Cavernous venous malformations in and around the central nervous system. Part 1: Dural and extradural

J Neurosurg 140:735–745, 2024

Cavernous-type malformations are venous lesions that occur in multiple locations throughout the body, and when present in the CNS, they have canonically been referred to as cavernomas, cavernous angiomas, and cerebral cavernous malformations. Herein all these lesions are referred to as “cavernous venous malformations” (CavVMs), which is congruent with the current International Society for the Study of Vascular Anomalies classification system.

Even though histologically similar, depending on their location relative to the dura mater, these malformations can have different features. In Part 1 of this review, the authors discuss and review pertinent clinical knowledge with regard to CavVMs as influenced by anatomical location, starting with the dural and extradural malformations. They particularly emphasize dural CavVMs (including those in the cavernous sinus), orbital CavVMs, and spinal CavVMs. The genetic and histopathological features of CavVMs in these locations are reviewed, and commonalities in their presumed mechanisms of pathogenesis support the authors’ conceptualization of a spectrum of a single disease entity. Illustrative cases for each subtype are presented, and the pathophysiological and genetic features linking dural and extradural to intradural CavVMs are examined.

A new classification is proposed to segregate CavVMs based on the location from which they arise, which guides their natural history and treatment.

Transcranial superior orbitotomy for the treatment of intraorbital intraconal tumors

Neurosurg Rev (2012) 35:573–582

In this article, the authors are presenting their experience and the results with the surgical treatment of intraorbital intraconal tumors based on a review of 33 constitutive cases. Our data were evaluated in comparison to other major series, and possible factors that might influence surgical outcome and survival are discussed. Thirty-three patients diagnosed with intraorbital intraconal tumors between 1998 and 2009 were treated by transcranial approach. Of these patients, there were 14 males (42.4 %) and 19 females (57.8 %). The age ranged between 2 and 70 years (mean036±16.6 years). The follow-up period ranged between 2 and 13 years (mean07.3±3.2 years). The most common presenting symptoms were exophthalmus and decreased visual acuity, which was seen in 21 (63.6 %) and 19 patients (57.6 %), respectively. Total resection was achieved in 23 patients (69.7 %) while subtotal resection was done in ten patients (30.3 %). Cavernoma and optic nerve sheath meningioma were the most common histologic variants, which were found in 11 (33.3 %) and 10 (30.3 %) patients, respectively. In the long-term follow-up, 54.5 % of the patients showed total ophthalmologic improvement, 9.1 % showed partial improvement, 21.2 % demonstrated unchanged ophthalmologic status, and 15.2 % showed worse ophthalmologic outcome.

Transcranial approach for the treatment of intraorbital intraconal tumors is an effective approach for the management of these pathologies. The effectiveness is clearly demonstrated by the clinical results and outcomes of these patients’ groups.