Neurofibromatosis 1 is an autosomal dominant disorder characterized by cafe-au-lait spots, cutaneous neurofibroma, and bony deformities. Vascular abnormality such as stenosis, aneurysm, or rupture associated with neurofibromatosis 1 is rare. Rupture of vertebral artery aneurysm into the thoracic cavity is extremely rare. The outcomes of patients with aneurysmal ruptures are very poor when spontaneous hemothorax occur. A 31-year-old woman presented to the emergency department with left shoulder pain and with both lower chest wall pain and left supraclavicular area swelling. The chest computed tomography scan revealed about 4-cm pseudo-aneurysm probable arising from the left vertebral artery with large hematoma at left supraclavicular area. Neurofibromatosis 1 is generally being regarded as a benign disease but has the potential for serious vascular complications. When aneurysms were ruptured, cervical hematoma, hemothorax, or hypotension was developed. It is potentially a life-threatening condition, so it must require emergent management. Emergency physicians must remember the relation of neurofibromatosis 1 and serious vascular complications and ensure rapid access to rule out vascular lesions, so as to prevent the life-threatening condition.
Bibliographical noteFunding Information:
The manuscript, as submitted or its essence in another version, is not under consideration for publication elsewhere and will not be published elsewhere while under consideration by the Hong Kong Journal of Emergency Medicine. The authors have no commercial associations or sources of support that might pose a conflict of interest. All authors have made substantive contributions to the study, and all authors endorse the data and conclusions. D.H.L. and Y.H.C. participated in the design of the study and conceived the study. D.H.L. and Y.H.C. were major contributors in writing the manuscript. J.H.L., K.K., and J.Y.L. helped to draft and revise the manuscript. All authors read and approved the final manuscript. The author(s) received no financial support for the research, authorship, and/or publication of this article.
© The Author(s) 2018.
- vertebral artery aneurysm