Perioperative Management for Port Catheter Procedures in Pediatric Patients with Severe Hemophilia and Inhibitors

Abstract

Background The objective of this systematic study was to assess the perioperative management and outcome of surgery in pediatric patients with hemophilia A/B and inhibitors compared to nonhemophilic pediatric patients.

Methods The surgical outcome of 69 port catheter operations in patients with hemophilia who developed inhibitory antibodies against the administered factor was compared to 51 procedures in the control group. In the patients with hemophilia and inhibitors, a standardized protocol for recombinant activated factor VII was used to prevent perioperative bleeding.

Results Hemophilic pediatric patients with inhibitors showed no significant differences in perioperative management (blood transfusion: p = 0.067, duration of surgery: p = 0.69; p = 0.824) in comparison to patients without hemophilia. The length of hospital stay was significantly longer in pediatric patients with hemophilia and inhibitors (20 days vs. 4 days for insertion; 12 days vs. 1 day for explantation). Moreover, no statistically significant difference was found for secondary bleeding (three patients with hemophilia vs. none in the control group; p = 0.11) or surgical complications (five hemophilia patients vs. none with grade I complication; one hemophilia patient vs. none with grade II complications; p = 0.067).

Conclusion This study has demonstrated that port catheter insertion and removal is safe in these patients. Moreover, it shows the importance of a coordinated approach with a multidisciplinary team.

