Korean J Thorac Cardiovasc Surg.
2005 Dec;38(12):873-877.
Lateral Thoracic Expansion Surgery for Jeune's Syndrome
- Affiliations
-
- 1Department of Thoracic and Cardiovascular Surgery, Asan Medical Center, University of Ulsan College of Medicine. sipark@amc.seoul.kr
- 2Department of Pediatrics, College of Medicine, Korea University.
- 3Department of Anesthesiology and Pain Medicine, Asan Medical Center, University of Ulsan College of Medicine.
Abstract
- 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.
