A 13-year-old girl born at 32 weeks gestation with congenital hydrocephalus and VP shunt presents with abdominal distension, pain and vomiting. Her shunt was inserted at 12 days of age. This was followed by a shunt infection that was treated by shunt externalization and antibiotic therapy. The shunt was later reinserted back into the peritoneum. At the age of four, the shunt was revised because of a distal malfunction. There were no other abdominal or shunt surgeries.
On examination, the patient was awake and alert, afebrile and neurologically stable with no evidence of raised intracranial pressure. The abdomen was markedly distended with diffuse tenderness and decreased bowel sounds. Ultrasound revealed significant dilatation of the small bowel loops and echogenic peritoneal fluid. CT showed large loculated/multiseptated abdominal cysts pushing the bowels and liver superiorly [Figure 1].
The VP shunt was externalized. The CSF was clear and the APC partially drained 1.8 L of yellowish fluid, and her abdominal symptoms subsided. CSF gram stain did not reveal any organisms and all cultures were negative. The leukocyte and erythrocyte count was 1 and 0, respectively. CSF protein and glucose levels were normal [Table 1] The shunt was converted to a ventricular-pleural (VPl) system and the patient discharged home. Follow-up chest X-ray revealed an increasing right-sided pleural effusion with no evidence of a shunt malfunction. Thoracentesis was performed and cultures were negative for bacteria, including slow growing and rare organisms such as tuberculosis. The VPl shunt was converted to a ventricular atrial (VA) shunt, with no complications. The patient has remained well for over 3.5 years.