PS1101 PERIOPERATIVE MANAGEMENT AND OUTCOMES IN CHILDREN WITH CONGENITAL BLEEDING DISORDERS: A RETROSPECTIVE REVIEW AT A SINGLE HEMOPHILIA TREATMENT CENTER

Background: Establishing hemostasis for surgical procedures in children with inherited bleeding disorders is challenging. Providers are often hesitant to undertake surgeries in children with bleeding disorders out of fear of bleeding complications. However, appropriate presurgery/preprocedure factor replacement can decrease pre‐, peri‐, and postoperative bleeding complications and allow almost any necessary surgical procedure to proceed. Aims: We review the results of surgical procedures in children with inherited bleeding disorders at our center and review the preoperative management and hemorrhagic complications. Methods: We conducted a retrospective electronic medical record review from October 2006 to November 2018. Primary focus was review of factor replacement strategies and bleeding complications. Data were collected from patients under 18 years of age. Results: In total, 152 procedures were performed in 99 children at our center. The distribution of bleeding disorders is as follows; hemophilia A 79, hemophilia B 15, Von Willebrand disease 3 and FVII deficiency 2. The median age of the patients was 13 years (range, 0∼18). Duration of hospitalization was median 8 days (range, 0∼44) and period from surgery to discharge was median 7 days (range, 0∼42). Nineteen procedures (12.5%) in 8 children were performed in the presence of high titer inhibitors to coagulation factor. Procedures included synovectomy (84/152, 55.3%), orthopaedic procedures except synovectomy (22/152, 14.5%), central venous catheter placement or revision (9/152, 5.9%), otolaryngology procedures (7/152, 4.6%), circumcision (6/152, 3.9%), laparoscopic appendectomy (5/152, 3.3%) and miscellaneous (19/152, 12.5%). All patients received preoperative factor replacement (100% in hemophilia patients) followed by various factor replacement regimens postoperatively. No deaths or life‐threatening bleeding occurred with any procedure. Seven of 152 procedures (4.6%) were complicated by bleeding. Tonsillectomy was the most common procedure complicated by hemorrhage 3 of 7 (42.9%). The complete list of all 168 surgical procedures is shown in Table 2.All patients received preoperative factor replacement (100% in hemophilia patients) followed by various factor replacement regimens postoperatively. No deaths or life‐threatening bleeding occurred with any procedure. Seven of 152 procedures (4.6%) were complicated by bleeding. Tonsillectomy was the most common procedure complicated by hemorrhage 3 of 7 (42.9%). Summary/Conclusion: Surgical procedures are safe in children with bleeding disorders with adequate planning and factor replacement. Bleeding remains a problem in a subset of patients and requires ongoing hematological involvement and oversight. Delayed bleeding following tonsillectomy was especially common and suggests a need for close follow‐up and ongoing factor coverage for this group of patients.