LUMBAR: Association between Cutaneous Infantile Hemangiomas of the Lower Body and Regional Congenital Anomalies

被引:112
作者
Iacobas, Ionela [1 ]
Burrows, Patricia E. [2 ]
Frieden, Ilona J. [3 ]
Liang, Marilyn G. [4 ]
Mulliken, John B. [5 ]
Mancini, Anthony J. [6 ,7 ]
Kramer, Daniela [8 ]
Paller, Amy S. [6 ,7 ]
Silverman, Robert [9 ]
Wagner, Annette M. [6 ,7 ]
Metry, Denise W. [10 ]
机构
[1] Baylor Coll Med, Dept Pediat Hematol Oncol, Houston, TX 77030 USA
[2] Baylor Coll Med, Dept Pediat Intervent Radiol, Houston, TX 77030 USA
[3] Univ Calif San Francisco, Dept Dermatol, San Francisco, CA 94143 USA
[4] Harvard Univ, Sch Med, Dept Dermatol, Boston, MA 02115 USA
[5] Harvard Univ, Sch Med, Dept Surg, Childrens Hosp, Boston, MA 02115 USA
[6] Northwestern Univ, Dept Dermatol, Feinberg Sch Med, Chicago, IL 60611 USA
[7] Northwestern Univ, Dept Pediat, Feinberg Sch Med, Chicago, IL 60611 USA
[8] Hosp Ninos Luis Calvo Mackenna, Santiago, Chile
[9] Georgetown Univ, Dept Pediat, Washington, DC 20057 USA
[10] Baylor Coll Med, Dept Dermatol, Houston, TX 77030 USA
关键词
PHACE SYNDROME; SPINAL DYSRAPHISM; MALFORMATION;
D O I
10.1016/j.jpeds.2010.05.027
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
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. (J Pediatr 2010; 157:795-801).
引用
收藏
页码:795 / U140
页数:14
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