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Vascular and Neurogenic-Cobb Syndrome

Bajaj A* Mini Review Consultant Histopathologist, A.B.Diagnostics, India Volume 5 Issue 1 *Corresponding author: Anubha Bajaj, Consultant Histopathologist, A.B.Diagnostics, A-1, Received Date: November 09, 2020 Ring Road, Rajouri Garden, New Delhi, 110027, India, Tel: 00911141446785; Email: anubha. Published Date: November 20, 2020 [email protected] DOI: 10.23880/oajg-16000206

Abstract

Cobb syndrome is an exceptional, non-inherited, genetic disorder characteristically constituted by vascular anomalies and

neurological deficits. Spinal arteriovenous malformations appear in concurrence with cutaneous vascular lesions within the corresponding dermatome. Dermatome specific port wine stain upon the trunk, arteriovenous malformation, angioma, angiokeratoma, angiolipoma, cavernous haemangioma or lymphatic malformation is discerned in accompaniment with (MRI),hyperreflexia, computerized limb paresis, tomography muscular (CT) cramps, scan, plain sensory radiography loss, bladder or angiography. and bowel dysfunction, Cobb syndrome sudden can paraplegia be appropriately or subarachnoid managed haemorrhage. Spinal vascular lesions of Cobb syndrome can be adequately determined with magnetic resonance imaging

with sclerotherapy, endovascular embolization, oral corticosteroids or surgical extermination of vascular lesions. Keywords: Cobb Syndrome; Magnetic Resonance Imaging; Computerized Tomography

Mini Review birth whereas neurological symptoms emerge around 5 Cobb syndrome is denominated as an extremely exceptional genetic disorder characteristically constituted by years. Incriminated children lack a family history of Cobb syndrome. Of obscure aetiology, Cobb syndrome probably emerges from somatic mutations within the neural crest vascular anomalies and neurological deficits. Cobb syndrome or mesoderm with consequent, antecedent, anatomic withas a a non- cutaneous inherited lesion. disorder Cobb syndrome delineates can a also concurrence appear as of a manifestations within the developing embryo [1,2]. Port spinal arteriovenous malformation in a metamere coincident notwine demonstrate stain of varying a palpable magnitude or canaudible be demonstrated thrill overlying on metameric connections [1]. Additionally termed as “spinal buttock, trunk or thigh. Vascular anomalies may or may arteriovenouscomponent of multiplemetameric arteriovenous syndrome” (SAMS)shunts demonstratingor “cutaneous meningospinal angiomatosis”, Cobb syndrome was initially incriminated zones. Vascular aberrations appear blue at described by Berenbauch in 1890. However, the syndrome birth and fade gradually [1,2]. Cutaneous manifestations of Cobb syndrome vary from macular, port wine stain to diverse appearing in association with cutaneous nevi as described by categories of papular or nodular vascular lesions such as Cobbexemplifies in 1915 a specific [1,2]. clinical setting of spinal haemangiomata vascularangioma, lesions angiokeratoma, contingent angiolipoma to Cobb andsyndrome lymphangioma do not resolvecircumscriptum. or involute Unlike spontaneously. capillary or cavernous Cutaneous haemangioma, vascular Disease Characteristics concerns or susceptibility to traumatic haemorrhage Cobb syndrome or cutaneous meningospinal malformations are observed incidentally or due to cosmetic angiomatosis enunciates spinal arteriovenous [1,2]. Commencement of vascular supply to embryological cutaneous lesions within the corresponding dermatome. bloodvertebrae vessels and engendering spinal cord cutaneomeningospinal emerges from segmental angiomas dorsal malformations occurring in concurrence with vascular arteries. Thus, a common metameric origin of arteriovenous

