Cobb Syndrome: A Case Report and Systematic Review of the Literature

doi:10.1016/j.pediatrneurol.2008.08.001

Pediatric Neurology

Volume 39, Issue 6, December 2008, Pages 423-425

https://doi.org/10.1016/j.pediatrneurol.2008.08.001 Get rights and content

We report on a case of Cobb syndrome, a rare metameric disorder, characterized by a vascular abnormality of the spinal cord, with an associated vascular skin lesion of the same metamere, in an 8-year-old girl presenting with leg weakness. Magnetic resonance imaging revealed a spinal arteriovenous malformation at the level of T₁₀-L₅. This finding, together with a large, pigmented nevus at this level, is diagnostic for Cobb syndrome. A systematic review of the literature on Cobb syndrome, with a focus on treatment and outcomes, is presented. Current treatment options include combinations of embolization, neurosurgical intervention, corticosteroid therapy, and radiotherapy.

Section snippets

Case Report

We describe a previously well 8-year-old girl who presented at the emergency department after a 24-hour history of headache, vomiting, and severe muscle ache in her lower limbs and neck. There was no abnormal bladder or bowel functioning.

There was no significant past or family history. She has one older sister who was well. She was Australian-born, of Sri Lankan heritage. She was previously very active, and did not report any preceding signs.

On examination, she held herself in a lordosis with

Discussion

Cobb syndrome, or cutaneomeningospinal angiomatosis, was first described in 1895, and was reported by Cobb in 1915 [1]. It is a rare, nonfamilial condition coupling cutaneous vascular lesions with arteriovenous malformations involving the spinal cord in the same metamere as the cutaneous lesion [2]. The most common dermatologic manifestations comprise nevus flammeus and angiokeratomas. The incidence of Cobb syndrome is unknown, but may be more common than the 39 cases in the literature suggest,

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Segmental cutaneous hemangioma and spinal arteriovenous malformation (Cobb syndrome): Case report and historical perspective
J Neurosurg
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J.H. Shim et al.
A case of Cobb syndrome associated with lymphangioma circumscriptum—Review
Dermatology
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E. Veznedaroglu et al.
Endovascular treatment of spinal cord arteriovenous malformations
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G. Rodesch et al.
Angio-architecture of spinal cord arteriovenous shunts at presentation. Clinical correlations in adults and children. The Bicêtre experience on 155 consecutive patients seen between 1981-1999
Acta Neurochir (Wien)
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Report of a new case of Cobb syndrome—Meningospinal cutaneous angiomatosis
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Cobb syndrome
Dermatologica
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Cobb syndrome treated by staged intravascular embolisation and surgery
Med J Malaysia
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Basappa
Cobb syndrome
J Assoc Physicians India
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L.J. Kim et al.
Classification and surgical management of spinal arteriovenous lesions: Arteriovenous fistulae and arteriovenous malformations
Neurosurgery
(2006)

There are more references available in the full text version of this article.

Cited by (34)

