Background: The ventriculoperitoneal (VPS) shunt is the most common procedure in the treatment of hydrocephalus in children. Abdominal cerebrospinal fluid pseudocysts are a rare complication of the ventriculoperitoneal shunt with an incidence ranging from less than 1% to 10% and are more prevalent in children. The malfunction of the ventriculoperitoneal shunt can cause headaches, nausea, vomiting, altered level of consciousness and abdominal pain due to the accumulation of cerebrospinal fluid. There is no consensus on which type of treatment is better in this case, but there are several available methods. Aim: To report an unusual case of a giant abdominal cerebrospinal fluid pseudocyst as a complication of the VPS. Case Presentation: Female 1 y/3 months old patient, less than 7 kg, that has been diagnosed with hydrocephalus prenatally, confirmed postnatally associated with an esophageal atresia and distal tracheoesophageal fistula (AE/FTE, Gross III), was admitted to our service with progressive abdominal distention without obstructive intestinal signs or peritoneal inflammatory signs. The CT scan of the abdomen showed a large liquid collection (estimated volume of 600 ml), centered on the umbilical region, diagnosed as a giant abdominal cerebrospinal fluid (CSF) pseudocyst from the VPS. All of data and information were obtained from her medical records at the infirmary of the Conjunto Hospitalar de Sorocaba (CHS), São Paulo. Conclusion: Abdominal CSF as a cause of these giant pseudocysts should be considered as a diagnostic hypothesis for cases of large abdominal distensions without intestinal involvement in patients with a VPS. This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.
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