Cutaneous manifestations of Cobb syndrome appear at can be contemplated. Vascular cutaneous nevus cogitated Vascular and Neurogenic-Cobb Syndrome J Gynecol 2 Open Access Journal of Gynecology in Cobb syndrome is contingent to several, diverse vascular as with muscular paralysis. Morphological or structural physiological reflexes besides sensory and motor deficits pathologies. Intra-spinal lesions are usually high-flow, symptoms can appears as intermittent episodes, gradual syndromearteriovenous can malformationsbe subdivided andinto exceptionally vascular neoplasia appear anomalies of central nervous system can ensue. Neurological as low -flow angiomas. Vascular anomalies with Cobb progressive symptoms or as a sudden onset of pertinent such as haemangiomas and vascular malformations [3,4]. clinical settings. Structural anomalies of the urinary tract can Vascular malformations are generally subcategorized occur. Concordance of a port-wine stain with spinal vascular as a) slow or low- flow lesions and b) fast or high- flow lesions is a significant feature in accurate discernment vascular malformations. Low- flow vascular malformations of Cobb syndrome and is commonly detected whereas birthdemonstrate or during an early intermingling childhood, ofare capillary,gradually venousprogressive and limbrepresenting paresis, symptomsmuscular crampssuch as inpain extremities, and motor sensory deficits loss are lymphatic components. The malformations can appears at non-specific [4,5]. Subjects can depict a sudden onset of Cobb syndrome can demonstrate complications such as a areand therapeutically dimensions may amenable increase withto compression crying or adoption garments, of extending up to pelvis besides bladder and bowel dysfunction. Valsalva’s manoeuver. Low-flow vascular malformations concurrence of kyphosis and scoliosis, bone and joint pain sclerotherapy or surgical extermination of the lesion. Cogent andon accountFoix-Alajouanine of spinal syndrome. incrimination, Exceptionally, vascular thrombosiscongestive therapy can alleviate clinical symptoms of pain, swelling or within arteriovenous malformations, necrotic myelopathy life threatening vascular malformations as denominated with Cobb syndrome is accompanied with diverse clinical airway compromise [3,4]. High- flow vascular malformations heart failure and gangrene can ensue [5]. Symptomatic symptomsare comprised such of asarterial pain, componentsulceration, ischemicwithin arteriovenous alterations, Cutaneous lesions can indicate Cobb syndrome, especially anomalies or arteriovenous fistula and can manifest representations and disease course is often unpredictable. upon the cutis with accompanying vascular murmur or thrill. paraplegia or subarachnoid haemorrhage [4-8]. haemorrhage, congestive heart failure and warm pink patches when appearing in concurrence with sudden onset of

High-flow vascular malformations can be adequately treated with surgical eradication of the lesion or embolization [3,4]. Neurological deficits with flaccidity and paresis of upper and lower limbs is enunciated. Deep tendon reflexes or plantar reflexes can be absent. Cobb syndrome is typically discerned following onset of cogent neurological symptoms which appear gradually over a period of weeks or years although a sudden commencement of rapidly progressive muscle paresis can ensue. Neurological representations can vary from monoparesis to sudden onset paraplegia or quadriplegia. Bladder and bowel dysfunction is common although delayed Figure 1: and appears with disease progression. Physical signs such as cell extravasation, cutaneous adnexal structures and a meningismus, headache, fever, gluteal and limb hypertrophy superimposed Port acanthotic wine stain epithelium with vascular [9]. dilatation, red activatingare infrequent mechanisms [4,5]. Cord incurring compression myelopathy due to spinalsuch as angioma venous hypertension,can induce specific compression neurological and cord symptoms ischemia inarising addition due to steal syndrome [4].

Clinical Elucidation Although the disorder appears is late childhood, no represents multiple vascular lesions. Capillary vascular age of disease occurrence is exempt. Cobb syndrome malformations, as designated with port wine stain, demonstrates a dermatome specific distribution upon Figure 2: the trunk. Arteriovenous malformations, angioma, angiokeratoma, angiolipoma, cavernous haemangioma or Capillary malformation with vascular distension, lymphatic malformations are also delineated. Neurological encompassing, mildly inflamed fibrous tissue stroma and involvement exemplifies as hyperreflexia with overactive acanthosis of the superimposed epithelium [10]. Bajaj A. Vascular and Neurogenic-Cobb Syndrome J Gynecol 2020, 5(1): 000206. Copyright© Bajaj A. 3 Open Access Journal of Gynecology

Figure 3: Cavernous haemangioma with enlarged Figure 6: Cavernous haemangioma with enlarged, patent

tissuevascular stroma spaces [11]. lined with flattened endothelium, red cell vascular configurations, red cell extravasation and a mildly aggregation and an intervening, mildly inflamed fibrous inflamed, intervening fibrotic stroma [14].

Figure 7: Figure 4: lined by plump endothelium with intra-cavitary projections by plump endothelium with projections, partially solid Tufted angioma depicting vascular arrangements Tufted angioma with vascular spaces lined and a circumscribing, inflamed fibrous tissue stroma [15]. vascular configurations and an encompassing, inflamed fibrous tissue stroma [12].

Figure 8: Figure 5: Cherry angioma demonstrating vascular disseminated within the upper dermis with a superimposed Port wine stain with dilated capillaries collapsed vascular articulations and a superimposed configurations, red cell accumulation, and patent and squamous epithelium showing acanthosis, hyperkeratosis and elongation of rete ridges [16]. squamous epithelium [13]. Bajaj A. Vascular and Neurogenic-Cobb Syndrome J Gynecol 2020, 5(1): 000206. Copyright© Bajaj A. 4 Open Access Journal of Gynecology

especiallyTherapeutic a spinal vascular Options configuration [6].

