title: Spontaneous hemothorax in patients with von Recklinghausen's disease.
abstract: Spontaneous massive intrathoracic bleeding is rare except for the rupture of aortic aneurysm or pleural adhesions in association with pneumothorax. We encountered two cases of critical massive hemothorax in patients with von Recklinghausen's disease (type I neurofibromatosis). Case 1; a 59-year-old female suddenly experienced severe back pain followed by syncope and shock. The hemothorax was caused by a bleeding of diffuse type neurofibroma of the parietal pleura and she underwent thoracotomy and surgical ligation of the bleeding vessels. Case 2; a 46-year-old male suddenly suffered back pain and fainted while driving. An intercostal aneurysmal rupture caused a spontaneous hemothorax and he underwent chest tube drainage followed by endovascular coil embolization. We reviewed 23 cases reported in the literature, including our two cases. Spontaneous hemothorax in patients with von Recklinghausen's disease is a life-threatening syndrome and may require emergency surgical or endovascular embolization.
authors.affiliation: Department of Oncological Science, Surgery II, Oita University Faculty of Medicine, 1-1 Idaigaoka, Hasama-machi, Yufu, Oita 879-5593, Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Miura
authors.forename: Yozo
authors.initials: Y
authors.lastname: Kawano
authors.forename: Masao
authors.initials: M
authors.lastname: Chujo
authors.forename: Michiyo
authors.initials: M
authors.lastname: Miyawaki
authors.forename: Hiromu
authors.initials: H
authors.lastname: Mori
authors.forename: Katsunobu
authors.initials: K
authors.lastname: Kawahara
mesh_terms: D000208:Acute Disease; D000792:Angiography; D004621:Embolization, Therapeutic / Q000379:methods; D005260:Female; D005500:Follow-Up Studies; D006488:Hemostasis, Surgical / Q000379:methods*; D006491:Hemothorax / Q000209:etiology* / Q000503:physiopathology / Q000601:surgery*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009456:Neurofibromatosis 1 / Q000150:complications* / Q000175:diagnosis*; D012720:Severity of Illness Index; D013908:Thoracotomy / Q000379:methods; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome