Rare asymptomatic giant cerebral cavernous malformation in adults: two case reports and a literature review

Cavernous malformations are benign vascular malformations. Giant cavernous malformations are very rare. All reported cases have been symptomatic because of the large size and compression of the surrounding brain tissue. We report two asymptomatic cases of giant cavernous malformation that were both misdiagnosed as neoplasms because of their atypical presentations. The first case was a 54-year-old man whose computed tomography and magnetic resonance imaging scans revealed an inhomogeneous lesion of 6 cm diameter and mild enhancement in the left frontal lobe. A left lateral supraorbital and transcortical approach was applied and the lesion was completely removed. The second case was a 36-year-old man with an irregular large mass in the parasellar region. Craniopharyngioma was suspected and gross total resection was performed. Post-surgical pathological analyses confirmed the diagnoses as cavernous malformations. Both patients recovered uneventfully. The rare asymptomatic giant cavernous malformations reported here in adults had benign behavior for this specific disease entity. The different clinical characteristics of ordinary cavernous malformation and adult and pediatric giant cavernous malformation imply complex and distinct genetic backgrounds. Concerns should be raised when considering giant cavernous malformation as a differential diagnosis for atypical large lesions. Surgical resection is recommended as the primary treatment option.

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An Occipital Headache as the First Presentation of Multiple Third, Fourth, and Lateral Ventricular Cavernous Malformations: A Case Report and Review of Literature

Galen Medical Journal ◽

10.31661/gmj.v6i1.666 ◽

2017 ◽

Vol 6 (1) ◽

pp. 61-65

Author(s):

Alireza Vakilian ◽

Amir Moghadam Ahmadi ◽

Habib Farahmand

Keyword(s):

Surgical Intervention ◽

Cavernous Malformation ◽

Vascular Malformations ◽

Case Reports ◽

Review Of Literature ◽

Rare Presentation ◽

Cavernous Malformations ◽

Cavernous Hemangiomas ◽

Occipital Headache ◽

Tomography Scan

Background: Cavernous hemangiomas are common benign vascular malformations. Their existence in the intraventricular region is very rare. Case Reports: A 43-year old woman with an occipital headache was admitted to the emergency ward. Brain computed tomography scan showed mild hydrocephalus and multiple intraventricular isodense lesions. Imaging findings, especially of Gradient Resonance Echo imaging, were in favor of multiple intraventricular cavernous malformations. Conclusion: This is a rare presentation of multiple cavernous malformation as occipital headache without needing surgical intervention in this phase. Coexistence of periventricular plaques like Radiologically isolated syndrome of Multiple sclerosis is another unique aspect in this report. [GMJ.2017;6(1):61-65]

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Unique enlarging cavernous malformation secondary to abnormal arteriovenous shunting through an associated developmental venous anomaly

The Neuroradiology Journal ◽

10.1177/19714009211042883 ◽

2021 ◽

pp. 197140092110428

Author(s):

Nimisha Parikh ◽

Richard Williamson ◽

Matthew Kulzer ◽

Albert Sohn ◽

Warren M Chang ◽

...

Keyword(s):

Unusual Case ◽

Cavernous Malformation ◽

Vascular Malformations ◽

Venous Hypertension ◽

Developmental Venous Anomaly ◽

Venous Anomaly ◽

Arteriovenous Shunting ◽

Cavernous Malformations ◽

Progressive Growth

Cavernous malformations are angiographically occult vascular malformations. They are often associated with a developmental venous anomaly through poorly understood mechanisms. We present an unusual case of a gradually enlarging cavernous malformation associated with a developmental venous anomaly with arteriovenous shunting, suggesting venous hypertension or reflux as a potential cause of progressive growth.

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Transclival Approach for Resection of a Pontine Cavernous Malformation: 2-Dimensional Operative Video

Operative Neurosurgery ◽

10.1093/ons/opaa025 ◽

2020 ◽

Vol 19 (4) ◽

pp. E413-E413

Author(s):

Dennis London ◽

Seth Lieberman ◽

Omar Tanweer ◽

Donato Pacione

Keyword(s):

