Surgery in adults with congenital coagulation deficiencies: how do we do it?

Abstract

Background: We retrospectively analysed major surgical procedures and complications for a period of 11 years and tried to find out if the reasons for surgery were population specific or the same as in general population. Methods: Retrospective analysis included documentation of patients with hemophilia, von Willebrand’s disease, and F VII deficiency treated at the Dept. for Hematology and the Centre for Hemophilia, Ljubljana in the period from 1 January2000 to 31 December 2011. Data for major surgeries, i.e. those carried out under general anaesthesia, extraction of more than 4 teeth with carving, catheterisation with coronary angiography and coronary stunt were sought. Surgery was done in different departments of the hospital. We were searching for complications, surgical and non-surgical, inhibitor development, concentrate used and the way of application (bolus, infusion). Results: There were 81 operations performed in 11 years in 56 patients aged 24–75 years. Less than 1/3 of them were over 50 years (30 % = 24/56), and 1/8 over 60 years (13 % = 7/56).Urgent surgery was done in 5 % (4/81). One or more operations were done in the same patient: 1–3 in 96.4 % (54/56), 4 and 6 in one. Half of the surgeries 49.5 % (40/81) were orthopedic, the other half were abdominal surgeries (18.5 % = 15/81), neurosurgeries (6.2 % = 5/81), otorhinolaryngological (4.9 % = 4.9 %), and some thoracic, plastic, urological, cardiologic/cardiovascular, gynecological and delivery and trauma-related surgical interventions. Several concentrates were used, mostly as bolus: rF VIII, plasma FVIII, plasma F IX, plasma F VIII+von Willebrand factor, rF VIIa. The activity of coagulation factor was checked daily as necessary. Minor bleeding occurred in 2.4 % (2/81); there were no surgical complications, no inhibitor development and no thrombosis. Conclusions: Surgery in adults with congenital coagulation disorders is always challenging. The risk of complications is average or lower if there is an adequate level of medical treatement and organisation. Treatment concentrates are safe, no inhibitor was found. Measures other thanmedicines are quite effective for prophylaxis of thrombosis. Surgery is partly specific for this population (hemophilic polyarthropathy) but it also reflects common problems of the aging population.