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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

(a) OCH showing a well-circumscribed border with the orbital adipose tissue and a variably thick fibrous capsule. (b) Highpower view of the lesion showing cavernous, endothelium-lined vascular sinusoids. Extravasated red blood cells and hemosiderin are noted in between the cavernous sinusoids. In contrast to the CCM, a cellular chronic inflammatory response is seen between the vessels. (c) A focus of organizing hemorrhage with a cholesterol granuloma is shown. (d) An area of the hemangioma showing chronic inflammation and fibrosis between the cavernous sinusoids is seen
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Figure 4: (a) OCH showing a well-circumscribed border with the orbital adipose tissue and a variably thick fibrous capsule. (b) Highpower view of the lesion showing cavernous, endothelium-lined vascular sinusoids. Extravasated red blood cells and hemosiderin are noted in between the cavernous sinusoids. In contrast to the CCM, a cellular chronic inflammatory response is seen between the vessels. (c) A focus of organizing hemorrhage with a cholesterol granuloma is shown. (d) An area of the hemangioma showing chronic inflammation and fibrosis between the cavernous sinusoids is seen

Mentions: CCMs are low-flow, angiographically occult lesions that often present with seizures, headaches, and focal neurological deficits depending on the location of the malformation. Such lesions can typically be removed microsurgically with a high success rate. The CCM location determines its clinical presentation. Brainstem cavernous malformations can present with cranial nerve palsies and motor or sensory weakness. Similarly, temporal lobe cavernous malformations might present with seizures, as seen in our patient. Kwon et al. recently demonstrated a high postoperative long-term seizure-free rate of 82% in patients undergoing resection of cavernous malformations for seizures.[31] In this report, the patient underwent a left temporal approach for resection of the lesion, which placed him at risk of injury to Meyer's loop fibers of the optic radiation in the superior and middle temporal gyri. This is an important consideration with a known large OCH, which can also cause visual field compromise to the patient from optic nerve compression.[45] CCMs have been reported to occur along the optic pathway, such as the optic nerve, optic chiasm, and optic tract, where they can cause visual field disturbances from mass effect.[26] These visual field disturbances should be clinically differentiated from visual compromise caused by OCHs. Several genetic causes of CCMs have recently been identified, with mutations in KRIT1 (CCM1), CCM2, PDC10 (CCM3), and RASA1. Several of these genes are presumed to play roles in endothelial cell interactions with the extracellular matrix, as well as to impact endothelial permeability and proliferation.[1063] These syndromes typically show an autosomal dominant inheritance pattern with incomplete penetrance. The genetics behind OCHs, although not fully characterized, suggests that abnormalities of chromosome 13 may be more common in patients with OCH.[53] Potential overlapping genetic loci of OCHs with CCMs need further study and may suggest a common cytogenetic origin given their similarities in histopathology. In terms of histology, these entities show considerable overlap [Figures 3 and 4]. OCHs and CCMs are both marked by dilated vascular spaces lined by endothelium, but have distinct native tissue environments. Both lesions are non-arterial and can show evidence of thrombosis and chronic hemorrhage that is typically marked by the presence of hemosiderin-laden macrophages. Unlike CCMs, OCHs are encapsulated, and though they may have adhesions to the surrounding epimysium of the intraocular muscles, they are non-infiltrative. OCHs are purplish, ovoid lesions with a fibrous pseudocapsule. The abundance of fibrous connective tissue in the surrounding retrobulbar intraconal tissue space allows these lesions to have a clear plane around them, making the resection easier.[21] In contrast, CCMs are soft and mulberry-like with a lack of fibrous trabeculae, causing hemorrhage to extend into the surrounding tissue. A hemosiderin stain in the surrounding brain tissue is often the only margin available to the surgeon when resecting such pathologies. In some scenarios where CCMs are close to the cisternal or ventricular surface, hemorrhage may extend leading to subarachnoid hemorrhage and intraventricular hemorrhage, respectively.[16] Hejazi et al. studied the histopathology and radiology characteristics of 19 patients with OCH and compared them with 107 CCMs operated on within the same period.[25] They identified several differences between the two types of cavernous malformations, as highlighted in Table 1, suggesting that these lesions could represent two distinctive lines of differentiation of cavernous lesions. CCMs typically are associated with thin, irregular vascular walls, while rounded, fibrous walls are distinctive of OCHs, suggesting that surrounding tissue plays an important role in the angioarchitecture of these lesions and their presentation. The fibrous capsule of an OCH could represent the orbital fatty tissue reaction to the presence of the hemangioma.[24] Given a different environment in the brain, no such reaction is seen in CCMs; rather, they seem to be associated with developmental venous anomalies (DVAs). DVAs are noted on MRI in up to 25% of patients with CCMs and are hypothesized to be the source of hemorrhagic angiogenic proliferation that predisposes to CCM formation.[46]


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)

