Presentation
A 29-year-old man presented with cutaneous hemangioma and a progressive paraparesis and paresthesia of the lower extremities. [ncbi.nlm.nih.gov]
Signs and symptoms[edit] The disease is present at birth, but clinical manifestations are often not seen until later in life. [en.wikipedia.org]
Entire Body System
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Arm Pain
We present a 16-year-old boy with undiagnosed Cobb syndrome who developed acute chest and right arm pain followed by severe quadriparesis and respiratory failure; the patient recovered substantially after microsurgical treatment without embolization. [surgicalneurologyint.com]
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Soft Tissue Mass
tissue mass along the right anterior abdominal wall. [casereports.bmj.com]
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Localized Pain
The radiation of pain from the back to the stomach must make one think of aneurysm, gastric ulceration, cholecystitis, or appendicitis, knowing that younger patients have difficulty specifically localizing pain. [healio.com]
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Inguinal Mass
A physical examination showed a palpable 3 3 cm right inguinal mass without tenderness or movement and neck rigidity; muscle strength and tension were normal. [frontiersin.org]
Urogenital
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Incontinence
A 42-year-old man presented with an acute onset of gait disturbance, low-back pain, and urinary incontinence. [ncbi.nlm.nih.gov]
Cauda equina syndrome can present with severe back pain with saddle anesthesia accompanied by a disturbance of bowel and bladder function (overflow incontinence and poor rectal tone). [healio.com]
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Urinary Incontinence
A 42-year-old man presented with an acute onset of gait disturbance, low-back pain, and urinary incontinence. [ncbi.nlm.nih.gov]
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Overflow Incontinence
Cauda equina syndrome can present with severe back pain with saddle anesthesia accompanied by a disturbance of bowel and bladder function (overflow incontinence and poor rectal tone). [healio.com]
Neurologic
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Monoplegia
Examination On examination, the patient presented flaccid paraplegia, with areflexia of the lower limbs, monoplegia of the right arm, and monoparesis of the left arm 2/5 proximal, 1/5 distal. [surgicalneurologyint.com]
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Spinal Cord Syndrome
It is stressed the importance of clinical suspicion of cutaneo-meningospinal angiomatosis based on a spinal cord syndrome in the presence of a vascular skin nevus of the same metameric level. [ncbi.nlm.nih.gov]
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Foot Drop
Examination of the bilateral lower extremity showed marked steppage gait with bilateral external rotation deformities and bilateral foot drop. [casereports.bmj.com]
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Flaccid Paralysis
The first description of this syndrome was reported by Berenbruch in 1890 but it was not until 1915, when Cobb syndrome was first reported in an 8-year-old boy with flaccid paralysis (1), that there started to be noticeable recognition of Cobb syndrome [frontiersin.org]
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Brudzinsky Sign
With regards to the nervous system, Kernig's sign and Brudzinski's sign were both positive, but no typical signs of sensory disorder of the spinal cord were evident. [frontiersin.org]
Workup
Workup for each patient included pre- and postoperative contrast-enhanced MR imaging with vascular sequencing and spinal angiography. [ncbi.nlm.nih.gov]
A thorough workup is recommended by a vascular anomaly specialist after identification. [hemangioma.com]
In such cases, a brain subarachnoid hemorrhage workup is often the starting point. Head CT shows hemorrhage, and a bloody tap will show up if one does an LP. In these cases, a cerebral workup is negative. Do not miss a spinal source! [neuroangio.org]
X-Ray
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Periostal Elevation
Cobb chisel Cobb curet Cobb gouge Cobb method Cobb osteotome Cobb periosteal elevator Cobb retractor Cobb spinal elevator Cobb syndrome - cutaneous angiomas, usually in a dermatomal distribution on the trunk, associated with vascular abnormality of the [medical-dictionary.thefreedictionary.com]
Treatment
Management and treatment Treatment of the osteomuscular malformations involves embolisation (endovascular navigation and occlusion of the arteries feeding the malformation using a biological glue) and/or surgery. [orpha.net]
Her symptoms improved by combined treatment with liquid embolization and corticosteroid therapy. Although Cobb syndrome has been reported in older children, it is extremely rare in infants. [ncbi.nlm.nih.gov]
We emphasize the role of microsurgery because it is the only treatment modality that offers (in selected cases of compact AVMs) complete removal of the malformation and can achieve very good results if diagnosis is followed by treatment in a timely manner [surgicalneurologyint.com]
Prognosis
Prognosis The disease course is unpredictable and the lesions may remain asymptomatic for long periods of time. The documents contained in this web site are presented for information purposes only. [orpha.net]
Prognosis likely depends on the severity of the spinal pathology and the timeliness of diagnosis and intervention. Patients who present with rapidly progressing neurological deficits may have a worse prognosis. Early diagnosis is critical. [emedicine.medscape.com]
[…] symptoms of this disorder may undergo several imaging studies such as MRI, Radiography, Angiography, and CT scans, as well as a thorough work-up by specialists in the fields of Neurology, Neurosurgery, and Interventional Radiology to ensure the best prognosis [nicklauschildrens.org]
Treatment and prognosis Patients should be referred to a neurosurgeon and an interventional radiologist for embolization and decompression. [radiopaedia.org]
Etiology
Etiology The syndrome is not familial or hereditary and no chromosomal anomaly has been described. [orpha.net]
Epidemiology
Summary Epidemiology Less than 100 cases of Cobb syndrome have been reported in the literature. There is no sex predilection. Cobb syndrome represents less than 15% of cases of spinal cord arteriovenous malformations. [orpha.net]
Epidemiology Less than 100 cases of Cobb syndrome have been reported in the literature. There is no sex predilection. Cobb syndrome represents less than 15% of cases of spinal cord arteriovenous malformations. [rarediseases.info.nih.gov]
Pathophysiology
The pathophysiology is similar to spinal pial fistulas, presenting with cord venous congestion. Right T10 injection demonstrates premature opacification of a spinal vein (blue) via a fistula (purple) supplied by the anterior cerebral artery (red). [neuroangio.org]
[…] anatomical variations are hypothesized to predispose to a “flap-valve” occlusion of the natural maxillary sinus ostium wherein the sinus becomes completely occluded allowing gas resorption and development of negative pressure in the sinus antrum. [11] Pathophysiology [eyewiki.aao.org]
Regarding the manifestations in the head and neck area, pathophysiological knowledge could be included in the treatment planning. [egms.de]
Presentation, diagnosis, pathophysiology, and treatment of the neurological features of Sturge-Weber syndrome. Neurologist 2011;17(4):179–184. Crossref, Medline, Google Scholar 25 Welty LD. Sturge-Weber syndrome: a case study. [pubs.rsna.org]
Prevention
Multiple embolizations failed to prevent neurological deterioration, and the patient eventually became wheelchair dependent. [ncbi.nlm.nih.gov]
Recognition of the associated cutaneous lesions with spinal lesions will allow for prevention and treatment of neurological symptoms. [casereports.bmj.com]
The spinal vascular lesions can cause neurologic deficits, including paralysis. [2, 3] Early recognition of the association between the vascular skin lesions and associated spinal lesions may prevent or minimize neurological sequela. [emedicine.medscape.com]
The embolization that occurred was to stop the continued bleeding to prevent a mass effect, and to reduce/eliminate the artereovenous malformation to prevent further embolic complications. [healio.com]