What are PELVIS or SACRAL syndromes in infantile hemangioma?

Updated: Feb 06, 2019
  • Author: Richard J Antaya, MD; Chief Editor: William D James, MD  more...
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Answer

Segmental infantile hemangiomas involving the perineal area may be associated with other underlying congenital anomalies as delineated in the PELVIS or SACRAL syndromes. [39] The acronymic PELVIS syndrome describes the association of a perineal hemangioma with any of the following: external genital malformations, lipomyelomeningocele, vesicorenal abnormalities, imperforate anus, or skin tag. SACRAL syndrome is spinal dysraphism with anogenital, cutaneous, renal, and urologic anomalies, associated with an angioma of lumbosacral localization.

An isolated midline lumbosacral hemangioma may be a cutaneous marker for underlying occult spinal dysraphism, the most common being an intraspinal lipoma with resultant tethered cord. Spinal imaging should be performed in these cases. Symptoms may not occur for several years and which infants require corrective surgery has been debated, because as many as 10% of the population have asymptomatic tethering of the spinal cord. The decision should be left to a neurosurgeon with experience with this condition.


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