背景:成人特发性门罗孔闭塞(AIOFM)是导致脑积水的罕见疾病,双边演讲更罕见。在这里,我们报告了成年患者Monro孔特发性双侧狭窄的病例,并对当前的治疗方案和结果进行了系统的文献综述。
方法:我们对SCOPUS进行了系统评价,科学直接,和PubMed数据库符合PRISMA指南。人口统计数据,临床表现,影像学发现,AIOFM的类型,治疗,并收集结果。
结果:文献中共发现22例双侧AIOFM,包括我们的.中位年龄为38.5岁(范围:20-53),没有性偏好。最常见的症状是头痛(n=16,73%)和呕吐(n=10,45%)。1型AIOFM(狭窄)9例,2型(膜闭塞)13例。大多数患者接受了手术治疗,主要是内窥镜单侧椎间孔成形术和鼻中隔造口术(59%),其次是脑室-腹腔分流术(31%)。一名患者接受了医疗管理,只是为了减轻她的症状(癫痫发作)。在中位随访6个月时,大多数患者的总体结果良好。
结论:双侧AIOFM是一种很容易被错过的罕见疾病,所以神经外科医生应该认识到这种疾病。AIOFM类型的识别可以指导手术决策。治疗选择包括神经内镜手术,如鼻中隔造口术和椎间孔成形术,和脑室-腹腔分流术插入.
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.
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.
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.
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.