Perioperative Management of Hemophiliacs - a Retrospective Study from an Ihtc in a Lower Middle-Income Country
Authors: Pechetty Priyanka , Shanmukhaiah Chandrakala , Mohanty Shubhranshu Shekhar , Kulkarni Bipin , Keswani Pranav , Kadam Tushar Sandeep , Garale Mahadeo N , Pawar Shwetal Ghule , Patil Kirti Amol 
Published: December 2024

Background: The management of surgical procedures in individuals with haemophilia is a matter of considerable concern due to the potential impact on health outcomes. Inadequate haemostatic control may significantly contribute to morbidity and mortality. However, the success of surgical procedures is contingent not only upon the appropriate replacement of the deficient factor but also on pre- and post-operative interventions, laboratory monitoring, and patient care and rehabilitation. It is imperative to conduct surgical procedures for patients with haemophilia in fully equipped hospitals capable of delivering a comprehensive, multidisciplinary approach, often referred to as a “Comprehensive Haemophilia Treatment Centre.” Our study aims to assess the outcomes of surgical procedures for individuals with haemophilia at KEM Hospital, Mumbai, which is recognised as a comprehensive Haemophilia Treatment Centre in India.

Methods: This retrospective, observational study encompassed all surgical interventions conducted on patients with haemophilia between the specified dates. Data on baseline activated partial thromboplastin time (aPTT) and factor levels before the surgical procedures, inhibitor screening and titration results (if applicable), complete blood count, blood group, and liver function tests were retrospectively extracted from patient files. Specific information documented encompassed the nature and location of the surgical procedure, the quantity of factor concentrates administered pre-, intra-, and post-operatively, subsequent factor levels and aPTT findings post-replacement, any perioperative or postoperative complications, the necessity for packed red cell transfusion, and details of the treatment modality employed before surgery (on demand vs. prophylaxis). The dosage of factor concentrates administered to patients was quantified in units per kilogram.

Results: 198 procedures were analysed in the study, with 92 major and 106 minor surgeries. For major surgeries, 88.16% were for haemophilia A and 11.84% for haemophilia B. Among these, 4.3% tested positive for inhibitors (3.95% for factor 8 and 1.31% for factor 9). Of the cases, 64.79% had severe, 18.31% moderate, and 16.90% mild haemophilia. The median age at the time of surgery was 32.5 years (5 to 79). Orthopaedic procedures accounted for 74.2% of major surgeries, and general surgery accounted for 12.4%.

Regarding minor surgeries, 83.33% were for haemophilia A and 16.67% for haemophilia B. Inhibitors were present in 15.21% of cases. Among these, 62.92% were severe, 19.10% were moderate, and 17.98% were mild. The median patient age was 24 years (0.4 to 82). General surgical procedures were the most common at 37.8%, followed by orthopaedic procedures at 33.3%.

Factor consumption was 411.5 U/kg (ranging from 50 to 2960) in haemophilia A without inhibitors and 322.3 U/kg (ranging from 51.2 to 780) in patients with inhibitors. The median factor requirement for haemophilia B was 362.4 U/kg for patients without inhibitors and 662.7 U/kg for patients with inhibitors.

Complications occurred in 13 (6.5%) surgeries, but no deaths occurred. The complication rates did not significantly differ among surgical branches. Most patients had severe haemophilia, and there were no significant differences in complication rates between different types and severities of haemophilia. Major surgeries had more complications compared to minor procedures. Patients with complications required higher factor doses.

Conclusion: The results of our retrospective study demonstrate that in experienced comprehensive care centres, surgical procedures can effectively and successfully be performed in PwH, even in those with severe haemophilia and those with inhibitors