Agenesis of Retrohepatic Inferior Vena Cava with Congenital Cavo-Portal Shunt

Abstract

Background Congenital anomalies of the
inferior vena cava are extremely rare
with agenesis of retrohepatic segment of
the inferior vena cava (RHIVC) with azygos
continuation being a relatively wellknown
congenital anomaly. We present
the rare case of absence of the RHIVC,
with venous return entirely through the
liver parenchyma by way of a cavo-portal
shunt. This to our knowledge is only the
second such case reported in English
medical literature. Case report A eleven
year old girl with normal mile stones, presented
with abdominal pain of four days
duration. She gave no previous history of
medical problems. She was evaluated
and her abdominal examination and
blood investigations were unremarkable.
An Ultrasonogram revealed a cavo-portal
shunt, with absent retrohepatic segment
of IVC. CT angiogram was obtained
which confirmed the findings. As she was
asymptomatic, she was managed conservatively
and continues to be asymptomatic
on routine followup.Conclusion We
highlight an extremely
rare case of an absent segment of IVC with
congenital cavo-portal shunt, wherein the
patient was well compensated and was
managed by masterly inactivity