The Institute of Chest Wall Surgery
Surgery for A 7-year-old Child with Asphyxiating Thoracic Dystrophy (Jeune Syndrome)
Medical History
The patient is a 7-year-old girl who exhibited rapid breathing and severe hypoxia at birth. After examination, she was diagnosed with a severe chest wall deformity that significantly narrows her chest cavity, resulting in inhibited respiratory movement, thus she has to receive oxygen inhalation in order to alleviate her symptoms. After several resuscitation attempts, her condition gradually stabilized. However, she remained frail and was prone to frequent colds. She has suffered from pneumonia multiple times, with each episode accompanied by hypoxia, necessitating oxygen therapy to alleviate her symptoms.
Preoperative Examination
The patient’s thoracic cage is generally constricted, with a chest circumference of only 44.5 cm. The center of her anterior chest wall protrudes while the sides are concave. The patient was diagnosed with asphyxiating thoracic dystrophy.
Surgical Overview
The Wang procedure was performed to correct the depressions on both sides of the chest wall. The surgery lasted 2 hours and was completed successfully. After the surgery,the overall size of the patient's thoracic cage increased significantly, and the deformity was effectively corrected, yielding satisfactory results.
Related Photos
Asphyxiating thoracic dystrophy(ATD), or Jeune syndrome, is a profoundly rare and life-threatening condition. Characterized by a narrow chest, it significantly restricts lung expansion, leading to severe breathing difficulty from birth. Many infants with ATD tragically succumb to asphyxiation during the neonatal period, and few affected children survive to reach adolescence, with adult patients being even rarer.
Over the last decade, specialists of ICWS have conducted extensive research on this disease.Their work has not only uncover the underlying causes but also led to the development of various effective surgical methods.
In this particular case, the Wang procedure was performed to alleviate the depressions on the lateral chest wall. This surgical procedure aims to expand the thoracic cavity, thus reducing the pressure on the lungs. Due to its minimal invasiveness and simplicity, the Wang procedure is considered one of the ideal surgical approachs for managing ATD.