Catheter-related thrombosis in children with hemophilia A: evidence of a multifactorial disease

Ettingshausen, Carmen Escuriola; Kurnik, Karin; Schobeß, R.; Kreuz, W.; Halimeh, Susan; Pollman, Hartmut; Nowak‐Göttl, Ulrike

doi:10.1182/blood.v99.4.1499

Cited by 33 publications

(32 citation statements)

References 15 publications

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“…Forty‐eight studies reported between 1982 and 2003 with 2704 patients receiving a total of 2973 CVADs were included in the meta‐analysis (Table 1) [2–4,6,7,9–22,27–55]. None of the studies was unpublished.…”

Section: Resultsmentioning

confidence: 99%

Central venous access devices in haemophilia

Valentino

Ewenstein

Navickis³

et al. 2004

Haemophilia

138

182

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Central venous access devices (CVADs) can facilitate repeated and/or urgent administration of coagulation factors in haemophilic patients. We conducted a systematic review and meta-analysis of complication rates and risk factors for poor outcome. Forty-eight studies with a total of 2704 patients and 2973 CVADs were included. The primary indications for CVADs were immune tolerance therapy (34.9% of patients), difficult venous access (31.8%) and prophylaxis (29.1%). Fully implanted CVADs were employed in 77.4% of cases and external CVADs in 22.6%. A total of 1190 infections were reported, and the pooled incidence of infection was 0.66 per 1000 CVAD days [confidence interval (CI), 0.44-0.97 per 1000 CVAD days]. Among patients developing infection, the pooled time to first infection was 295 days (CI, 181-479 days). Presence of inhibitors was an independent risk factor for infection with an incidence rate ratio (IRR) of 1.67 (CI, 1.15-2.43). Infection was less likely in patients >6 years of age (IRR, 0.46; CI, 0.27-0.79) and recipients of fully implanted CVADs (IRR, 0.31; CI, 0.12-0.86). Available information on thrombosis was limited, with only 55 cases being reported. Eventually, 31.3% of CVADs were removed, and infection was the reason for removal in 69.9% of cases and thrombosis in 4.1%. The pooled time period CVADs remained indwelling prior to removal or the expiration of the study observation period was 578 days per CVAD (CI, 456-733 days per CVAD). CVADs can confer major benefits in patients with haemophilia requiring long-term venous access, and serious complications are rare.

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Section: Resultsmentioning

confidence: 99%

Central venous access devices in haemophilia

Valentino

Ewenstein

Navickis³

et al. 2004

Haemophilia

138

182

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“…Most of these studies were retrospective and venography was usually not routinely performed. This has been done in a few studies which showed an unexpectedly high frequency of thrombosis [61,62]. The series by Price et al revealed radiological signs of thrombosis in 13/16 (81%) children although the clinical relevance of these findings was not clear in all cases [61].…”

Section: Central Venous Lines -Why Such Diverse Experiences?mentioning

confidence: 99%

Haemophilia in the first years of life

et al. 2008

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Summary. Surgery in infants and young children with haemophilia, when preceded by accurate diagnosis and accompanied by safe and effective factor prophylaxis, is not associated with a significant risk of haemorrhage. Haemophilic newborns undergoing circumcision or major surgery prior to diagnosis and in the absence of appropriate haemostatic prophylaxis remain as a concern. Inhibitor development has replaced haemorrhage as the major surgical complication in the developed world, largely because of the intensity of treatment used to secure haemostasis. For that reason only, essential surgery should be performed. Intracranial haemorrhage (ICH) during the neonatal period affects 3.5-4.0% of all haemophilia boys in countries with a good standard of health care, which is considerably (40-80 times) higher than expected in the normal population. Because of the high frequency of sporadic cases, ICH in the neonatal period can only be partially prevented by improved carrier diagnosis and counselling. Infections and thrombosis are the major serious complications of central venous lines. Large differences are seen in the frequency of these complications, the most plausible explanations are probably related to the protocol used for device care, the quality of education and the compliance of the users, an issue addressed in an on-going study.

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“…Koerper et al [8] and Ettingshausen et al [9] have reported their experiences of catheter‐related thrombosis in subjects with haemophilia. When data from these two reports are pooled with the studies described earlier, a frequency of 46% (range 22–81%) is obtained for CVAD‐associated thrombosis in persons with haemophilia (Table 3).…”

Section: Central Venous Access Device‐associated Deep Vein Thrombosismentioning

confidence: 99%

Overview of the use of implantable venous access devices in the management of children with inherited bleeding disorders

Komvilaisak¹,

Connolly

Naqvi³

et al. 2006

Haemophilia

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Frequent infusion of factor concentrates may be challenging in young boys with haemophilia, especially if their disease is complicated by inhibitors. A central venous access device (CVAD) is often placed in young patients in need of repeated infusions for prophylaxis or immune tolerance induction. Although user friendly and capable of providing reliable venous access, these devices are associated with a high complication rate over time. In the haemophilia population, major complications include CVAD-associated infections and deep venous thrombosis, which is most often silent. Established risk factors for catheter-related infection include age less than 6 years at the time of CVAD placement and use of an external CVAD when compared with a totally implantable device such as a port. Avoidance of CVAD-related infections is facilitated by strict adherence to aseptic technique. The risk of deep venous thrombosis appears related to the duration for which the catheter is in place, with the risk increasing beyond 4 years. The promotion of a strict clinic policy in which CVADs are left in place for as short a time as possible should decrease the risk of complications. In rare cases where a totally implantable CVAD cannot be placed for technical reasons, an arteriovenous fistula may provide reliable venous access. In all cases, however, venous access via peripheral veins is preferred over CVADs.

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Catheter-related thrombosis in children with hemophilia A: evidence of a multifactorial disease

Cited by 33 publications

References 15 publications

Central venous access devices in haemophilia

Central venous access devices in haemophilia

Haemophilia in the first years of life

Overview of the use of implantable venous access devices in the management of children with inherited bleeding disorders

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