4.4 Article Proceedings Paper

Lessons from a liver hemangioma registry: subtype classification

Journal

JOURNAL OF PEDIATRIC SURGERY
Volume 47, Issue 1, Pages 165-170

Publisher

W B SAUNDERS CO-ELSEVIER INC
DOI: 10.1016/j.jpedsurg.2011.10.037

Keywords

Hemangioma; Hepatic hemangioma; Infantile hemangioma; Liver; Vascular anomaly

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Purpose: A previously proposed classification of hepatic hemangioma postulated 3 types of lesions: focal, multifocal, and diffuse. A registry (www.liverhemangioma.org) was created to track patients to validate this classification scheme. Methods: Registry records entered between 1995 and 2010 were reviewed. Results: Of 121 patients with hepatic hemangioma, 119 were focal (n = 33), multifocal (n = 68), and diffuse (n = 18). Two patients shared features of multifocal and diffuse. The focal group had a balanced sex distribution, whereas multifocal and diffuse were more common in women (66.2% and 70.0%, respectively). Hepatic hemangioma was detected prenatally in 30% (9/30) of patients with focal disease. Detection was not possible in multifocal or diffuse hepatic hemangioma, indicating postnatal proliferation typical of common cutaneous infantile hemangioma. Cutaneous hemangiomas accompanied 77.4% (48/62) of multifocal hepatic hemangioma, 53.3% (8/15) of diffuse hepatic hemangioma, and 15.3% (4/26) of focal hepatic hemangioma. Hypothyroidism was documented in all (16/16) patients with diffuse hepatic hemangioma, 21.4% (9/42) with multifocal hepatic hemangioma, but in no patients with focal hepatic hemangioma (0/17). Conclusions: Analysis of the Liver Hemangioma Registry confirms that focal lesions are biologically distinct from multifocal and diffuse hepatic hemangioma. Feared diffuse hepatic hemangioma may evolve from previously undetected multifocal hepatic hemangioma. Screening ultrasounds in infants with multiple cutaneous infantile hemangioma may allow for earlier detection and therapy. (C) 2012 Elsevier Inc. All rights reserved.

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