Jeune syndrome

Case contributed by Daniel M Conroy
Diagnosis almost certain

Presentation

Child admitted with difficulty breathing requiring intubation.

Patient Data

Age: 5 months
Gender: Male
x-ray
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Info

Frontal radiograph showing narrowed shortened ribs and restricted lung volumes.

An enteric tube is also visible.

Post Operative Image

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Patient has undergone surgical rib lengthening procedure to improve lung volumes. Bilateral chest drains in-situ.

Case Discussion

This case shows classic features of Jeune syndrome (asphyxiating thoracic dystrophy).

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