2022 Volume 21 Issue 3 Pages 157-161
Thisreport presentsa 53-year-old woman with neurofibromatosis type 1 (NF-1). She was brought to the emergency room because of sudden onset of abdominal pain. Contrast-enhanced CT on admission showed a superior mesenteric artery aneurysm and intra-abdominal bleeding. Diagnosis of intra-abdominal hemorrhage due to aneurysm rupture was made. During the course of the disease, multiple intra-abdominal hemorrhages occurred, requiring three endovascular treatmentsand two laparotomiesfor partial jejunal resection and pancreaticoduodenectomy. The pathological specimen removed at the time of pancreaticoduodenectomy showed extensive hemorrhage and proliferating neurofibroma cells in the interstitium, including the perivascular area. Major hemorrhage associated with NF-1 vascular lesions is relatively rare, and there have been few reportsfrom the field of dermatology except for those related to diffuse neurofibromas; however,there have been reportsfrom other fields. It is necessary to explain to patients that they should not be unnecessarily anxious, and that they should go to a core hospital where multidisciplinary treatment is available if they require surgery with major invasion. Patients should be transported to a core hospital in case of emergency. Skin Research, 21 : 157-161, 2022
