Images in Clinical Medicine
Spontaneous Pneumothorax in Tuberous Sclerosis
N Engl J Med 2008; 358:177January 10, 2008
- Article
A 28-year-old woman with tuberous sclerosis presented with sudden onset of dyspnea and discomfort in the right chest and back. On examination she had tachycardia, tachypnea, and hypoxia, with an absence of breath sounds on the right side. An anteroposterior chest radiograph showed a complete pneumothorax (Panel A, arrowheads), which resolved with a tube thoracostomy. Computed tomographic scans of the chest from previous hospitalizations showed a diffuse cystic intrapulmonary disease pattern consistent with pulmonary lymphangioleiomyomatosis (Panel B, arrows). After successful pulmonary re-expansion, the patient was discharged, but she returned within 1 week with bilateral pneumothoraxes. Pleurodesis was successful on the right side but failed on the left side. The patient was considered for lung transplantation but was not a candidate, given the coexisting conditions from tuberous sclerosis. Pulmonary lymphangioleiomyomatosis, a complication of tuberous sclerosis, is frequently manifested as recurrent episodes of spontaneous pneumothorax. The prognosis for patients with lymphangioleiomyomatosis is poor, with progressive respiratory insufficiency often leading to death. This patient was transferred to an inpatient hospice care facility after being released from the hospital.
Rajeev L. Narayan, M.D.
Columbia University, College of Physicians and Surgeons, New York, NY 10032-3784
RN2187@columbia.edu