Cavernous Malformation ◽

Case Reports ◽

Abducens Nerve ◽

Petrous Bone ◽

Fat Graft ◽

Csf Leak ◽

Endoscopic Endonasal ◽

Cavernous Malformations ◽

Lumbar Drain ◽

Magnetic Resonance Imaging Mri

Abstract Cerebral cavernous malformations are common vascular anomalies consisting of a cluster of capillaries without intervening brain tissue.1 A variety of approaches for resection have been undertaken,2 and a handful of case reports have described the endoscopic, endonasal, transclival approach.3 We present a case of a 51-yr-old woman with lupus and hepatitis B-associated cirrhosis who presented with diplopia, dysphagia, and ataxia. She had a left abducens nerve palsy and magnetic resonance imaging (MRI) showed a left pontine cavernous malformation. After a repeat hemorrhage, she consented to surgical resection. The lesion appeared to come to the medial pontine pial surface. Tractography indicated a rightward displacement of the left corticospinal tract. Therefore, an endoscopic, transnasal, transclival approach was chosen. A lumbar drain was placed preoperatively. The clivus and ventral petrous bone were drilled using the vidian canal to help identify the anterior genu of the petrous carotid artery. The clival dura was opened, revealing the abducens nerve exiting the ventral pons. The cavernoma was visible on the surface lateral to the nerve. It was removed using blunt dissection and the remaining cavity inspected. The skull base was reconstructed using an abdominal dermal-fat graft and Alloderm covered by a nasoseptal flap. Postoperatively she had transient swallowing difficulty. The lumbar drain was kept open for 5 d. Cerebrospinal fluid (CSF) leak was ruled out using an intrathecal fluorescein injection. She was discharged home, but presented 2 wk postoperatively with aseptic meningitis, which was treated supportively. Postoperative imaging did not show residual cavernoma.

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The Juxtaposition of a Capillary Telangiectasia, Cavernous Malformation, and Developmental Venous Anomaly in the Brainstem of a Single Patient: Case Report

Neurosurgery ◽

10.1097/00006123-200111000-00044 ◽

2001 ◽

Vol 49 (5) ◽

pp. 1246-1250 ◽

Cited By ~ 13

Author(s):

Richard E. Clatterbuck ◽

İlhan Elmacı ◽

Daniele Rigamonti

Keyword(s):

Cavernous Malformation ◽

Vascular Malformations ◽

Magnetic Resonance Imaging Examination ◽

Developmental Venous Anomaly ◽

Venous Malformations ◽

Venous Anomaly ◽

Single Patient ◽

Cavernous Malformations ◽

Swallowing Problems ◽

Capillary Telangiectasia

ABSTRACT OBJECTIVE AND IMPORTANCE Capillary telangiectasias, cavernous malformations, and developmental venous anomalies are all vascular malformations that occur on the capillary-venous side of the cerebral circulation. The associations of capillary telangiectasias with venous malformations, cavernous malformations with venous malformations, and capillary telangiectasias with cavernous malformations have all been described; however, the association of all three lesions in a single patient is extremely rare. CLINICAL PRESENTATION A 52 year-old Caucasian woman presented to our clinic with an extended history of confusion, distorted visual perceptions, photophobia, neck pain, swallowing problems, and poor balance. The patient's examination was remarkable for difficulty concentrating, mild rotatory nystagmus, subtle decreased sensation over the left side of the face and body, and brisk reflexes. Review of the patient's magnetic resonance imaging examination demonstrated a cavernous malformation, a capillary telangiectasia, and a developmental venous anomaly located adjacent to one another in the brainstem. INTERVENTION Given the patient's complex constellation of symptoms and relatively mild neurological findings, it was difficult to ascribe any one of them to a specific vascular malformation. Conservative management of this patient's vascular malformations was decided upon. CONCLUSION Juxtaposition of these three different vascular lesions in the brainstem of an otherwise normal individual suggests a relationship among them. Although there are several theories that link similar associations through physiological mechanisms such as venous hypertension, we propose that a developmental event disrupting local capillary-venous pattern formation is a plausible alternative.

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Are cavernous sinus hemangiomas and cavernous malformations different entities?

Neurosurgical FOCUS ◽

10.3171/foc.2006.21.1.7 ◽

2006 ◽

Vol 21 (1) ◽

pp. 1-5 ◽

Cited By ~ 42

Author(s):

L. Fernando Gonzalez ◽

Gregory P. Lekovic ◽

Jennifer Eschbacher ◽

Stephen Coons ◽

Randall W. Porter ◽

...