syndromeOn account remain of limitedimprecise. discernible Cobb instances,syndrome definitivecan be appropriatelymanagement andmanaged therapeutic with sclerotherapy, recommendations endovascular of Cobb

canembolization partially improve and surgical and cutaneous extermination nevus can of partly the vascular regress lesion. Oral steroids can be administered [7,8]. Paraparesis

following administration of oral prednisolone. Endovascular Figure 9: Haemangioma with dilated vascular procedureembolization wherein of feeding postoperative blood vessels neurological with the deterioration employment isof absent. n-butyl-2-cyanoacrylate Continual therapy (NBCA) with canoral be prednisolone adopted, a and cogent physiotherapy is advantageous. Supportive configurations impacted with red cells and an intermingled, inflamedInvestigative fibrous tissue Assay stroma [17]. therapeutic measures in the form of physical therapy and Cobbapplication syndrome of compression is contingent garmentsto proportionate for capillary, incrimination venous multiple, cutaneous vascular lesions and neurological or lymphatic anomalies can be adopted [7,8]. Severity of Cobb syndrome is discerned due to concurrence of preliminary disease discernment and suitable therapeutic interventionand seriousness is mandated of spinal andto circumvent neurological the implication. disorder or A xanthochromia,deficits. Cobb syndrome minimal can cell be diagnosticallycount and a confirmedlymphocytic on minimise neurological complications. Optimal management cogent imaging studies. Lumbar puncture can demonstrate vascularpredominance. lesions Proteinsassociated are with elevated Cobb syndrome whereas glucosecan be of Cobb syndrome is debatable. Appropriation of a superior levels of cerebrospinal fluid can be normal [5,6]. Spinal therapeutic algorithm can be challenging. Definitive surgical (MRI), although computerized tomography (CT) scan, optionsextermination are palliation of the lesion without is of surgical limited intervention, benefit as morbidity surgical plainadequately radiography determined or angiography with a magnetic can resonancealso be employed imaging due to surgical procedures is of concern. Cogent therapeutic lumbar spine can reveal an enlarged arteriovenous eradication and a contemporary procedure of endovascular [5,6]. Magnetic resonance imaging (MRI) of dorsal and embolization [7,8]. Efficacy of endovascular therapy for with accompanying haematoma within spinal cord conus. spinal arteriovenous malformations in Cobb syndrome malformation or haematomyelia within the spinal cord requires evaluation. Ambulation with alleviation of extensive artery to enter the dorsal spinal canal through cogent paraplegia can be achieved with successful endovascular Dilated and tortuous blood vessels can arise from intercostal usuallyembolization extensive in enlarged and appropriate spinal arteriovenous cure may not malformations be achieved discerned upon post- contrast T1 weighted images [5,6]. of Cobb syndrome. Spinal arteriovenous malformations are Computerizedneural foramina. tomography Intrathecal (CT) vascular and enhancementmagnetic resonance can be therapeutic modalities and surgical intervention can induce upon multiple, spinal levels. Thus, adoption of combined lesion. Magnetic resonance imaging (MRI) is superior to imaging (MRI) are advantageous in assessing the extent of a cessation of disease progression and minimize neurological alongsequelae the by spinal decimating cord [7,8]. factors Surgical such asextermination mass effect, venouscan be computerized tomography (CT) for delineating deformed hypertension and enunciation of vascular steal syndrome Magneticblood vessels, resonance angiomas imaging and (MRI) feeding can demonstrate arteries. Ultimate signal alterationsassessment withinof Cobb the syndrome intramedullary is contingent canal to and angiography. majority Endovascularbeneficially combinedembolization with can endovascular be employed embolizationconcurrently for treating enlarged spinal arteriovenous malformations. angiographyof blood vessels with andintravascular is contemplated contrast. as However, a superior, selective safer with pre-embolization steroid therapy. Various treatment diagnostic option, in contrast to adoption of an invasive modalities can be efficaciously employed with steroids to architecture, pathophysiology and adaptable embolization circumvent disease progression with acquisition of certain spinal angiography facilitates exemplification of complex degree of neurological function and decline in quantifiable embolization procedures [7,8]. Administration of systemic procedures of cogent vasculature [5,6]. Lumbar puncture is corticosteroids is efficacious in adjunctive cutaneous contraindicated in the evaluation of suspected spinal lesion vascular malformation such as Kasabach-Merritt syndrome. Bajaj A. Vascular and Neurogenic-Cobb Syndrome J Gynecol 2020, 5(1): 000206. Copyright© Bajaj A. 5 Open Access Journal of Gynecology

Corticosteroids are empirically administered in the (2011) Combined endovascular and surgical resection exceptionally delineated Cobb syndrome and therapeutic a patient with Cobb syndrome. J Neurointerv Surg 3(3): endovascular embolization and oral corticosteroids can 293-296.of a giant lumbosacral arteriovenous malformation in decimateoutcomes morbidity require and further ensure evaluation. a rapid recovery Concurrence [7,8]. of 7.

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Bajaj A. Vascular and Neurogenic-Cobb Syndrome J Gynecol 2020, 5(1): 000206. Copyright© Bajaj A.