Secondary Asphyxiating Thoracic Dysplasia Due to Multiple Chondromas: A Novel Surgical Report

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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Publication Details

Journal
Interdisciplinary CardioVascular and Thoracic Surgery
Published
2025-08-15
DOI
https://doi.org/10.1093/icvts/ivaf191
Primary Topic
Medical Imaging and Pathology Studies
Type
article
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article

Secondary Asphyxiating Thoracic Dysplasia Due to Multiple Chondromas: A Novel Surgical Report

Weiguang Long, Rajkamal Vishnu, Yang Liu, Wenli Wang
Interdisciplinary CardioVascular and Thoracic Surgery
Medical Imaging and Pathology Studies
article

Secondary Asphyxiating Thoracic Dysplasia Due to Multiple Chondromas: A Novel Surgical Report

Weiguang Long, Rajkamal Vishnu, Yang Liu, Wenli Wang
article en

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.

Interdisciplinary CardioVascular and Thoracic SurgeryVol. 40(9)
Tamil Nadu Dr. M.G.R. Medical University (IN), Center for Orthopaedics (US), Guangdong Provincial People's Hospital (CN), Guangzhou Chest Hospital (CN)
Openalex Percentile: Top 68%
Medical Imaging and Pathology Studies
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