Cobb Syndrome: Case Report and Review of the Literature

Dilmé-Carreras, Elisabet; Iglesias‐Sancho, Maribel; Márquez-Balbás, Gemma; Sola-Ortigosa, Joaquin; Umbert-Millet, P.

doi:10.1159/000313510

Cited by 19 publications

(41 citation statements)

References 25 publications

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“…They stated that the neurological manifestations of this syndrome are caused by spinal angioma and that the most common initial symptoms are pain and motor deficit . The range of presentations in symptomatic Cobb syndrome is diverse, and the course is unpredictable, thus making firm prognoses difficult . In our patient, initial symptom was radicular pain in the lumbar region, and followed by urinary symptoms due to neurogenic bladder and ultimately paraplegia.…”

mentioning

confidence: 72%

“…Cobb syndrome (cutaneomeningospinal angiomatosis) is a rare metameric disorder characterised by an association with cutaneous angiomas in the same metamere . The syndrome was not recognised until Cobb's description of it in 1915 .…”

mentioning

confidence: 99%

“…The common neurological manifestations of this syndrome are pain and motor deficits caused by spinal angioma. Nevus flammeus and angiokeratomas are the known skin manifestations of Cobb syndrome . We report a 32‐year‐old male, who presented to the dermatology clinic of Alzahra hospital, Isfahan University of Medical Sciences, with a malodor cauliflower‐like bulky lesion at the intergluteal cleft and lower back from 6 months ago.…”

mentioning

confidence: 99%

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Unusual cause of lower extremity wounds: Cobb syndrome

Abtahi‐Naeini

Saffaei

Pourazizi

2015

International Wound Journal

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Cobb syndrome (Cutaneomeningospinal Angiomatosis) is a rare segmental neurocutaneous syndrome associated with metameric cutaneous and spinal cord arteriovenous malformations (AVMs). In this syndrome, capillary malformation or angiokeratoma-like lesions are formed in a dermatomal distribution, with an AVM in the corresponding segment of the spinal cord. The spinal cord lesions can cause neurological disorder and paraplegia, which typically develop during young adulthood. We report a 32-year-old male with the Cobb syndrome associated with lower extremity painful wounds and acute-onset paraplegia due to metameric vascular malformations.

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confidence: 72%

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confidence: 99%

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confidence: 99%

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Unusual cause of lower extremity wounds: Cobb syndrome

Abtahi‐Naeini

Saffaei

Pourazizi

2015

International Wound Journal

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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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“…Adult and young patients typically present with sudden onset of radicular pain in the lower extremities and associated numbness. Less commonly, sudden onset of weakness or rectal/ and bladder dysfunction is the presenting symptom [8]. The clinical course may also progress over several years to paraplegia and sphincter dysfunction [7].…”

Section: Case Reportmentioning

confidence: 99%

Spinal Arteriovenous Metameric Syndrome: Role of Physiotherapy: A Case Report

Lama¹,

Wajapey²,

Pyatha³

2018

IJPR

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Background: Spinal arteriovenous metameric syndrome (SAMS), also known as Cobb syndrome is a rare metameric developmental disorder presenting as an extradural-intradural vascular malformation that involves bone, muscle, skin, spinal cord, and nerve roots.Case Report: An 11-year-old girl presented with history of progressive paraparesis, lower extremity sensory loss, along with bowel and bladder incontinence who was diagnosed with Cobb syndrome. She underwent endovascular embolization of one arterial feeder and two metameric components. Post embolization patient experienced further decline in muscle strength. Physiotherapy regimen was implemented for 4-5 days per week for period of two months. The patient was discharged with a home exercise programme.Result: At the time of discharge, muscle power was improved. Patient was able to walk with the help of walker. Conclusion:Spinal metameric arteriovenous syndrome is a complex nonhereditary genetic vascular disorder associated with variety of neurologic deficits. Physiotherapy management will play a major role in minimizing disability hence improve clinical outcome.

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Cobb Syndrome: Case Report and Review of the Literature

Cited by 19 publications

References 25 publications

Unusual cause of lower extremity wounds: Cobb syndrome

Unusual cause of lower extremity wounds: Cobb syndrome

Spinal juvenile (Type III) extradural-intradural arteriovenous malformations

Spinal Arteriovenous Metameric Syndrome: Role of Physiotherapy: A Case Report

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