Asphyxiating Thoracic Dystrophy

Introduction

Asphyxiating thoracic dystrophy (ATD), also known as Jeune syndrome, is an extremely rare chest wall deformity characterized by an abnormally developed thoracic cavity. Both the anterior-posterior and left-right diameters are significantly shorter than those of a normal chest, and this condition often involves dysfunction of multiple organs. The constriction of the chest severely impairs respiratory function, making ATD a critical condition. Most patients succumb to hypoxia soon after birth.

Causes

The cause of primary ATD remains unclear. It may be related to genetic factor, gene mutation or abnormal growth direction of the ribs and costal cartilages.

Secondary ATD is caused by chest wall deformity surgery. Such patients have undergone open surgery for thoracic deformity, during which their costal cartilages were excised. After the surgery, due to the inability of the remaining bone structure to regenerate, the chest cavity becomes constricted, and the intrathoracic organs are constantly compressed, thereby affecting various physiological functions of the patients.

Symptoms

Common symptoms of ATD include a narrow chest, breathing difficulty, and recurrent respiratory infection. As the disease progresses, it can affect other organs, leading to complications such as renal failure and liver dysfunction. Additionally, patients with this condition often exhibit short stature and disproportionate limb growth, with approximately 20% of them presenting with polydactyly (extra fingers or toes).

Diagnostic Methods

Diagnosis involves assessing the patient’s clinical manifestations and physical signs, along with imaging examinations such as X-ray, chest CT scan, and 3D reconstruction.

Surgical Procedures

Patients with ATD present both protrusion deformity in the middle of the chest wall and depression deformity on the lateral chest wall. Consequently, combining the Wenlin procedure with the Wang procedure allows for the simultaneous correction of both types of the deformity.

Chest wall reconstruction:

The bony structure of the chest wall is reconstructed with MatrixRIB to expand the volume of the thoracic cavity and improve its appearance. This shaping material does not need to be removed, which can avoid the pain of a secondary operation.m or flat chest, conventional surgical correction of the chest wall deformity can be adopted to significantly increase mediastinal space and relieve the compression.

Wenlin procedure