A rare case of inferior vena cava occlusion secondary to abdominal pseudocyst associated with ventriculoperitoneal shunt-case report and review of literature

Background and importance Abdominal pseudocyst (APC) is an uncommon but well-recognised complication of ventriculo-peritoneal (VP) shunt. Diagnosis is based on clinical features of shunt malfunction, including headaches, vomiting and drowsiness, and abdominal swelling. APCs can grow to large sizes resulting in compression of abdominal viscera; however, inferior vena cava (IVC) compression is extremely rare, and only one other case associated with VP shunt had been reported. Clinical presentation We report a case of a 12-year-old girl with a background of open myelomeningocoele repair, kyphoscoliosis and right-sided VP shunt in situ who presented with bilateral lower limb swelling and abdominal distension. She exhibited no features of raised intracranial pressure but had bilateral pitting oedema up to the groin. Abdominal ultrasound and CT scan showed a massive septated pseudocyst (20 x 18 x 8 cm) compressing the IVC. The APC was drained, and the shunt was externalised, with conversion to a ventriculo-atrial (VA) shunt 1 week later after cultures of the cystic fluid, cerebrospinal fluid and shunt tube came back sterile. The patient remained well with no recurrence of the abdominal fluid and no VA shunt complication at 36-month follow-up. Conclusion This patient had specific risk factors for development of a massive APC and the subsequent IVC compression, including prior surgeries, spinal deformity, abnormal abdominal anatomy and poor abdominal muscle tone and sensation. Early recognition of this complication and prompt alleviation of the mass effect of IVC compression can prevent long-term neurological and vascular sequalae.