Wednesday, November 15, 2017

                                Hemangiomas in children
Department of Pediatric Dentistry

Lutheran Medical Center

Resident’s Name: Wayne Dobbins                                                                 Date: 11/15/2017
Article Title: Hemangiomas in children
Author(s): Drolet, B. A., Esterly, N. B. and Frieden, I. J
Journal: New England Journal of Medicine
Date: July 15, 1999
Major Topic: Special care for special patients
Type of Article:
Main Purpose: Discussion of Hemangiomas, pathogenesis, clinical manifestations, complications, management
Key Points/Summary:

·                Most common soft tissue tumor of infancy (5%-10% of 1 year olds)
·                Definitions:
o                 Vasculogenesis: process by which precursors of endothelial cells give rise to blood vessels
o                 Angiogenesis: development of new vessels from existing vasculature
·                During proliferative phase, hemangiomas are made up of densely packed endothelial cells forming small capillaries
o                 Cellular markers of angiogenesis can be identified using immunochemical analysis (proliferating-cell nuclear antigen, type IV collagenase, basic fibroblastic growth factor, vascular endothelial growth factor, urokinase, E-slectin)
o                 Urinary levels of fibroblastic growth factor are elevated in infants; it is a means to monitor treatment efficacy
o                 Risk of hemangioma 10X higher in children of parents who had chorionic-villus sampling
·                Differs from most other tumors: has phase of rapid proliferation followed by spontaneous involution
Clinical manifestations:
o                 Newborn: pale macule with thread-like telangiectases
o                 As tumor proliferates: bright red, slightly elevated, noncompressible plaque
o                 Deeper in skin: soft, warm masses with slightly bluish color
o                 Female infants 3x as likely than male infants; 20% of affected have multiple lesions
o                 Superficial hemangiomas reach full size by ~6-8mths; deeper ones may proliferate to ~12-14mth
o                 20%-40% leave residual changes of the skin; large superficial hemangiomas of the face can leave disfiguring scars

·                Complications
o                 Ulceration – most frequent; carries risk of infection, hemorrhage, and scarring
§               Usually due to ischemia and necrosis 
§               In cases of hemorrhage, direct pressure usually sufficient
§               Superinfection may lead to cellulitis, osteomyelitis, or septicemia 
o                 Kasabach-Merritt Phenomenon – complication of rapidly enlarging vascular lesions
§               Not typical – instead have kaposiform hemangioendotheliomas (tufted angiomas) with high mortality rate
o                 Regionally Important Lesions
§               Multiple hemangiomas and large facial hemangiomas may be associated with visceral hemangiomas
·                                                                      Visceral hemangiomas (particularly of liver) higher mortality rate (40%-80%) – need monitoring
§               Slowly proliferating legions may pose threat if compromise any vital structure (periorbital region, area involving ear may also affect speech development, airway)
o                 Dysmorphic features – 2 particular conditions due to hemangiomas
§               Extensive hemangiomas of the neck and face may be associated with PHACES syndrome (Posterior fossa malformations, Hemangiomas of the cervicofacial region, Arterial anomalies, Cardiac anomalies, Eye anomalies, Sternal or abdominal clefting or ectopic cordis); predominance Girls(9):(1)Boys
§               Hemangiomas of the lumbosacral region may be marker for occult spinal malformations and anomalies of anorectal and urogenital regions – imaging of spine indicated
·                Treatment/Management
o                 1940’s-50’s – Irradiation used; decried by experts due to aggressive treatment to treat self-limiting tumors
o                 But other options available: lasers, corticosteroids systemically or directly into lesion, Interferon alpha, and prospect of new inhibitors 
o                 Whether to treat depends on possible complications due to hemangioma that may be life threatening or controversial in cases of hemangiomas that may cause permanent disfigurement
o                 Systemic corticosteroids are mainstay in treatment of hemangiomas (2-3mg/kg of prednisolone or prednisone) – results in shrinkage (1/3 smaller, 1/3 reduced growth, 1/3 no affect)
§               Side effects include: irritability, GI upset, immunosuppression, HTN, growth retradation
o                 Corticosteroids injected directly into lesion (triamcinolone 3-5mg/kg per tx session)
o                 Recombinent interferon Alpha – inhibitor of angiogenesis; for failed treatment using steroids
§               Side effects include: irritability, neutropenia, and abnormalities of liver enzymes; especially worrisome spastic diplegia (potentially irreversible, affects 20% of patients)
o                 Lasers – optimum delivery systems not yet found
o                 Cryotherapy – concerns with scarring (but used widely in Europe and South America)
o                 Bleomycin injected directly into lesion
o                 Surgical excision early or to repair residual cosmetic deformities later 
Assessment of Article:  Level of Evidence/Comments:

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