Solving a complex, rare, and challenging scenario in a normal pressure hydrocephalus with recurrent shunt dysfunction and multiple revisions - The opposing role of evolving low-pressure hydrocephalus and idiopathic raised intra-abdominal pressure.

Background: Maintenance of pressure gradient within the intracranial chamber, shunt hardware, and the abdominal cavity plays a significant role in the optimal functioning of the ventriculo peritoneal shunt. We report a rare and a complex scenario in a patient with normal pressure hydrocephalus (NPH) who had recurrent and refractory ventricular peritoneal shunt dysfunction. Following a meticulous analysis, this was attributed to a very rare, and, first to be documented in the literature, a combination of an evolved very low pressure hydrocephalus (VLPH) system and asymptomatic raised intra-abdominal pressure (IAP).