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<p>Surgical interventions in patients with congenital bleeding disorders(CBD) present unique challenges due to the increased risk of perioperative and postoperative bleeding. The data on spectrum of surgical procedures and their outcomes among CBD patients in the developing world is limited. This study aims to evaluate the surgical procedures performed in such patients and assess the outcomes, primariy clinically relevant periperative bleeding. A retrospective observational study was conducted on 22 consecutive surgical procedures performed in 20 patients with congenital bleeding disorders from January 2020 till July 2025 at a tertiary center in South India. Data collected included patient demographics, type and severity of bleeding disorder, inhibitor status, type of surgery, perioperative management, bleeding events, and postoperative outcomes. The cohort included 20 patients − 17 patients with hemophilia A (7 severe, 5 moderate, 5 mild), 1 with hemophilia B (severe), 1 with combined Factor VIII and Factor XI deficiency, and 1 with Factor VII deficiency. Of the nine pediatric patients, four were on emicizumab prophylaxis. Two pediatric patients had high titer inhibitors against factor VIII, of whom one was on emicizumab prophylaxis. Surgical procedures encompassed total knee replacement, knee arthroplasty, incision and drainage of infected leg hematoma, inguinal hernia repair, laparoscopic appendicectomy, ureteroscopy with lithotripsy, and circumcision. Tranexamic acid was used as an adjunctive agent in all the cases except during ureteroscopy. Targeted factor levels were achieved in all the cases. The median factor consumption among orthopedic cases in severe hemophilia A was 825 U/kg/procedure. The median factor consumption per procedure for minor surgical cases among severe hemophilia A cohort was 270 U/kg and 45 U/kg, respectively for those not on emicizumab and, on emicizumab, respectively. In inhibitor positive cases, the factor eight inhibitor bypassing agent(FEIBA) use was much less in patients on emicizumab versus those not on emicizumab(25 U/kg vs. 825 U/kg). The overall bleeding rate was 3/22 (13.6%). Of the three bleeding events, there was one instance each of grade 2, grade 3, grade 4 bleeding by World Health Organization bleeding scale. The bleeding rate in non-inhibitor cases was 2/20(10%), and in inhibitor cases 1/2(50%). The bleeding rate among orthopedic and non-orthopedic cases were 1/4(25%) and 2/18(11%), respectively. With appropriate perioperative management, including individualized factor replacement therapy, patients with congenital bleeding disorders can safely undergo a wide range of surgical procedures. Emicizumab prophylaxis significantly reduces factor VIII/FEIBA usage in patients with hemophilia A undergoing surgical procedures. Multidisciplinary coordination is essential to optimize outcomes.</p>

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Achieving Successful Surgical Outcomes in Patients with Congenital Bleeding Disorders: Experience from a Resource-Limited Setting

  • 期刊论文

  • 发表日期：

    2026年5月20日

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  • Henna Pokka,
  • Raghuveer S. Prabhu,
  • S. N. Rahmathullah,
  • Javed Ahammad

摘要

Surgical interventions in patients with congenital bleeding disorders(CBD) present unique challenges due to the increased risk of perioperative and postoperative bleeding. The data on spectrum of surgical procedures and their outcomes among CBD patients in the developing world is limited. This study aims to evaluate the surgical procedures performed in such patients and assess the outcomes, primariy clinically relevant periperative bleeding. A retrospective observational study was conducted on 22 consecutive surgical procedures performed in 20 patients with congenital bleeding disorders from January 2020 till July 2025 at a tertiary center in South India. Data collected included patient demographics, type and severity of bleeding disorder, inhibitor status, type of surgery, perioperative management, bleeding events, and postoperative outcomes. The cohort included 20 patients − 17 patients with hemophilia A (7 severe, 5 moderate, 5 mild), 1 with hemophilia B (severe), 1 with combined Factor VIII and Factor XI deficiency, and 1 with Factor VII deficiency. Of the nine pediatric patients, four were on emicizumab prophylaxis. Two pediatric patients had high titer inhibitors against factor VIII, of whom one was on emicizumab prophylaxis. Surgical procedures encompassed total knee replacement, knee arthroplasty, incision and drainage of infected leg hematoma, inguinal hernia repair, laparoscopic appendicectomy, ureteroscopy with lithotripsy, and circumcision. Tranexamic acid was used as an adjunctive agent in all the cases except during ureteroscopy. Targeted factor levels were achieved in all the cases. The median factor consumption among orthopedic cases in severe hemophilia A was 825 U/kg/procedure. The median factor consumption per procedure for minor surgical cases among severe hemophilia A cohort was 270 U/kg and 45 U/kg, respectively for those not on emicizumab and, on emicizumab, respectively. In inhibitor positive cases, the factor eight inhibitor bypassing agent(FEIBA) use was much less in patients on emicizumab versus those not on emicizumab(25 U/kg vs. 825 U/kg). The overall bleeding rate was 3/22 (13.6%). Of the three bleeding events, there was one instance each of grade 2, grade 3, grade 4 bleeding by World Health Organization bleeding scale. The bleeding rate in non-inhibitor cases was 2/20(10%), and in inhibitor cases 1/2(50%). The bleeding rate among orthopedic and non-orthopedic cases were 1/4(25%) and 2/18(11%), respectively. With appropriate perioperative management, including individualized factor replacement therapy, patients with congenital bleeding disorders can safely undergo a wide range of surgical procedures. Emicizumab prophylaxis significantly reduces factor VIII/FEIBA usage in patients with hemophilia A undergoing surgical procedures. Multidisciplinary coordination is essential to optimize outcomes.