SACRAL syndrome: spinal dysraphism, anogenital, cutaneous, renal and urologic anomalies, associated with an angioma of lumbosacral localization.
BACKGROUND
Publications concerning perineal infantile hemangiomas are scarce, and comprise no large series.


OBJECTIVE
Studying clinical features of hemangiomas of the perineal area, complications and associated malformations.


METHODS
Retrospective analysis of all hemangiomas localized in the perineal area, encountered at the Children's Hospital in Bordeaux from 1994.


RESULTS
Of 49 perineal hemangiomas (34 girls, 15 boys), 5 patients had accompanying malformation, mainly lipomyelomeningocele with tethered cord. The superficial hemangiomas were more represented in males and presented sooner than the nodular counterpart. The average rate of ulceration was 73%, ulcerations developed earlier in the superficial forms than their nodular counterparts (2 vs. 4 months).


CONCLUSIONS
Superficial perineal hemangiomas are more often complicated by ulceration, and are associated with developmental anomalies. As a counterpart for the PHACE syndrome in facial hemangioma, we propose the acronym SACRAL for perineal hemangiomas: Spinal dysraphism, Anogenital anomalies, Cutaneous anomalies, Renal and urologic anomalies, associated with Angioma of Lumbosacral localization.
Stockman
Annelies
A
Pediatric Dermatology Unit, Hôpital Pellegrin-Enfants, Bordeaux, France.
Boralevi
F
F
Taïeb
Alain
A
Léauté-Labrèze
Christine
C
D001003:Anal Canal / Q000002:abnormalities*; D003937:Diagnosis, Differential; D005260:Female; D005602:France / Q000453:epidemiology; D006391:Hemangioma / Q000150:complications* / Q000453:epidemiology / Q000473:pathology; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D008160:Lumbosacral Plexus; D008297:Male; D010502:Perineum; D012189:Retrospective Studies; D017678:Sex Distribution; D012868:Skin Abnormalities / Q000150:complications* / Q000175:diagnosis; D016135:Spinal Dysraphism / Q000150:complications* / Q000175:diagnosis; D013577:Syndrome; D014564:Urogenital Abnormalities / Q000150:complications* / Q000175:diagnosis