Abstract
In some cases of Hemophilia A patients who are treated with factor VIIII replacement therapy, factor VIIII inhibitor appears, making bleeding control difficult. The authors describe their experience with factor VIII bypassing therapy by using a factor VIII inhibitor to repair a cleft palate.
The case was a 19-months-old old male child with congenital cleft lip, alveolus, and palate. The lip was repaired at the age of four months, and at that time, prolonged bleeding was noticed. Investigations revealed that the patient had Hemophilia A. Thereafter, the patient was treated with factor VIII replacement therapy, but factor VIII inhibitor appeared, and consequently the factor VIII replacement therapy became ineffective.
The patient was given the ideal amount of prdthrombin complex concentrate (PCC), which is the first choice for Hemophilia A with factor VIII inhibitor, and the cleft palate was repaired using the push back method and vomer flap.
But a day after the operation, bleeding could not be controlled in the bone exposed area of the hard palate, and activated prdthrombin complex concentrate (APCC), which is the second choice for Hemophilia A with factor VIII inhibitor, was started, and five days after the operation, the vomer flap was returned.
Bleeding was controlled after the second operation by using a combination of PCC and APCC, and the patient was discharged 37 days after the operation.
Up to now, therapy for Hemophilia A with a factor VIII inhibitor has been described only in some minor cases, like tooth extraction. In the present case, bleeding control was an unknown quantity. In advance, the authors confirmed that APTT reached 86 seconds at six hours after instillation of PCC, but controllable APTT time was level of fifty second.
The authors describe that the instillation quantity of PCC and APCC should be decided on a case-by-case basis because of few relationship between the clinical effect and shortening of PT and APTT.
