Combined Wang Procedure and MatrixRIB Reconstruction for Severe Type II Asphyxiating Thoracic Dysplasia: A Case...

Asphyxiating thoracic dysplasia (ATD), also known as Jeune syndrome, is a rare, potentially fatal genetic disorder characterized by a constricted thoracic cavity that can lead to respiratory failure. Standard thoracic expansion techniques are often inadequate for severe variants with deep lateral depressions. We report the case of an 11-year-old boy with type II ATD who presented with severe bilateral chest wall depressions and chronic hypoxia. The patient underwent a combined surgical approach using the Wang procedure and MatrixRIB plates. The third through eighth costochondral junctions were resected bilaterally, followed by rigid reconstruction. The postoperative course was uncomplicated, with a marked increase in chest volume and resolution of the chest wall depressions. The patient was weaned from mechanical ventilation and discharged home on postoperative day 25 without supplemental oxygen. At the 3-month follow-up, he remained asymptomatic, with no signs of hypoxia. This case highlights the technical feasibility and potential utility of combined reconstruction techniques for managing type II ATD.