(a) OCH showing a well-circumscribed border with the orbital adipose tissue and a variably thick fibrous capsule. (b) Highpower view of the lesion showing cavernous, endothelium-lined vascular sinusoids. Extravasated red blood cells and hemosiderin are noted in between the cavernous sinusoids. In contrast to the CCM, a cellular chronic inflammatory response is seen between the vessels. (c) A focus of organizing hemorrhage with a cholesterol granuloma is shown. (d) An area of the hemangioma showing chronic inflammation and fibrosis between the cavernous sinusoids is seen
© Copyright Policy - open-access
Related In: Results  -  Collection

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getmorefigures.php?uid=PMC4109172&req=5

Figure 4: (a) OCH showing a well-circumscribed border with the orbital adipose tissue and a variably thick fibrous capsule. (b) Highpower view of the lesion showing cavernous, endothelium-lined vascular sinusoids. Extravasated red blood cells and hemosiderin are noted in between the cavernous sinusoids. In contrast to the CCM, a cellular chronic inflammatory response is seen between the vessels. (c) A focus of organizing hemorrhage with a cholesterol granuloma is shown. (d) An area of the hemangioma showing chronic inflammation and fibrosis between the cavernous sinusoids is seen
Mentions: CCMs are low-flow, angiographically occult lesions that often present with seizures, headaches, and focal neurological deficits depending on the location of the malformation. Such lesions can typically be removed microsurgically with a high success rate. The CCM location determines its clinical presentation. Brainstem cavernous malformations can present with cranial nerve palsies and motor or sensory weakness. Similarly, temporal lobe cavernous malformations might present with seizures, as seen in our patient. Kwon et al. recently demonstrated a high postoperative long-term seizure-free rate of 82% in patients undergoing resection of cavernous malformations for seizures.[31] In this report, the patient underwent a left temporal approach for resection of the lesion, which placed him at risk of injury to Meyer's loop fibers of the optic radiation in the superior and middle temporal gyri. This is an important consideration with a known large OCH, which can also cause visual field compromise to the patient from optic nerve compression.[45] CCMs have been reported to occur along the optic pathway, such as the optic nerve, optic chiasm, and optic tract, where they can cause visual field disturbances from mass effect.[26] These visual field disturbances should be clinically differentiated from visual compromise caused by OCHs. Several genetic causes of CCMs have recently been identified, with mutations in KRIT1 (CCM1), CCM2, PDC10 (CCM3), and RASA1. Several of these genes are presumed to play roles in endothelial cell interactions with the extracellular matrix, as well as to impact endothelial permeability and proliferation.[1063] These syndromes typically show an autosomal dominant inheritance pattern with incomplete penetrance. The genetics behind OCHs, although not fully characterized, suggests that abnormalities of chromosome 13 may be more common in patients with OCH.[53] Potential overlapping genetic loci of OCHs with CCMs need further study and may suggest a common cytogenetic origin given their similarities in histopathology. In terms of histology, these entities show considerable overlap [Figures 3 and 4]. OCHs and CCMs are both marked by dilated vascular spaces lined by endothelium, but have distinct native tissue environments. Both lesions are non-arterial and can show evidence of thrombosis and chronic hemorrhage that is typically marked by the presence of hemosiderin-laden macrophages. Unlike CCMs, OCHs are encapsulated, and though they may have adhesions to the surrounding epimysium of the intraocular muscles, they are non-infiltrative. OCHs are purplish, ovoid lesions with a fibrous pseudocapsule. The abundance of fibrous connective tissue in the surrounding retrobulbar intraconal tissue space allows these lesions to have a clear plane around them, making the resection easier.[21] In contrast, CCMs are soft and mulberry-like with a lack of fibrous trabeculae, causing hemorrhage to extend into the surrounding tissue. A hemosiderin stain in the surrounding brain tissue is often the only margin available to the surgeon when resecting such pathologies. In some scenarios where CCMs are close to the cisternal or ventricular surface, hemorrhage may extend leading to subarachnoid hemorrhage and intraventricular hemorrhage, respectively.[16] Hejazi et al. studied the histopathology and radiology characteristics of 19 patients with OCH and compared them with 107 CCMs operated on within the same period.[25] They identified several differences between the two types of cavernous malformations, as highlighted in Table 1, suggesting that these lesions could represent two distinctive lines of differentiation of cavernous lesions. CCMs typically are associated with thin, irregular vascular walls, while rounded, fibrous walls are distinctive of OCHs, suggesting that surrounding tissue plays an important role in the angioarchitecture of these lesions and their presentation. The fibrous capsule of an OCH could represent the orbital fatty tissue reaction to the presence of the hemangioma.[24] Given a different environment in the brain, no such reaction is seen in CCMs; rather, they seem to be associated with developmental venous anomalies (DVAs). DVAs are noted on MRI in up to 25% of patients with CCMs and are hypothesized to be the source of hemorrhagic angiogenic proliferation that predisposes to CCM formation.[46]

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