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The Journal of Thoracic and Cardiovascular Surgery, Vol 83, 100-104, Copyright © 1982 by The American Association for Thoracic Surgery and The Western Thoracic Surgical Association

ARTICLES

Spontaneous pneumothorax associated with tuberous sclerosis

TL Babcock and BA Snyder

Tuberous sclerosis is a rare hereditary anomaly of variable penetrance characterized by adenoma sebaceum, mental retardation, and epilepsy. Pulmonary disease with spontaneous pneumothorax is a rare complication with this disease. We have recently seen two patients with this complication. A review of the literature reveals 17 other patients with spontaneous pneumothorax and tuberous sclerosis. Eight of these died of pneumothorax. Fifty-three percent of the patients, including all those who died, had normal intelligence. The pathological and roentgenographic findings of the disease are discussed. Recommendations for therapeutic management are given.


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[Abstract] [Full Text] [PDF]


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