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Co-occurrence of a cerebral cavernous malformation and an orbital cavernous hemangioma in a patient with seizures and visual symptoms: Rare crossroads for vascular malformations.

Choudhri O, Feroze AH, Lad EM, Kim JW, Plowey ED, Karamchandani JR, Chang SD - Surg Neurol Int (2014)

Bottom Line: As a result of hemorrhage and mass effect, patients may present with focal neurologic deficits, seizures, and other symptoms necessitating treatment.We provide a brief overview of the two related pathologies in the context of a patient presenting to our care with concomitant lesions, which were both resected in full without complication.Recognition of disparate symptomatologies is important for properly managing these patients.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurosurgery, Stanford University Medical Center, Stanford, California, Canada.

ABSTRACT

Background: Cerebral cavernous malformations (CCMs) are angiographically occult vascular malformations of the central nervous system. As a result of hemorrhage and mass effect, patients may present with focal neurologic deficits, seizures, and other symptoms necessitating treatment. Once symptomatic, most often from hemorrhage, CCMs are treated with microsurgical resection. Orbital cavernous hemangiomas (OCHs) are similar but distinct vascular malformations that present within the orbital cavity. Even though CCMs and OCHs are both marked by dilated endothelial-lined vascular channels, they are infrequently seen in the same patient.

Case description: We provide a brief overview of the two related pathologies in the context of a patient presenting to our care with concomitant lesions, which were both resected in full without complication.

Conclusion: This is the first known report that describes a case of concomitant CCM and OCH and explores the origins of two pathologies that are rarely encountered together in neurosurgical practice. Recognition of disparate symptomatologies is important for properly managing these patients.

No MeSH data available.


Related in: MedlinePlus

MRI of the CCM and OCH pre- and post-resection (at 3-year follow-up). (a) Axial T2 MRI showing left temporal heterogeneous popcorn lesion (arrow) extending into the atrium of the left occipital horn of the lateral ventricle consistent with a CCM. A left orbital lesion consistent with an orbital hemangioma is also visualized (arrowhead). (b) Coronal T2 image showing OCH. (c) Sagittal T1 image showing the CCM and OCH before resection. (d-f) Axial T2, coronal T2, and sagittal T1 MRIs post-resection demonstrating complete resection of the CCM and OCH
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Figure 1: MRI of the CCM and OCH pre- and post-resection (at 3-year follow-up). (a) Axial T2 MRI showing left temporal heterogeneous popcorn lesion (arrow) extending into the atrium of the left occipital horn of the lateral ventricle consistent with a CCM. A left orbital lesion consistent with an orbital hemangioma is also visualized (arrowhead). (b) Coronal T2 image showing OCH. (c) Sagittal T1 image showing the CCM and OCH before resection. (d-f) Axial T2, coronal T2, and sagittal T1 MRIs post-resection demonstrating complete resection of the CCM and OCH

Mentions: A 35-year-old male with a 4-year history of tonic–clonic seizures presented to the emergency room with new-onset headaches, vomiting, and bilateral paresthesias in the hands. Past medical history was significant for congenital glaucoma and a previous left OCH, removed at age 7 via a lateral orbitotomy. His neurologic exam was significant for sluggishly reactive left pupil in addition to mild abducens palsy, esotropia, and mild ptosis of the left eye, consistent with a cranial nerve III and VI palsy. Visual acuity was 20/20 in the right eye and limited to counting fingers with the left eye at baseline. Neuroimaging revealed a 15-mm hemorrhagic mass in the left temporo-occipital region consistent with a CCM and a significant residual left OCH measuring approximately 30 mm [Figure 1a-c]. Given the poor control of seizures despite medication and the epileptogenic potential of the CCM, the patient expressed interest in surgical resection of the left temporo-occipital lesion, but wished to continue with observation of the orbital hemangioma. Benefits and risks of surgical removal of the CCM, with an emphasis on the proximity of the lesion to Meyer's loop and potential for loss of vision, were discussed in full, and the patient consented to the procedure. The mass was successfully resected by left temporal craniotomy without complication. The postoperative visual field deficits were unchanged. Histopathologic evaluation confirmed the diagnosis of CCM. During biannual follow-up examinations, the patient reported resolution of seizures and successful tapering of his seizure medications. Annual MRI demonstrated stable postsurgical changes. However, at the 3-year follow-up, the patient presented with complaints of supraorbital fullness and an associated sensation of increased ocular pressure of the left orbit. Given these new ophthalmic symptoms, he was referred for oculoplastic ophthalmologic evaluation. Interval MRI demonstrated that the left orbital mass had increased in size, consistent with recurrence of the left orbital tumor that was resected 30 years before [Figure 1]. On examination, his visual acuity in the right eye was 20/20 and in the left eye was limited to counting fingers at 4 feet. Both pupils were reactive to light and accommodation, albeit sluggish in the left eye with a relative afferent pupillary defect. Ocular alignment was notable for a sensory exotropia in the left eye. Examinations of extraocular motility, anterior segment, and dilated fundus of the right eye were all within normal limits. There was notable hypoglobus and severe limitation on supraduction of the left eye. Anterior segment examination of the left eye was significant for blepharoptosis, central corneal haze, and a mild posterior subcapsular cataract. The dilated fundus examination of the left eye revealed marked disc pallor consistent with optic nerve atrophy and retinal vessel dilatation. The patient requested surgical removal of the supraorbital tumor due to the progression of ocular abnormalities and tumor growth. Given the anatomic location of the orbital tumor, the decision was made to perform a left lateral orbitotomy for the removal of the presumed recurrent cavernous hemangioma.


