Spinal arteriovenous malformation associated with spinal metameric syndrome: a treatable cause of long-term paraplegia?

Linfante, Italo; Capone, Francesca; Dabus, Guilherme; Gonzalez‐Arias, Sergio; Lau, Patricio E.; Samaniego, Edgar A

doi:10.3171/2011.12.spine11636

Cited by 13 publications

(11 citation statements)

References 31 publications

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“…[356] Most of the cases reported “typical” presentation of angiomatous-cutaneous stains and spinal AVM corresponding to the same metamere, whereas other cases reported Cobb syndrome cases with cutaneous cavernous angiomas (CAs) associated with spinal Cas. [9] A few cases have been presented as acute paraplegia, but none as acute quadriparesis.…”

Section: Discussionmentioning

confidence: 99%

“…[9] A few cases have been presented as acute paraplegia, but none as acute quadriparesis. [1610] Although the most widely accepted diagnosis criteria is that proposed by Kissel and Dureux,[4] which includes a skin nevus in the same segment as the spinal angioma (with or without visceral angiomatosis), the type and pathology of spinal angioma is still ill-defined. The genetic framework of this syndrome also remains unclear, although one study suggested an inherited disposition based in a family case of Cobb syndrome.…”

Section: Discussionmentioning

confidence: 99%

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Cervicothoracic cutaneomeningospinal angiomatosis in adults (Cobb's syndrome): A case report of acute quadriparesis

Papalini¹,

Papalini²

2017

Surg Neurol Int

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Background:Cutaneomeningospinal angiomatosis or Cobb syndrome is a rare, not well understood phacomatosis that features metameric cutaneous and spinal arteriovenous malformations (AVMs). The first case was described in Boston in 1915, and since then, few more cases have been reported in the English literature. No case was found to be from Argentina.Case Description:The authors present a 16-year-old boy with acute quadriparesis and respiratory failure who was diagnosed as Cobb syndrome and treated with microsurgery alone with very good results.Conclusion:Authors believe that microsurgery, alone or combined with embolization, should be the mainstay of treatment. They also acknowledge Harvey Cushing's contribution to the description of the syndrome and propose the syndrome to be renamed as Cobb–Cushing syndrome.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Cervicothoracic cutaneomeningospinal angiomatosis in adults (Cobb's syndrome): A case report of acute quadriparesis

Papalini¹,

Papalini²

2017

Surg Neurol Int

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“…[1][2][3][4]8,10,[12][13][14][20][21][22][23][24][25]29,[32][33][34][35][36][37]39,[41][42][43]45,48,50 For these 51 patients, the mean age at presentation was 15.0 ± 10.5 years (± SD), and there was a slight male sex predilection (63% male, 1.7:1 male/ female ratio) ( Table 2). Presentation modalities included progressive neurological deficits in 35% of cases, acute hemorrhage in 31%, an acute neurological deficit without hemorrhage in 22% of cases, and incidental discovery in the remaining 12%.…”

Section: Resultsmentioning

confidence: 99%

Spinal juvenile (Type III) extradural-intradural arteriovenous malformations

Gross¹,

Du²

2014

SPI

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Object Owing to their rarity, demographics, natural history, and treatment, results for spinal juvenile (Type III) extradural-intradural arteriovenous malformations (AVMs) are frequently only provided in case report format. Methods A pooled analysis was performed utilizing the PubMed database through April 2013. Individualized patient data were extracted to elucidate demographics, hemorrhage risk, and treatment result information. Results Twenty-nine studies describing 51 patients were included. The mean age at presentation was 15.0 ± 10.5 years with a slight male predilection (63%, 1.7:1 sex ratio). Presentation modality included progressive deficits in 35%, hemorrhage in 31%, acute deficits not attributed to hemorrhage in 22%, and asymptomatic/incidental in 12% of patients. The annual hemorrhage rate was 2.1%; statistically significant risk factors for hemorrhage included presentation age (HR 0.39 [95% CI 0.18–0.87]) and associated aneurysms (HR 8.74 [95% CI 1.76–43.31]). Seventy-seven percent of patients underwent treatment; after a mean follow-up of 2.6 ± 3.2 years, 73% were improved, 10% were the same, and 17% were worse neurologically. Of 25 cases with described angiographic results, 8 lesions were obliterated (32%). Of these 25 patients, 8 had AVMs with associated aneurysms, and the aneurysm was obliterated in all 8 patients. Over the course of 57.9 patient-years of follow-up, including 55.3 patient-years for partially treated AVMs, no hemorrhages were described, reflecting a trend toward protection from hemorrhage after treatment (p = 0.12, likelihood ratio test). Conclusions Spinal juvenile (Type III) extradural-intradural AVMs commonly present symptomatically. Associated arterial aneurysms increase their hemorrhage risk, and protection from hemorrhage may be achieved from partial obliteration of these lesions, particularly if targeted toward associated aneurysms.

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“…7,13,18 Gross et al reviewed 27 publications and analyzed 352 cases of intramedullary spinal cord CAs. 7 They reported that the mean age at presentation was 42 years, without sex predominance.…”

Section: Discussionmentioning

confidence: 99%

Coexistence of multiple cavernous angiomas in the spinal cord and skin: a unique case of Cobb syndrome

Matsui¹,

Mineharu²,

Satow³

et al. 2014

SPI

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Cobb syndrome is a rare, noninherited, neurocutaneous disease characterized by vascular abnormality of the spinal cord and is associated with vascular lesions in the skin at the same metamere. The majority of spinal vascular lesions are arteriovenous malformations, and skin lesions are mostly port-wine angiomas. The authors report the first case of multiple intramedullary cavernous angiomas (CAs) accompanied by skin CAs within the same metamere. A 42-year-old man presented with an acute onset of gait disturbance, low-back pain, and urinary incontinence. Magnetic resonance imaging of the thoracolumbar spine showed homogeneously enhanced lesions on a contrast-enhanced T1-weighted image and a hypointense area on a T2*-weighted image surrounding this enhanced lesion, between the T-12 and S-1 levels. Purple protruding skin lesions were detected on the left side of his gluteal region. The patient received a laminectomy followed by evacuation of a hematoma and partial removal of the tumor, which completely resolved his neurological symptoms. Pathological examinations showed that the spinal and skin lesions were CAs, suggesting that these vascular lesions developed congenitally. Cavernous angiomas associated with Cobb syndrome present with multiple lesions spanning more than 3 vertebral levels, making it difficult to completely resect these tumors. Although Cobb syndrome is an uncommon disease entity, it should be considered if a patient manifesting with neurological deficits has skin vascular lesions, including CAs.

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Spinal arteriovenous malformation associated with spinal metameric syndrome: a treatable cause of long-term paraplegia?

Cited by 13 publications

References 31 publications

Cervicothoracic cutaneomeningospinal angiomatosis in adults (Cobb's syndrome): A case report of acute quadriparesis

Cervicothoracic cutaneomeningospinal angiomatosis in adults (Cobb's syndrome): A case report of acute quadriparesis

Spinal juvenile (Type III) extradural-intradural arteriovenous malformations

Coexistence of multiple cavernous angiomas in the spinal cord and skin: a unique case of Cobb syndrome

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