Twenty-five central venous lines (two external, 23 subcutaneous ports)
were placed in 19 boys with haemophilia A (n=17) or B (n=2). The mean
age of the boys was 4.9 years (range 0.2-15.3 years). The haemophilia
was severe (factor level <1%) in 18 boys and moderate (factor level 3
%) in one. Three boys had circulating inhibitors and three were positi
ve for human immunodeficiency virus (HIV)-1 antibody. Central venous l
ines were placed to facilitate intermittent factor replacement therapy
(n=6), long-term factor prophylaxis (n=9), induction of an immune tol
erance protocol (n=2) or therapy for acquired immunodeficiency syndrom
e (AIDS)-related complications (n=2). The ports remained in place for
15 795 days (mean 687 days, range 11-2059 days). The frequency of port
-related sepsis xas 48% (11/23 ports in eight boys) or 0.7 port infect
ions per 1000 patient days. Ports were removed from five boys with an
unresolved infection (four with Staphylococcus aureus sepsis and one w
ith Pseudomonas sp. sepsis). Other complications requiring port remova
l included a catheter tip placed too high in the venous system (n=1),
severe persistent pain associated with needle access of the port (n=1)
and a subclavian vein thrombosis (n=1). Both the benefits and risks o
f a subcutaneous port should be considered when deciding whether to pl
ace this device in a very young child with haemophilia.