Cobb syndrome
Cobb syndrome | |
---|---|
Synonyms | Cutaneomeningospinal angiomatosis |
Pronounce | N/A |
Specialty | N/A |
Symptoms | Back pain, neurological deficits, skin lesions |
Complications | Paralysis, bladder dysfunction, bowel dysfunction |
Onset | Childhood or adolescence |
Duration | Chronic |
Types | N/A |
Causes | Congenital |
Risks | Spinal cord vascular malformations |
Diagnosis | MRI, CT scan, angiography |
Differential diagnosis | Sturge-Weber syndrome, Klippel-Trenaunay syndrome |
Prevention | N/A |
Treatment | Surgery, embolization, radiotherapy |
Medication | N/A |
Prognosis | Variable, depends on extent of neurological involvement |
Frequency | Rare |
Deaths | N/A |
A rare congenital disorder involving vascular malformations
Classification | |
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External resources |
Cobb syndrome, also known as cutaneomeningospinal angiomatosis, is a rare congenital disorder characterized by the presence of vascular malformations affecting the skin, spinal cord, and vertebrae. This condition is named after Dr. Stanley Cobb, who first described it in 1915.
Presentation[edit | edit source]
Cobb syndrome typically presents with a combination of cutaneous and spinal manifestations. The hallmark of the condition is the presence of a cutaneous hemangioma or vascular malformation on the skin, usually located on the back. This skin lesion is often associated with underlying spinal cord and vertebral involvement.
Cutaneous Manifestations[edit | edit source]
The skin lesions in Cobb syndrome are usually present at birth or appear in early childhood. They are typically red or purple in color and may vary in size. These lesions are often located in the midline of the back, corresponding to the level of the spinal cord involvement.
Neurological Manifestations[edit | edit source]
The neurological symptoms of Cobb syndrome arise from the involvement of the spinal cord. Patients may experience neurological deficits such as weakness, sensory loss, or paralysis in the areas of the body below the level of the spinal lesion. The severity of these symptoms can vary depending on the extent of the spinal cord involvement.
Pathophysiology[edit | edit source]
Cobb syndrome is caused by the presence of abnormal blood vessels, known as arteriovenous malformations (AVMs), that affect the skin, spinal cord, and vertebrae. These AVMs can lead to increased blood flow and pressure in the affected areas, resulting in damage to the spinal cord and surrounding structures.
Diagnosis[edit | edit source]
The diagnosis of Cobb syndrome is based on the clinical presentation and imaging studies. Magnetic resonance imaging (MRI) of the spine is the preferred method for visualizing the spinal cord and identifying any vascular malformations. Angiography may also be used to assess the blood vessels and confirm the presence of AVMs.
Treatment[edit | edit source]
The management of Cobb syndrome involves addressing both the cutaneous and neurological components of the disorder. Treatment options may include:
- Surgical intervention: In some cases, surgery may be necessary to remove or reduce the size of the vascular malformations, particularly if they are causing significant neurological symptoms.
- Endovascular therapy: This minimally invasive procedure involves the use of catheters to deliver agents that can block or reduce blood flow to the AVMs.
- Symptomatic management: Physical therapy and rehabilitation may be required to manage neurological deficits and improve the patient's quality of life.
Prognosis[edit | edit source]
The prognosis for individuals with Cobb syndrome varies depending on the severity of the spinal cord involvement and the success of treatment interventions. Early diagnosis and appropriate management are crucial for improving outcomes and minimizing neurological complications.
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Contributors: Deepika vegiraju, Prab R. Tumpati, MD