A Case of Spontaneous Hemothorax Due to Rupture of Pseudoaneurysm in Type 1 Neurofibromatosis.
10.4046/trd.2001.50.1.122
- Author:
Sun Jong KIM
;
Hoon JEONG
;
Sung Soon LEE
;
Chae Man LIM
;
Sang Do LEE
;
Younsuk KOH
;
Woo Sung KIM
;
Dong Soon KIM
;
Won Dong KIM
;
Tae Sun SHIM
- Publication Type:Case Report
- Keywords:
Hemothorax;
neurofibromatosis;
Vasculopathy;
Pseudoaneurysm
- MeSH:
Aneurysm, False*;
Aortic Rupture;
Aortography;
Ehlers-Danlos Syndrome;
Endometriosis;
Female;
Hemothorax*;
Humans;
Male;
Mammary Arteries;
Neoplasm Metastasis;
Neurofibromatoses;
Neurofibromatosis 1*;
Rupture*;
Shock;
Skin
- From:Tuberculosis and Respiratory Diseases
2001;50(1):122-126
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
A non-traumatic, spontaneous hemothorax is rare. The most common causes are coagulopathy, due to anticoagulation treatment, and cancers with a metastasis to the pleural surface. Other unusual causes include thoracic endometriosis, ruptured aortic aneurysm, pulmonary arterio-venous malformation, coagulopathy, Osler-Rendeu-Weber syndrome, Ehlers-Danlos syndrome et cetera. A type 1 neurofibromatosis(Von Recklinghausen's disease) is an autosomal dominant disease that is characterized by multiple skin tumors(neurofibroma) and abnormal skin pigmentation(cafe-au-lait spots). Some are accompanied by vasculopathy, and are present with a spontaneous hemothorax. Such cases are unusual but fatal. We have recently experienced a case where a young male patient with neurofibromatosis initially presented with hypovolemic shock due to a spontaneous hemothorax. Later, aortography revealed that the cause of the hemothorax was a rupture of a pseudoaneurysm of the right internal mammary artery and as a result, an embolization was performed. Here we report this case with a review of the appropriate literature.
