Ann Dermatol.  2023 May;35(Suppl1):S88-S92. 10.5021/ad.21.183.

A Case of Perineal Hemangioma, External Genitalia Malformations, Lipomyelomeningocele, Vesicorenal Abnormalities, Imperforate Anus, and Skin Tag (PELVIS) Syndrome with Extensive Perineal Infantile Hemangioma

• Hwang HW
• Lee SB
• Shin J
• Choi GS
• Byun JW

• KMID: 2542748
• DOI: http://doi.org/10.5021/ad.21.183

Abstract

PELVIS syndrome describes the constellation of perineal hemangioma, external genitalia malformations, lipomyelomeningocele, vesicorenal abnormalities, imperforate anus, and skin tag. A 2-month-old girl presented with infantile hemangioma on her perineum and genitalia with imperforate anus, rectovaginal fistula and perineal skin tag at birth. Under the impression of PELVIS syndrome, consequential spinal sonography was conducted and revealed an intrasacral meningocele without clinical neurologic deficit. The anorectal malformation was surgically corrected, she was taking oral propranolol for the cutaneous lesion, and she showed improvement and no complications.

Keyword

Congenital abnormalities; Hemangioma; Imperforate anus; Infant; Propranolol