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Diagnosis and Management of Infantile Hemangioma D CORE Metadata, citation and similar papers at core.ac.uk Provided by Hofstra Northwell Academic Works (Hofstra Northwell School of Medicine) Donald and Barbara Zucker School of Medicine Journal Articles Academic Works 2015 Diagnosis and Management of Infantile Hemangioma D. H. Darrow A. K. Greene A. J. Mancini A. J. Nopper T. M. O Northwell Health Follow this and additional works at: https://academicworks.medicine.hofstra.edu/articles Part of the Otolaryngology Commons Recommended Citation Darrow D, Greene A, Mancini A, Nopper A, O T. Diagnosis and Management of Infantile Hemangioma. 2015 Jan 01; 136(4):Article 575 [ p.]. Available from: https://academicworks.medicine.hofstra.edu/articles/575. Free full text article. This Article is brought to you for free and open access by Donald and Barbara Zucker School of Medicine Academic Works. It has been accepted for inclusion in Journal Articles by an authorized administrator of Donald and Barbara Zucker School of Medicine Academic Works. CLINICAL REPORT Guidance for the Clinician in Rendering Pediatric Care Diagnosis and Management of Infantile Hemangioma: Executive Summary David H. Darrow, MD, DDS, Arin K. Greene, MD, Anthony J. Mancini, MD, Amy J. Nopper, MD, the SECTION ON DERMATOLOGY, SECTION ON OTOLARYNGOLOGY–HEAD & NECK SURGERY, AND SECTION ON PLASTIC SURGERY INTRODUCTION Infantile hemangiomas (IHs) are the most common tumors of childhood. Unlike other tumors, they have the capacity to involute after proliferation, often leading primary care providers to assume they will resolve without intervention or consequence. However, a subset of IHs may be associated with complications, resulting in pain, functional impairment, or permanent disfigurement. As a result, the primary care provider is often called on to decide which lesions should be referred for early consultation with a specialist. This document provides a summary of the guidance contained in the clinical report “Diagnosis and Management of Infantile Hemangioma,” published concurrently in the online version of Pediatrics (Pediatrics. This document is copyrighted and is property of the American 2015;136[4]:e1060–e1104, available at: www.pediatrics.org/content/ Academy of Pediatrics and its Board of Directors. All authors have filed conflict of interest statements with the American Academy of 136/4/e1060.full). The report is uniquely based on input from the many Pediatrics. Any conflicts have been resolved through a process specialties involved in the treatment of IH. Its purpose is to update the approved by the Board of Directors. The American Academy of Pediatrics has neither solicited nor accepted any commercial pediatric community about recent discoveries in IH pathogenesis, clinical involvement in the development of the content of this publication. associations, and treatment and to provide a knowledge base and Clinical reports from the American Academy of Pediatrics benefit from framework for clinical decision-making in the management of IH. expertise and resources of liaisons and internal (American Academy of Pediatrics) and external reviewers. However, clinical reports from the American Academy of Pediatrics may not reflect the views of the liaisons or the organizations or government agencies that they NOMENCLATURE represent. • Infantile hemangiomas (IHs) are vascular neoplasms characterized by The guidance in this statement does not indicate an exclusive course abnormal proliferation of endothelial cells and aberrant blood vessel of treatment or serve as a standard of medical care. Variations, taking into account individual circumstances, may be appropriate. architecture. In contrast, vascular malformations are structural anoma- All clinical reports from the American Academy of Pediatrics lies and inborn errors of vascular morphogenesis. The latter include automatically expire 5 years after publication unless reaffirmed, capillary malformations (port wine stains), venous malformations, revised, or retired at or before that time. lymphatic malformations (formerly known as lymphangiomas or cystic www.pediatrics.org/cgi/doi/10.1542/peds.2015-2482 hygromas), and arteriovenous malformations. DOI: 10.1542/peds.2015-2482 • Congenital hemangiomas are biologically and behaviorally distinct from PEDIATRICS (ISSN Numbers: Print, 0031-4005; Online, 1098-4275). IHs. They are fully grown at birth and present as 2 varieties: rapidly involuting (RICH) and noninvoluting (NICH). Copyright © 2015 by the American Academy of Pediatrics • Pyogenic granuloma, or lobular capillary hemangioma, is a reactive FINANCIAL DISCLOSURE: The authors have indicated they do not have a financial relationship relevant to this article to disclose. proliferative vascular lesion. Although classified with the vascular POTENTIAL