PELVIS and LUMBAR syndromes

Author: Daniel Jun Yi Wong, Medical Student, University of Melbourne, Australia, 2013.

What is PELVIS syndrome?

PELVIS syndrome is a an association between segmental infantile haemangiomas of the perineum (around the genitals and anus) and congenital anomalies involving the genitalia, urinary tract, spine, anus, and rectum.

The acronym PELVIS was proposed in 2006 by Dr. Celine Girard and colleagues.[1] It stands for:

Another group has recently proposed the acronym LUMBAR to describe a collection of similar signs where the hallmark feature is a segmental lumbosacral haemangioma over the middle of the lower back.[2]

There are similarities between LUMBAR and PELVIS syndromes and PHACE syndrome, which may suggest they are regional variations of the same pathological process.

How is PELVIS syndrome diagnosed?

Currently, there are no standardised diagnostic criteria or consensus on the collection of signs required to make a diagnosis of PELVIS syndrome. However, the presence of a segmental haemangioma over the perineum and/or lower back should lead to a search for associated congenital anomalies.

What investigations are required?

Investigations may include:

What is the treatment?

Each child has a unique clinical presentation and will require individual treatment.

Medical care of the infant will be undertaken by a multidisciplinary team that may include plastic surgeon, paediatric urologist, paediatric dermatologist, radiologist, neurosurgeon, and neurologist.

Related information

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