Keywords

hemophilia A - hemophilia B - port catheter - inhibitors - antibodies - rFVIIa

Publication History

Received: 22 January 2024

Accepted: 26 May 2024

Article published online:
12 September 2024

• References

• 1 Richards M, Williams M, Chalmers E. et al; Paediatric Working Party of the United Kingdom Haemophilia Doctors' Organisation. A United Kingdom Haemophilia Centre Doctors' Organization guideline approved by the British Committee for Standards in Haematology: guideline on the use of prophylactic factor VIII concentrate in children and adults with severe haemophilia A. Br J Haematol 2010; 149 (04) 498-507
• 2 Meunier S, Trossaërt M, Berger C. et al; Groupe Prophylaxie CoMETH. French guidelines. Long-term prophylaxis for severe haemophilia A and B children to prevent haemophiliac arthropathy [in French]. Arch Pediatr 2009; 16 (12) 1571-1578
• 3 Oldenburg J. Optimal treatment strategies for hemophilia: achievements and limitations of current prophylactic regimens. Blood 2015; 125 (13) 2038-2044
• 4 Saint-Remy JM, Lacroix-Desmazes S, Oldenburg J. Inhibitors in haemophilia: pathophysiology. Haemophilia 2004; 10 (Suppl. 04) 146-151
• 5 Baghaipour MR, Steen Carlsson K. Strategies for inhibitor treatment and costs in the short and long term: a critical evaluation of recent clinical studies. Eur J Haematol 2015; 94 (Suppl. 77) 30-37
• 6 Batorova A, Morongova A, Tagariello G, Jankovicova D, Prigancova T, Horakova J. Challenges in the management of hemophilia B with inhibitor. Semin Thromb Hemost 2013; 39 (07) 767-771
• 7 Oldenburg J, Mahlangu JN, Kim B. et al. Emicizumab prophylaxis in hemophilia A with inhibitors. N Engl J Med 2017; 377 (09) 809-818
• 8 Pipe SW, Shima M, Lehle M. et al. Efficacy, safety, and pharmacokinetics of emicizumab prophylaxis given every 4 weeks in people with haemophilia A (HAVEN 4): a multicentre, open-label, non-randomised phase 3 study. Lancet Haematol 2019; 6 (06) e295-e305
• 9 Ewenstein BM, Valentino LA, Journeycake JM. et al. Consensus recommendations for use of central venous access devices in haemophilia. Haemophilia 2004; 10 (05) 629-648
• 10 Valentino LA, Kapoor M. Central venous access devices in patients with hemophilia. Expert Rev Med Devices 2005; 2 (06) 699-711
• 11 Goldmann G, Holoborodska Y, Oldenburg J. et al. Perioperative management and outcome of general and abdominal surgery in hemophiliacs. Am J Surg 2010; 199 (05) 702-707
• 12 Lingohr P, Bensoukehal S, Matthaei H. et al. Value and risk of laparoscopic surgery in hemophiliacs-experiences from a tertiary referral center for hemorrhagic diatheses. Langenbecks Arch Surg 2014; 399 (05) 609-618
• 13 Rogenhofer S, Hauser S, Breuer A. et al. Urological surgery in patients with hemorrhagic bleeding disorders Hemophilia A, Hemophilia B, von Willebrand disease: a retrospective study with matched pairs analysis. World J Urol 2013; 31 (03) 703-707
• 14 Jenkins PV, Collins PW, Goldman E. et al. Analysis of intron 22 inversions of the factor VIII gene in severe hemophilia A: implications for genetic counseling. Blood 1994; 84 (07) 2197-2201
• 15 Dindo D, Demartines N, Clavien PA. Classification of surgical complications: a new proposal with evaluation in a cohort of 6336 patients and results of a survey. Ann Surg 2004; 240 (02) 205-213
• 16 Ingerslev J, Hvid I. Surgery in hemophilia. The general view: patient selection, timing, and preoperative assessment. Semin Hematol 2006; 43 (1, Suppl 1): S23-S26
• 17 Chapin J, Bamme J, Hsu F, Christos P, DeSancho M. Outcomes in patients with hemophilia and von Willebrand disease undergoing invasive or surgical procedures. Clin Appl Thromb Hemost 2017; 23 (02) 148-154
• 18 Ljung RC, Knobe K. How to manage invasive procedures in children with haemophilia. Br J Haematol 2012; 157 (05) 519-528
• 19 Quintana-Molina M, Martínez-Bahamonde F, González-García E. et al. Surgery in haemophilic patients with inhibitor: 20 years of experience. Haemophilia 2004; 10 (Suppl. 02) 30-40
• 20 O'Connell N, Mc Mahon C, Smith J. et al. Recombinant factor VIIa in the management of surgery and acute bleeding episodes in children with haemophilia and high responding inhibitors. Br J Haematol 2002; 116 (03) 632-635
• 21 Goudemand J, Tagariello G, Lopaciuk F. Cases of surgery in high-responder haemophilia patients. Haemophilia 2004; 10 (Suppl. 02) 46-49
• 22 Matino D, Makris M, Dwan K, D'Amico R, Iorio A. Recombinant factor VIIa concentrate versus plasma-derived concentrates for treating acute bleeding episodes in people with haemophilia and inhibitors. Cochrane Database Syst Rev 2015; 2015 (12) CD004449
• 23 Ingerslev J, Christiansen K, Calatzis A, Holm M, Sabroe Ebbesen L. Management and monitoring of recombinant activated factor VII. Blood Coagul Fibrinolysis 2000; 11 (Suppl. 01) S25-S30
• 24 Shapiro AD, Gilchrist GS, Hoots WK, Cooper HA, Gastineau DA. Prospective, randomised trial of two doses of rFVIIa (NovoSeven) in haemophilia patients with inhibitors undergoing surgery. Thromb Haemost 1998; 80 (05) 773-778
• 25 Castaman G, Peyvandi F, Kremer Hovinga JA. et al. Surgical experience from the STASEY study of emicizumab prophylaxis in people with hemophilia A with factor VIII inhibitors. TH Open 2024; 8 (01) e42-e54
• 26 Jiménez-Yuste V, Rodríguez-Merchán EC, Matsushita T, Holme PA. Concomitant use of bypassing agents with emicizumab for people with haemophilia A and inhibitors undergoing surgery. Haemophilia 2021; 27 (04) 519-530