Management challenges of perianal necrotizing fasciitis complicated by haemophilia A: A clinical case report

INTRODUCTION AND IMPORTANCE: Haemophilia A (HA) is a hereditary X-linked recessive hemorrhagic disorder that results from a deficiency or dysfunction of coagulation factor VIII (FVIII) caused by gene mutations. CASE PRESENTATION: This case report presents the challenging management of a 37-year-old man who developed perianal necrotizing fasciitis accompanied by severe infection, necrosis, and septic shock. The patient underwent emergency surgery. However, significant bleeding occurred during and after the surgery. CLINICAL DISCUSSION: Despite initial treatment with fresh frozen blood plasma infusion satisfactory efficacy was not achieved. Investigation into the patient's family history revealed a haemophiliac niece, prompting further testing for haemophilia. Ultimately, the patient was diagnosed with haemophilia A. Hemorrhage controlled was obtained through coagulation factor VIII infusion. With subsequent treatment, the patient experienced significant recovery, and normal anal function was restored. CONCLUSION: In summary, routine coagulation examination may not effectively evaluate coagulation dysfunction in patients with severe infectious diseases. Comprehensive preoperative evaluations are necessary for acute anorectal surgeries, with emphasis on screening for haemophilia.