Cavernous Malformations of the Optic Nerve and Optic Pathway: A Case Series and Systematic Review of the Literature

Abstract

BACKGROUND Although rare, cavernous malformations (CMs) of the optic nerve and anterior optic pathway (optic pathway cavernous malformations [OPCMs]) can occur, as described in several single case reports in the literature. OBJECTIVE To describe the technical aspects of microsurgical management of CMs of the optic pathway on the basis of an extensive single-center experience and review of the literature. METHODS A systematic literature review was performed to augment an earlier review, using PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines. In addition, an institutional database was searched for all patients undergoing surgical resection of OPCMs. Patient information, surgical technique, and clinical and radiographic outcomes were assessed. RESULTS Since the previous report, 14 CMs were resected at this institution or by the senior author at another institution. In addition, 34 cases were identified in the literature since the systematic review in 2015, including some earlier cases that were not discussed in the previous report. Most OPCMs were resected via pterional, orbital-pterional, and orbitozygomatic craniotomies. Visual outcomes were similar to those in earlier reports, with 70% of patients reporting stable to normal vision postoperatively. CONCLUSION OPCMs can occur throughout the anterior visual pathway and may cause significant symptoms. Surgery is feasible and should be considered for OPCMs presenting to a surface of the nerve. Favorable results can be obtained with resection, although optimal results are obtained with patients who present with milder symptoms without longstanding damage to the optic apparatus.

Acute management of ruptured cavernous malformation of the optic nerve: illustrative case

“…Observations Intracranial cavernous malformations occur in 0.4% to 0.8% of the population, and CMONs are estimated to make up less than 1% of all central nervous system cavernous malformations. [1][2][3][4] Patients with CMON often present with sudden-onset visual disturbances termed "chiasmal apoplexy" due to extralesional hemorrhage, as in our case. 1,[5][6][7][8][9][10] Some patients present less acutely with progressive visual disturbances, likely secondary to progressive expansion caused by intralesional hemorrhage.…”

Optochiasmatic cavernoma: Surgical treatment and outcomes

“…Cavernomas of the central nervous system are benign low-flow vascular malformations that are encountered in 0.2-0.5% of the population with a small female predominance [ 5 , 13 , 20 ]. They comprise 10-20% of vascular malformations of the central nervous system [ 6 , 7 , 9 ].…”

“…Reports on this pathology are limited. 118 cases have been reported since 1958 [ 20 ]. It is a very rare cause of visual symptoms and findings caused by optic nerve dysfunction.…”

“…It is a very rare cause of visual symptoms and findings caused by optic nerve dysfunction. Abrupt visual deterioration is a common symptom of the disease which is sometimes predisposed by factors like alcohol consumption, pregnancy, labour etc [ 5 , 13 , 20 ]. Visual deterioration can be either due to enlargement or abrupt bleeding of cavernoma.…”

“…118 cases have been reported since 1958. 20) It is a very rare cause of visual symptoms and findings caused by optic nerve dysfunction. Abrupt visual deterioration is a common symptom of the disease which is sometimes predisposed by factors like alcohol consumption, pregnancy, labour etc.…”