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Published on: May 1, 2015
Hemangioma-related syndromes
Manuel Valdebran1, Lara Wine Lee
1Division of Pediatric Dermatology, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina, Charleston, South Carolina, USA.
Infantile hemangiomas can have serious complications. Recognizing segmental hemangiomas is key for diagnosing PHACE and LUMBAR syndromes, and visceral hemangiomatosis, improving patient outcomes.
Area of Science:
- Pediatric Dermatology
- Vascular Anomalies
- Genetics and Syndromology
Background:
- Infantile hemangiomas (IH) are common benign vascular tumors in infants.
- Complications and associated anomalies require careful clinical evaluation.
- Syndromic associations of IH are increasingly recognized.
Purpose of the Study:
- To review recent clinical advances in syndromes associated with segmental infantile hemangiomas.
- To highlight the importance of recognizing visceral hemangiomatosis.
- To discuss diagnostic and screening recommendations for associated anomalies.
Main Methods:
- Review of current literature on infantile hemangiomas and associated syndromes.
- Analysis of clinical findings and diagnostic criteria for PHACE and LUMBAR syndromes.
- Discussion of the clinical implications of visceral hemangiomatosis.
Main Results:
- Longitudinal studies improve diagnosis and screening for PHACE and LUMBAR syndromes.
- Characterization of a wider spectrum of anomalies aids in classifying at-risk patients.
- Visceral hemangiomatosis recognition is crucial, with potential links to consumptive hypothyroidism.
Conclusions:
- Clinicians must be aware of syndromic complications of IH.
- Segmental hemangiomas, especially in the head/neck (PHACE) or lower body (LUMBAR), signal potential developmental anomalies.
- Visceral hemangiomas, often hepatic, may lead to heart failure or consumptive hypothyroidism.
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