[Infant respiratory distress revealing Jeune syndrome]

Jeune syndrome (asphyxiating thoracic dystrophy) is a rare autosomal recessive osteochondrodysplasia with a variable degree of severity, clinically characterized by respiratory distress with a narrow chest and limb shortness. The reported incidence is one to five in 500,000 live births. Most patients develop severe respiratory failure during the first 2 years of life, leading to death in 60-80 % of cases. Survivors may suffer from renal, hepatic, or pancreatic complications. Expanding thoracic surgery can be used for severe cases. We describe the case of an 18-month-old boy who developed mild respiratory distress. The patient showed typical radiological features of Jeune syndrome: narrow thorax with short ribs and trident appearance of the pelvis. This case underscores the value of the right interpretation of the chest radiograph of the infant with a respiratory distress syndrome.