Keyword(s):

Cavernous Sinus ◽

Cavernous Malformation ◽

Vascular Malformations ◽

Response To Treatment ◽

Vascular Tumors ◽

Cerebral Cavernous Malformations ◽

Imaging Studies ◽

Cavernous Malformations ◽

Cavernous Hemangiomas

✓Cavernous hemangiomas that occur within the cavernous sinus (CS) are different from cerebral cavernous malformations (CMs) clinically, on imaging studies, and in their response to treatment. Moreover, CMs are true vascular malformations, whereas hemangiomas are benign vascular tumors. Because of these differences, the authors suggest that these two entities be analyzed and grouped separately. Unfortunately, despite these differences, much confusion exists in the literature as to the nature, behavior, and classification of these two distinct lesions. This confusion is exacerbated by subtle histological differences and the inconsistent use of nomenclature. The authors use the term “cavernous malformation” to refer to intraaxial lesions only; they prefer to use the term “cavernous sinus hemangioma” to refer to extraaxial, intradural hemangiomas of the CS.

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A Novel CCM2 Missense Variant Caused Cerebral Cavernous Malformations in a Chinese Family

Frontiers in Neuroscience ◽

10.3389/fnins.2020.604350 ◽

2021 ◽

Vol 14 ◽

Author(s):

Guoqing Han ◽

Li Ma ◽

Huanhuan Qiao ◽

Lin Han ◽

Qiaoli Wu ◽

...

Keyword(s):

Vascular Malformations ◽

Case Reports ◽

Chinese Family ◽

Incomplete Penetrance ◽

Cerebral Cavernous Malformations ◽

Missense Mutations ◽

Inherited Disease ◽

Cavernous Malformations ◽

Direct Dna Sequencing ◽

Number Of Patients

Cerebral cavernous malformations (CCMs) are common vascular malformations in the central nervous system. Familial CCMs (FCCMs) are autosomal dominant inherited disease with incomplete penetrance and variable symptoms. Mutations in the KRIT1, CCM2, and PDCD10 genes cause the development of FCCM. Approximately 476 mutations of three CCM-related genes have been reported, most of which were case reports, and lack of data in stable inheritance. In addition, only a small number of causative missense mutations had been identified in patients. Here, we reported that 8/20 members of a Chinese family were diagnosed with CCMs. By direct DNA sequencing, we found a novel variant c.331G > C (p.A111P) in exon 4 of the CCM2 gene, which was a heterozygous exonic variant, in 7/20 family members. We consider this variant to be causative of disease due to a weaken the protein–protein interaction between KRIT1 and CCM2. In addition, we also found the exon 13 deletion in KRIT1 coexisting with the CCM2 mutation in patient IV-2, and this was inherited from her father (patient III-1H). This study of a Chinese family with a large number of patients with CCMs and stable inheritance of a CCM2 mutation contributes to better understanding the spectrum of gene mutations in CCMs.

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Large cavernous malformations in infant

JBNC - JORNAL BRASILEIRO DE NEUROCIRURGIA ◽

10.22290/jbnc.v32i2.1961 ◽

2021 ◽

Vol 32 (2) ◽

pp. 149-155

Author(s):

João Pedro Einsfeld Britz ◽

Ildo Sonda ◽

Renato Luis Calloni ◽

Yan Bicca ◽

Arthur Aguzzoli

Keyword(s):

Cavernous Malformation ◽

Vascular Malformations ◽

Pediatric Population ◽

Previous History ◽

Resonance Imaging ◽

Cavernous Malformations ◽

History Of ◽

The Central Nervous System ◽

Radiological Aspect ◽

Clonic Seizures

Cavernous malformations are rare vascular malformations in the central nervous system. We present the case of a 2-month-old female patient who presented tonic-clonic seizures, with no previous history of seizures. Magnetic resonance imaging showed a 5.6 cm tumor in the left parieto-occipital region. The radiological aspect of the tumor initially suggested an anaplastic meningioma. After surgical treatment and anatomopathological analysis, it was found to be a cavernous malformation. Cavernous malformations, or cavernomas, are rare lesions and even more rare is the occurrence of large cavernomas. In the pediatric population, although still quite rare, they usually are presented as larger cavernomas. Surgical resection is considered the most effective treatment.

