• Childhood Kidney Diseases
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• v.2 no.2
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• pp.196-199
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• 1998

The classical manifestation of asphyxiating thoracic dystrophy comprise narrow chest, short limbs, and pelvic changes. The degree of respiratory distress varied from negligible to lethal. The development of progressive renal failure may indicate that the condition is asphyxiating thoracic dystrophy. We have experienced a case of asphyxiating thoracic dystrophy who died from chronic renal failure in a 5-month old infant. Abriefreviewofassociatedliteratureisalsopresented.

• Journal of Chest Surgery
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• v.38 no.12 s.257
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• pp.873-877
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• 2005

Jeune's asphyxiating thoracic dystrophy is a rare, complex malformation with a broad spectrum of clinical expression. The degree of chest wall deformity is the most important prognostic factor and the only part which is correctable. A 11 month-old male infant was diagnosed as having Jeune's syndrome and received right side lateral thoracic expansion surgery. But because respiratory distress symptom was sustained postoperatively, we performed left side procedure 3 months after the initial operation. Respiratory distress symptom got worse after fracturing the left titanium plate which was inserted to fix the expanded thoracic wall and reimplantation was performed. The patient was discharged 6 months after the initial operation. He was readmitted and received ventilator care for respiratory failure and died 10 months after the initial operation.