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

Anna Woestemeier, Silvia Horneff, Vincent Marlon Lüder, Jennifer Nadal, Arne Koscielny, Jörg C. Kalff, Johannes Oldenburg, Georg Goldmann, Philipp Lingohr
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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.

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严重血友病和抑制剂儿科患者端口导管手术的围手术期管理
背景 这项系统性研究的目的是评估与非血友病儿科患者相比,A/B 型血友病和抑制剂儿科患者的围手术期管理和手术结果。方法 对血友病患者的 69 例端口导管手术与对照组的 51 例手术的手术结果进行比较。在血友病患者和抑制剂患者中,采用了重组活化因子 VII 的标准化方案,以防止围手术期出血。结果 患有抑制剂的血友病儿科患者与非血友病患者相比,在围手术期管理方面没有明显差异(输血:P = 0.067;手术时间:P = 0.69;P = 0.824)。患有血友病和抑制剂的儿科患者住院时间明显更长(插入手术 20 天对 4 天;剥离手术 12 天对 1 天)。此外,在继发性出血(3 名血友病患者与对照组无;P = 0.11)或手术并发症(5 名血友病患者与无一级并发症患者;1 名血友病患者与无二级并发症患者;P = 0.067)方面也没有发现明显的统计学差异。结论 本研究表明,在这些患者中插入和拔出端口导管是安全的。此外,该研究还显示了多学科团队协调合作的重要性。
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