Tonsil, adenoid surgery safe for hemophilia kids if plan is in place
Study finds almost no bleeding complications with strong management
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Children with hemophilia who underwent surgery to remove the tonsils or adenoids experienced almost no bleeding complications when surgeons followed a structured bleeding management plan, according to a 14-year single medical center review.
Of 18 pediatric hemophilia patients who underwent the surgery, one child had a minor bleeding episode, a rate far below what has been reported in earlier studies of this high-risk group.
“This protocol provides reassurance to caregivers and surgeons about the safety of the procedure, ensuring comprehensive care tailored to the unique needs of these patients,” the team wrote.
The study, “Bleeding Outcomes After Adenotonsillar Surgery in Pediatric Patients With Hemophilia: Perioperative Management and Outcomes,” was published in Pediatric Blood & Cancer.
Common procedure carries bleeding risk
Hemophilia is caused by a shortage of certain blood clotting factors, resulting in unusually easy and prolonged bleeding.
The two most severe types are hemophilia A, which involves a deficiency of clotting factor VIII, and hemophilia B, which involves a deficiency of clotting factor IX. A related but rarer condition, hemophilia C, involves a deficiency of factor XI and can also raise bleeding risk, though generally to a lesser degree.
Removing the tonsils and/or adenoids (adenotonsillectomy) is common in children, but it carries a risk of postsurgical bleeding. In the general pediatric population, this bleeding risk typically ranges from about 2% to 5%. Children with hemophilia face a much higher risk, with some earlier studies reporting rates above one-third of patients.
However, there is no single recommended protocol for managing hemophilia patients through this type of surgery, and most existing data come from small case reports.
The researchers described their own management approach to adenotonsillectomy in people with hemophilia and evaluated how well it worked in practice.
An examination of medical records identified 1,580 total adenotonsillar surgeries from 2010 to 2024. Among them, seven patients had hemophilia A, seven had hemophilia B, and four had hemophilia C. They ranged in age from three months to 18, and most (78%) were boys.
Each child was evaluated by both an ear, nose, and throat specialist and a hematologist (blood doctor) one to three months before surgery, with blood testing and severity classification done two to four weeks beforehand.
About an hour before surgery, patients received clotting factor replacement aimed at reaching 80% to 100% of normal activity, along with a dose of Cyklokapron (tranexamic acid), which helps stabilize blood clots. Factor IX was used for hemophilia B, factor VIII for hemophilia A, and plasma infusions for hemophilia C, since there is no specific concentrate available for that type.
After surgery, factor levels were checked daily and adjusted over time. Levels were kept between 60% and 80% for hemophilia A and 40% to 60% for hemophilia B or C during the first three days, then gradually lowered over the following two weeks as healing progressed. Tranexamic acid therapy was continued for a full 14 days after surgery.
Eight patients (44%) had adenoidectomy alone, and 10 patients (56%) had tonsillectomy, with or without adenoidectomy. Adenoid removal was most often done using a cold steel instrument.
Tonsil removal was most often performed using an extracapsular technique (removing the entire tonsil), which is generally considered to carry a higher bleeding risk than the intracapsular method (which leaves a thin layer of tissue behind). Surgery took a mean of 43 minutes for tonsillectomy and 28 minutes for adenoidectomy.
One bleeding event was recorded across the entire group, a minor nosebleed on the second day after surgery in a child with hemophilia B who had undergone adenoidectomy. This represented 5.5% of the group, and the patient was treated without surgery, using additional clotting factor and intravenous tranexamic acid.
No patient needed to return to the operating room for bleeding, and there were no cases of dehydration or breathing problems. Three children developed temporary fevers that resolved on their own. The broader group of patients without hemophilia who had these procedures at the same center had a post-surgical bleeding rate requiring hospitalization of 1.8%.
“Perioperative management in pediatric patients may help reduce the incidence of [post-tonsillectomy bleeding],” the team wrote. “This study supports the need for stringent perioperative protocols and further research into optimal management strategies for this high-risk population.”
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