Introduction. Cavernoma, also known as cavernous angioma or cavernous malformation (CM), is a vascular malformation characterized by the presence of sinusoid- like capillary vessels containing blood in very sluggish circulation. Cavernomas can develop in several areas of the central nervous system, in the cerebrum, cerebellum or spinal cord, but they have been most frequently reported at subcortical sites on the frontal and temporal lobes. CM involving cranial nerves are rarely reported: optic nerve and chiasm, third nerve, seventh and eight nerve in the internal auditory canal and seventh nerve in the temporale bone are the only locations reported. Chiasmatic cavernomas are rare. They can be asymptomatic lesions or they can cause gradually progressive visual or pituitary disturbances to those patients presenting acutely with a chiasmal apoplexy syndrome. There are no clear histological or radiological differences between optochiasmal CM and those found elsewhere in the CNS. It’s reasonable to assume that these lesions have a similar natural history in terms of hemorrhage rate as those in other location in the CNS. Objects. The aim of the study was to evaluate the clinical, neuroradiological and surgical aspects concerning optochiasmatic cavernomas. We present a case and discuss the diagnostic and therapeutic aspects, together with a review of the literature. Case Description. A 48-year-old female was admitted to our Department with a 6-month-history of progressive decrease of the vision. At admission the neurological examination showed bitemporal hemianopsia, with decreased pupillary reaction to the light in both eyes. The examination of fundus oculi showed a light papilla on both eyes. The patient didn’t present any pituitary disfunction. A Brain MRI was performed showing a 18 mm hypointense lesion on T2 weighted images inside the optic chiasm in the suprasellar region. On T1 weighted images the lesion appeared as an intra-axial lesion intrinsic to the optic chiasm without contrast enhancement after gadolinium administration. The patient underwent cerebral angiography which didn’t reveal any vascular malformation. Results and conclusions. Surgery was performed using a right pterional trans-sylvian approach. Intraoperatively, there was evidence of subacute hemorrhage surrounding the arachnoide of the optic chiasm, which was drained. Lamina terminalis was opened, then the chiasm was opened at the most anterior part in order to permorf a complete resection of the lesion. The postoperative course was uneventful. The patient did not present any additional neurological deficit. Her pituitary function was unchanged. A postoperative ophtalmological examination performed 5 days after surgery revealed a minimal improvement of vision in the left eye without any change in the visual field. Chiasmatic cavernomas are rare. When symptomatic, they are generally treated surgically. These tumours are intrinsic lesions of the nerves and requires neurotomy for excision. During surgery, the gliotic interspace between the vascular malformation and the neural tissue provides a cleavage for dissection which permits total excision, without causing new deficits. A possible operative strategy is the simple biopsy that has been proposed to minimize operative risk. In our opinion a more extensive procedure to decompress the chiasm while obtaining a pathological diagnosis should be possible by the skilled neurosurgeon with appropriate microsurgical experience. The pterional approach is preferred for removal of optochiasmatic cavernous malformations even if other surgical approaches are described in the literature such as orbitozygomatic, subfrontal or eyebrow keyhole approaches. The choice of the approach should be planned in order to perform a procedure with minimal manipulation of the optic apparatus, preventing postoperative visual deficits.

Chiasmatic cavernoma: case report and review of the literature

ALAFACI, Concetta;GRANATA, Francesca;CUTUGNO, MARIANO;CAFFO, Maria;TOMASELLO, Francesco
2013-01-01

Abstract

Introduction. Cavernoma, also known as cavernous angioma or cavernous malformation (CM), is a vascular malformation characterized by the presence of sinusoid- like capillary vessels containing blood in very sluggish circulation. Cavernomas can develop in several areas of the central nervous system, in the cerebrum, cerebellum or spinal cord, but they have been most frequently reported at subcortical sites on the frontal and temporal lobes. CM involving cranial nerves are rarely reported: optic nerve and chiasm, third nerve, seventh and eight nerve in the internal auditory canal and seventh nerve in the temporale bone are the only locations reported. Chiasmatic cavernomas are rare. They can be asymptomatic lesions or they can cause gradually progressive visual or pituitary disturbances to those patients presenting acutely with a chiasmal apoplexy syndrome. There are no clear histological or radiological differences between optochiasmal CM and those found elsewhere in the CNS. It’s reasonable to assume that these lesions have a similar natural history in terms of hemorrhage rate as those in other location in the CNS. Objects. The aim of the study was to evaluate the clinical, neuroradiological and surgical aspects concerning optochiasmatic cavernomas. We present a case and discuss the diagnostic and therapeutic aspects, together with a review of the literature. Case Description. A 48-year-old female was admitted to our Department with a 6-month-history of progressive decrease of the vision. At admission the neurological examination showed bitemporal hemianopsia, with decreased pupillary reaction to the light in both eyes. The examination of fundus oculi showed a light papilla on both eyes. The patient didn’t present any pituitary disfunction. A Brain MRI was performed showing a 18 mm hypointense lesion on T2 weighted images inside the optic chiasm in the suprasellar region. On T1 weighted images the lesion appeared as an intra-axial lesion intrinsic to the optic chiasm without contrast enhancement after gadolinium administration. The patient underwent cerebral angiography which didn’t reveal any vascular malformation. Results and conclusions. Surgery was performed using a right pterional trans-sylvian approach. Intraoperatively, there was evidence of subacute hemorrhage surrounding the arachnoide of the optic chiasm, which was drained. Lamina terminalis was opened, then the chiasm was opened at the most anterior part in order to permorf a complete resection of the lesion. The postoperative course was uneventful. The patient did not present any additional neurological deficit. Her pituitary function was unchanged. A postoperative ophtalmological examination performed 5 days after surgery revealed a minimal improvement of vision in the left eye without any change in the visual field. Chiasmatic cavernomas are rare. When symptomatic, they are generally treated surgically. These tumours are intrinsic lesions of the nerves and requires neurotomy for excision. During surgery, the gliotic interspace between the vascular malformation and the neural tissue provides a cleavage for dissection which permits total excision, without causing new deficits. A possible operative strategy is the simple biopsy that has been proposed to minimize operative risk. In our opinion a more extensive procedure to decompress the chiasm while obtaining a pathological diagnosis should be possible by the skilled neurosurgeon with appropriate microsurgical experience. The pterional approach is preferred for removal of optochiasmatic cavernous malformations even if other surgical approaches are described in the literature such as orbitozygomatic, subfrontal or eyebrow keyhole approaches. The choice of the approach should be planned in order to perform a procedure with minimal manipulation of the optic apparatus, preventing postoperative visual deficits.
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11570/2603372
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