LUMBAR syndrome
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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