Desmopressin and Epsilon Amino-Caproic Acid (EACA) in Adenotonsillectomy (T _& A): Are We Under-Treating Patients with Mild Type 1 von Willebrand Disease (VWD) and Mild Platelet Function Defects (PFD)?.

The advent of safer treatment for patients with mild congenital bleeding disorders has led to increasing numbers of surgical procedures such as T _& A, one of the most common surgical procedures in the pediatric age group. Recommendations for prophylaxis of surgical hemorrhagic complications vary significantly even among clinicians from the same institution. We examined the records of 700 patients with mild type 1 VWD and mild PFD seen in the Comprehensive Center for Bleeding Disorders from January 1995-December 2003. Seventy-three underwent adenotonsillectomy; 8 were excluded from the analysis for lack of follow-up and one was treated with Humate-P because of failure to respond to desmopressin. Of the 64 included in the analysis, 28 had mild type 1 VWD. Thirty-six had a mild PFD characterized by failure of platelets to release ATP (all 36 patients) in response to ADP and disaggregation following initial aggregation in 19. All 64 patients were given 0.3 mcg/kg desmopressin IV over 30 min. about 1 hr prior surgery; post-operatively, patients received additional desmopressin for 0–3 days and oral EACA for 0–14 days. Thirteen of the 64 (20%) patients had excessive bleeding in the peri-operative period (1 intra-operative and 12 post-operative); whereas published rates of bleeding in normal patients range from 1–4% of cases. Bleeding time and historical bleeding scores were not different between the bleeder and non-bleeder groups. Among VWD patients, there was no difference in baseline VWF:RCo levels or rise in VWF:RCo levels following a challenge dose of desmopressin in those who did not bleed compared to those who did. However, bleeding rates were significantly higher among patients with PFD; nine of 36 (25%) of patients with a PFD had a hemorrhagic complication compared to 4 of 28 (14%) of those with VWD (p<0.02). Four of the post-operative bleeds occurred after EACA had been discontinued; 1 of the bleeds occurred prior to the institution of EACA on day 3. (This patient had also not received post-operative desmopressin.) None of the patients who received 14 days of EACA had post-operative bleeding. Over all, patients with VWD received more prolonged treatment with both desmopressin and EACA than those with PFD. In summary, patients with PFD are perceived as having less risk for post-operative bleeding than those with VWD as evidenced by their less aggressive preventive treatment. However, in this large series, the risk of hemorrhage following T _& A in patients with PFD is at least as high as in those with mild type 1 VWD. Neither group fared as well as patients without a diagnosis of congenital coagulopathy. We conclude that formal randomized clinical trials are warranted in order to optimize surgical prophylaxis to prevent hemorrhagic complications in patients with mild PFD and mild type 1 VWD.