Operative management of asphyxiating thoracic dystrophy after pectus repair.

Pectus excavatum repair usually results in unchanged or improved pulmonary function. However, a small subset of patients will experience severely impaired pulmonary function after pectus repair caused by restrictive lung disease, and no adequate surgical approach has been described for this condition. A procedure is described that is a variation of an operation for Jeune's thoracic dystrophy, that resulted in marked respiratory improvement in this setting. A 14-year-old boy had undergone standard pectus excavatum repair at age 4, from which he recovered uneventfully. Beginning at age 10 to 12 years progressive restrictive pulmonary disease, recurrent pneumonia, and cor pulmonale developed, which resulted in almost constant shortness of breath and the need for continuous nasal positive pressure support. Pulmonary function test results were markedly abnormal and worsening. He underwent an operative procedure consisting of sternal split that was wedged open permanently with rib struts, opening of pleura bilaterally, and six rib resections bilaterally. His postoperative recovery was satisfactory, and his pulmonary functions have shown steady improvement. He is now completely off oxygen and pressure support, has improved exercise tolerance, and has returned to school. Severe restrictive lung disease after pectus repair can be successfully managed with aggressive operative procedures. Patients should have close follow-up after pectus repair for the development of this potentially debilitating disorder to allow earlier repair.