads for free

    Get the Pediatric Radiology News as RSS feed !


    Radiolopolis

    The dynamic Radiology community for education, research and practice


     

    There are 7 cases available...

    Go to the top of the page   ID: 20080327175230 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
     
    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:

    Generalized diseases  

     
     Pediatric Radiology CasesMost likely etiology:

    unknown  

     
     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:


    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:

    Surgery / Histo  

     
     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:

    1. 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

    2. 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

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

    4. 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: Generalized diseases
    or in the region: Generalized diseases
    or in the tissue/organ:
    or with the etiology: unknown
     
     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


     

    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



    Henoch-Schoenlein Purpura
    Other cases by these authors:

    Search K. Gerlach in Medline K. Gerlach (5)   
    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


     
     
    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: 20060322175555 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
     
    Primary sclerosing cholangitis (PSC)
    A Nordwig, N Lorenz, B Biereder, T Haufe, M Kabus. Primary sclerosing cholangitis (PSC). PedRad [serial online] vol 6, no. 3.
    URL: www.PedRad.info/?search=20060322175555


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

    A. Nordwig, N. Lorenz, B. Biereder, T. Haufe, M. Kabus (Städtisches Krankenhaus Dresden-Neustadt-Deutschland)  

     
     Pediatric Radiology CasesEmail Address:

    Viewable for logged on visitors (Log on)  

     
     Pediatric Radiology CasesAge:

    15 Years  

     
     Pediatric Radiology CasesGender:

    Male  

     
     Pediatric Radiology CasesRegion-Organ:

    N/A  

     
     Pediatric Radiology CasesMost likely etiology:

    N/A  

     
     Pediatric Radiology CasesHistory:

    - 15 year old boy, clinically and neurologically unremarkable
    - 03/2005 Diagnosis of rohn's disease, therapy: Azathioprin, Mesalazin
    - 11/2005 generalised pruritus, mild scleric icterus
    - Labs: Leukocytes 4,1 Gpt/l, Thrombocytes 152 Gpt/l, ASAT 2,28 µmol/s*l, ALAT 3,11 µmol/s*l, Gamma-GT 4,11 µmol/s*l, Bilirubin gesamt 40 µmol/l, Bilirubin direct 17 µmol/l, Bile acides 418 µmol/l , BSG, Alpha1-Glycoprotein, AP, Coagulation, Albumin, Protein electrophoresis, Cholinesterase, Ammonia, Alpha1-Antitrypsin, Iron, Ferritin, Copper, Coeruloplasmin within normal limits.
    - Autoantibody screening: pANCA positive, ANA, AMA, SMA, LKM, PBCNA, LC1 negative.
     

     
     Pediatric Radiology CasesPathomorphology or Pathophysiology of this disease :

    PSC is associated in 87% with Ulcerative colitis and in 13% with Crohn's disease and precedes the cIBD (chronic inflammatory bowel disease) symptoms in 50% (Wilschanski M et al, 1995). The pathogenesis of the chronic inflammation of the bile ducts is not clear. Discussed are chronische portal bacterimia, toxic bile metabolites from the intestinal flora, ischemic vascular factors and autoimmun processes which lead to an inflammation and scarring with stenosis of the bile ducts. Affected are initially in most cases small intrahepatic and later on also extrahepatic bile ducts.  

     
     Pediatric Radiology CasesRadiological findings:


    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: Ductus choledochus above mildly dilated Vena portae with changing caliber. Increased echogenicity of the wall of the Ductus choledochus.



    Ultrasound 2 <- view Ultrasound 2

    Ultrasound 2: Ductus choledochus above mildly dilated Vena portae with changing caliber. Increased echogenicity of the wall of the Ductus choledochus.



    Ultrasound 3 <- view Ultrasound 3

    Ultrasound 3: Increased liver echogenicity; Bile ducts partially up to the periphery irregularly dilated.



    Ultrasound 4 <- view Ultrasound 4

    Ultrasound 4: Minimal distended gallbladder with significant wall thickening and shadowing.



    MRI 1 <- view MRI 1

    MRI 1: MRCP: No dilatation of the intra- and extrahepatic bile ducts. Slightly irregularity of the walls of the Ductus choledochus. No changes in caliber of the intrahepatic bile ducts.





    X-Ray 1 <- view X-Ray 1

    X-Ray 1: ERCP: Rarefication of the intrahepatic bile ducts with multiple stenoses of different lenght especially in the right liver lobe. Extrahepatic bile ducts are not significantly dilated, Ductus cysticus serpentinuous.



     

     
     Pediatric Radiology CasesDiagnosis confirmation:

    N/A  

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

    Drug induced hepatopathy (Azathioprin), autoimmunehepatitis, primary biliary cirrhosis.  

     
     Pediatric Radiology CasesCourse / Prognosis / Frequency / Other :

    PSC leads often to liver transplantation. Feared is PSC within the transplanted organ. Course and grade of PSC are independent of the activity of cIBD. Therapy of choice is ursodesoxycholic acid with a positive, but only transient effect on the symptoms and lab results (Feldstein AE et al, 2003). There is no proven influence on mortality or liver transplant rate by ursodesoxycholic acid (Breuers U et al, 1992). Very high doses of ursodesoxycholic acid improve possible the log time survival in a PSC patient (40 mg/kg KG). The early endoscopic dilatation of stenotic bile ducts and eventual stent implant is effective in addition to drug therapy (Stiehl A et al, 2002).  

     
     Pediatric Radiology CasesComments of the author about the case:

    N/A  

     
     Pediatric Radiology CasesFirst description / History:

    N/A  

     
     Pediatric Radiology CasesLiterature:

    N/A  

     
     Pediatric Radiology CasesKeywords:

    Primary sclerosing cholangitis, PSC, Crohn, liver disease, Crohn's disease, generalised pruritus, icterus, scleric icterus  

     
     Pediatric Radiology Cases Cite this article:

    A Nordwig, N Lorenz, B Biereder, T Haufe, M Kabus. Primary sclerosing cholangitis (PSC). PedRad [serial online] vol 6, no. 3.
    URL: www.PedRad.info/?search=20060322175555  

     
     Pediatric Radiology Cases Read similar articles: scleric icterus&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 ] There are MRI-images available for this case. [ MRI ] View all modalities [ All ]   
     
    Primary sclerosing cholangitis (PSC)
    A Nordwig, N Lorenz, B Biereder, T Haufe, M Kabus. Primary sclerosing cholangitis (PSC). PedRad [serial online] vol 6, no. 3.
    URL: www.PedRad.info/?search=20060322175555


     

    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



    Primary sclerosing cholangitis (PSC)
    Other cases by these authors:

    Search K. Gerlach in Medline K. Gerlach (5)   
    Search P. Göbel in Medline P. Göbel (6)   
    Search A. Nordwig in Medline A. Nordwig (20)   
    Search N. Lorenz in Medline N. Lorenz (1)   
    Search B. Biereder in Medline B. Biereder (1)   
    Search T. Haufe in Medline T. Haufe (4)   
    Search M. Kabus in Medline M. Kabus (1)   

    Primary sclerosing cholangitis (PSC)  
     
    Primary sclerosing cholangitis (PSC)
    A Nordwig, N Lorenz, B Biereder, T Haufe, M Kabus. Primary sclerosing cholangitis (PSC). PedRad [serial online] vol 6, no. 3.
    URL: www.PedRad.info/?search=20060322175555


     

    Which diagnosis have other collegues guessed?


