Extensive Thrombosis of the Inferior Vena Cava and Left Renal Vein in a Neonate

Abstract

Antenatal renal vein thrombosis is a rarely described diagnostic finding, with variable consequences on kidney function. We present the case of an affected fetus, born at 35-week gestation, with intrauterine oligohydramnios and two small kidneys. A renal ultrasound carried out after birth confirmed the presence of prenatal abnormalities. Renal vein thrombosis was not diagnosed at the time. The baby died 20 days later of kidney failure, metabolic acidosis, and polypnea with severe hypotrophy. Autopsy revealed atrophied kidneys and adrenal glands. The vena cava had thrombosis occupying most of its length. The right renal vein was normal, while the left renal vein was threadlike and not permeable. Histologically, there was necrosis of the left adrenal gland with asymmetrical bilateral renal impairment and signs of ischemic and hemorrhagic lesions. A review of thrombophilia was carried out and a heterozygous mutation in Factor V was found in both the mother and the child.

Inferior vena cava syndrome in neonates: An evidence‐based systematic review of the literature

“…Individuals heterozygous for factor V Leiden have an increased thrombotic propensity that escalates several‐fold with homozygosity . In our review, all six cases with factor V Leiden mutation were heterozygous, and it was reported as the sole prothrombotic risk factor . Factor V Leiden either may occur as a heterozygous mutation in the mother and child or may coexist with the prothrombin G20210A mutation .…”

“…Four hundred seventy‐two articles were identified and 33 were included; 28 case reports, 3 case series, and 2 retrospective cohort studies (Supporting Information Table S1), with a combined total of 34 patients from case reports/case series and 27 from cohort studies (Figure ). Selected articles included all patients with radiological evidence of IVCS ( n = 61; 57 of which were clinically symptomatic).…”

“…Overall, there were four (6.5%) cases of hemorrhage, and no recurrences of IVC thrombosis with treatment. Mortality following neonatal IVCS was 23% ( n = 14/61) . The most common cause of death was renal failure ( n = 3/13), one patient of which had extensive thrombosis of the left renal vein .…”

“…Mortality following neonatal IVCS was 23% ( n = 14/61) . The most common cause of death was renal failure ( n = 3/13), one patient of which had extensive thrombosis of the left renal vein . Other causes of mortality were attributed to unsuccessful thrombolytic therapy ( n = 2/13) .…”

“…17,49 In our review, all six cases with factor V Leiden mutation were heterozygous, and it was reported as the sole prothrombotic risk factor. 17,20,22,27,29,39 Factor V Leiden either may occur as a heterozygous mutation in the mother and child 22 or may coexist with the prothrombin G20210A mutation. 20,39 In one case, antithrombin deficiency (activity 37%) was identified as a co-predisposing factor for Initial Literature Search:…”