Lateral thoracic expansion for Jeune's syndrome: Midterm results

Background. In 1995, we reported the use of lateral thoracic expansion in a patient with symptomatic Jenne's asphyxiating thoracic dystrophy. We have subsequently used lateral thoracic expansion 16 times on 10 patients during 7 years. This article reports our outcomes and provides surgical details. Methods. Charts of all patients undergoing lateral thoracic expansion were reviewed. Eight of the 10 patients had symptomatic Jeune's syndrome. The ot her 2 had similar thoracic deformities limiting thoracic capacity. In half of the patients the procedures were performed bilaterally. Results. All patients older than 1 year of age were symptomatically benefit ed by lateral thoracic expansion. Functional and anatomic measurements docu mented thoracic enlargement in several patients who had comparable preopera tive and postoperative studies. However, 2 infants with significant underly ing airway disease did not improve and went on to succumb to that aspect of their disease despite enlargement of the thorax. Fracture of the titanium ministruts has been a recurrent problem, and we now use larger struts. Conclusions. Lateral thoracic expansion is a safe and effective procedure i n selected patients with Jeune's syndrome older than 1 year of age as judge d by shortterm and midterm follow-up. More experience and longer follow-up are required to discern the place of the lateral thoracic expansion in the overall management of these patients. (C) 2001 by The Society of Thoracic S urgeons.