    • Primary sclerosing cholangitis
      Votes: 16 (88 %)


    • Caroli syndrome
      Votes: 1 (5 %)


    • Obstructing concrement in the ductus choledochus
      Votes: 0 (0 %)


    • Hepatitis
      Votes: 0 (0 %)


    • Liver cirrhosis
      Votes: 1 (5 %)



        Total answers: 18

     
    Primary sclerosing cholangitis (PSC)
    A Nordwig, N Lorenz, B Biereder, T Haufe, M Kabus. Primary sclerosing cholangitis (PSC). PedRad [serial online] vol 6, no. 3.
    URL: www.PedRad.info/?search=20060322175555


     

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




    1 = less interesting)
     
    Primary sclerosing cholangitis (PSC)
    A Nordwig, N Lorenz, B Biereder, T Haufe, M Kabus. Primary sclerosing cholangitis (PSC). PedRad [serial online] vol 6, no. 3.
    URL: www.PedRad.info/?search=20060322175555


     


    Go to the top of the page   ID: 20051005175954 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
     
    Xanthogranulomatous Pyelonephritis
    M Zieger. Xanthogranulomatous Pyelonephritis. PedRad [serial online] vol 5, no. 10.
    URL: www.PedRad.info/?search=20051005175954


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

    M. Zieger (Radiologisches Institut Olgahospital Stuttgart Deutschland)  

     
     Pediatric Radiology CasesEmail Address:

    Viewable for logged on visitors (Log on)  

     
     Pediatric Radiology CasesAge:

    3 Years  

     
     Pediatric Radiology CasesGender:

    Male  

     
     Pediatric Radiology CasesRegion-Organ:

    N/A  

     
     Pediatric Radiology CasesMost likely etiology:

    N/A  

     
     Pediatric Radiology CasesHistory:

    3.5 year old boy, "never been sick until now".
    Feels fatigued for the last few weeks with leg and abdominal pain. Oral antibiotic therapy without success. Urine after urinary catheterization sterile.  

     
     Pediatric Radiology CasesPathomorphology or Pathophysiology of this disease :

    The etiology is uncler, but chronic obstructions with infections are commonly seen. Calcifications are seen in 80% of the patients. Xanthogranulomatous pyelonephritis is often associated with diabetes or immunocomprimised patients, maybe even fatty acid metabolic disorders.
    Pathology:
    Localized tissue destruction, circumducted infiltration is possible. The process holds yellowish tissiue with necroses and hemorrhages.
    Microscopically, there are spongy, fat-laden macrophages and chronic and acute inflammatory responses at the same time.  

     
     Pediatric Radiology CasesRadiological findings:


    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: Ductus choledochus above mildly dilated Vena portae with changing caliber. Increased echogenicity of the wall of the Ductus choledochus.



    Ultrasound 2 <- view Ultrasound 2

    Ultrasound 2: Ductus choledochus above mildly dilated Vena portae with changing caliber. Increased echogenicity of the wall of the Ductus choledochus.



    Ultrasound 3 <- view Ultrasound 3

    Ultrasound 3: Increased liver echogenicity; Bile ducts partially up to the periphery irregularly dilated.



    Ultrasound 4 <- view Ultrasound 4

    Ultrasound 4: Minimal distended gallbladder with significant wall thickening and shadowing.



    MRI 1 <- view MRI 1

    MRI 1: MRCP: No dilatation of the intra- and extrahepatic bile ducts. Slightly irregularity of the walls of the Ductus choledochus. No changes in caliber of the intrahepatic bile ducts.





    X-Ray 1 <- view X-Ray 1

    X-Ray 1: ERCP: Rarefication of the intrahepatic bile ducts with multiple stenoses of different lenght especially in the right liver lobe. Extrahepatic bile ducts are not significantly dilated, Ductus cysticus serpentinuous.




    Ultrasound 1 <- view Ultrasound 1

    Ultrasound 1: Nephromegaly, Calcifications with acoustic shadowing, calyectasia, lymphnodes in the renal hilus. The renal configuration is normal, but there are abscess-like lesions (nephromegaly in papillary region, the renal cortex is maintained).



    Ultrasound 2 <- view Ultrasound 2

    Ultrasound 2: Renal hilus transversal:
    enlarged lymphnodes



    Ultrasound 3 <- view Ultrasound 3

    Ultrasound 3: Overview kidney:
    Nephromegaly, Calcifications with acoustic shadowing, calyectasia, lymph nodes in the renal hilus. The renal configuration is normal, but there are abscess-like lesions (in papillary region, the renal cortex is maintained).




    Ultrasound 4 <- view Ultrasound 4

    Ultrasound 4: Detailed image of the parenchymal structure





    OP-Situs 1 <- view OP-Situs 1

    OP-Situs 1: Renal situs opened longitudinal

     

     
     Pediatric Radiology CasesDiagnosis confirmation:

    N/A  

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

    Tumor  

     
     Pediatric Radiology CasesCourse / Prognosis / Frequency / Other :

    Surgery: Nephrectomy  

     
     Pediatric Radiology CasesComments of the author about the case:

    N/A  

     
     Pediatric Radiology CasesFirst description / History:

    1916 by Schlagenhaufer
    “Über eigentümliche Staphylmykosen der Nieren und des pararenalen Bindegewebes” 1916; 19: 139-48 Frankfurt Z Pathol  

     
     Pediatric Radiology CasesLiterature:

    N/A  

     
     Pediatric Radiology CasesKeywords:

    Kidney, Kidneys, Xanthogranuloma , Pyelonephritis, xanthogranulomatous Pyelonephritis, inflammation of the Kidney  

     
     Pediatric Radiology Cases Cite this article:

    M Zieger. Xanthogranulomatous Pyelonephritis. PedRad [serial online] vol 5, no. 10.
    URL: www.PedRad.info/?search=20051005175954  

     
     Pediatric Radiology Cases Read similar articles: inflammation of the Kidney &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 OP-Situs-images available for this case. [ OP-Situs ] View all modalities [ All ]   
     
    Xanthogranulomatous Pyelonephritis
    M Zieger. Xanthogranulomatous Pyelonephritis. PedRad [serial online] vol 5, no. 10.
    URL: www.PedRad.info/?search=20051005175954


     

    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



    Xanthogranulomatous Pyelonephritis
    Other cases by these authors:

    Search K. Gerlach in Medline K. Gerlach (5)   
    Search P. Göbel in Medline P. Göbel (6)   
    Search A. Nordwig in Medline A. Nordwig (20)   
    Search N. Lorenz in Medline N. Lorenz (1)   
    Search B. Biereder in Medline B. Biereder (1)   
    Search T. Haufe in Medline T. Haufe (4)   
    Search M. Kabus in Medline M. Kabus (1)   
    Search M. Zieger in Medline M. Zieger (17)   

    Xanthogranulomatous Pyelonephritis  
     
    Xanthogranulomatous Pyelonephritis
    M Zieger. Xanthogranulomatous Pyelonephritis. PedRad [serial online] vol 5, no. 10.
    URL: www.PedRad.info/?search=20051005175954


     

    Which diagnosis have other collegues guessed?


    • Primary sclerosing cholangitis
      Votes: 16 (88 %)


    • Caroli syndrome
      Votes: 1 (5 %)


    • Obstructing concrement in the ductus choledochus
      Votes: 0 (0 %)


    • Hepatitis
      Votes: 0 (0 %)


    • Liver cirrhosis
      Votes: 1 (5 %)


    • Nephroblastoma
      Votes: 2 (5 %)


    • Xanthogranulomatous Pyelonephritis
      Votes: 14 (38 %)


    • Renal carcinoma
      Votes: 0 (0 %)


    • Refluxnephropathy
      Votes: 0 (0 %)


    • Glomerulonephritis
      Votes: 2 (5 %)



        Total answers: 36

     
    Xanthogranulomatous Pyelonephritis
    M Zieger. Xanthogranulomatous Pyelonephritis. PedRad [serial online] vol 5, no. 10.
    URL: www.PedRad.info/?search=20051005175954


     

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




    1 = less interesting)
     
    Xanthogranulomatous Pyelonephritis
    M Zieger. Xanthogranulomatous Pyelonephritis. PedRad [serial online] vol 5, no. 10.
    URL: www.PedRad.info/?search=20051005175954


     


    Go to the top of the page   ID: 20040123095758 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
     
    Bladder Diverticulum with Double Kidneys and Duplicated Ureter
    Dirk Schaper, P Göbel. Bladder Diverticulum with Double Kidneys and Duplicated Ureter. PedRad [serial online] vol 4, no. 1.
    URL: www.PedRad.info/?search=20040123095758


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

    Dirk Schaper, P. Göbel (Kinderchirurgie St. Barbara-Krankenhaus Halle/S.)  

