A Large Case Series on Surgical Outcomes in Congenital FXIII Deficiency Patients in Iran

Background

FXIII requires low plasma FXIII levels for normal hemostasis. The lack of accepted standardized surgical guidelines necessitates dependence on the treating physicians' and centers' experiences.

Aim

Our aim is to evaluate surgical outcomes on a large group of congenital FXIII deficiency (FXIIID) patients.

Methods

A case series study was conducted on congenital FXIIID patients in two major referral centers located in Iran from 2010 to 2016. All patients were on prophylaxis using plasma factor XIII concentrate (10 U kg⁻¹, every 28 days) except three patients prior to surgery. One single dose of 10 U kg⁻¹ and 30 U kg⁻¹ plasma factor XIII concentrate before a minor procedure and circumcision were given, respectively. Two doses of plasma factor XIII concentrate, one 30 U kg⁻¹ prior to the procedure and the second dose of 30 U kg⁻¹ on post-operative day 3, were given for major surgery. The plasma factor XIII concentrate dose was 50 U kg⁻¹ both before and after the procedure for neurosurgery.

Results

One hundred sixty-two FXIIID patients underwent minor, major and obstetrical and gynecological surgeries. The median age of the patients was 14 years (ages ranged 15 days to 47 years). The male-to-female ratio was 89/73. Five post-operative complications, two bleeding and three thrombosis, were recorded for two patients who were undiagnosed prior to surgery.

Conclusion

Our study showed excellent hemostasis in FXIIID patients undergoing surgeries. Of these surgeries, we observed only 1.8% post-operative complications. Surgery can be performed safely in FXIIID patients, and the proposed treatment regimens lead to adequate hemostatic coverage with minimal risk for both minor and major surgeries.