Orthopedics - Oleksa A.P. 2006

Joint Pathophysiology
Skeletal Malformations and Congenital Anomalies
Jeune's Thoracodystrophic Syndrome

This autosomal recessive trait is associated with asphyxiating thoracic Dysplasia. A narrow Thorax and short Ribs lead to frequent postnatal respiratory complications, very often resulting in the patient's death (Jeunes M., Beraud C., Carron R., 1955).

However, some children survive this period, subsequently exhibiting a progressive increase in chest circumference (Herdman R.C., Langer L., 1968).

Radiological findings in affected children typically include a narrow thorax; short ribs with irregular osteochondral junctions; hypoplastic iliac wings with normal, horizontal acetabular roofs that overhang the femoral heads; abnormal epiphyses and metaphyses; shortening of the radius (ulna); and occasional hand and FOOT polydactyly.

Management of surviving children involves comprehensive supportive therapy, massage, and corrective bracing. Surgical interventions are indicated for polydactyly and limb-lengthening Procedures to prevent progressive Clubhand and valgus foot deformity.



Last update: 10/08/2026

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