     
     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:

    5 year-old boy without clinical complaints. Incidental findings on sonogram (Ultrasound 1) in the course of treatment for balanitis. Right-sided duplicated urinary tract system without urinary obstruction. Left: unremarkable kidney.  

     
     Pediatric Radiology CasesPathomorphology or Pathophysiology of this disease :

    N/A  

     
     Pediatric Radiology CasesRadiological findings:


    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: Ductus choledochus above mildly dilated Vena portae with changing caliber. Increased echogenicity of the wall of the Ductus choledochus.



    Ultrasound 2 <- view Ultrasound 2

    Ultrasound 2: Ductus choledochus above mildly dilated Vena portae with changing caliber. Increased echogenicity of the wall of the Ductus choledochus.



    Ultrasound 3 <- view Ultrasound 3

    Ultrasound 3: Increased liver echogenicity; Bile ducts partially up to the periphery irregularly dilated.



    Ultrasound 4 <- view Ultrasound 4

    Ultrasound 4: Minimal distended gallbladder with significant wall thickening and shadowing.



    MRI 1 <- view MRI 1

    MRI 1: MRCP: No dilatation of the intra- and extrahepatic bile ducts. Slightly irregularity of the walls of the Ductus choledochus. No changes in caliber of the intrahepatic bile ducts.





    X-Ray 1 <- view X-Ray 1

    X-Ray 1: ERCP: Rarefication of the intrahepatic bile ducts with multiple stenoses of different lenght especially in the right liver lobe. Extrahepatic bile ducts are not significantly dilated, Ductus cysticus serpentinuous.




    Ultrasound 1 <- view Ultrasound 1

    Ultrasound 1: Nephromegaly, Calcifications with acoustic shadowing, calyectasia, lymphnodes in the renal hilus. The renal configuration is normal, but there are abscess-like lesions (nephromegaly in papillary region, the renal cortex is maintained).



    Ultrasound 2 <- view Ultrasound 2

    Ultrasound 2: Renal hilus transversal:
    enlarged lymphnodes



    Ultrasound 3 <- view Ultrasound 3

    Ultrasound 3: Overview kidney:
    Nephromegaly, Calcifications with acoustic shadowing, calyectasia, lymph nodes in the renal hilus. The renal configuration is normal, but there are abscess-like lesions (in papillary region, the renal cortex is maintained).




    Ultrasound 4 <- view Ultrasound 4

    Ultrasound 4: Detailed image of the parenchymal structure





    OP-Situs 1 <- view OP-Situs 1

    OP-Situs 1: Renal situs opened longitudinal


    Ultrasound 1 <- view Ultrasound 1

    Ultrasound 1: Abdomen axial, bladder with an unclear, echogenic structure to the left in the trigonal region.




    X-Ray 1 <- view X-Ray 1

    X-Ray 1: Voiding cystourethrogram (VCUG) ap, left-sided vesico-ureteral-reflux (VUR) III°







    X-Ray 2 <- view X-Ray 2

    X-Ray 2: Voiding cystourethrogram (VCUG) oblique view, during urination. Bulging of the bladder dorsolaterally with oblique VUR III° on the left side, The left pelvicocaliceal system resembles a wilting flower .






    X-Ray 3 <- view X-Ray 3

    X-Ray 3: Voiding cystourethrogram (VCUG): further enlargening of the structure during micturition.






    Ultrasound 2 <- view Ultrasound 2

    Ultrasound 2: Abdomen axial, anechoic structure on the left side, changing in size during the exam, no depiction of the ureter.




    Ultrasound 3 <- view Ultrasound 3

    Ultrasound 3: Lower abdomen axial: Again demonstrated is the anechoic strcuture on the left, which is changing in size during the continuous examination. When the bladder contracts, enlargening.




    Ultrasound 4 <- view Ultrasound 4

    Ultrasound 4: Sonogram during urination, an obvious enlargening of the pathology




    Ultrasound 5 <- view Ultrasound 5

    Ultrasound 5: Bladder continues emptying, pathology becomes larger.




    Ultrasound 6 <- view Ultrasound 6

    Ultrasound 6: Bladder is empty, pathology further enlarged.




    OP-Situs 1 <- view OP-Situs 1

    OP-Situs 1: Intraoperative findings



     

     
     Pediatric Radiology CasesDiagnosis confirmation:

    N/A  

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

    Ureterocele, parostial diverticlum, megaureter  

     
     Pediatric Radiology CasesCourse / Prognosis / Frequency / Other :

    Since the sonographic image was initally unclear, a cystoscopy was performed. This showed a widened ostium in a prominent mucous membrane without signs of the diverticulum's entry side and without proof of a second ostium. In the VCUG, reflux to the lower pole moiety was demonstrated. The left pelvicocaliceal system resembled a wilting flower which allows the suspicion of a duplicated renal system.


    In the MAG3 scintigraphy, the functioning part of the left kidney was measured as 25.5%. No proof of an upper moiety.

    This resulted in an operation to resect the diverticulum and to replant the ureter. Intraoperatively, a second, hypoplastic ureter is seen, which does not show urine flow and is not able to be probed. The diverticulum is large, the ureter of the lower moiety connects inserts into the central portion of the diverticulum. The connection with the second ureter could not be seen. A resection of the diverticulum and the neoimplantation of the ureter into the lower moiety was performed. The hypoplastic ureter has been resected.  

     
     Pediatric Radiology CasesComments of the author about the case:

    The overall pathology is difficult to interpret. Is this a bladder diverticulum, in which both ureters join, or is this a protruded ureterocele? Because of the intraoperative situation and the obvious hypoplasia of the upper pole moiety uereter, it seems rather unlikely that this is a protruded ureterocele. Furthermore, a histological examination does not describe a ureterocele.

    In the follow-up examination and after the ureter was re-implanted, the left kidney is not dilated. An upper pole moiety is still not able to be demonstrated.
     

     
     Pediatric Radiology CasesFirst description / History:

    N/A  

     
     Pediatric Radiology CasesLiterature:

    5. Medline: Medline
    Minevich E, Moayed A, Wacksman J, Lewis AG, Sheldon CA.
    Unusual anatomic presentation of ectopic ureteroceles
    J Pediatr Surg. 1999 Mar; 34(3): 474-6

    6. Medline: Medline
    Bellah RD, Long FR, Canning DA
    Ureterocele eversion with vesicoureteral reflux in duplex kidneys: findings at voiding cystourethrography
    AJR Am J Roentgenol. 1995 Aug; 165(2): 409-13

    7. Medline: Medline
    Beasley SW, Kelly JH
    Inverted Y duplication of the ureter in association with ureterocele and bladder diverticulum
    J Urol. 1986 Oct; 136(4): 899-900  

     
     Pediatric Radiology CasesKeywords:

    duplicated kidney, duplicated collecting system, double kidney, bladder diverticulum, double, kidney, bladder, diverticulum, duplication, duplication of renal collecting system, renal duplication, ureteral duplication, ureter duplication  

     
     Pediatric Radiology Cases Cite this article:

