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Vol. 23. Issue 2.
Pages 78-80 (March - April 1987)
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Vol. 23. Issue 2.
Pages 78-80 (March - April 1987)
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Neumotorax espontaneo y esclerosis tuberosa
Spontaneous pneumothorax and tuberous sclerosis
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A. Pacheco galvan, J.P. Valdazo de diego, M.T. Naya nieto, L. Fogue calvo, J. Lago viguera, J. zapatero Gaviria
Servicio de Neumología. Hospital Ramón y Cajal. Madrid
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Cuando un neumotorax espontáneo incide en una mujer en edad reproductiva debe descartarse la esclerosis tuberosa. La afectación pulmonar en la esclerosis tuberosa tiene una frecuencia menor del 1% y se manifiesta en dos aspectos: un patrón en panal en la radiografía de tórax con posibilidad de neumotórax espontáneo debido a la rotura del espacio pleural de bullas subyacentes, y un síndrome obstructivo espirométrico. El dato anatómico-patológico básico es la proliferación hamartomatosa de músculo liso en capilares, linfáticos y paredes de bulla. La posibilidad de frenar el posible estímulo hormonal sobre la proliferación de músculo liso ha abierto recientes y esperanzadores resultados.

Tuberous sclerosis is to be considered whenever a wornan in child-bearing age develops spontaneous pneumothorax. Pulmonary involvement in tuberous sclerosis appears with a frequency less than 1% and has two aspects: a «honecomb pattern» in thorax X-ray examination where the possibility exists of spontaneous pneumothorax due to rupture of underlying bullae into the pleural cavity, and an obstructive syndrome as assessed by spirometry. The main pathological finding is hamartomatous proliferation of smooth rnuscle in capillary and lymphatic vessels as well as on bullae walls. The approach aimed to blunting the possible hormonal stimulus on smooth muscle proliferation has recently provided some promising results.

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Bibliografia
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Spontaneous pneumothorax associated with tuberous sclerosis.
J Thor Cardiovasc Surg, 83 (1982), pp. 100-104
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Pulmonary tuberous sclerosis: report of three patients and a review of the literature.
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Early diagnosis genetic marker sought for tuberous sclerosis.
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Tuberous sclerosis.
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Lung biopsy interpretation.
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Pulmonary lymphangiomyomatosis associated with tuberous sclerosis Treatment with Tamoxifen and tetracicline-pleurodesis.
Chest, 88 (1985), pp. 473-475
Copyright © 1987. Sociedad Española de Neumología y Cirugía Torácica
Archivos de Bronconeumología
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