Elsevier

The Journal of Pediatrics

Volume 157, Issue 5, November 2010, Pages 795-801.e7
The Journal of Pediatrics

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

https://doi.org/10.1016/j.jpeds.2010.05.027Get rights and content

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.

Section snippets

Methods

Institutional review board (IRB) approval was obtained for this study, allowing for the collection of non-identifiable patient information from outside institutions. Because of the known rarity of this association, a call for unpublished cases of lumbosacral or perineal IH associated with at least 1 other regionally present, congenital anomaly was made through the Society for Pediatric Dermatology membership listserve. Specific information requested included patient's sex, a description of the

Results

Table I (available at www.jpeds.com) and Figures 3 and 4 describe the clinical features of 24 new, unpublished cases. There were 15 female and 9 male patients, resulting in a sex ratio of 1.7 to 1. The most common IH location was region B (sacral; 20/24; 83.3%), followed by regions A (lumbar; 18/24; 75%) and C (perineum/genitals; 16/24; 66.6%). Region D (lower extremity) was affected in 10 of 24 cases (41.7%). One case (case 1) had an additional retroperitoneal IH. Most patients (66%) had

Discussion

The term “segmental” or what some authors refer to as “regional” describes IHs that show linear patterning, geographic patterning, or both over a specific cutaneous territory, respecting embryologic anatomic boundaries, rather than appearing to arise from a single focal point. Segmental IHs show reproducible patterns associated with developmental segments, which arise from the neuroectoderm and later correspond to a specific region of skin and soft tissues.7 Clinically, segmental IH can

References (24)

  • A.N. Haggstrom et al.

    Segmental hemangioma: an important clinical term

    Am J Med Genet A

    (2008)
  • A. Patrizi et al.

    Sacral medial telangiectatic vascular nevus: a study of 43 children

    Dermatology

    (1996)
  • Cited by (0)

    The authors declare no conflicts of interest.

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