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

F Frade¹, N Kadlub, V Soupre

¹Service de chirurgie maxillo-faciale et plastique, faculté de médecine Pierre-et-Marie-Curie Paris-6, hôpital d'enfant Armand-Trousseau, centre hospitalier STARTT, AP-HP, 26, rue du Docteur-Arnold-Netter, 75571 Paris cedex 12, France.

Archives De Pediatrie : Organe Officiel De La Societe Francaise De Pediatrie

|November 22, 2011

Summary

Segmented hemangiomas in infants can indicate serious underlying conditions like LUMBAR syndrome, involving multiple organ systems. Early recognition of these associations is crucial for timely diagnosis and management.

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Area of Science:

Pediatric Dermatology
Medical Genetics
Vascular Malformations

Background:

Pelvic hemangiomas associated with pelvic malformations are known as SACRAL or PELVIS syndrome.
LUMBAR syndrome describes lower-body hemangiomas with cutaneous defects, urogenital, anorectal, arterial, and renal anomalies, plus myelopathy and bony deformities.
Segmented hemangiomas frequently present with extracutaneous abnormalities.

Observation:

Two female infants with infantile hemangioma and genital malformations were studied.
One case involved spinal anomalies, while the other presented with the anus in the genital position.

Findings:

The study reports two cases illustrating the spectrum of infantile hemangioma associations.
These cases highlight the connection between segmented hemangiomas and congenital malformations.

Implications:

PELVIS, SACRAL, and LUMBAR syndromes represent a spectrum of conditions linking segmented hemangiomas to lower-body malformations.
These syndromes, analogous to PHACE syndrome, are often incomplete, necessitating comprehensive evaluation.
Understanding these associations aids in diagnosing and managing complex pediatric cases.