Having your tonsils removed gets more complicated if you have a bleeding disorder

Adenoidectomy and tonsillectomy (adenotonsillectomy) remain two of the most commonly performed surgeries in the United States. For the most part, these procedures are safe and effective; however, risk of post-operative bleeding remains a concern. In patients with bleeding disorders, the inherent risk of post-operative bleeding for adenotonsillectomy procedures poses a difficult dilemma to the otolaryngologist—head and neck surgeon (ear, nose, and throat specialist).

Studies estimate that one to four percent of normal patients have bleeding associated with tonsil removal, regardless of the procedure used. Patients with bleeding disorders have a much higher risk of bleeding during and after the surgery.

A team of otolaryngologist—head and neck surgeons and hematologists have investigated a special protocol in tonsillectomy patients with Von Willebrand disease, a genetic bleeding disorder characterized by tendency to bleed from mucous membranes for a prolonged time due to abnormal or absent von Willebrand factor. This disorder affects upwards of two percent of the population, necessitating the development of a regimen that can reduce this population’s risks of bleeding.

Past studies have explored the use of clotting agents and non-transfusional therapies for adenotonsillectomy patients with von Willebrand disease. The findings in these research efforts revealed an average post-operative hemorrhage of around ten percent, a rate deemed excessive given the high risks of bleeding in this population.

This new study reports on a new protocol individualized for patients with von Willebrand disease and those with similar bleeding disorders. The authors of “Tonsillectomy and Adenoidectomy in Children with Bleeding Disorders,” are Alessandro de Alarcón MD, Margaret Sennett PNP,, Steven Early MD, and Charles Gross MD, all from the University of Virginia Health Sciences System, Charlottesville, VA; and Pedro de Alarcón MD, from St. Jude Children’s Research Hospital ,Memphis, TN. Their findings are being presented as a poster/paper study at the 107th meeting of the Triological Society http://www.triological.org, being held April 30 - May 3, 2004, at the JW Marriott Desert Ridge Resort & Spa, Phoenix, AZ.

Methodology: A retrospective chart review identified patients with bleeding disorders who had operations performed by the Otolaryngology service at the University of Virginia from 1998-2004. Patient hospital charts, operative records, and clinic charts were reviewed for demographics, type of surgery, specific treatment protocols, and complications. Eleven patients were identified that met study criteria. Three patients with Factor VIII deficiency, one patient with platelet disorder, and eight patients with von Willebrand Disease (VWD) were identified.

All patients with VWD were diagnosed with mild type 1 VWD. Patients with VWD were all proven responders to desmopressin (DDAVP), (desmopressin acetate), a synthetic analogue of the natural pituitary hormone 8-arginine vasopressin (ADH), that supports coagulation. The treatment protocol was:

1. Preoperatively document adequate response to DDAVP with rise in Ristocetin Cofactor and Factor VIII at separate clinic visit
2. Preoperative labs
3. Administer Intranasal DDAVP (Stimate®) or DDAVP IV at 0.3 mcg/kg 30 minutes prior to operating, day one, and day five after surgery.
4. Document appropriate response with rise of Ristocetin and Factor VIII to 70-90 percent normal. If inadequate response administer Humate®.
5. Restrict fluids to 1/2 maintenance for 12 hours post-surgery and after each dose of DDAVP.
6. Admit for 23 hour observation.
7. Administer aminocaproic acid (Amicar®) 50mg/kg four times a day for five days.

The treatment prescribed for Factor VIII deficiency consisted of:

1. Preoperatively, a substantial dose of 100 percent Factor VIII was given 30 minutes prior to surgery.
2. Intraoperative Factor VIII was then administered at a rate of 4u/k/hr intra-operatively and for days one through four after surgery. This was then followed by 3u/k/hr of Factor VIII for two days followed by home IV therapy Factor VIII, three times a day, for 4-6 days.

Twelve patients were included in the study, eight patients with von Willebrand disease (VWD) mild type 1, three with Factor VIII deficiency, and one with platelet dysfunction responsive to DDAVP. Nine adenotonsillectomies were performed: five in patients with VWD, three with Factor VIII deficiency, and one with platelet dysfunction. One patient with VWD had tonsillectomy performed alone.

Results: Age range of the study population was two-ten with a mean of 6.25 years. Four of ten patients had tonsillectomy performed by electrocautery, six had tonsillectomy performed by cold steel technique. Intraoperative blood loss ranged from 10cc to 50 cc with an average of 32.9 overall. Average blood loss for the tympanoplasties was 10cc, for the tonsillectomies and adenoidectomies 37.5cc. Hospital admission ranged from 23 hours to six days. The VWD population had an average admission stay of 23 hours while the Factor VIII deficiency patients had a range of three to six days and average of 4.3 days. There were no post-operative bleeding complications.

The finds revealed that use of DDAVP pre-operatively constitutes an effective peri and post operative prophylactic regimen for mild to moderate type 1 VWD patients undergoing adenotonsillectomy. In this study there were no complications and the admission time was relatively short. Factor VIII deficiency results in more severe bleeding. Although only a small number of patients with this disorder were in the study, the treatment protocol proved efficacious in reducing the risk of post-operative bleeding.

Conclusions: This research suggests a new standard of care for the management of mild bleeding disorders in patients undergoing adenotonsillectomy. Cooperation between the otolaryngologist and the hematologist in the management of the patient with a bleeding disorder needing surgery is essential.