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An unusual case of uninterrupted inferior vena cava with accessory hemizygous channel: An incidental finding in a child.

ABSTRACT: Azygos or hemizygous continuation of inferior vena cava (IVC) is diagnosed in the presence of intrahepatic interruption of IVC. We report a case of a 4-year-old, male child presenting with a history of poor weight gain. A detailed evaluation of the child revealed a diagnosis of hemizygous continuation of uninterrupted, but severely obstructed, IVC. This incidental finding has rarely been reported in the literature.

SUBMITTER: Agrawal G

PROVIDER: S-EPMC7879915 | biostudies-literature | 2020 Oct-Dec

REPOSITORIES: biostudies-literature

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An unusual case of uninterrupted inferior vena cava with accessory hemizygous channel: An incidental finding in a child.

Agrawal Gaurav G Das Anupam A Gupta Gaurav G

Annals of cardiac anaesthesia 20201001 4

Azygos or hemizygous continuation of inferior vena cava (IVC) is diagnosed in the presence of intrahepatic interruption of IVC. We report a case of a 4-year-old, male child presenting with a history of poor weight gain. A detailed evaluation of the child revealed a diagnosis of hemizygous continuation of uninterrupted, but severely obstructed, IVC. This incidental finding has rarely been reported in the literature. ...[more]

PMID: 33109815

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Project description:Introduction Kidney and liver cysts in autosomal dominant polycystic kidney disease (ADPKD) can compress the inferior vena cava (IVC), but IVC compression prevalence and its risk factors are unknown. Methods Patients who have ADPKD (n = 216) with abdominal magnetic resonance imaging (MRI) studies and age-/sex-matched controls (n = 216) were evaluated for IVC compression as well as azygous vein diameter (a marker of collateral blood flow) and IVC aspect ratio (left-to-right dimension divided by anterior-to-posterior dimension with a value of 1 corresponding to a circular (high pressure) IVC caudal to compression. Results Severe IVC compression (?70%) was observed in 33 (15%) ADPKD subjects and mild compression (?50% to <70%) was observed in 33 (15%) subjects; whereas controls had no IVC compression (P < 0.001). Severe IVC compression was associated with larger azygous vein (4.0 ± 1.3 mm versus 2.3 ± 0.8 mm without IVC compression; P < 0.001) and a more circular IVC cross-section upstream (mean IVC aspect ratio: 1.16 ± 0.27 vs. 1.69 ± 0.67, P < 0.001), suggesting higher pressure upstream from the compression. IVC compression was associated with older age, lower estimated glomerular filtration rate (eGFR), greater height-adjusted total kidney volumes, greater height-adjusted liver volume (ht-LV), and greater liver and renal cyst fractions (P < 0.001). No subject younger than 30 years had IVC compression, but ADPKD subjects ?40 years old had 12-fold higher risk of IVC compression (95% confidence interval [CI]: 4.2–42.4), with highest predicted probability for Mayo Clinic classes 1D (59%; 95% CI: 39%–76%) and 1E (74%; 95% CI: 49%–90%) after adjustment (P < 0.001). Women with ht-LV ? 2000 ml/m had 83% (95% CI: 59%–95%) prevalence of IVC compression. Complications of IVC compression included deep vein thrombosis (DVT) and symptomatic hypotension. Conclusions IVC compression is common in ADPKD patients >40 years old, with Mayo Clinic class 1D/E, and in females with ht-LV > 2000 ml/m. Graphical abstract

Dataset Information

An unusual case of uninterrupted inferior vena cava with accessory hemizygous channel: An incidental finding in a child.

Publications

An unusual case of uninterrupted inferior vena cava with accessory hemizygous channel: An incidental finding in a child.

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