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

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:  = 0.067, duration of surgery:  = 0.69;  = 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;  = 0.11) or surgical complications (five hemophilia patients vs. none with grade I complication; one hemophilia patient vs. none with grade II complications;  = 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.