title: Central venous access devices in children with congenital coagulation disorders: complications and long-term outcome.
abstract: Reliable venous access is essential to facilitate the administration of prophylactic factor concentrate or blood products in children with congenital coagulation disorders and immune tolerance therapy (ITT) regimens in those who develop high responding inhibitors. Poor venous access is even more problematic in very young children, the vast majority of whom will require the insertion of central venous access devices (CVADs). Previous studies have suggested that infection rates are low and that there are few long-term complications associated with CVAD usage. We have reviewed 86 CVADs that have been inserted, since 1988, in 58 children with congenital bleeding disorders, aged 6 d to 16.5 years, attending Great Ormond Street Hospital, London, and the National Children's Hospital, Dublin. The devices have remained in situ for 2 weeks to 92 months (median 22.5 months). Early (0-2 weeks) complications of CVAD insertion included nine bleeding episodes, one extravasation of factor concentrate, three allergic reactions to factor concentrate and five catheter infections. Overall, CVAD infection was the commonest problem encountered, with 52 devices (60%) becoming infected. Twenty-seven CVADs (31%) required removal. Infection rates in children without inhibitors (29/68) were 1/20 patient-months or 1. 6 infections/1000 patient-days, but infection rates for those with inhibitors were 1/8.5 patient-months or 4.3/1000 patient-days. Staphylococcus epidermidis was the predominant organism (25/52) isolated. Blockage of CVAD (four) and catheter disconnection (four) were the most frequently occurring non-infectious long-term complications. Skin erosion of the port was also seen in three children, in one child at 20 months, in one at 29 months and in one at 34 months after insertion. This study demonstrates a high CVAD infection rate and highlights the long-term complications of CVAD usage.
authors.affiliation: National Paediatric Centre for Inherited Coagulation Disorders, National Children's Hospital, Tallaght, Dublin 24, Ireland.
authors.forename: C
authors.initials: C
authors.lastname: McMahon
authors.forename: J
authors.initials: J
authors.lastname: Smith
authors.forename: K
authors.initials: K
authors.lastname: Khair
authors.forename: R
authors.initials: R
authors.lastname: Liesner
authors.forename: I M
authors.initials: IM
authors.lastname: Hann
authors.forename: O P
authors.initials: OP
authors.lastname: Smith
mesh_terms: D000293:Adolescent; D001778:Blood Coagulation Disorders / Q000151:congenital / Q000628:therapy*; D002405:Catheterization, Central Venous / Q000009:adverse effects*; D002408:Catheters, Indwelling / Q000009:adverse effects; D002648:Child; D002675:Child, Preschool; D004342:Drug Hypersensitivity / Q000209:etiology; D004868:Equipment Failure; D005260:Female; D006470:Hemorrhage / Q000209:etiology; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007902:Length of Stay; D008297:Male; D012189:Retrospective Studies; D013203:Staphylococcal Infections / Q000209:etiology; D016896:Treatment Outcome