SACRAL Syndrome: Spinal Dysraphism, Anogenital, Cutaneous, Renal and Urologic Anomalies, Associated with an Angioma of Lumbosacral Localization

Abstract

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). Conclusion: 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.

SACRAL Syndrome

“…We agree that the association between cutaneous hemangioma of the lumbosacral area and occult dysraphism was first reported by Golberg and Esterly [1] , and this paper was referenced as number 6 in our article. However, concerning the 2006 paper published by our French colleagues in the Archives of Dermatology [2] , where they proposed the acronym PELVIS, we want to clarify that this work was not yet published when our paper was submitted.…”

“…We read the article SACRAL Syndrome: Spinal Dysraphism, Anogenital, Cutaneous, Renal and Urologic Anomalies, Associated with an Angioma of Lumbosacral Localization [1] . The association between cutaneous hemangiomas in the lumbosacral area and underlying developmental anomalies was first reported by Goldberg and Esterly over 20 years ago and the acronym PELVIS was recently published to emphasize this well-recognized association [2,3] .…”

The role of propranolol in the treatment of infantile hemangioma

“…6,7 In most cases only parental education and reassurance are required. Although 85---90% of all infantile hemangiomas eventually undergo spontaneous involution, a minority can still cause disfigurement and serious complications, depending on their location (obstruction of airways and vision), size, and speed of regression, which can be associated with painful ulcerations and hemorrhage or even highoutput heart failure.…”

Vascular anomalies of the male genitalia

“…An ulcerated IH of the male genitalia is at risk for permanent scarring and disfigurement that could potentially lead to a negative self-image. The association of perineal IH with urogenital, anorectal, and spinal malformations is well known [9][10][11][12][13][14]. The IHs seen in conjunction with anogenital malformations are frequently superficial with a distinctive reticular network [9].…”