This Article  • Full text  • PDF  • Send to a friend  • Save in My Folder  • Save to citation manager  • Permissions  Citing Articles  • Citation map  • Citing articles on ISI (7)  • Contact me when this article is cited  Related Content  • Similar articles in this journal  Topic Collections  • Pediatrics  • Congenital Malformations  • Neonatology and Infant Care  • Hemangiomas  • Alert me on articles by topic

Céline Girard, MD; Michèle Bigorre, MD; Bernard Guillot, MD; Didier Bessis, MD

Arch Dermatol. 2006;142:884-888.

Background  Large perineal hemangiomas can be associated with congenital anomalies, including anorectal, urinary tract, spine, and external genitalia malformations.

Observations  We describe 2 infants with large sacral and perineal hemangiomas, with severe malformation of the external genitalia with ambiguous genitalia in one case and urinary tract malformations and imperforate anus in the other case. These cases are discussed, along with 9 previously reported cases with similar findings.

Conclusions  Large perineal hemangiomas may constitute a distinctive group of associated anorectal, neurologic, renal or urinary tract, and genital defects. We propose the acronym PELVIS syndrome to emphasize the characteristic findings of this syndrome: perineal hemangioma, external genitalia malformations, lipomyelomeningocele, vesicorenal abnormalities, imperforate anus, and skin tag.

Author Affiliations: Department of Dermatology, Hôpital Saint-Eloi (Drs Girard, Guillot, and Bessis), and Department of Plastic Pediatric Surgery, Hôpital Arnaud de Villeneuve (Dr Bigorre), Centre Hospitalier Régional Universitaire, Montpellier, France.