Breaking the Deadlock: Pathophysiological Insights into Spontaneous Shunt Knotting and Volvulus—A Case Report and Literature Review

A bstract Ventriculoperitoneal shunt surgery is the primary treatment for hydrocephalus; however, while common complications include infection and dysfunction, shunt-related acute abdomen syndrome due to catheter knotting is exceedingly rare, with fewer than 30 cases reported in the literature. We present a 9-year-old male with congenital hydrocephalus and a history of neonatal surgery for esophageal atresia and Nissen fundoplication who, despite having a stable neurological course for 8 years, suffered from multiple hospitalizations due to nonspecific gastrointestinal symptoms. He eventually presented with acute nausea, vomiting, and abdominal distension, where radiography revealed distal shunt knotting and acute ileus. Emergency laparotomy confirmed a small bowel volvulus caused by the knotted catheter, and following externalization, the patient was successfully treated with a ventriculoatrial shunt. Based on our clinical observations and literature review, we identified that knotting occurs at intestinal “corner points” where chronic peristaltic movements force redundant catheter lengths to rotate and eventually self-entangle. To overcome the limitations of 2D imaging and improve diagnostic accuracy, we emphasize the potential of developing three-dimensional computed tomography reconstruction techniques, similar to those used in aneurysm surgery, to better visualize the spatial orientation of the catheter. We conclude that managing distal catheter length and maintaining a high clinical suspicion regarding the “neurologically silent phase” are the cornerstones of preventing such life-threatening complications, particularly in patients with complex abdominal histories.