Familial Cavernous Angiomas

Bicknell, Joseph M.; Carlow, Thomas J.; Kornfeld, Mario; Støvring, Jørgen; Turner, Paul

doi:10.1001/archneur.1978.00500350050010

Cited by 159 publications

(43 citation statements)

References 13 publications

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“…1,5,38,42) Some might have a familial distribution. 4,20,33) In our case, the lesion was neither multiple nor familial. However, the entire neuraxis should be screened because there is a high incidence of multiple lesions.…”

Section: Discussionmentioning

confidence: 46%

Infantile Cervical Intramedullary Cavernous Angioma Manifesting as Hematomyelia -Case Report-

Miyoshi

Yasuhara

Omori

et al. 2010

Neurol. Med. Chir.(Tokyo)

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A 2-month-old infant presented with a cervical intramedullary spinal cord cavernous angioma manifesting as left hemiparesis caused by hematomyelia. Osteoplastic laminotomy of the cervical spine was carried out with subsequent microsurgical excision of the intramedullary spinal cord cavernous angioma. Magnetic resonance imaging revealed no residual tumor. At 25 months after the surgery, she presented no neurological deficit without recurrence and cervical deformity. This case of infantile intramedullary spinal cord cavernous angioma presenting with cervical hematomyelia shows osteoplastic laminotomy of the cervical spine might be helpful to prevent consequent cervical deformity in pediatric cases.

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Section: Discussionmentioning

confidence: 46%

Infantile Cervical Intramedullary Cavernous Angioma Manifesting as Hematomyelia -Case Report-

Miyoshi

Yasuhara

Omori

et al. 2010

Neurol. Med. Chir.(Tokyo)

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“…4,11,41,43 In an early review of 43 surgical case reports and small surgical series, Canavero et al 6 reported a 74% rate of complete resection. This rate has improved to 91% in our study since the goal of surgery has become complete resection.…”

Section: Surgical Resultsmentioning

confidence: 99%

Intramedullary spinal cord cavernous malformations

Gross¹,

Du²,

Popp³

et al. 2010

FOC

119

106

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Although originally the subject of rare case reports, intramedullary spinal cord cavernous malformations (CMs) have recently surfaced in an increasing number of case series and natural history reports in the literature. The authors reviewed 27 publications with 352 patients to consolidate modern epidemiological, natural history, and clinical and surgical data to facilitate decision making when managing these challenging vascular malformations. The mean age at presentation was 42 years without a sex predilection. Thirty-eight percent of the cases were cervical, 57% thoracic, 4% lumbar, and 1% unspecified location. Nine percent of the patients had a family history of CNS CMs. Twenty-seven percent of the patients had an associated cranial CM. On presentation 63% of the patients had motor deficits, 65% had sensory deficits, 27% had pain, and 11% had bowel or bladder dysfunction. Presentation was acute in 30%, recurrent in 16%, and progressive in 54% of cases. An overall annual hemorrhage rate was calculated as 2.5% for 92 patients followed up for a total of 2571 patient-years. Across 24 reviewed surgical series, a 91% complete resection rate was found. Transient morbidity was seen in 36% of cases. Sixty-one percent of patients improved, 27% were unchanged, and 12% were worse at the long-term follow-up. Using this information, the authors review surgical nuances in treating these lesions and propose a management algorithm.

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“…It was long suspected that a genetic link to CMs existed; 7,9,24,28 however, definitive evidence remained elusive until 1982 when Hayman et al 21 published their seminal paper on CM inheritance. They reported on a kindred of 122 individuals, of whom 5 had cerebral vascular malformations and 3 had CMs.…”

Section: Risk Factors That Increase Hemorrhage Riskmentioning

confidence: 99%

Update on the natural history of cavernous malformations and factors predicting aggressive clinical presentation

Washington¹,

McCoy²,

Zipfel

2010

FOC

137

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Cavernous malformations (CMs) are angiographically occult, low-pressure neurovascular lesions with distinct imaging and clinical characteristics. They present with seizure, neurological compromise due to lesion hemorrhage or expansion, or as incidental findings on neuroimaging studies. Treatment options include conservative therapy, medical management of seizures, surgical intervention for lesion resection, and in select cases stereotactic radiosurgery. Optimal management requires a thorough understanding of the natural history of CMs including consideration of issues such as mode of presentation, lesion location, and genetics that may impact the associated neurological risk. Over the past 2 decades, multiple studies have been published, shedding valuable light on the clinical characteristics and natural history of these malformations. The purpose of this review is to provide the reader with a concise consolidation of this published material such that they may better understand the risks associated with CMs and their implications on patient treatment.

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Familial Cavernous Angiomas

Cited by 159 publications

References 13 publications

Infantile Cervical Intramedullary Cavernous Angioma Manifesting as Hematomyelia -Case Report-

Infantile Cervical Intramedullary Cavernous Angioma Manifesting as Hematomyelia -Case Report-

Intramedullary spinal cord cavernous malformations

Update on the natural history of cavernous malformations and factors predicting aggressive clinical presentation

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