Spinal vascular lesions: anatomy, imaging techniques and treatment
2021, European Journal of Radiology Open
Citation Excerpt :
Dermatological manifestations mainly include nevus flammeus and angiokeratomas; spinal vascular lesions consist of high-flow arteriovenous shunts like SDAVFs and AVMs, whose extension may involve meninges, vertebral bodies and soft tissue within the same metamere [79], as well as low-flow non-arterialized vascular malformations [80]. Symptoms may result from spinal cord compression, ischemia, venous hypertension or subarachnoid haemorrhage [81]; in case of vascular cutaneous malformation with metameric distribution, Cobb syndrome should be considered. Vascular myelopathies are critical conditions with acute/subacute presentation and severe consequences on the quality of life.
Vascular lesions of the spinal cord are rare but potentially devastating conditions whose accurate recognition critically determines the clinical outcome. Several conditions lead to myelopathy due to either arterial ischemia, venous congestion or bleeding within the cord. The clinical presentation varies, according with the different aetiology and mechanism of damage.
The aim is to provide a comprehensive review on the radiological features of the most common vascular myelopathies, passing through the knowledge of the vascular spinal anatomy and the clinical aspects of the different aetiologies, which is crucial to promptly address the diagnosis and the radiological assessment.
Bilateral optic disc pits in a pediatric patient with Cobb syndrome
2020, American Journal of Ophthalmology Case Reports
Citation Excerpt :
Patients can present at any age, but typically present in late childhood, with three reported cases in infancy.3,7,21,22 The vascular cutaneous lesions, which can be segmental in distribution, typically take the form of port-wine angiomas (neveus flammeus), and are brought to medical attention due to cosmetic concerns or due to acute bleeding with trauma.1,4,5 Of greater concern is that the underlying paraspinous AVMs may result in hemiparesis or quadraparesis due to ischemia, hemorrhage, spinal cord compression or vascular steal syndrome.1,4,6
To describe a patient with Cobb Syndrome, a rare congenital disorder characterized by cutaneous and spinal arteriovenous malformations, who was found to have bilateral optic nerve pits.
A 15-year-old boy diagnosed with Cobb Syndrome, manifesting as a large cutaneous port-wine stain associated with an underlying left paraspinous arteriovenous malformation resulting in severe scoliosis, presented for a screening ophthalmological exam. The patient had no visual symptoms. On examination, his visual acuity was 20/20 in each eye; however, bilateral optic disc pits were discovered on biomicroscopy and confirmed by spectral-domain optical coherence tomography.
The unusual finding of bilateral optic disc pits in this rare congenital neurocutaneous disorder represents the first report of ophthalmic abnormalities in association with Cobb syndrome. Patients with Cobb Syndrome may be considered for screening ophthalmological exam for the detection of subclinical optic nerve abnormalities.
Endovascular stent graft exclusion of a thoracic arteriovenous malformation in a patient with Cobb syndrome
2016, Journal of Vascular Surgery Cases
Cobb syndrome is a rare neurocutaneous disorder characterized by spinal vascular abnormalities in association with a vascular lesion of the skin at the same metamere. Patients present after the onset of symptoms such as paraplegia, back pain, and, less commonly, fatigue due to heart failure. Available treatment options to date have included neurosurgical resection and endovascular embolization. We present a patient with Cobb syndrome with a progressively symptomatic arteriovenous malformation involving the thoracic vertebrae and left hemithorax, with extensive blood supply from the T3 to T10 intercostal arteries, and demonstrate successful endovascular thoracic stent graft placement and coil embolization.
Exclusively epidural spinal metameric arteriovenous shunts: Case report and literature review
2015, Spine Journal
Spinal arteriovenous metameric syndrome (SAMS) is a subgroup of spinal arteriovenous malformations (AVMs). Most SAMS cases have intra- and extradural AVMs and suffer from hematomyelia, subarachnoid hemorrhage, or venous congestive myelopathy.
To present a rare case of SAMS in which spinal AVMs were exclusively epidural. We reviewed previous literature and evaluated the feasibility of a treatment strategy using endovascular interventions, followed by surgical obliteration.
A case report and literature review of SAMS.
We report a case of a 15-year-old boy suffering from SAMS in which epidural venous ectasia because of extradural AVMs caused spinal cord compression.
The patient was successfully treated with multiple sessions of transarterial embolization followed by open surgery. After the treatment, his neurologic deficits resolved. Postoperative angiography confirmed complete obliteration of extradural AVMs.
Although exclusively epidural spinal AVM is an uncommon type of SAMS, combined endovascular and surgical interventions can be an effective treatment for AVMs to achieve better radiologic outcomes and complete resolution of patient symptoms.
Diagnostic Imaging: Spine
2015, Diagnostic Imaging: Spine
Neurodermatology
2014, Handbook of Clinical Neurology
Since the skin and the central and/or peripheral nervous system share a common source (the ectoderm), numerous genetic and acquired diseases (infectious, tumoral or autoimmune disorders) equally affect both. Neurologic diseases or symptoms such as stroke, cerebral or medullary vascular malformations, peripheral, brain or medullary tumors, epilepsy, ataxia, neurologic infections, or cognitive disorders (dementia, mental retardation) may be associated with specific cutaneous manifestations of which the discovery can facilitate the final diagnosis, thereby leading to specific treatment and/or genetic investigations. Careful examination of the skin, hair, and nails by the neurologist is consequently of the utmost importance; when unusual abnormalities of the skin are discovered or when greater expertise is required, consultation with a dermatologist is frequently advisable.

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Case ReportCobb Syndrome: A Case Report and Systematic Review of the Literature

Section snippets

Case Report

Discussion

Segmental cutaneous hemangioma and spinal arteriovenous malformation (Cobb syndrome): Case report and historical perspective

J Neurosurg

A case of Cobb syndrome associated with lymphangioma circumscriptum—Review

Dermatology

Endovascular treatment of spinal cord arteriovenous malformations

Neurosurgery

Angio-architecture of spinal cord arteriovenous shunts at presentation. Clinical correlations in adults and children. The Bicêtre experience on 155 consecutive patients seen between 1981-1999

Acta Neurochir (Wien)

Report of a new case of Cobb syndrome—Meningospinal cutaneous angiomatosis

Acta Neurol Lat Am

Cobb syndrome

Dermatologica

Cobb syndrome treated by staged intravascular embolisation and surgery

Med J Malaysia

Cobb syndrome

J Assoc Physicians India

Classification and surgical management of spinal arteriovenous lesions: Arteriovenous fistulae and arteriovenous malformations

Neurosurgery

Case Report
Cobb Syndrome: A Case Report and Systematic Review of the Literature