Introduction Patients with intestinal failure are living longer as the rates of intestinal failure-associated liver disease (IFALD) and sepsis have declined due to advancements in care. Today one of the leading causes of mortality among these patients is loss of central venous access due to venous thromboembolism (VTE), which can preclude eligibility for transplantation. Children with intestinal failure have VTE rates that far exceed any other pediatric population, including children with cancer, but the reasons behind these high rates of VTE are not understood. In this study, we sought to characterize the incidence of VTE in children with intestinal failure and as well as potential factors associated with VTE.

Methods A single institution retrospective chart review of patients referred to our intestinal rehabilitation multidisciplinary team was performed on all patients from 2022-2024. Demographics, central venous catheter and parenteral history, as well as the number of venous and arterial thromboses were collected at time of intestinal rehabilitation referral to our institution. Univariate analysis comparing groups was performed with Mann Whitney U or Chi square/Fishers exact test with p <0.05 being significant.

Results One hundred and twenty-five patients with intestinal failure were included with a median age of 8 (2, 52) months and 90% having a diagnosis of short bowel syndrome. There was an overall incidence of 0.63 VTEs per 1000 catheter days. Twenty-two percent (28/125) of patients had a history of VTE upon referral to our multidisciplinary intestinal rehabilitation team. There was a total of 49 clots between the 28 patients with 22/35 (63%) of these VTEs being asymptomatic and found incidentally. Among those who experienced a VTE, 13/28 (46%) would experience a second VTE event. On univariate analysis, a higher number of catheter days, an increased number of surgical procedures, and a higher number of central line-associated bloodstream infections (CLABSIs) were significantly associated with VTE. Only 9/23 (39%) patients who had developed a VTE had a thrombophilia evaluation with 3 patients having a diagnosis of thrombophilia. In our cohort, only 3 (2%) of patients experienced an arterial thrombosis.

Conclusion Preserving central venous catheter access is critical for the survival of children who remain dependent on parenteral nutrition. Given the high prevalence of VTEs in this population, the fact that the majority are asymptomatic, and the high rate of secondary thrombosis, routine screening and surveillance is critical. Further studies to identify the role of secondary thromboprophylaxis are needed.

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