    Dirk Schaper, P Göbel. Bladder Diverticulum with Double Kidneys and Duplicated Ureter. PedRad [serial online] vol 4, no. 1.
    URL: www.PedRad.info/?search=20040123095758  

     
     Pediatric Radiology Cases Read similar articles: ureter duplication&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 ] There are OP-Situs-images available for this case. [ OP-Situs ] View all modalities [ All ]   
     
    Bladder Diverticulum with Double Kidneys and Duplicated Ureter
    Dirk Schaper, P Göbel. Bladder Diverticulum with Double Kidneys and Duplicated Ureter. PedRad [serial online] vol 4, no. 1.
    URL: www.PedRad.info/?search=20040123095758


     

    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



    Bladder Diverticulum with Double Kidneys and Duplicated Ureter
    Other cases by these authors:

    Search K. Gerlach in Medline K. Gerlach (5)   
    Search P. Göbel in Medline P. Göbel (6)   
    Search A. Nordwig in Medline A. Nordwig (20)   
    Search N. Lorenz in Medline N. Lorenz (1)   
    Search B. Biereder in Medline B. Biereder (1)   
    Search T. Haufe in Medline T. Haufe (4)   
    Search M. Kabus in Medline M. Kabus (1)   
    Search M. Zieger in Medline M. Zieger (17)   
    Search Dirk Schaper in Medline Dirk Schaper (59)   
    Search P. Göbel in Medline P. Göbel (6)   

    Bladder Diverticulum with Double Kidneys and Duplicated Ureter  
     
    Bladder Diverticulum with Double Kidneys and Duplicated Ureter
    Dirk Schaper, P Göbel. Bladder Diverticulum with Double Kidneys and Duplicated Ureter. PedRad [serial online] vol 4, no. 1.
    URL: www.PedRad.info/?search=20040123095758


     

    Which diagnosis have other collegues guessed?


    • Primary sclerosing cholangitis
      Votes: 16 (88 %)


    • Caroli syndrome
      Votes: 1 (5 %)


    • Obstructing concrement in the ductus choledochus
      Votes: 0 (0 %)


    • Hepatitis
      Votes: 0 (0 %)


    • Liver cirrhosis
      Votes: 1 (5 %)


    • Nephroblastoma
      Votes: 2 (5 %)


    • Xanthogranulomatous Pyelonephritis
      Votes: 14 (38 %)


    • Renal carcinoma
      Votes: 0 (0 %)


    • Refluxnephropathy
      Votes: 0 (0 %)


    • Glomerulonephritis
      Votes: 2 (5 %)


    • Ureterocele
      Votes: 2 (3 %)


    • Parostial diverticlum
      Votes: 10 (17 %)


    • Megaureter
      Votes: 1 (1 %)


    • Bladder diverticulum in double kidney and duplicated ureter
      Votes: 7 (12 %)



        Total answers: 56

     
    Bladder Diverticulum with Double Kidneys and Duplicated Ureter
    Dirk Schaper, P Göbel. Bladder Diverticulum with Double Kidneys and Duplicated Ureter. PedRad [serial online] vol 4, no. 1.
    URL: www.PedRad.info/?search=20040123095758


     

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




    1 = less interesting)
     
    Bladder Diverticulum with Double Kidneys and Duplicated Ureter
    Dirk Schaper, P Göbel. Bladder Diverticulum with Double Kidneys and Duplicated Ureter. PedRad [serial online] vol 4, no. 1.
    URL: www.PedRad.info/?search=20040123095758


     


    Go to the top of the page   ID: 20030122112757 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
     
    Asphyxiating thoracic dysplasia (Jeune syndrome)
    J Blöcker, G Hahn. Asphyxiating thoracic dysplasia (Jeune syndrome). PedRad [serial online] vol 3, no. 1.
    URL: www.PedRad.info/?search=20030122112757


     
     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:

    J. Blöcker, G. Hahn (Dresden)  

     
     Pediatric Radiology CasesEmail Address:

    Viewable for logged on visitors (Log on)  

     
     Pediatric Radiology CasesAge:

    <1 Years  

     
     Pediatric Radiology CasesGender:

    N/A  

     
     Pediatric Radiology CasesRegion-Organ:

    N/A  

     
     Pediatric Radiology CasesMost likely etiology:

    N/A  

     
     Pediatric Radiology CasesHistory:

    Prenatally, an obvious dysplastic thorax and polyhydramy was seen in ultrasound. At the time of birth, eutrophic newborn 37+1, 45 cm, 2590 grams, 45 cm, 35 cm head circumference. Clinically noticeable thorax dysplasia (long, narrow thorax) with congenital lung hypoplasia and respiratory insufficiency (long-term respiration).

    Initally, patent ductus arteriosus and a small patent foramen ovale, which was no longer seen after 14 days.
    Oligury (during treatment with furosemide, spironolactone, etacrynic acid, diuresis was normal), obvious flank edema.  

     
     Pediatric Radiology CasesPathomorphology or Pathophysiology of this disease :

    -Autosomal-recessive illness.
    -Pathogenesis unknown.

    Typical findings:
    -short, horizontally running rib with widening of the costochondral connections; distally emphasized shortened and thickened long bones; three point configuration of the lower edge of the pelvis. Horizontal acetabula; early ossification of the femur head epiphyses
    -cystic tubular dysplasia; renal insufficiency with proteinuria and arterial hypertension; Nephronophtisis, polycystic kidney, usually starting in grade school.

     

     
     Pediatric Radiology CasesRadiological findings:


    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: Ductus choledochus above mildly dilated Vena portae with changing caliber. Increased echogenicity of the wall of the Ductus choledochus.



    Ultrasound 2 <- view Ultrasound 2

    Ultrasound 2: Ductus choledochus above mildly dilated Vena portae with changing caliber. Increased echogenicity of the wall of the Ductus choledochus.



    Ultrasound 3 <- view Ultrasound 3

    Ultrasound 3: Increased liver echogenicity; Bile ducts partially up to the periphery irregularly dilated.



    Ultrasound 4 <- view Ultrasound 4

    Ultrasound 4: Minimal distended gallbladder with significant wall thickening and shadowing.



    MRI 1 <- view MRI 1

    MRI 1: MRCP: No dilatation of the intra- and extrahepatic bile ducts. Slightly irregularity of the walls of the Ductus choledochus. No changes in caliber of the intrahepatic bile ducts.





    X-Ray 1 <- view X-Ray 1

    X-Ray 1: ERCP: Rarefication of the intrahepatic bile ducts with multiple stenoses of different lenght especially in the right liver lobe. Extrahepatic bile ducts are not significantly dilated, Ductus cysticus serpentinuous.




    Ultrasound 1 <- view Ultrasound 1

    Ultrasound 1: Nephromegaly, Calcifications with acoustic shadowing, calyectasia, lymphnodes in the renal hilus. The renal configuration is normal, but there are abscess-like lesions (nephromegaly in papillary region, the renal cortex is maintained).



    Ultrasound 2 <- view Ultrasound 2

    Ultrasound 2: Renal hilus transversal:
    enlarged lymphnodes



    Ultrasound 3 <- view Ultrasound 3

    Ultrasound 3: Overview kidney:
    Nephromegaly, Calcifications with acoustic shadowing, calyectasia, lymph nodes in the renal hilus. The renal configuration is normal, but there are abscess-like lesions (in papillary region, the renal cortex is maintained).




    Ultrasound 4 <- view Ultrasound 4

    Ultrasound 4: Detailed image of the parenchymal structure





    OP-Situs 1 <- view OP-Situs 1

    OP-Situs 1: Renal situs opened longitudinal


    Ultrasound 1 <- view Ultrasound 1

    Ultrasound 1: Abdomen axial, bladder with an unclear, echogenic structure to the left in the trigonal region.




