IMPLANTABLE CENTRAL VENOUS ACCESS DEVICES IN CHILDREN WITH METABOLIC DISEASE

We have inserted 20 totally implantable central venous devices in 17 p atients with severe metabolic disease over a 43-month span. Patient ag es ranged from 2 months to 17 years (mean, 4.2 years). The underlying pathology was Gaucher's disease in six patients, vitamin D-dependent r ickets type II in five, propionic acidemia in two, and methlymalonic a cidemia, 3-hydroxyl-3-methylglutaryl coenzyme A (CoA) lyase deficiency , fructose 1,6 diphosphatase deficiency, and urea cycle disorder in on e child each. There were seven complications (six due to catheter-rela ted infection and one due to occlusion of the system) during a total o f 7,278 patient-catheter days. The infection rate was 0.8 per 1.000 da ys. Six catheters were removed due to complications and two due to com pletion of treatment. There were no operative complications or deaths. Our experience demonstrates that a totally implantable device may be useful in children with metabolic disease who need long-term venous ac cess. Attention should be given to minimize the infection rate to redu ce the rate of catheter removal. Copyright (C) 1998 by W.B. Saunders C ompany.