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Chikungunya-induced inflammatory myositis: a case report in India

Tropical Doctor ◽

10.1177/0049475519843781 ◽

2019 ◽

Vol 49 (3) ◽

pp. 241-243 ◽

Cited By ~ 1

Author(s):

Nishant Dev ◽

Rahul Kumar ◽

Arun Gogna ◽

Shruti Sharma

Keyword(s):

Case Report ◽

Optic Neuritis ◽

Rna Virus ◽

Case Reports ◽

Neurological Complications ◽

Inflammatory Myositis ◽

Chikungunya Fever ◽

Aedes Mosquitoes ◽

First Case ◽

Atypical Presentations

Chikungunya fever is a benign and self-limiting disease caused by an RNA virus belonging to genus alphavirus and transmitted by infected Aedes mosquitoes. However, a number of atypical presentations involving various systems have been reported. Among the neurological complications, encephalitis, myelitis, Guillain-Barre syndrome and optic neuritis are commonly seen. However, its presentation as isolated inflammatory myositis causing quadriplegia is extremely rare. We report a 35-year-old woman with quadriplegia caused by chikungunya-induced inflammatory myositis. The diagnosis was confirmed with clinical examination, electromyography study, muscle biopsy findings and exclusion of other causes. There have been case reports of inflammatory myositis in association with various infections as well as in association with other neurological presentations in chikungunya. However, this may be the first case report of isolated inflammatory myositis associated with chikungunya fever.

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Using the Lateral Pontine Safe Entry Zone for Resection of Deep-Seated Cavernous Malformations in the Lateral Pons: 2-Dimensional Operative Video

Operative Neurosurgery ◽

10.1093/ons/opaa142 ◽

2020 ◽

Vol 19 (5) ◽

pp. E518-E519

Author(s):

Daniel D Cavalcanti ◽

Joshua S Catapano ◽

Paulo Niemeyer Filho

Keyword(s):

Trigeminal Nerve ◽

Cranial Nerve ◽

Cavernous Malformation ◽

Vascular Malformations ◽

Cranial Nerves ◽

Cavernous Malformations ◽

Entry Zone ◽

History Of ◽

Tumor Biopsies ◽

Safe Entry Zone

Abstract The retrosigmoid approach is one of the main approaches used in the surgical management of pontine cavernous malformations. It definitely provides a lateral route to large central lesions but also makes possible resection of some ventral lesions as an alternative to the petrosal approaches. However, when these vascular malformations do not emerge on surface, one of the safe corridors delimited by the origin of the trigeminal nerve and the seventh-eight cranial nerve complex can be used.1-5 Baghai et al2 described the lateral pontine safe entry zone in 1982, as an alternative to approaches through the floor of the fourth ventricle when performing tumor biopsies. They advocated a small neurotomy performed right between the emergence of the trigeminal nerve and the facial-vestibulocochlear cranial nerves complex. Accurate image guidance, intraoperative cranial nerve monitoring, and comprehensive anatomical knowledge are critical for this approach.4,5 Knowing the natural history of a brainstem cavernous malformation after bleeding,6 we sought to demonstrate in this video: (1) the use of the retrosigmoid craniotomy in lateral decubitus for resection of deep-seated pontine cavernous malformations; (2) the wide opening of arachnoid membranes and dissection of the superior petrosal vein complex to improve surgical freedom and prevent use of fixed cerebellar retraction; and (3) the opening of the petrosal fissure and exposure of the lateral pontine zone for gross total resection of a cavernous malformation in a 19-yr-old female with a classical crossed brainstem syndrome. She had full neurological recovery after 3 mo of follow-up. The patient consented in full to the surgical procedure and publication of the video and manuscript.

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Brainstem cavernous malformations: a review with two case reports

Arquivos de Neuro-Psiquiatria ◽

10.1590/s0004-282x2009000500030 ◽

2009 ◽

Vol 67 (3b) ◽

pp. 917-921 ◽

Cited By ~ 4

Author(s):

Adolfo Ramírez-Zamora ◽

José Biller

Keyword(s):

Radiation Therapy ◽

De Novo ◽

Vascular Malformations ◽

Case Reports ◽

Clinical Manifestations ◽

Pregnant Patient ◽

Complex Nature ◽

Cavernous Malformations ◽

Symptomatic Intracranial Hemorrhage ◽

Brainstem Cavernous Malformation

Central nervous system (CNS) cavernous malformations (CMs) are developmental malformations of the vascular bed with a highly variable clinical course due to their dynamic nature. We present one case of "de novo" brainstem cavernous malformation after radiation therapy adding to the increasing number of reported cases in the medical literature, and the case of a pregnant patient with symptomatic intracranial hemorrhage related to brainstem CMs to illustrate the complex nature in management of these patients, followed by a review of clinical and radiographic characteristics. CMs account for 8-15% of all intracranial and intraspinal vascular malformations. Although traditionally thought to be congenital in origin, CMs may present as acquired lesions particularly after intracranial radiation therapy. Clinical manifestations are protean and surgical treatment should be considered for patients with progressive neurologic deficits.

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