    X-Ray 1 <- view X-Ray 1

    X-Ray 1: Voiding cystourethrogram (VCUG) ap, left-sided vesico-ureteral-reflux (VUR) III°







    X-Ray 2 <- view X-Ray 2

    X-Ray 2: Voiding cystourethrogram (VCUG) oblique view, during urination. Bulging of the bladder dorsolaterally with oblique VUR III° on the left side, The left pelvicocaliceal system resembles a wilting flower .






    X-Ray 3 <- view X-Ray 3

    X-Ray 3: Voiding cystourethrogram (VCUG): further enlargening of the structure during micturition.






    Ultrasound 2 <- view Ultrasound 2

    Ultrasound 2: Abdomen axial, anechoic structure on the left side, changing in size during the exam, no depiction of the ureter.




    Ultrasound 3 <- view Ultrasound 3

    Ultrasound 3: Lower abdomen axial: Again demonstrated is the anechoic strcuture on the left, which is changing in size during the continuous examination. When the bladder contracts, enlargening.




    Ultrasound 4 <- view Ultrasound 4

    Ultrasound 4: Sonogram during urination, an obvious enlargening of the pathology




    Ultrasound 5 <- view Ultrasound 5

    Ultrasound 5: Bladder continues emptying, pathology becomes larger.




    Ultrasound 6 <- view Ultrasound 6

    Ultrasound 6: Bladder is empty, pathology further enlarged.




    OP-Situs 1 <- view OP-Situs 1

    OP-Situs 1: Intraoperative findings




    X-Ray 1 <- view X-Ray 1

    X-ray 1: Thorax a. p. : short, horizontally running rib with widening of the costochondral connections.







    X-Ray 2 <- view X-Ray 2

    X-ray 2: pelvis a. p. : typical three point configuration of the lower edge




    X-Ray 3 <- view X-Ray 3

    X-ray 3: baby image: long bones (Femur/Humerus) appear shortened in comparison to the long thorax







     

     
     Pediatric Radiology CasesDiagnosis confirmation:

    N/A  

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

    Forms of the short-rib-polydactaly-syndrome.  

     
     Pediatric Radiology CasesCourse / Prognosis / Frequency / Other :

    Frequency:
    - Over 50 published cases

    Course/Prognosis:
    - Early infancy is often not outlived
    - Later, the kidney insufficiency determines the prognosis
    - The dyspnea lessens, the thoracic dysplasia remains the same.
    - Usually, normal intelligence  

     
     Pediatric Radiology CasesComments of the author about the case:

    Sonographic: Skull and abdomen unnoticeable.  

     
     Pediatric Radiology CasesFirst description / History:

    N/A  

     
     Pediatric Radiology CasesLiterature:

    8. Medline: Medline
    Wiedemann,Kunze
    Atlas der klinischen Syndrome 5. Auflage
    Schattauer GmbH 2001  

     
     Pediatric Radiology CasesKeywords:

    Asphyxiating thoracic dysplasia, Asphyxial thorax dysplasia, Jeune, Thorax, Asphyxiating, Dysplasia  

     
     Pediatric Radiology Cases Cite this article:

    J Blöcker, G Hahn. Asphyxiating thoracic dysplasia (Jeune syndrome). PedRad [serial online] vol 3, no. 1.
    URL: www.PedRad.info/?search=20030122112757  

     
     Pediatric Radiology Cases Read similar articles: Dysplasia&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 ]   
     
    Asphyxiating thoracic dysplasia (Jeune syndrome)
    J Blöcker, G Hahn. Asphyxiating thoracic dysplasia (Jeune syndrome). PedRad [serial online] vol 3, no. 1.
    URL: www.PedRad.info/?search=20030122112757


     

    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



    Asphyxiating thoracic dysplasia (Jeune syndrome)
    Other cases by these authors:

    Search K. Gerlach in Medline K. Gerlach (5)   
    Search P. Göbel in Medline P. Göbel (6)   
    Search A. Nordwig in Medline A. Nordwig (20)   
    Search N. Lorenz in Medline N. Lorenz (1)   
    Search B. Biereder in Medline B. Biereder (1)   
    Search T. Haufe in Medline T. Haufe (4)   
    Search M. Kabus in Medline M. Kabus (1)   
    Search M. Zieger in Medline M. Zieger (17)   
    Search Dirk Schaper in Medline Dirk Schaper (59)   
    Search P. Göbel in Medline P. Göbel (6)   
    Search J. Blöcker in Medline J. Blöcker (18)   
    Search G. Hahn in Medline G. Hahn (22)   

    Asphyxiating thoracic dysplasia (Jeune syndrome)  
     
    Asphyxiating thoracic dysplasia (Jeune syndrome)
    J Blöcker, G Hahn. Asphyxiating thoracic dysplasia (Jeune syndrome). PedRad [serial online] vol 3, no. 1.
    URL: www.PedRad.info/?search=20030122112757


     

    Which diagnosis have other collegues guessed?


    • Primary sclerosing cholangitis
      Votes: 16 (88 %)


    • Caroli syndrome
      Votes: 1 (5 %)


    • Obstructing concrement in the ductus choledochus
      Votes: 0 (0 %)


    • Hepatitis
      Votes: 0 (0 %)


    • Liver cirrhosis
      Votes: 1 (5 %)


    • Nephroblastoma
      Votes: 2 (5 %)


    • Xanthogranulomatous Pyelonephritis
      Votes: 14 (38 %)


    • Renal carcinoma
      Votes: 0 (0 %)


    • Refluxnephropathy
      Votes: 0 (0 %)


    • Glomerulonephritis
      Votes: 2 (5 %)


    • Ureterocele
      Votes: 2 (3 %)


    • Parostial diverticlum
      Votes: 10 (17 %)


    • Megaureter
      Votes: 1 (1 %)


    • Bladder diverticulum in double kidney and duplicated ureter
      Votes: 7 (12 %)


    • Short rib-polydactyly syndrome
      Votes: 4 (5 %)


    • Asphyxiating thoracic dysplasia (Jeune Syndrome)
      Votes: 6 (8 %)


    • Tantaphoric dysplasia
      Votes: 2 (2 %)


    • Chondrodysplasia punktata
      Votes: 2 (2 %)



        Total answers: 70

     
    Asphyxiating thoracic dysplasia (Jeune syndrome)
    J Blöcker, G Hahn. Asphyxiating thoracic dysplasia (Jeune syndrome). PedRad [serial online] vol 3, no. 1.
    URL: www.PedRad.info/?search=20030122112757


     

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




    1 = less interesting)
     
    Asphyxiating thoracic dysplasia (Jeune syndrome)
    J Blöcker, G Hahn. Asphyxiating thoracic dysplasia (Jeune syndrome). PedRad [serial online] vol 3, no. 1.
    URL: www.PedRad.info/?search=20030122112757


     


    Go to the top of the page   ID: 20020303175754 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
     
    Multiple ossifications within cranial sutures (WORMIAN bones)
    Roland Talanow. Multiple ossifications within cranial sutures (WORMIAN bones). PedRad [serial online] vol 2, no. 3.
    URL: www.PedRad.info/?search=20020303175754


     
     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:

    Roland Talanow (Cleveland/USA)  

     
     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:

    5 year-old girl without clinical symptoms. Incidental finding on a skull radiograph, which was done because of cranial trauma.  

     
     Pediatric Radiology CasesPathomorphology or Pathophysiology of this disease :

    Abnormal ossifications within the skull sutures. They are more frequent present in skull dysplasias (i.e. Osteogenesis imperfecta).  

     
     Pediatric Radiology CasesRadiological findings:


    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: Ductus choledochus above mildly dilated Vena portae with changing caliber. Increased echogenicity of the wall of the Ductus choledochus.



