Inadvertent surgeries for patients with haemophilia - Emergent need for creating awareness

Introduction: Majority of patients with hemophilia (PwH) has musculoskeletal (MSK) abnormalities and a large proportion of surgeries done in PwH are for MSK related complications. Often PwH have their initial presentation to orthopedicians with joint swelling even prior to bleeding from any other sites. Yet, haemophilia is an under-recognized entity among orthopedicians. Materials & Methods: This is a retrospective analysis of institutional haemophilia registry data of patients who has been operated for different MSK related abnormalities with special focus on those patients inadvertently operated by orthopedic surgeons without a prior diagnosis of haemophilia. Pre-operative evaluation and circumstances which led to the predicament of those patients were reviewed. All patients subsequently underwent a full coagulation work up including factor assay and inhibitor screen on suspecting a bleeding diathesis. Results: Out of a total 212 patients in the registry, 32 (15%) patients required surgical intervention and 16 (50%) of them were for MSK complications. Eleven (69%) patients underwent elective procedures with prior work up and five 5 (31%) patients underwent orthopaedic procedures inadvertently without a prior diagnosis of haemophilia. All the 5 patients (Haemophilia A-3 and Haemophilia B-2) achieved haemostasis with replacement therapy with varied surgical outcomes. Conclusions: Hemophilic arthropathy must be considered as a differential diagnosis in mono-articular arthritis. Careful elicitation of history and appropriate preoperative screening with prothrombin time (PT) and activated partial thromboplastin time (aPTT) is the antecedent to make a definitive diagnosis in a patient with suspected increased risk of bleeding.