CONFLICT OF INTEREST: The authors have indicated they neoplasms and often misdiagnosed as IH, pyogenic granuloma is distinct have no potential conflicts of interest to disclose. in its clinical appearance and behavior. Downloaded from by guest on February 7, 2017 FROM THE AMERICAN ACADEMY OF PEDIATRICS PEDIATRICS Volume 136, number 4, October 2015 • Lesions diagnosed as “cavernous with enlarged draining veins. In- that does not uniformly hemangiomas” are usually, in fact, voluting IHs reveal fibrofatty proliferate. “ ” deep IHs or venous malformations. stroma, residual ghost vessels, • IHs may also be classified accord- • Kasabach–Merritt phenomenon (a mast cells, and apoptotic bodies. ing to their anatomic configuration: consumptive coagulopathy) is not • Immunohistochemical staining of • Localized (focal) IH: discrete associated with IH but rather with IH is positive for glucose trans- lesions, arise from single focal other vascular neoplasms (kaposi- porter 1 (GLUT1), CD31, CD34, point form hemangioendothelioma and factor VIII–related antigen, and • Segmental IH (Fig 3): larger tufted angioma). others; GLUT1 is the most useful lesions, usually plaque-like, cov- and widely used marker for the diagnosis of IH. ering regions probably de- EPIDEMIOLOGY termined by neuroectodermal • The incidence of IH is estimated at CLINICAL PRESENTATION placodes approximately 5% of infants, and • Indeterminate IH: cannot de- • IHs usually appear before 4 weeks the female/male ratio ranges from finitively be categorized as lo- of age and complete most of their 1.4:1 to 3:1. calized or segmental growth by 5 months of age, al- • • IH risk factors include white race, though the proliferative phase may Multifocal IH: focal lesions oc- prematurity, low birth weight, ad- continue up to 12 months of age. curring at more than 1 anatomic vanced maternal age, multiple gesta- Deep IHs tend to appear later and site; may be a marker for hepatic tion pregnancy, placenta previa, and grow somewhat longer. The in- IH when 5 or more cutaneous preeclampsia. Other risk factors may volution phase usually begins be- lesions are present include in utero diagnostic proce- tween 6 and 12 months of age, and • Segmental IH may be associated dures (chorionic villus sampling and the majority of regression occurs amniocentesis), use of fertility drugs with extracutaneous before 4 years of age. As IHs in- manifestations. or erythropoietin, breech pre- volute, they flatten and shrink, with fi • sentation, and being rst born. fading of their color. A small subset of children with IH present with congenital anomalies • Up to 70% of IHs leave behind re- recognized as clinical associations. PATHOGENESIS AND HISTOPATHOLOGY sidual skin changes, including tel- These include: angiectasia, fibrofatty tissue, • The pathogenesis of IH remains to • PHACE, which is a congenital redundant skin, atrophy, dyspig- be clearly elucidated. Both intrinsic mentation, and scar (especially in vasculopathy with features of factors (such as angiogenic and lesions with history of past Posterior fossa defects, Heman- vasculogenic factors) and extrinsic ulceration). giomas, cerebrovascular Arterial factors (including tissue hypoxia • fi anomalies, Cardiovascular and developmental field dis- Premonitory ndings in early IH anomalies including Coarctation turbances) probably contribute to include localized blanching of skin of the aorta, and Eye anomalies. development of IH. and macular telangiectatic erythema. In PHACE, the IH is large and • Endothelial progenitor cells may • segmental, characteristically lo- develop into IH from clonal expan- During the proliferative phase, IHs fi cated on the face, scalp, or neck. sion, resulting in vasculogenesis; may be classi ed based on their • alternatively, fetal progenitor cells depth, as follows (Fig 1): LUMBAR, which refers to Lower • fi arising from disruption of the pla- Super cial IH: Surface appears body IH and other cutaneous centa during gestation or birth may red, and there is little or no dis- defects, Urogenital anomalies give rise to these tumors. cernible subcutaneous and Ulceration, Myelopathy, Bony component. • A unifying theory suggests that IH deformities, Anorectal malfor- • results from aberrant proliferation Deep IH: Surface appears blue or mations, Arterial anomalies, and fl and differentiation of a pluripotent esh-colored, and the tumor Renal anomalies. The associated progenitor cell, which migrates to resides deep below the skin IHs are usually segmental lum- locations in which growth of surface. bosacral or anogenital lesions. placenta-like tissue is favorable. • Combined IH: both superficial LUMBAR, also described under • Proliferative IHs histologically re- and deep components. the acronyms “SACRAL” and veal well-defined masses of capil- • Abortive (arrested
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