    Ultrasound 2 <- view Ultrasound 2

    Ultrasound 2: Ductus choledochus above mildly dilated Vena portae with changing caliber. Increased echogenicity of the wall of the Ductus choledochus.



    Ultrasound 3 <- view Ultrasound 3

    Ultrasound 3: Increased liver echogenicity; Bile ducts partially up to the periphery irregularly dilated.



    Ultrasound 4 <- view Ultrasound 4

    Ultrasound 4: Minimal distended gallbladder with significant wall thickening and shadowing.



    MRI 1 <- view MRI 1

    MRI 1: MRCP: No dilatation of the intra- and extrahepatic bile ducts. Slightly irregularity of the walls of the Ductus choledochus. No changes in caliber of the intrahepatic bile ducts.





    X-Ray 1 <- view X-Ray 1

    X-Ray 1: ERCP: Rarefication of the intrahepatic bile ducts with multiple stenoses of different lenght especially in the right liver lobe. Extrahepatic bile ducts are not significantly dilated, Ductus cysticus serpentinuous.




    Ultrasound 1 <- view Ultrasound 1

    Ultrasound 1: Nephromegaly, Calcifications with acoustic shadowing, calyectasia, lymphnodes in the renal hilus. The renal configuration is normal, but there are abscess-like lesions (nephromegaly in papillary region, the renal cortex is maintained).



    Ultrasound 2 <- view Ultrasound 2

    Ultrasound 2: Renal hilus transversal:
    enlarged lymphnodes



    Ultrasound 3 <- view Ultrasound 3

    Ultrasound 3: Overview kidney:
    Nephromegaly, Calcifications with acoustic shadowing, calyectasia, lymph nodes in the renal hilus. The renal configuration is normal, but there are abscess-like lesions (in papillary region, the renal cortex is maintained).




    Ultrasound 4 <- view Ultrasound 4

    Ultrasound 4: Detailed image of the parenchymal structure





    OP-Situs 1 <- view OP-Situs 1

    OP-Situs 1: Renal situs opened longitudinal


    Ultrasound 1 <- view Ultrasound 1

    Ultrasound 1: Abdomen axial, bladder with an unclear, echogenic structure to the left in the trigonal region.




    X-Ray 1 <- view X-Ray 1

    X-Ray 1: Voiding cystourethrogram (VCUG) ap, left-sided vesico-ureteral-reflux (VUR) III°







    X-Ray 2 <- view X-Ray 2

    X-Ray 2: Voiding cystourethrogram (VCUG) oblique view, during urination. Bulging of the bladder dorsolaterally with oblique VUR III° on the left side, The left pelvicocaliceal system resembles a wilting flower .






    X-Ray 3 <- view X-Ray 3

    X-Ray 3: Voiding cystourethrogram (VCUG): further enlargening of the structure during micturition.






    Ultrasound 2 <- view Ultrasound 2

    Ultrasound 2: Abdomen axial, anechoic structure on the left side, changing in size during the exam, no depiction of the ureter.




    Ultrasound 3 <- view Ultrasound 3

    Ultrasound 3: Lower abdomen axial: Again demonstrated is the anechoic strcuture on the left, which is changing in size during the continuous examination. When the bladder contracts, enlargening.




    Ultrasound 4 <- view Ultrasound 4

    Ultrasound 4: Sonogram during urination, an obvious enlargening of the pathology




    Ultrasound 5 <- view Ultrasound 5

    Ultrasound 5: Bladder continues emptying, pathology becomes larger.




    Ultrasound 6 <- view Ultrasound 6

    Ultrasound 6: Bladder is empty, pathology further enlarged.




    OP-Situs 1 <- view OP-Situs 1

    OP-Situs 1: Intraoperative findings




    X-Ray 1 <- view X-Ray 1

    X-ray 1: Thorax a. p. : short, horizontally running rib with widening of the costochondral connections.







    X-Ray 2 <- view X-Ray 2

    X-ray 2: pelvis a. p. : typical three point configuration of the lower edge




    X-Ray 3 <- view X-Ray 3

    X-ray 3: baby image: long bones (Femur/Humerus) appear shortened in comparison to the long thorax








    X-Ray 1 <- view X-Ray 1

    X-Ray 1: Within the sutures, mostly longitudinal ossification centers. The skull shape is not altered.




    X-Ray 2 <- view X-Ray 2

    X-Ray 2: Within the sutures, mostly longitudinal ossification centers (arrows). The skull shape is not altered.




    X-Ray 3 <- view X-Ray 3

    X-Ray 3: Lateral view

     

     
     Pediatric Radiology CasesDiagnosis confirmation:

    N/A  

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

    Skull fracture  

     
     Pediatric Radiology CasesCourse / Prognosis / Frequency / Other :

    N/A  

     
     Pediatric Radiology CasesComments of the author about the case:

    N/A  

     
     Pediatric Radiology CasesFirst description / History:

    According to H.W. Hayek (see literature), wormian bones were named after the danish anatomist, Olaus Wormius (18th century).  

     
     Pediatric Radiology CasesLiterature:

    9. Medline: Medline
    H.W. Hayek
    Schädel; in Kinderradiologie Band 1, Herausgeber W.Schuster, D.Färber
    Springer-Verlag 1966  

     
     Pediatric Radiology CasesKeywords:

    Wormian, bones, ossifications, cranial, multiple ossifications, cranial suture, Wormian bones  

     
     Pediatric Radiology Cases Cite this article:

    Roland Talanow. Multiple ossifications within cranial sutures (WORMIAN bones). PedRad [serial online] vol 2, no. 3.
    URL: www.PedRad.info/?search=20020303175754  

     
     Pediatric Radiology Cases Read similar articles: Wormian bones&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 ]   
     
    Multiple ossifications within cranial sutures (WORMIAN bones)
    Roland Talanow. Multiple ossifications within cranial sutures (WORMIAN bones). PedRad [serial online] vol 2, no. 3.
    URL: www.PedRad.info/?search=20020303175754


     

    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



    Multiple ossifications within cranial sutures (WORMIAN bones)
    Other cases by these authors:

    Search K. Gerlach in Medline K. Gerlach (5)   
    Search P. Göbel in Medline P. Göbel (6)   
    Search A. Nordwig in Medline A. Nordwig (20)   
    Search N. Lorenz in Medline N. Lorenz (1)   
    Search B. Biereder in Medline B. Biereder (1)   
    Search T. Haufe in Medline T. Haufe (4)   
    Search M. Kabus in Medline M. Kabus (1)   
    Search M. Zieger in Medline M. Zieger (17)   
    Search Dirk Schaper in Medline Dirk Schaper (59)   
    Search P. Göbel in Medline P. Göbel (6)   
    Search J. Blöcker in Medline J. Blöcker (18)   
    Search G. Hahn in Medline G. Hahn (22)   
    Search Roland Talanow in Medline Roland Talanow (25)   

    Multiple ossifications within cranial sutures (WORMIAN bones)  
     
    Multiple ossifications within cranial sutures (WORMIAN bones)
    Roland Talanow. Multiple ossifications within cranial sutures (WORMIAN bones). PedRad [serial online] vol 2, no. 3.
    URL: www.PedRad.info/?search=20020303175754


     

    Which diagnosis have other collegues guessed?


