Kwang Seok Han1, Kyung Mi Lee2, Bum Joon Kim3, Byung Duk Kwun1, Seok Keun Choi1, Sung Ho Lee4. 1. Department of Neurosurgery, College of Medicine, Kyung Hee University, Seoul, Korea. 2. Department of Radiology, College of Medicine, Kyung Hee University, Seoul, Korea. 3. Department of Neurology, College of Medicine, Kyung Hee University, Seoul, Korea. 4. Department of Neurosurgery, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea. Electronic address: nooname79@gmail.com.
Abstract
BACKGROUND: Although vascular abnormality is an uncommon comorbidity of neurofibromatosis type 1 (NF1), it is potentially fatal. We present spontaneous hemothorax caused by rupture of a vertebral artery (VA) aneurysm in a patient with NF1. CASE DESCRIPTION: A 36-year-old man with a history of NF1 was transferred to the emergency department with dyspnea. Chest computed tomography scan revealed hemothorax in the left lung field with mediastinal shifting and aneurysmal dilatation of the left VA at the C6 vertebra level. Immediate drainage of the hematoma by chest tube insertion was performed. Diagnostic angiogram showed a 3- to 4-cm fusiform aneurysm of the VA. After the angiogram, cardiopulmonary arrest occurred after a rebleed of the VA aneurysm. The aneurysmal segment of the VA was urgently occluded with detachable coils. Postoperatively, the patient was in intensive care for 1 month because of fulminant pneumonia. After the patient regained consciousness, he was found to have right hemiparesis from a small infarction at the pons. The patient's function improved to near normal after 1 year of recovery. CONCLUSIONS: Hemothorax caused by VA rupture in a patient with NF1 is an extremely rare condition that can be fatal. Careful examination with suspicion for early detection and treatment is required for this urgent condition. Endovascular coiling was safe even for an unstable patient with massive bleeding.
BACKGROUND: Although vascular abnormality is an uncommon comorbidity of neurofibromatosis type 1 (NF1), it is potentially fatal. We present spontaneous hemothorax caused by rupture of a vertebral artery (VA) aneurysm in a patient with NF1. CASE DESCRIPTION: A 36-year-old man with a history of NF1 was transferred to the emergency department with dyspnea. Chest computed tomography scan revealed hemothorax in the left lung field with mediastinal shifting and aneurysmal dilatation of the left VA at the C6 vertebra level. Immediate drainage of the hematoma by chest tube insertion was performed. Diagnostic angiogram showed a 3- to 4-cm fusiform aneurysm of the VA. After the angiogram, cardiopulmonary arrest occurred after a rebleed of the VA aneurysm. The aneurysmal segment of the VA was urgently occluded with detachable coils. Postoperatively, the patient was in intensive care for 1 month because of fulminant pneumonia. After the patient regained consciousness, he was found to have right hemiparesis from a small infarction at the pons. The patient's function improved to near normal after 1 year of recovery. CONCLUSIONS: Hemothorax caused by VA rupture in a patient with NF1 is an extremely rare condition that can be fatal. Careful examination with suspicion for early detection and treatment is required for this urgent condition. Endovascular coiling was safe even for an unstable patient with massive bleeding.