[PELVIS or LUMBAR syndrome: the same entity. Two case reports]

Arch Pediatr. 2012 Jan;19(1):55-8. doi: 10.1016/j.arcped.2011.10.004. Epub 2011 Nov 17.
[Article in French]

Abstract

Introduction: The association of a pelvic hemangioma and malformations in the pelvic region are described as SACRAL or PELVIS syndrome. More recently, the acronym of LUMBAR syndrome has been used to describe the association of lower-body hemangioma and other cutaneous defects, urogenital anomalies, ulceration, myelopathy, bony deformities, anorectal malformations, arterial anomalies, and renal anomalies. We report herein 2 representative cases.

Patients: Two girls presented with infantile hemangioma associated with genital malformation. One case was associated with spinal anomalies and the other one with the anus in the genital position.

Conclusion: Segmented hemangiomas are commonly associated with extracutaneous abnormalities. By analogy with PHACE syndrome, PELVIS/SACRAL/LUMBAR syndromes describe the association of segmented hemangioma of the lower extremities associated with other trunk or lower-body malformations. The syndrome is often incomplete.

Publication types

  • Case Reports
  • English Abstract

MeSH terms

  • Abnormalities, Multiple*
  • Adrenergic beta-Antagonists / administration & dosage
  • Anal Canal / abnormalities*
  • Female
  • Follow-Up Studies
  • Genitalia, Female / abnormalities*
  • Hemangioma / complications*
  • Hemangioma / diagnosis
  • Hemangioma / drug therapy
  • Humans
  • Infant, Newborn
  • Perineum
  • Sacrum / abnormalities*
  • Skin Neoplasms / complications*
  • Skin Neoplasms / diagnosis
  • Skin Neoplasms / drug therapy
  • Syndrome
  • Treatment Outcome

Substances

  • Adrenergic beta-Antagonists