Meningioma En Plaque associated with Cerebrospinal Fluid Rhinorrhea: A Systematic Review of Literature with Case Presentation.

Abstract

Background: Meningiomas are the most frequently diagnosed benign intracranial tumors. However, meningioma en plaque (MEP) is a rare subset accounting for 2.5% of all meningiomas and is characterized by flat, carpet-like proliferation along the dura, typically arising in the spheno-orbital region; therefore, causes proptosis, decreased visual acuity, and orbital pain. We present a unique case of MEP presenting with cerebrospinal fluid (CSF) rhinorrhea and conduct a systematic review of literature.

Methods: Following PRISMA guidelines, a systematic search was conducted in PubMed and Embase databases. Keywords and standardized index terms related to MEP were used. The search was performed without restriction on the publication date. Screening, data extraction, and quality assessment were carried out. Data on demographics, clinical presentations, management modalities, and treatment outcomes were analyzed.

Results: The search yielded 487 titles, with 36 studies eligible for inclusion. A total of 530 patients with MEP were reported, with a mean age of 50.1±11.62 years. Proptosis was the most common symptom (95%), followed by visual impairment (57.3%), orbital pain (38.3%), ophthalmoplegia (28.6%), and headache (23%). Our patient represented the only case with a spontaneous CSF leak. Surgical resection was performed in 85%, adjuvant radiotherapy in 15.7%, and one patient received primary radiotherapy, while 8 patients were closely followed up with no intervention.

Conclusion: MEP associated with spontaneous CSF rhinorrhea is extremely rare and poses diagnostic and therapeutic challenges. Conservative management for select cases of MEP can be a good choice, refraining the patient from surgical complications, especially for difficult-access skull base areas.