Is Asphyxiating Thoracic Dystrophy (Jeunes Syndrome) Deadly and Should We Insist on Treating It? Reconstructive Surgery On Demand

摘要

Our aim is to present the treatment of one of the skeletal manifestations of Jeune's syndrome (JS), the hypoplastic chest, which can result in thoracic insufficiency syndrome and present “on-demand” stage surgical technique using mandible locking plate system for the fixation of ribs. The diagnosis “Jeune's syndrome” was presented clinically in a 3-month-old girl from a family in which the first child died of JS at the age of 18 months. After close follow-up for several months and preoperative planning, we decided to make reconstructive chest operation with atypical use of a double-angled mandible locking plate for fixation. The plate was shaped as a “crown” to ensure the three dimension stability, from the dorsal part of the most curved ribs (paravertebrally) to the sternum after the resection of this area. Operation was done at the period of worsened breathing. For nearly 1 year, the rib cage preserved its stability and the child was in good condition. During the next 3 months, the upper part of the deformation started to grow inward fast. Second operation was “on demand,” and the implants used were mandible locking plates curved anterolaterally to effectuate extension of the rib cage and the sternum. In both the reconstructive operations, we spared the rectus and pectoral muscles and achieved good enlargement of the thoracic volume. The postoperative period is smooth and the child is active, without complications. We believe that in the future, the treatment should be “on demand” according to the course of the illness and the results of the follow-up examinations and adequate to the progress of chest wall deformity.