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.

Giant Cerebellar Cavernomas in Pediatric Patients: Systematic Review with Illustrative Case.

INTRODUCTION: Giant cavernomas (GCMs) are angiographically occult vascular malformations of the central nervous system measuring at least 4 cm in diameter. These are rare lesions, especially in the cerebellum. As such, the clinical and radiologic features, surgical management, and outcomes of treatment for this condition are unknown. METHODS: We performed a systematic review of SCOPUS and PubMed databases for case reports and case series of histopathologically proven GCMs arising from the cerebellum in the pediatric population, and included a case managed at our center. Data on clinical presentation, imaging features, surgical treatment and outcomes of management were collected. RESULTS: A total of 10 cases were identified from the systematic review, including our case report. The patients' median age was 6 months, with a slight male sex predilection (1.5:1). The most common neurologic manifestations were bulging fontanelle in infants, and headache and focal cerebellar deficits in older children. Surgical excision was done in all cases, while 50% of the cases had CSF diversion surgery done prior to excision. In cases with reported outcome, complete neurologic recovery was achieved in seven while partial recovery was reported in one case. No deaths were reported at median follow-up of 12 months. CONCLUSION: Our case report and systematic review show that giant cerebellar cavernoma is a rare differential diagnosis for symptomatic hemorrhagic cerebellar masses in the pediatric age group. Mainstay of treatment is surgical excision, which can be associated with favorable outcome in most cases.