LUMBAR syndrome

Acronym for Lower body hemangioma, Urogenital anomalies, Ulceration, Myelopathy, Bony deformities, Anorectal malformations, arterial anomalies and Renal anomalies.

Association of a regional hemangioma (linear boundaries or reticulated appearance), in the lumbar, sacral, perineal region and/or to the level of a lower limb with one of the following regional malformations:

-        anomaly of the spinal cord (83%): tethered cord,  sacrolombar lipoma, dysraphism

-        anorectal malformation (if lumbar area): imperforate or anteriorized anus, fistula, cloaca

-        urogenital malformation (if sacral or perineal zone): single kidney, vesicoureteral reflux, vaginal duplication or atresia, hypospadias, ambiguous genitalia

-        bone malformation: pes talipes, scoliosis, length anomaly of the affected limb

-        arterial malformations (if lower limb): stenosis, abnormal anatomic path, persistence of embryonic anastomoses

-        skin sores (70%) at the level of the hemangioma especially if it has progressed

A few cases of ischemia and necrosis of the distal part of a limb have been reported.

Female predominance (1.7: 1), as in the PHACE syndrome.


Anesthetic implications:

exclude the presence of vertebral or spinal deformity before making a neuraxial block; maintain a normal perfusion pressure in the affected limb (arterial stenoses or malformations).


References : 


Updated: January 2019