Wenlin Wang, Rajkamal Vishnu, Weiguang Long, Yang Liu
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引用次数: 0
Abstract
Asphyxiating thoracic dysplasia (ATD), also known as Jeune syndrome, is a rare and serious genetic condition; its incidence in adult populations is even rarer. A 25-year-old male had a 10-year history of chest wall deformity and progressive dyspnoea. A complex chest wall reconstruction, along with the excision of bone tumours, was performed in view of critical hypoxia. Mechanical ventilation was persistently required postoperatively. However, the patient did improve, and eventually proper chest configuration was restored with a special surgical technique. Histopathological analysis demonstrated the presence of multiple osteochondromas of the ribs. To the best of our knowledge, this is the first reported case of secondary ATD caused by osteochondromas of the ribs.
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