THE SUCCESSFUL MAJOR SURGERY IN A PATIENT WITH INHERITED FVII DEFICIENCY AND A HUGE NASOPHARYNGEAL ANGIOFIBROMA

Abstract

The bleeding phenotype of patients with inherited FVII deficiency is variable, and epistaxis is one of the most frequent symptoms. Interestingly, the bleeding risk does not correlate with the level of FVII activity. The severity of FVII deficiency and the type of surgery are not determinants of the optimal management of surgery, the doses and the duration of rFVIIa therapy are widely variable. The aim of this study is to present our successful experience in a 16-year-old boy with inherited FVII deficiency and a huge nasopharyngeal angiofibroma with a very high risk of bleeding The patient was referred with recurrent epistaxis in the last 6 months and he was diagnosed as an inherited FVII deficiency (FVIIC:29%, FVII inhibitor negative with positive family history). Tranexamic acid (10days) and rFVIIa (2doses) were used with success in the management of this surgery. Since this surgery may cause life-threatening bleeding, endovascular particle embolization was done to the important vessels feeding the mass one day before surgery without rFVIIa support. No bleeding or thrombosis were observed in our patient. In conclusion, a life-threatening major surgery was successfully done for a patient with inherited FVII deficiency and a huge angiofibroma. However, perioperative management of patients with FVII deficiency still remains a major challenge and clinical trials are needed to provide evidence-based optimal management of surgeries. And, angiofibroma should be thought in the differential diagnosis of epistaxis.