( Cutaneomeningospinal angiomatosis, SAMS 1-31)

Very rare. Spinal cord vascular anomaly (arteriovenous malformation) associated within the same metamere to:

-         a cutaneous vascular malformation (port-wine stain, venular lesions or angiokeratoma) 

-         a muscular and/or osseous arteriovenous malformation 

Usually, the malformation is asymptomatic, but it may present in childhood as monoparesis, brutal quadriplegia, meningismus due to an acute hemorrhagic accident or venous congestion. The neurological symptomatology depends on the localization of the  malformation: either cervical, thoracic, lumbar, or sacral area. Hypertrophy of a buttock or lower limb can also be observed. 

Adjacent metameres can be involved: this is the reason why sometimes it is called  SAMS 1-31 syndrome, acronym for Spinal Arteriovenous Metameric Syndrome 1-31.

Treatment: embolization, rarely surgery.

Anesthetic implications: 

consider this pathology in case of a metameric paravertebral skin lesion: in case of port-wine stain of metameric topography alongside the neuraxis, do not realize a  neuraxial block without prior neuraxial imaging (angioNMR). Check hemostasis (preoperative D-dimers and fibrinogen level to detect a possible local chronic DIC), risk of hemorrhage or air embolism.

References : 

-        Clark MT, Brooks EL, Chong W, Pappas C, Fahey M. 
Cobb syndrome : a case report and systematic review of the literature. 
Pediatr Neurol 2008; 39: 423-5.

-         Soeda A,Sakai N, Iihara K, Nagata I. 
Cobb syndrome in an infant: treatment with endovascular embolization and corticosteroid therapy: a case report.
Neurosurgery 2003; 52: 711-5.

-         Dilmé-Carreras E, Iglesias-Sancho M, Marquez-Balbas G, Sola-Ortigosa J, Umbert-Milet P. 
Cobb syndrome: case report and review of literature. 
Dermatology 2010; 221: 110-2. 

-        Minagar M, Dehghan-Tezerjani M, Khan ZH.
Anesthetic considerations in Cobb syndrome : a case report.
A&A Practice 2019 ; 13 : 137-9.

Updated: September 2019