Original article
Diffuse neonatal hemangiomatosis: An evidence-based review of case reports in the literature

Presented at the Annual Meeting of the Society for Pediatric Dermatology Baltimore, MD, July 10, 2011.
https://doi.org/10.1016/j.jaad.2012.01.018Get rights and content

Background

The term “diffuse neonatal hemangiomatosis” has been used historically to describe multifocal vascular lesions affecting the skin and viscera in infants.

Objective

We hypothesized that many cases reported as diffuse neonatal hemangiomatosis did not have infantile hemangiomas (IH), but represented more recently described neonatal vascular diseases.

Methods

A literature search was performed using PubMed database (1950-2009) with the terms “neonatal hemangiomatosis,” “benign hemangiomatosis,” and “diffuse hemangiomatosis.” A total of 180 articles were identified. Exclusion criteria included disease onset later than 3 years of age and absence of multifocal skin involvement. In all, 73 cases were selected and categorized into 3 groups: IH/probable IH; multifocal lymphangioendotheliomatosis with thrombocytopenia (MLT)/probable MLT; and multifocal vascular lesions, not otherwise specified.

Results

Of the 73 cases, 43 had IH/probable IH, 17 had MLT/probable MLT, and 13 had multifocal vascular lesions, not otherwise specified. The clinical outcomes of these groups differed in that two of 43 (5%) patients with IH died whereas 11 of 17 (65%) patients with MLT died (odds ratio 37.6, confidence interval 5.6-387.6, P value < .0001).

Limitations

This was a literature-based meta-analysis, which inherently has limitations of incomplete and inconsistently presented information.

Conclusions

Many cases reported in the literature as diffuse neonatal hemangiomatosis represent newly described multifocal vascular anomalies such as MLT, which has a strikingly higher mortality than IH. We propose the term “multifocal infantile hemangioma–with or without extracutaneous disease” instead of “diffuse neonatal hemangiomatosis” for multiple cutaneous IH. Accurate diagnosis of multifocal neonatal vascular lesions is imperative to facilitate appropriate evaluation, treatment, and prognosis.

Section snippets

Methods

A literature search was performed using the National Library of Medicine PubMed database of the National Institutes of Health and Ovid MEDLINE database (1950-2009) using the search terms “neonatal hemangiomatosis,” “benign, hemangiomatosis,” and “diffuse hemangiomatosis,” along with the alternate spelling “haemangiomatosis” for all 3 search terms. All eligible articles were reviewed in detail and clinical characteristics were recorded. The authors identified key features distinguishing IH from

Results

Using the above search criteria, 180 articles were identified. Of the 180 articles, 95 were excluded for one or more of the following reasons: the article was not in English or was unavailable, case duplication, no case-specific information (ie, review articles without case reports), or a diagnosis other than multifocal vascular anomaly was made. The remaining 85 eligible articles were reviewed and a total of 110 cases were identified. Twenty-four cases were further excluded because they had

Discussion

Our study highlights the confusion in classifying and diagnosing multifocal vascular tumors of infancy and demonstrates that the term “diffuse neonatal hemangiomatosis” was used in the past to include IH, MLT, and likely other less well-characterized multifocal conditions.

The majority of cases reviewed were found to have IH, which is the most common vascular tumor of infancy (Fig 1). Infants with multiple cutaneous IH are recognized to have a higher risk of extracutaneous disease, the liver

References (19)

There are more references available in the full text version of this article.

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Funding sources: None.

Disclosure: Dr Frieden is a consultant for Pierre-Fabre Dermatology. Drs Glick, Garzon, Mully, and Drolet have no conflicts of interest to declare.

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