    • Primary sclerosing cholangitis
      Votes: 16 (88 %)


    • Caroli syndrome
      Votes: 1 (5 %)


    • Obstructing concrement in the ductus choledochus
      Votes: 0 (0 %)


    • Hepatitis
      Votes: 0 (0 %)


    • Liver cirrhosis
      Votes: 1 (5 %)


    • Nephroblastoma
      Votes: 2 (5 %)


    • Xanthogranulomatous Pyelonephritis
      Votes: 14 (38 %)


    • Renal carcinoma
      Votes: 0 (0 %)


    • Refluxnephropathy
      Votes: 0 (0 %)


    • Glomerulonephritis
      Votes: 2 (5 %)


    • Ureterocele
      Votes: 2 (3 %)


    • Parostial diverticlum
      Votes: 10 (17 %)


    • Megaureter
      Votes: 1 (1 %)


    • Bladder diverticulum in double kidney and duplicated ureter
      Votes: 7 (12 %)


    • Short rib-polydactyly syndrome
      Votes: 4 (5 %)


    • Asphyxiating thoracic dysplasia (Jeune Syndrome)
      Votes: 6 (8 %)


    • Tantaphoric dysplasia
      Votes: 2 (2 %)


    • Chondrodysplasia punktata
      Votes: 2 (2 %)



        Total answers: 70

     
    Multiple ossifications within cranial sutures (WORMIAN bones)
    Roland Talanow. Multiple ossifications within cranial sutures (WORMIAN bones). PedRad [serial online] vol 2, no. 3.
    URL: www.PedRad.info/?search=20020303175754


     

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




    1 = less interesting)
     
    Multiple ossifications within cranial sutures (WORMIAN bones)
    Roland Talanow. Multiple ossifications within cranial sutures (WORMIAN bones). PedRad [serial online] vol 2, no. 3.
    URL: www.PedRad.info/?search=20020303175754


     


    Go to the top of the page   ID: 20011128175838 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
     
    Tuberous sclerosis of the brain / Bourneville-Pringle's disease
    Wolfgang Hirsch, RP Spielmann. Tuberous sclerosis of the brain / Bourneville-Pringle's disease. PedRad [serial online] vol 1, no. 11.
    URL: www.PedRad.info/?search=20011128175838


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

    Wolfgang Hirsch, R.P. Spielmann (Halle)  

     
     Pediatric Radiology CasesEmail Address:

    Viewable for logged on visitors (Log on)  

     
     Pediatric Radiology CasesAge:

    N/A  

     
     Pediatric Radiology CasesGender:

    N/A  

     
     Pediatric Radiology CasesRegion-Organ:

    N/A  

     
     Pediatric Radiology CasesMost likely etiology:

    N/A  

     
     Pediatric Radiology CasesHistory:

    Mental retardation, epilepsy, typical changes in the skin: Adenoma sebaceum: facial angiofibromas in the nasolabial fold - starting at age 1-5. Multiple ungual fibromas, depigmentation of the trunk.  

     
     Pediatric Radiology CasesPathomorphology or Pathophysiology of this disease :

    In this contribution, only the cerebral lesions of a 9 year-old boy are shown.

    Fundamentally, however, Multi-organ illness (phakomatosis) with manifestations possible in:

    Skin: Adenoma sebaceum: facial angiofibromas in the nasolabial fold;

    Eye: retinal hamartomas that can calcify;

    Kidney: Angiomyofibromas, see there;

    Lung and Abdomen: Lymphangioleiomyofibromatosis, see there.

    Heart: benign rhabdomyomas.

     

     
     Pediatric Radiology CasesRadiological findings:


    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: Ductus choledochus above mildly dilated Vena portae with changing caliber. Increased echogenicity of the wall of the Ductus choledochus.



    Ultrasound 2 <- view Ultrasound 2

    Ultrasound 2: Ductus choledochus above mildly dilated Vena portae with changing caliber. Increased echogenicity of the wall of the Ductus choledochus.



    Ultrasound 3 <- view Ultrasound 3

    Ultrasound 3: Increased liver echogenicity; Bile ducts partially up to the periphery irregularly dilated.



    Ultrasound 4 <- view Ultrasound 4

    Ultrasound 4: Minimal distended gallbladder with significant wall thickening and shadowing.



    MRI 1 <- view MRI 1

    MRI 1: MRCP: No dilatation of the intra- and extrahepatic bile ducts. Slightly irregularity of the walls of the Ductus choledochus. No changes in caliber of the intrahepatic bile ducts.





    X-Ray 1 <- view X-Ray 1

    X-Ray 1: ERCP: Rarefication of the intrahepatic bile ducts with multiple stenoses of different lenght especially in the right liver lobe. Extrahepatic bile ducts are not significantly dilated, Ductus cysticus serpentinuous.




    Ultrasound 1 <- view Ultrasound 1

    Ultrasound 1: Nephromegaly, Calcifications with acoustic shadowing, calyectasia, lymphnodes in the renal hilus. The renal configuration is normal, but there are abscess-like lesions (nephromegaly in papillary region, the renal cortex is maintained).



    Ultrasound 2 <- view Ultrasound 2

    Ultrasound 2: Renal hilus transversal:
    enlarged lymphnodes



    Ultrasound 3 <- view Ultrasound 3

    Ultrasound 3: Overview kidney:
    Nephromegaly, Calcifications with acoustic shadowing, calyectasia, lymph nodes in the renal hilus. The renal configuration is normal, but there are abscess-like lesions (in papillary region, the renal cortex is maintained).




    Ultrasound 4 <- view Ultrasound 4

    Ultrasound 4: Detailed image of the parenchymal structure





    OP-Situs 1 <- view OP-Situs 1

    OP-Situs 1: Renal situs opened longitudinal


    Ultrasound 1 <- view Ultrasound 1

    Ultrasound 1: Abdomen axial, bladder with an unclear, echogenic structure to the left in the trigonal region.




    X-Ray 1 <- view X-Ray 1

    X-Ray 1: Voiding cystourethrogram (VCUG) ap, left-sided vesico-ureteral-reflux (VUR) III°







    X-Ray 2 <- view X-Ray 2

    X-Ray 2: Voiding cystourethrogram (VCUG) oblique view, during urination. Bulging of the bladder dorsolaterally with oblique VUR III° on the left side, The left pelvicocaliceal system resembles a wilting flower .






    X-Ray 3 <- view X-Ray 3

    X-Ray 3: Voiding cystourethrogram (VCUG): further enlargening of the structure during micturition.






    Ultrasound 2 <- view Ultrasound 2

    Ultrasound 2: Abdomen axial, anechoic structure on the left side, changing in size during the exam, no depiction of the ureter.




    Ultrasound 3 <- view Ultrasound 3

    Ultrasound 3: Lower abdomen axial: Again demonstrated is the anechoic strcuture on the left, which is changing in size during the continuous examination. When the bladder contracts, enlargening.




    Ultrasound 4 <- view Ultrasound 4

    Ultrasound 4: Sonogram during urination, an obvious enlargening of the pathology




    Ultrasound 5 <- view Ultrasound 5

    Ultrasound 5: Bladder continues emptying, pathology becomes larger.




    Ultrasound 6 <- view Ultrasound 6

    Ultrasound 6: Bladder is empty, pathology further enlarged.




    OP-Situs 1 <- view OP-Situs 1

    OP-Situs 1: Intraoperative findings




    X-Ray 1 <- view X-Ray 1

    X-ray 1: Thorax a. p. : short, horizontally running rib with widening of the costochondral connections.







    X-Ray 2 <- view X-Ray 2

    X-ray 2: pelvis a. p. : typical three point configuration of the lower edge




    X-Ray 3 <- view X-Ray 3

    X-ray 3: baby image: long bones (Femur/Humerus) appear shortened in comparison to the long thorax








    X-Ray 1 <- view X-Ray 1

    X-Ray 1: Within the sutures, mostly longitudinal ossification centers. The skull shape is not altered.




    X-Ray 2 <- view X-Ray 2

    X-Ray 2: Within the sutures, mostly longitudinal ossification centers (arrows). The skull shape is not altered.




