SACRAL syndrome: spinal dysraphism, anogenital, cutaneous, renal and urologic anomalies, associated with an angioma of lumbosacral localization

Dermatology. 2007;214(1):40-5. doi: 10.1159/000096911.


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.

MeSH terms

  • Anal Canal / abnormalities*
  • Diagnosis, Differential
  • Female
  • France / epidemiology
  • Hemangioma / complications*
  • Hemangioma / epidemiology
  • Hemangioma / pathology
  • Humans
  • Incidence
  • Infant
  • Infant, Newborn
  • Lumbosacral Plexus
  • Male
  • Perineum
  • Retrospective Studies
  • Sex Distribution
  • Skin Abnormalities / complications*
  • Skin Abnormalities / diagnosis
  • Spinal Dysraphism / complications*
  • Spinal Dysraphism / diagnosis
  • Syndrome
  • Urogenital Abnormalities / complications*
  • Urogenital Abnormalities / diagnosis