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Objective: The etiology of infantile inferior vena cava (IVC) occlusion remains unclear as it relates to congenital atresia or acute venous thromboembolism (VTE). The natural history of IVC occlusion, including the risk of recurrent VTE and post-thrombotic syndrome, similarly remains poorly defined. This study aims to better elucidate these features to aid with treatment recommendations.
Methods: A single-institution retrospective review of pediatric IVC occlusion was performed between 2000-2015. Patients with superior vena cava interruptions were excluded. The electronic medical record was surveyed and primary endpoints included VTE, hemorrhage and death.
Results: 47 patients were diagnosed with IVC occlusion (mean age 3 years); the majority of which were infants at the time of diagnosis (N=27). In this infantile cohort median age at diagnosis was 28 days and median weight 2.3kg. Specific infantile risk factors included prematurity (33%), sepsis (48%); and ICU admission (96%). 12 infants (44%) were symptomatic and seven (26%) had renal vein thrombosis. No infant was treated with thrombolysis and 63% managed with anticoagulation. One infant suffered non-fatal hemorrhagic stroke during early anticoagulation.
The majority of IVC occlusions were diagnosed with MRI; duplex ultrasound and CT imaging were additionally employed. Additional risk factors across the cohort included hypercoaguable state (15%), malignancy (17%), and family history (17%). The incidence of VTE was 13% (N=6) at a median age of 15 years (range 9-17); five of these six patients had identifiable hypercoaguable state. Three patients underwent successful thrombolysis for extensive acute iliofemoral DVT and two required caval recanalization (stenting) for lower extremity swelling with relief of symptoms. Two patients presented with venous aneurysm (one ruptured) requiring treatment. During a mean follow-up of 3 years (range 0-17 years) there were no late hemorrhagic complications with prolonged anticoagulation, no episodes of recurrent VTE following treatment and one case of post-thrombotic syndrome. Twelve patients (25%) expired from causes unrelated to IVC occlusion.
Conclusions: Conventional VTE risk factors accompany pediatric IVC occlusion. Lower extremity DVT complicates a minority of cases, often during the teenage years. Indefinite anticoagulation for pediatric IVC occlusion is likely unnecessary for those patients that lack identifiable hypercoaguable state.