Co-occurrence of a cerebral cavernous malformation and an orbital cavernous hemangioma in a patient with seizures and visual symptoms: Rare crossroads for vascular malformations.

Choudhri O, Feroze AH, Lad EM, Kim JW, Plowey ED, Karamchandani JR, Chang SD - Surg Neurol Int (2014)

MRI of the CCM and OCH pre- and post-resection (at 3-year follow-up). (a) Axial T2 MRI showing left temporal heterogeneous popcorn lesion (arrow) extending into the atrium of the left occipital horn of the lateral ventricle consistent with a CCM. A left orbital lesion consistent with an orbital hemangioma is also visualized (arrowhead). (b) Coronal T2 image showing OCH. (c) Sagittal T1 image showing the CCM and OCH before resection. (d-f) Axial T2, coronal T2, and sagittal T1 MRIs post-resection demonstrating complete resection of the CCM and OCH
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4109172&req=5

Figure 1: MRI of the CCM and OCH pre- and post-resection (at 3-year follow-up). (a) Axial T2 MRI showing left temporal heterogeneous popcorn lesion (arrow) extending into the atrium of the left occipital horn of the lateral ventricle consistent with a CCM. A left orbital lesion consistent with an orbital hemangioma is also visualized (arrowhead). (b) Coronal T2 image showing OCH. (c) Sagittal T1 image showing the CCM and OCH before resection. (d-f) Axial T2, coronal T2, and sagittal T1 MRIs post-resection demonstrating complete resection of the CCM and OCH
Mentions: A 35-year-old male with a 4-year history of tonic–clonic seizures presented to the emergency room with new-onset headaches, vomiting, and bilateral paresthesias in the hands. Past medical history was significant for congenital glaucoma and a previous left OCH, removed at age 7 via a lateral orbitotomy. His neurologic exam was significant for sluggishly reactive left pupil in addition to mild abducens palsy, esotropia, and mild ptosis of the left eye, consistent with a cranial nerve III and VI palsy. Visual acuity was 20/20 in the right eye and limited to counting fingers with the left eye at baseline. Neuroimaging revealed a 15-mm hemorrhagic mass in the left temporo-occipital region consistent with a CCM and a significant residual left OCH measuring approximately 30 mm [Figure 1a-c]. Given the poor control of seizures despite medication and the epileptogenic potential of the CCM, the patient expressed interest in surgical resection of the left temporo-occipital lesion, but wished to continue with observation of the orbital hemangioma. Benefits and risks of surgical removal of the CCM, with an emphasis on the proximity of the lesion to Meyer's loop and potential for loss of vision, were discussed in full, and the patient consented to the procedure. The mass was successfully resected by left temporal craniotomy without complication. The postoperative visual field deficits were unchanged. Histopathologic evaluation confirmed the diagnosis of CCM. During biannual follow-up examinations, the patient reported resolution of seizures and successful tapering of his seizure medications. Annual MRI demonstrated stable postsurgical changes. However, at the 3-year follow-up, the patient presented with complaints of supraorbital fullness and an associated sensation of increased ocular pressure of the left orbit. Given these new ophthalmic symptoms, he was referred for oculoplastic ophthalmologic evaluation. Interval MRI demonstrated that the left orbital mass had increased in size, consistent with recurrence of the left orbital tumor that was resected 30 years before [Figure 1]. On examination, his visual acuity in the right eye was 20/20 and in the left eye was limited to counting fingers at 4 feet. Both pupils were reactive to light and accommodation, albeit sluggish in the left eye with a relative afferent pupillary defect. Ocular alignment was notable for a sensory exotropia in the left eye. Examinations of extraocular motility, anterior segment, and dilated fundus of the right eye were all within normal limits. There was notable hypoglobus and severe limitation on supraduction of the left eye. Anterior segment examination of the left eye was significant for blepharoptosis, central corneal haze, and a mild posterior subcapsular cataract. The dilated fundus examination of the left eye revealed marked disc pallor consistent with optic nerve atrophy and retinal vessel dilatation. The patient requested surgical removal of the supraorbital tumor due to the progression of ocular abnormalities and tumor growth. Given the anatomic location of the orbital tumor, the decision was made to perform a left lateral orbitotomy for the removal of the presumed recurrent cavernous hemangioma.

Bottom Line: As a result of hemorrhage and mass effect, patients may present with focal neurologic deficits, seizures, and other symptoms necessitating treatment.We provide a brief overview of the two related pathologies in the context of a patient presenting to our care with concomitant lesions, which were both resected in full without complication.Recognition of disparate symptomatologies is important for properly managing these patients.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurosurgery, Stanford University Medical Center, Stanford, California, Canada.

ABSTRACT

Background: Cerebral cavernous malformations (CCMs) are angiographically occult vascular malformations of the central nervous system. As a result of hemorrhage and mass effect, patients may present with focal neurologic deficits, seizures, and other symptoms necessitating treatment. Once symptomatic, most often from hemorrhage, CCMs are treated with microsurgical resection. Orbital cavernous hemangiomas (OCHs) are similar but distinct vascular malformations that present within the orbital cavity. Even though CCMs and OCHs are both marked by dilated endothelial-lined vascular channels, they are infrequently seen in the same patient.

Case description: We provide a brief overview of the two related pathologies in the context of a patient presenting to our care with concomitant lesions, which were both resected in full without complication.

Conclusion: This is the first known report that describes a case of concomitant CCM and OCH and explores the origins of two pathologies that are rarely encountered together in neurosurgical practice. Recognition of disparate symptomatologies is important for properly managing these patients.

No MeSH data available.


Related in: MedlinePlus