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Neurofibromatosis and Spontaneous Hemothorax in Pregnancy

Division of Maternal-Fetal Medicine, Department of Obstetrics and Gynecology, The Washington Hospital Center, Washington, D.C.

Abstract

Two cases of spontaneous hemothorax in pregnant women with neurofibromatosis are reported. Effects of pregnancy on the clinical course of neurofibromatosis are known to include worsening of cutaneous lesions, increased incidence of hypertension, and renal artery rupture. However, vessel wall rupture into the pleural cavity in areas of vascular neurofibromatosis is previously unreported. Contributing factors may include normal hemodynamic changes of the puerperium and pathological alterations of preexisting areas of neurofibromatosis. Because the incidence of vascular neurofibromatosis is not known and is difficult to diagnose, the serious morbidity and mortality encountered in this group place all pregnant patients with neurofibromatosis in a high-risk obstetric category.