Hemophilia A is an X-linked hereditary bleeding disorder caused by coagulation factor VIII deficiency, and although males are usually affected, there have been a few reports of excessive bleeding in female carriers. Here we report a case of a hemophilia A carrier diagnosed after postadenotonsillectomy hemorrhage. A 4-year-old girl with sleep apnea was referred to one of the authors. Adenotonsillar hypertrophy was detected, and her parents requested surgical treatment. Preoperative blood tests

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... owed an elevated activated partial thromboplastin time (APTT) of 41.5s (normal range: 25.8-37.7s, normal control: 27.0s), but other routine laboratory data yielded no abnormal fundings. Adenotonsillectomy was carried out uneventfully under general anesthesia with little intraoperative blood loss. However, there was significant wound hemorrhage within several hours after the operation, and reoperation was performed, but oozing from the wound persisted. A second reoperation with blood transfusion was performed on postoperative day 5, and detailed questioning about the family history revealed that her mother's brother had mild hemophilia. The patient was tested for clotting function, and was diagnosed as a hemophilia A carrier. Coagulation factor VIII activity was 68%, the lower limit of the normal range, but the vWF (von Wiilebrand factor) level was 152% and within the normal range. Infusion of factor VIII concentrate was started and there was immediate and complete resolution of the oozing. The patient was discharged 17 days after the adenotonsillectomy We wish to emphasize that whenever the APTT of female patients as well as male patients is prolonged 10 seconds or more above normal control in a preoperative blood test, clotting factor tests should be performed in addition to careful questioning about the family history. Preoperative diagnosis and adequate correction of factor VIII activity, if necessary, allow surgery, such as tonsillectomy to be performed safely in hemophilia patients and hemophilia carriers.