USE OF CENTRAL VENOUS CATHETERS IN CHILDREN WITH SEVERE CONGENITAL COAGULOPATHY

From two U.K. centres 23 children with severe congenital coagulopathy had a total of 27 port-a-cath devices inserted to facilitate factor VI II or IX prophylaxis (eight patients), domiciliary therapy (seven pati ents), immunotolerance (four patients), or a combination thereof (four patients), Six children had a factor VIII inhibitor at the time of in sertion. The mean age at operation was 30 months, with a range of 9-76 months, The cumulative length of follow-up is 639 months with a mean of 27.8 months and a range of 5-79 months. Haemostasis was achieved pe ri- and post-operatively with high-purity concentrate in the majority of patients without an inhibitor. All those with an inhibitor had porc ine factor VIII, except one who had recombinant factor VIIa. The post- operative complication rate was 27% (6/23): three had a port-site haem atoma (one required removal and replacement), two had post-operative i nfection, and one had swelling caused by extravasation. To date there have been 13 documented infections in 10/23 patients (five with inhibi tor): a rate of 0.24 per follow-up year or 0.67 per 1000 patient-days, Six were caused by Gram-positive and seven by Gram-negative organisms . Six infections could not be eradicated by antibiotics and the port-a -cath system had to be removed; in three it was replaced by a second p ort-a-cath. Although there are risks involved in the use of port-a-cat hs in this population, both clinicians and parents involved in the car e of these children believe that the benefits are considerable and the potential hazards are acceptable.