Hemorrhagic shock due to ruptured lower limb vascular malformation in a neurofibromatosis type 1 patient:A case report

BACKGROUND Neurofibromatosis type 1(NF-1)is a common autosomal dominant genetic disorder.It is characterized by café-au-lait spots and cutaneous neurofibromas.Although NF-1 typically involves the skin,nerves,bones,and eyes,vascular manifestation in the form of devastating hemorrhage can occur rarely.CASE SUMMARY We present the case of a 47-year-old female with NF-1 who had a ruptured right lower limb arterial malformation.She presented with sudden right lower limb swelling for two hours and symptoms of hemorrhagic shock on admission.The physical examination revealed a right lower limb presenting as elephantiasis and visible dark-brown pigmentation over a large area.Computed tomography angiography showed right lower limb arteriovenous malformation.Therefore,the patient underwent emergency right lower limb digital subtraction angiography(DSA)and vascular embolization after blood transfusions.However,after DSA,vascular embolization,and repeated blood transfusions,the anemia and right lower limb swelling and tenderness did not improve.As a result,the patient underwent right lower extremity above-knee amputation.After amputation,the patient's hemoglobin level improved significantly without blood transfusion,and she was discharged from the hospital after the incision healed.Postoperative pathological examination suggested neurogenic tumors.No other complications had occurred 1-year follow-up.CONCLUSION Vascular malformation and rupture are fatal complications of NF-1.Embolization may not provide complete relief,the patient might need to undergo neurofibroma resection or amputation.

Behcet’s disease manifesting as esophageal variceal bleeding: A case report

BACKGROUND Behcet’s disease(BD)is a chronic disease characterized by oral and vulvar ulcers as well as eye and skin damage and involves multiple systems.It presents as an alternating process of repeated attacks and remissions.Esophageal venous rupture and bleeding caused by BD is rarely reported at home and abroad.This paper reports a case of bleeding from oesophageal varices caused by BD,aiming to provide an additional dimension for considering the cause of bleeding from esophageal varices in the future.CASE SUMMARY A 38-year-old female patient was admitted due to a gradual increase in shortness of breath and chest tightness after the activity,and was admitted to our hospital for treatment.After admission,relevant examinations showed that the patient had multiple blood clots.Four days after admission,she suddenly experienced massive hematemesis.Emergency esophagogastroduodenoscopy revealed bleeding from esophageal and gastric varices.The patient had no history of viral hepatitis or drinking habits,and no history of special genetic diseases or congenital vascular diseases.There is no obvious abnormality in liver function.After reviewing the medical history,it was found that the patient had recurred oral ulcers since childhood,ulcers were visible in the perineum during menstruation,and there was an intermittent red nodular rash and uveitis.The current skin acupuncture reaction is positive,combined with the evaluation of the external hospital and our hospital,the main diagnosis is BD.She received methylprednisolone,cyclophosphamide,immunomodulation,acid suppression,gastric protection,and anticoagulation and anti-infection treatments,and was discharged from the hospital.During the 1-year follow-up period,the patient did not vomit blood again.CONCLUSION This case highlights bleeding from esophageal varices caused by BD, aiming toprovide an additional dimension concerning the cause of bleeding fromesophageal varices in the future.