Angela Gregory (Birmingham Childrens Hospital/Birmingham/ UK), Rupan Banga (Birmingham Childrens Hospital/Birmingham/ UK)  

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6 Years  

Female  

Neck-Lymphatic tissue  

congenital  

A 6 year old girl presented to casualty with a 4 day history of sore throat and dysphagia. Examination was unremarkable and initial management for tonsillitis was commenced. Within 24 hours, she dramatically worsened and was admitted to paediatric ICU with respiratory distress and progressive neck swelling.  

Cystic hygromas usually affects the head and neck (75%), and the axilla (20%). More infrequent sites have been reported to include the mediastinum, groin, and retroperitoneum.

Cystic Hygromas are thought to arise due to a congential blockage or arrest of the normal development of the primordial lymphatic channels. They have a multiloculated cystic appearance [1].  

Imaging confirmed a very large multiloculated retropharyngeal cystic mass measuring 13.5cm in length, 3.7cm AP and 7cm width. The lesion extended from the clivus to the aortic arch, displacing the trachea anteriorly.


<- view X-Ray 1

X-Ray 1: Lateral neck X ray showing a massive retropharyngeal mass pushing the trachea anteriorly.




<- view CT 1

CT 1: Sagittal CT image of the retropharyngeal swelling demonstrating the loculated nature of the cyst.




<- view CT 2

CT 2: Coronal CT demonstrating a retropharyngeal cystic lesion.




<- view CT 3

CT 3: Axial CT demonstrating a retropharyngeal cystic lesion.

 

Surgery / Histo  

Oesphageal duplication cyst, Retropharyngeal cystic hygroma, Retropharyngeal abscess
 

Most cystic hygromas are evident at birth, with about 90% presenting by age 2 years. However, they may become evident with rapid enlargement especially with infection at any age. Complete surgical excision is the recommended treatment of choice. Other treatment options include  

Cystic hygroma belongs to a group of diseases now recognized as lymphatic malformations. Although most hygromas (75%) involve the anterior neck, they can also affect other sites, including the axillae, mediastinum, retroperitoneum, abdominal viscera, groin, bones, and scrotum. We report an unusual case of a patient presenting with a cystic hygroma involving the retropharyngeal space.  

N/A  

1. Medline:
1. Chervenak FA, Isaacson G, Blakemore KJ, Breg WR,Hobbins JC, Berkowitz RL, Tortora M, Mayden K,Mahoney MJ. Fetal cystic hygroma. Cause and natural
history. N Engl J Med 1983;309:822-5.

2. Kennedy TL, Whitaker M, Pellitteri P, Wood WE. Cystic hygroma/lymphangioma: a rational approach to management. Laryngoscope. 2001 Nov;111(11 Pt 1):1929-37.  

cystic hygroma, respiratory distress, retropharyngeal, child, childhood, pediatric radiology  

Angela Gregory, Rupan Banga. Retropharyngeal cystic hygroma. PedRad [serial online] vol 10, no. 2.
URL: www.PedRad.info/?search=20100217140928