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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, b) CCM comprised cavernous, endothelium-lined vascular sinusoids with foci of calcification (a) and ossification. (b) Little intervening brain tissue between the cavernous vessels was noted. (c) High-power view of the vascular walls of the CCM demonstrates delicate mural hyalinization, scattered extravasated erythrocytes and hemosiderin, and scant inflammation. (d) Brain parenchyma at the periphery of the lesion showing typical hemosiderin deposits, macrophages, axonal spheroids, and gliosis
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Figure 3: (a, b) CCM comprised cavernous, endothelium-lined vascular sinusoids with foci of calcification (a) and ossification. (b) Little intervening brain tissue between the cavernous vessels was noted. (c) High-power view of the vascular walls of the CCM demonstrates delicate mural hyalinization, scattered extravasated erythrocytes and hemosiderin, and scant inflammation. (d) Brain parenchyma at the periphery of the lesion showing typical hemosiderin deposits, macrophages, axonal spheroids, and gliosis

Mentions: Subsequently, the neurosurgical team bluntly dissected around the lesion, which appeared to be most consistent with a recurrent cavernous hemangioma. The feeding arteries to the lesion were cauterized and cut sharply. The lesion was biopsied and submitted to pathology for frozen and permanent section. The remainder of the lesion was then removed en bloc as two specimens. The superior aspect was dissected first followed by the medial and lateral aspects, while the inferior and deep aspects were the final portions to be removed. There were no subsequent post-procedural complications, with visual acuity remaining unchanged from preoperative status. Histopathologic review confirmed the working diagnosis of recurrent OCH [Figure 3]. At his 2-week follow-up, the patient reported complete resolution of supraorbital discomfort. However, he continued to note ptosis of the left eye. Examination demonstrated a diffuse limitation of the extraocular movements secondary to edema, a well-healed upper eyelid incision, and complete ptosis of the left upper eyelid with mild upper lid edema. Six weeks following orbitotomy, visual acuity, ocular motility, and anterior segment examination findings remained stable compared with preoperative status. Over the course of the next several months, the degree of left upper lid ptosis continued to improve, ultimately returning to baseline 6 months postoperatively. At his most recent follow-up examination, 5 years after resection of the intraparenchymal cavernous malformation and 2 years after resection of the orbital hemangioma, the patient's clinical status remains stable. MRI showed typical postsurgical changes at each operative site with no new abnormal signal or enhancement suggestive of recurrence, additional lesions, or hemorrhage [Figure 1d–f].


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, b) CCM comprised cavernous, endothelium-lined vascular sinusoids with foci of calcification (a) and ossification. (b) Little intervening brain tissue between the cavernous vessels was noted. (c) High-power view of the vascular walls of the CCM demonstrates delicate mural hyalinization, scattered extravasated erythrocytes and hemosiderin, and scant inflammation. (d) Brain parenchyma at the periphery of the lesion showing typical hemosiderin deposits, macrophages, axonal spheroids, and gliosis
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 3: (a, b) CCM comprised cavernous, endothelium-lined vascular sinusoids with foci of calcification (a) and ossification. (b) Little intervening brain tissue between the cavernous vessels was noted. (c) High-power view of the vascular walls of the CCM demonstrates delicate mural hyalinization, scattered extravasated erythrocytes and hemosiderin, and scant inflammation. (d) Brain parenchyma at the periphery of the lesion showing typical hemosiderin deposits, macrophages, axonal spheroids, and gliosis
Mentions: Subsequently, the neurosurgical team bluntly dissected around the lesion, which appeared to be most consistent with a recurrent cavernous hemangioma. The feeding arteries to the lesion were cauterized and cut sharply. The lesion was biopsied and submitted to pathology for frozen and permanent section. The remainder of the lesion was then removed en bloc as two specimens. The superior aspect was dissected first followed by the medial and lateral aspects, while the inferior and deep aspects were the final portions to be removed. There were no subsequent post-procedural complications, with visual acuity remaining unchanged from preoperative status. Histopathologic review confirmed the working diagnosis of recurrent OCH [Figure 3]. At his 2-week follow-up, the patient reported complete resolution of supraorbital discomfort. However, he continued to note ptosis of the left eye. Examination demonstrated a diffuse limitation of the extraocular movements secondary to edema, a well-healed upper eyelid incision, and complete ptosis of the left upper eyelid with mild upper lid edema. Six weeks following orbitotomy, visual acuity, ocular motility, and anterior segment examination findings remained stable compared with preoperative status. Over the course of the next several months, the degree of left upper lid ptosis continued to improve, ultimately returning to baseline 6 months postoperatively. At his most recent follow-up examination, 5 years after resection of the intraparenchymal cavernous malformation and 2 years after resection of the orbital hemangioma, the patient's clinical status remains stable. MRI showed typical postsurgical changes at each operative site with no new abnormal signal or enhancement suggestive of recurrence, additional lesions, or hemorrhage [Figure 1d–f].

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