    X-Ray 3 <- view X-Ray 3

    X-Ray 3: Lateral view


    MRI 1 <- view MRI 1

    MRI 1: Transversal



    MRI 2 <- view MRI 2

    MRI 2: Transversal



    MRI 3 <- view MRI 3

    MRI 3: Transversal



    MRI 4 <- view MRI 4

    MRI 4: Transversal



    MRI 5 <- view MRI 5

    MRI 5: Coronal



    MRI 6 <- view MRI 6

    MRI 6: Transversal



    MRI 7 <- view MRI 7

    MRI 7: Transversal



    MRI 8 <- view MRI 8

    MRI 8: Transversal



    MRI 9 <- view MRI 9

    MRI 9: Transversal



    Intracerebrally, there are 4 types of changes to be found:

    1. Subependymal Hamartomas: They push themselves out under the ependyma into the ventricles, are very small, are principally seen in every ventricle (but most often in the lateral ventricles). Calcifications are seen in rising age. In small children these are hyperintense, but later become isointense to the white matter.

    2. Large-cell tumors: differentiate themselves from above only because of their size and the tendency to grow. They are also more often to be found in the area of the foramen of Monroi. Contrast uptake is possible, but not obligatory.

    3. (Sub)-Cortical Tuber: Round or polygonal, underneath the cortex, and are seen often causing widened gyri. In babies, T1 images are hyperintense and T2 is hypointense. Later they become isointense in T1 and hyperintense in T2. There is a tendency for calcification.

    4. White matter lesions: spanning or linear, these have the same signal intensity characteristics as the (sub)cortical tuber and are composed of dysplastic cell formations. Here too, calcifications are possible.

    Extracerebral manifestations: Kidney, lung, abdomen (see above. In these examples, only cerebral manifestations are shown.)

     

     
     Pediatric Radiology CasesDiagnosis confirmation:

    N/A  

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

    Relatively typical findings in summation. The difficulty and need to compare this to neurofibromatosis or other cerebral tumors occur only very seldomly.  

     
     Pediatric Radiology CasesCourse / Prognosis / Frequency / Other :

    N/A  

     
     Pediatric Radiology CasesComments of the author about the case:

    N/A  

     
     Pediatric Radiology CasesFirst description / History:

    N/A  

     
     Pediatric Radiology CasesLiterature:

    10. Medline: Medline
    Barkovich AJ
    Pediatric Neuroimaging
    2000  

     
     Pediatric Radiology CasesKeywords:

    Tuberous, sclerosis, brain, Bourneville-Pringle's disease, tuberous sclerosis, mental retardation, epilepsy, skin, Adenoma sebaceum, facial angiofibromas, nasolabial fold, ungual fibromas, depigmentation, kidney, Angiomyofibromas, retinal hamartoma, Lymphangioleiomyofibromatosis, phakomatosis  

     
     Pediatric Radiology Cases Cite this article:

    Wolfgang Hirsch, RP Spielmann. Tuberous sclerosis of the brain / Bourneville-Pringle's disease. PedRad [serial online] vol 1, no. 11.
    URL: www.PedRad.info/?search=20011128175838  

     
     Pediatric Radiology Cases Read similar articles: phakomatosis&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 ]   
     
    Tuberous sclerosis of the brain / Bourneville-Pringle's disease
    Wolfgang Hirsch, RP Spielmann. Tuberous sclerosis of the brain / Bourneville-Pringle's disease. PedRad [serial online] vol 1, no. 11.
    URL: www.PedRad.info/?search=20011128175838


     

    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



    Tuberous sclerosis of the brain / Bourneville-Pringle's disease
    Other cases by these authors:

    Search K. Gerlach in Medline K. Gerlach (5)   
    Search P. Göbel in Medline P. Göbel (6)   
    Search A. Nordwig in Medline A. Nordwig (20)   
    Search N. Lorenz in Medline N. Lorenz (1)   
    Search B. Biereder in Medline B. Biereder (1)   
    Search T. Haufe in Medline T. Haufe (4)   
    Search M. Kabus in Medline M. Kabus (1)   
    Search M. Zieger in Medline M. Zieger (17)   
    Search Dirk Schaper in Medline Dirk Schaper (59)   
    Search P. Göbel in Medline P. Göbel (6)   
    Search J. Blöcker in Medline J. Blöcker (18)   
    Search G. Hahn in Medline G. Hahn (22)   
    Search Roland Talanow in Medline Roland Talanow (25)   
    Search Wolfgang Hirsch in Medline Wolfgang Hirsch (33)   
    Search R.P. Spielmann in Medline R.P. Spielmann (2)   

    Tuberous sclerosis of the brain / Bourneville-Pringle's disease  
     
    Tuberous sclerosis of the brain / Bourneville-Pringle's disease
    Wolfgang Hirsch, RP Spielmann. Tuberous sclerosis of the brain / Bourneville-Pringle's disease. PedRad [serial online] vol 1, no. 11.
    URL: www.PedRad.info/?search=20011128175838


     

    Which diagnosis have other collegues guessed?


    • Primary sclerosing cholangitis
      Votes: 16 (88 %)


    • Caroli syndrome
      Votes: 1 (5 %)


    • Obstructing concrement in the ductus choledochus
      Votes: 0 (0 %)


    • Hepatitis
      Votes: 0 (0 %)


    • Liver cirrhosis
      Votes: 1 (5 %)


    • Nephroblastoma
      Votes: 2 (5 %)


    • Xanthogranulomatous Pyelonephritis
      Votes: 14 (38 %)


    • Renal carcinoma
      Votes: 0 (0 %)


    • Refluxnephropathy
      Votes: 0 (0 %)


    • Glomerulonephritis
      Votes: 2 (5 %)


    • Ureterocele
      Votes: 2 (3 %)


    • Parostial diverticlum
      Votes: 10 (17 %)


    • Megaureter
      Votes: 1 (1 %)


    • Bladder diverticulum in double kidney and duplicated ureter
      Votes: 7 (12 %)


    • Short rib-polydactyly syndrome
      Votes: 4 (5 %)


    • Asphyxiating thoracic dysplasia (Jeune Syndrome)
      Votes: 6 (8 %)


    • Tantaphoric dysplasia
      Votes: 2 (2 %)


    • Chondrodysplasia punktata
      Votes: 2 (2 %)



        Total answers: 70

     
    Tuberous sclerosis of the brain / Bourneville-Pringle's disease
    Wolfgang Hirsch, RP Spielmann. Tuberous sclerosis of the brain / Bourneville-Pringle's disease. PedRad [serial online] vol 1, no. 11.
    URL: www.PedRad.info/?search=20011128175838


     

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




    1 = less interesting)
     
    Tuberous sclerosis of the brain / Bourneville-Pringle's disease
    Wolfgang Hirsch, RP Spielmann. Tuberous sclerosis of the brain / Bourneville-Pringle's disease. PedRad [serial online] vol 1, no. 11.
    URL: www.PedRad.info/?search=20011128175838


     




    Discussion >> Write Comment <<



      There are no comments available regarding this case.





     Write a comment to this case (With * marked fields have to be filled)

    Type in this number: 3824

    Name: *

    E-Mail:

    Website:

    Subject: *

    Text: *




     
      Pediatric Radiology Discussion Get the Pediatric Radiology Discussion as RSS feed !
      To the discussion forum
      To the discussion forum

      Get the Pediatric Radiology Discussion as RSS feed !

      © 2001-2020   www.kinderradiologie-online.de
      In cooperation with © Radiolopolis - Radiology Search - Radiology Teacher - PubMed Reader - Annotate - Radiology Lectures & Tutorials - Radiology Boards Preparation - Pediatric Radiology Links - Pediatric Radiology Information - Emergency Radiology - Lung Cancers - USMLE Forums - Cancer Staging Information - Chest Radiology - Radiology Case Report Journal
      For suggestions and questions: information@kinderradiologie-online.de
      Add this site to your favorites (CTRL+D)