Adult idiopathic bilateral occlusion of foramen of Monro: Systematic review with illustrative case

Abstract

BackgroundAdult idiopathic occlusion of foramen of Monro (AIOFM) is a rare condition that results in hydrocephalus, and bilateral presentation is even rarer. Here we report a case of idiopathic bilateral stenosis of the foramen of Monro in an adult patient and performed a systematic literature review on the current treatment options and outcomes. MethodsWe performed a systematic review of SCOPUS, Science Direct, and PubMed databases in accordance with PRISMA guidelines. Data on demographics, clinical presentation, imaging findings, type of AIOFM, treatment, and outcomes were collected. ResultsA total of 22 cases of bilateral AIOFM were identified in the literature, including ours. The median age was 38.5 years (range: 20–53), with no sex predilection. The most common presenting symptoms were headache (n=16, 73%) and vomiting (n=10, 45%). There were 9 cases of Type 1 AIOFM (stenosis) and 13 cases of Type 2 (membrane occlusion). Majority of patients underwent surgical treatment, mostly endoscopic unilateral foraminoplasty and septostomy (59%), followed by ventriculoperitoneal shunt insertion (31%). One patient underwent medical management only to alleviate her presenting symptoms (seizures). The overall outcome was good for majority of patients at a median follow-up of 6 months. ConclusionBilateral AIOFM is a rare condition that may easily be missed, so neurosurgeons should be cognizant of this disease entity. Identification of the type of AIOFM may guide surgical decision-making. Treatment options include neuroendoscopic procedures such as septostomy and foraminoplasty, and ventriculoperitoneal shunt insertion.