TY - JOUR
T1 - LUMBAR
T2 - Association between cutaneous infantile hemangiomas of the lower body and regional congenital anomalies
AU - Iacobas, Ionela
AU - Burrows, Patricia E.
AU - Frieden, Ilona J.
AU - Liang, Marilyn G.
AU - Mulliken, John B.
AU - Mancini, Anthony J.
AU - Kramer, Daniela
AU - Paller, Amy S.
AU - Silverman, Robert
AU - Wagner, Annette M.
AU - Metry, Denise W.
N1 - Copyright:
Copyright 2018 Elsevier B.V., All rights reserved.
PY - 2010/11
Y1 - 2010/11
N2 - Objective: To define the clinical spectrum of regional congenital anomalies associated with large cutaneous hemangiomas of the lower half of the body, clarify risk for underlying anomalies on the basis of hemangioma location, and provide imaging guidelines for evaluation. Study design: We conducted a multi-institutional, retrospective case analysis of 24 new patients and review of 29 published cases. Results: Hemangiomas in our series tended to be "segmental" and often "minimal growth" in morphology. Such lesions were often extensive, covering the entire leg. Extensive limb hemangiomas also showed potential for extracutaneous anomalies, including underlying arterial anomalies, limb underdevelopment, and ulceration. The cutaneous hemangioma and underlying anomalies demonstrated regional correlation. Myelopathies were the most common category of associated anomalies. Conclusions: We propose the acronym "LUMBAR" to describe the association of Lower body hemangioma and other cutaneous defects, Urogenital anomalies, Ulceration, Myelopathy, Bony deformities, Anorectal malformations, Arterial anomalies, and Renal anomalies. There are many similarities between LUMBAR and PHACE syndrome, which might be considered regional variations of the same. Although guidelines for imaging are suggested, prospective studies will lead to precise imaging recommendations and help determine true incidence, risk and long-term outcomes.
AB - Objective: To define the clinical spectrum of regional congenital anomalies associated with large cutaneous hemangiomas of the lower half of the body, clarify risk for underlying anomalies on the basis of hemangioma location, and provide imaging guidelines for evaluation. Study design: We conducted a multi-institutional, retrospective case analysis of 24 new patients and review of 29 published cases. Results: Hemangiomas in our series tended to be "segmental" and often "minimal growth" in morphology. Such lesions were often extensive, covering the entire leg. Extensive limb hemangiomas also showed potential for extracutaneous anomalies, including underlying arterial anomalies, limb underdevelopment, and ulceration. The cutaneous hemangioma and underlying anomalies demonstrated regional correlation. Myelopathies were the most common category of associated anomalies. Conclusions: We propose the acronym "LUMBAR" to describe the association of Lower body hemangioma and other cutaneous defects, Urogenital anomalies, Ulceration, Myelopathy, Bony deformities, Anorectal malformations, Arterial anomalies, and Renal anomalies. There are many similarities between LUMBAR and PHACE syndrome, which might be considered regional variations of the same. Although guidelines for imaging are suggested, prospective studies will lead to precise imaging recommendations and help determine true incidence, risk and long-term outcomes.
KW - IH
KW - Infantile hemangioma
KW - MG
KW - MRA
KW - MRI
KW - MRV
KW - Magnetic resonance angiography
KW - Magnetic resonance imaging
KW - Magnetic resonance venography
KW - Minimal growth
KW - OMIM
KW - Online Mendelian Inheritance in Man database
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U2 - 10.1016/j.jpeds.2010.05.027
DO - 10.1016/j.jpeds.2010.05.027
M3 - Article
C2 - 20598318
AN - SCOPUS:77958028527
SN - 0022-3476
VL - 157
SP - 795-801.e7
JO - journal of pediatrics
JF - journal of pediatrics
IS - 5
ER -