Implantable central venous access device procedures in haemophilia patients without an inhibitor: systematic review of the literature and institutional experience

Abstract

Elective surgical procedures involving central venous access devices (CVADs) in patients with haemophilia are often necessary for adequate factor delivery but there are few data regarding haemostatic coverage and acute complication rates accompanying these procedures. To describe experience with CVAD insertion, revision and removal in young haemophilia patients at our institution and in the literature and to assess acute complications following CVAD procedures. PubMed, Medline and Cochrane databases were searched for articles, which included a description of factor coverage during CVAD procedures. A retrospective review of our comprehensive haemophilia database identified patients undergoing CVAD placement, revision and removal between January 1993 and August 2005. Manual and electronic searches of the published literature yielded 14 articles, which met inclusion criteria. Peri-operative factor administration varied greatly among the reports. Mean acute infection and haematoma rates were 8% and 12.5% respectively. A retrospective review identified 49 CVAD placements, revisions, or removals meeting inclusion criteria. Most patients received outpatient bolus factor replacement to achieve a level of 100% preoperatively, immediately postoperatively and on postoperative days 1, 2, 3, 5 and 7. Thirty-six procedures were performed without hospitalization. Ten patients developed 11 (22%) minor haematomas postoperatively. Major haemorrhage, acute infection, or pneumothorax was not encountered. Few published data exist regarding haemostatic coverage and complications following CVAD procedures. Our institutional experience using a consistent management approach was favourable. Further studies are required to define optimal haemostatic coverage during minor surgical procedures in haemophilia.

Enhanced pharmacokinetic properties of a glycoPEGylated recombinant factor IX: a first human dose trial in patients with hemophilia B

“…8,11,12 The need for frequent dosing, especially in pediatric patients, presents challenges in terms of repeated venous access that may necessitate the placement of indwelling catheters that is associated with frequent complications such as thromboses and infections. 13,14 Furthermore, frequent dosing interrupts daily activities and therefore, lack of compliance with a frequent injection regimen is one of the most commonly cited reasons for failure of prophylaxis. 15 Similar to patients with mild or moderate hemophilia B, patients with severe hemophilia B would also benefit from a single effective dose, instead of multiple doses, when they experience breakthrough bleeding episodes during prophylaxis.…”

Central venous access device insertion and perioperative management of patients with severe haemophilia A

“…Dosage is then gradually decreased to CVAD insertion in children with severe haemophilia A Fonseca et al 157 levels of 0.3-0.75 IU/ml for the following 1-5 days [3]. This is replicated in the results found at McMaster Children's Hospital.…”

“…The few reports in literature regarding perioperative replacement of FVIII for CVADs involves a preoperative circulating factor level of 1.0 IU/ml and postoperative administration to maintain this level at 0.5-1.0 IU/ml for 2-3 days [3]. Dosage is then gradually decreased to CVAD insertion in children with severe haemophilia A Fonseca et al 157 levels of 0.3-0.75 IU/ml for the following 1-5 days [3].…”

“…No postoperative complications were reported. Neunert et al [3] reported their institution's results, having used lesser dosages around CVAD surgeries and performed most of these surgeries in an outpatient setting. Patients from this institution received 50 IU/kg immediately before and several hours after surgery.…”

“…Patients from this institution received 50 IU/kg immediately before and several hours after surgery. This dosage was then continued once daily for the next 5 days [3].…”