Vascular Anomalies in Pediatrics

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Introduction

Most vascular anomalies involve the skin and are noted at birth. For centuries, vascular birthmarks were referred to by vernacular names derived from folk beliefs that a mother's emotions or patterns of ingestion could indelibly imprint her unborn fetus. Old medical texts are dotted with references to brightly colored foods appropriated to describe the appearance of an unusual cutaneous lesion. Depending on culture and sensitivity, the mother was blamed for eating too much or too little red fruit during her pregnancy. The present-day use of such terms as “cherry,” “port-wine stain,” and “strawberry” can be referenced to this false doctrine of maternal impressions.1

Virchow may be credited with the first effort to categorize vascular anomalies on the basis of histologic features.2 Unfortunately, a lack of specificity or identifying features continued to plague the field. Overlapping clinical, vernacular, and histopathologic terms contributed to persistent confusion, often resulting in misdiagnosis, incorrect treatment, and misdirected research.3 A reliable system of classification for vascular anomalies was ultimately developed by Mulliken and Glowacki, who divided vascular anomalies into 2 major categories: hemangiomas and malformations.4 In 1996, this division was modified to tumors and malformations and was formally accepted by the International Society for the Study of Vascular Anomalies.5 Vascular tumors consist of lesions secondary to endothelial hyperplasia, incorporating both hemangiomas and less common pediatric vascular tumors. Vascular malformations arise by dysmorphogenesis and exhibit normal endothelial cell turnover.3 Use of this division provided a clinically useful method of diagnosis and prognosis, as well as a guide to therapy. Despite this system, some anomalies seem to span both categories. It is hoped that with continued investigation into the biology and pathogenesis of these lesions, a more comprehensive molecular classification will soon be developed.

Section snippets

Incidence

Hemangiomas represent the most common infantile tumor with a documented perinatal incidence of 1.0% to 2.6%. Over the first year of life the incidence increases, with approximately 4% of all Caucasian infants affected.6 Dark-skinned babies are less frequently affected. A female to male ratio of 3:1 to 5:1 has been observed.3 Preterm infants with low birth weight (<1000 g) have an increased incidence of hemangioma, possibly as high a 30%, and with every 500-g decrease in birth weight, the risk

Vascular malformations

Vascular malformations are localized or diffuse errors of embryonic development affecting any segment of the vascular tree including arterial, venous, capillary, and lymphatic vessels. It is useful to subcategorize vascular malformations by the predominant type of channel abnormality and flow characteristics. According to this distinction, 2 major categories exist: (1) slow-flow anomalies (capillary malformations [CMs], LMs, and VMs) and (2) fast-flow anomalies (arteriovenous malformations

Summary

The last 3 decades have witnessed remarkable forays into understanding the pathogenesis of vascular anomalies. Improved definitions based on this genetic-anatomic-histologic classification have allowed the development of multidisciplinary approaches toward disease treatment and management. As the appreciation of the embryonic and developmental contributions to disease increases, so does the ability to develop novel strategies for management of previously insurmountably complex lesions.

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