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

BACKGROUND: Adult 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.

METHODS: We 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.

RESULTS: A 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.

CONCLUSION: Bilateral 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.