Copyright @ 2009 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited. Vascular Anomalies of the Upper Extremity in Children Deniz Dayicioglu, MD,* Erick G. Martell, MD,* Micheal Ogilvie, MD,* Aydin Gozu, MD,Þ Zubin J. Panthaki, MD, FACS,* and Milton B. Armstrong, MD, FACS* Abstract: Vascular malformations in the pediatric upper extremity remain a diagnostic and therapeutic challenge. Recent knowledge about diagnosis and treatment is provided including specific markers, imaging techniques, and management. Key Words: Arteriovenous malformation, hemangioma, upper extremity (J Craniofac Surg 2009;20: 1025Y1029) T here is considerable variation in the presentation and natural course of vascular anomalies in children. When encountered in the upper extremity, vascular anomalies offer diagnostic and thera- peutic challenges. The most common vascular anomalies are vascu- lar malformations and hemangiomas. Treatment is variable and based on the etiology of the lesion and severity of symptoms. Hemangiomas Hemangiomas are the most common tumors of infancy pre- senting in 1% to 2.6% of neonates in all races. Hemangiomas affect females more commonly, and the prevalence is increased in pre- mature infants. The natural history of hemangiomas involve rapid proliferation for the first several months of life with subsequent spontaneous regression, often leaving fibrofatty deposition and tel- angiectasis. Recent research has yielded some clues about the pathogenesis. Most believe that there is an imbalance between angiogenic and angiostatic factors. Markers such as GLUT1, merosin, and Lewis Y antigen are hemangioma specific, but their role in hemangiogenesis remains incompletely understood. Elevated mast cell counts are present in highly vascularized tumors, but their contributions also re- main to be elucidated. The concentration of mast cells varies through- out the life cycle of a hemangioma, with numbers peaking in the proliferative phase; however, they are not increased in the setting of vascular malformations. 1 Overall, hemangiomas affect the extrem- ities in 15% of cases, and they rarely produce functional deficits. Vascular Malformations They are thought to arise from developmental errors in angio- genic signaling during vascular development. Unlike hemangiomas, malformations are often recognized at birth, fail to involute, and typ- ically grow proportionally with the child, with many becoming more prominent with puberty. Vascular malformations are far less common than hemangiomas and demonstrate no sex predilection. These lesions are classified according to the predominant cell type as capillary malformation, venous malformation, lymphatic malformation, arterial malformation, venous malformation, or combined malformations (Table 1). They are also subcategorized as fast- and slow-flow lesions (Table 2). 1,2 Classification Vascular anomalies are among the most common, palpable on the hand in children with ganglion cysts and foreign bodies. 3 Vas- cular anomalies in the pediatric population were for a long time highly confusing because their classification was based on super- ficial descriptions of the lesions. 4 The classification system was uniquely altered in 1982 by Mulliken and Glowacki 5,6 when they used a biologic classification scheme based on clinical courses, en- dothelial proliferation activity, and potential presence of mast cell activity. Using this classification system, hemangiomas (infantile/juve- nile) were recognized as distinct entities from other vascular ano- malies, namely, vascular and lymphatic malformations or pyogenic granulomas. However, even with the use of this classification system, the differential diagnosis has proven hard to make, particularly be- tween proliferating hemangiomas and vascular malformations of similar histology. This is a problematic issue because accurate diag- nosis is necessary because current treatment modalities are quite different. Hemangiomas tend to involute over time and typically re- quire only watchful waiting, whereas vascular malformations are per- manent and often require some form of surgery. 7,8 Tumor Markers Several tumor markers have been researched to aid in the diagnosis of hemangiomas. North et al 9 described in 2000 the use of high endothelial immunoreactivity for the erythrocyte-type glucose transporter protein, GLUT1. The study illustrated that GLUT1 was highly specific for juvenile hemangiomas (in all 3 phases) as op- posed to all vascular malformations and concluded that GLUT1 could be used as a specific diagnostic marker. 9 Results from their study were later reproduced and confirmed by Leon-Villapalos et al. 7 Later in 2001, North et al 10 released their experience with other placenta-associated vascular antigens, namely, FcFRII, Lewis Yantigen, merosin, and GLUT1. Results of the study conveyed that all markers were highly expressed in hemangiomas but were absent in all vascular malformations and pyogenic granulomas. Although North et al 10 did not conclude that these endothelial markers should be used as differential diagnostic tools, they hypothesized a unique relation between hemangiomas and the placenta and suggested new origins for these tumors. ORIGINAL ARTICLE The Journal of Craniofacial Surgery & Volume 20, Number 4, July 2009 1025 From the *Department of Surgery, Division of Plastic and Reconstructive Surgery, Jackson Health System, University of Miami, Miami, Florida; and †Vakif Gureba Training Hospital Department of Plastic and Reconstructive Surgery, Istanbul, Turkey. Received April 13, 2008. Accepted for publication April 27, 2008. Address correspondence and reprint requests to Deniz Dayicioglu, MD, Division of Hand Surgery, Department of Plastic Surgery, University of Miami, 1611 NW 12th Ave, ET 3019, Miami, FL 33136; E-mail: denizdayici@yahoo.com Copyright * 2009 by Mutaz B. Habal, MD ISSN: 1049-2275 DOI: 10.1097/SCS.0b013e3181abb1f 3