Report of a case of Cobb syndrome: multimodality imaging.

Dalia Ibrahim,Shady Mashhour

doi:10.1259/bjrcr.20200145

Abstract

Cobb syndrome is a rare vascular disorder characterized by vascular skin lesions distributed in a dermatomal pattern, with corresponding muscular, osseous, paraspinal, and/or spinal vascular lesions occurring at the same body somite (metamere). We present a case of a 25-year-old man who presented with a history of right upper limb paresthesia followed by bilateral progressive upper and lower limb weakness and heaviness. Physical examination showed large cutaneous port wine stains on the right side of the chest, the nape, and along the whole right upper limb in a dermatomal distribution, with no corresponding limb hypertrophy or asymmetry. MRI and CT scan of the cervical spine showed aggressive vertebral hemangiomas involving the right side of C1 down to C4 vertebrae associated with extraosseous epidural lesion causing cervical cord compression, in addition to right paraspinal muscular low flow vascular malformations. Digital subtraction angiography of the neck vessels showed corresponding vascular blush and delayed contrast pooling in the affected regions. Cobb syndrome was diagnosed based on the dermatomal distribution of the cutaneous vascular lesions and the corresponding vertebral, epidural, and paraspinal vascular lesions occurring at the same metamere. The patient underwent a decompressive laminectomy at C2–C6 levels with removal of the epidural lesion, after which his symptoms had improved.

Highlights

Cobb syndrome is a rare vascular disorder characterized by vascular skin lesions distributed in a dermatomal pattern, with corresponding muscular, osseous, paraspinal, and/or spinal vascular lesions occurring at the same body somite
A 25-year-o ld male presented with paresthesia of the right upper limb, followed by bilateral progressive upper and lower limb heaviness and weakness evolving over a period of 3 months
The pathogenesis of neurological symptoms in Cobb syndrome is believed to be due to a variety of factors, including cord compression by the vascular malformations, blood steal syndrome resulting in cord ischemia, and venous hypertension.[3,4]

Summary

CASE PRESENTATION

A 25-year-o ld male presented with paresthesia (tingling and numbness) of the right upper limb, followed by bilateral progressive upper and lower limb heaviness and weakness evolving over a period of 3 months. Physical examination demonstrated large cutaneous port wine stains (capillary vascular malformations) involving the right side of the chest, the nape, and along the ventrolateral aspect of the whole right upper limb distal to the thumb in a dermatomal distribution, with no size discrepancy between both upper limbs (Figure 1). The patient demonstrated reduced muscle strength (Grade 3/5) of both hands and legs and decreased sensation (Grade 4/5) globally from his C3 dermatome down. He was hyperreflexic in the lower extremities. CT scan of the involved vertebrae demonstrated typical features of osseous hemangiomas including bone expansion and accentuation of the trabecular bone markings, forming the pathognomonic “polka–dot” sign (Figure 3)

Ibrahim and Mashhour

DISCUSSION

LEARNING POINTS