Surgical management in a classical haemophilia patient – A rare case report

Abstract

A young man presented with complaints of right upper quadrant pain for 6 months, past history revealed he is a known case of Haemophilia A, which is a challenge for surgeons and anaesthetist to manage intraoperative and postoperative bleeding, henceforth referred to Haematology, department of pathology. Soon after taking clinical history patient was subjected for haemostatic screening tests such as PT, APTT, mixing studies, inhibitors screening and factor VIII assay revealed patient is a case of several haemophilic with negative for inhibitors and elevated APTT values. Patient is prepared for surgery only after giving Anti haemophilic factor replacement and preoperatively achieving 100% haemostasis by raising Factor VIII assay to 100%. Intra and post operatively patient is constantly monitored for bleeding. Post operatively every 12hrs patient was infused with anti-haemophilic factor variable levels and tapering doses to maintain constant haemostasis as the factor VIII half-life is less than 12 hours. Patient was haemodynamically stable when discharged that is 7 day post-surgery. Thus, we presenting a case how to manage haemophiliacs in a simple way for any kind of surgery with availability of antihemophilic factors and expertise in maintaining the haemostasis as build up confidence among surgeons.