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*There are no relevant financial relationships to disclose *I will be discussing off-label use of medications Misuse of the nomenclature continues to exist Delay of recognizing complications and a need for intervention compromises patient care The standard of care is shifting to new medical interventions 1 Upon completion of this educational activity, you will be able to: 1) Review the nomenclature and correct misnomers in the definition of hemangiomas and vascular malformations. 2) Review the characteristics of a hemangioma that may indicate further evaluation and/or intervention is necessary. 3) Review the current trends in management of proliferative capillary hemangiomas. Greek suffix “–oma” meaning “swelling” or “tumor” 2 major categories: tumors (primarily hemangiomas) malformations lf an accurate diagnosis is essential history and physical examination radiologic examination biopsy 2 Infantile Hemangioma Vascular Tumors › Proliferative › RICH, NICH Pyogenic Granuloma Kaposiform Hemangioendotheliolma Capillary (Port Wine) Venous Artieriovenous Lymphatic Combined Lesions Vascular Malformations › Venous-Lymphatic › Capillary-Lymphaticovenous Hematology/Oncology Radiology Dermatology Surgery › Pediatric Surgery › Plastic Surgery › Otolaryngology Basic Scientists Patient/family support staff 3 Hemangiomas exhibit cellular proliferation g grow duringg infancyy involute in childhood never appear in an adolescent or adult Vascular Malformations dysplastic vessels no endothelial proliferation growth proportional to patient’s growth never regress most common tumor of childhood Prevalence 3-10% in Caucasian infants less in Asian, very low in African descent limited epidemiologic data as high as 30% incidence in LBW premature infants F>M 10% with elicited family history 60% in the H & N region characteristic time cycle › appear typically within first 2-4 weeks of life › begin involution between 12-18 months of life › nearly 90% resolved by 9 years of age proliferating hemangiomas › composed of foci of endothelial cells, pericytes, fibroblasts and mast cells mature and immature endothelial cells › express surface markers for alkaline phosphatase and factor VIII antigen mast cells › may produce angiogenic factor or secrete dysfunctional angiogenic inhibitors › numerous angiogenic factors have been identified and cloned bFGF (basic fibroblast growth factor) 4 disfigurement ulceration infection localized l li d hemorrhage h h g compression of vital structures high output cardiac failure Kassabach-Merritt Phenomenon (KMP) psychological stress size location presence of complications age of the patient rate of growth at the time of evaluation 1997 American Academy of Dermatology 5 interference with vital structures possibility b l t off permanentt scarring large facial hemangiomas ulcerated hemangiomas hemangiomas in a “beard distribution” periorbital hemangiomas lumbosacral hemangiomas g multiple, cutaneous hemangiomas PHACE syndrome hypothyroidism “Beard” distribution Orlow, et al J Peds 1997 6 Neurological and/or genitourinary defects Tethered cords Albright et al Pediatrics 83:977-980,1989 Renal anomalies Bony sacral anomalies Leptomeningocele Imperforate anus Associated with internal hemangiomas Most commonly in the liver Other areas include: CNS, eye, pancreas, GI tract, lung, spleen, and the airway Posterior fossa malformations Hemangiomas Arterial anomalies Cardiac anomalies Eye anomalies Sternal cleft or supraumbilical raphe syndrome 20% of infants with large cervicofacial hemangiomas will have one of associated anomalies of PHACE Pacual-Castroviejo et al Neuroradiology 16, 1978 Frieden et al Arch Dermatol 132:307 311,1996 7 http://www.radinfonet.com Large liver hemangioma associated with hypothyroidism Functionally active T4/T3 are degraded by type 3 deiodinase enzyme (D3) Increased levels of D3 leading to accelerated degradation of T4/T3 Huang SA et al N Engl J Med 2000 Jul 20;343(3):185-9 Observation Steroids Propranolol Interferon Chemotherapy Laser therapy Embolization Surgery Radiation 8 first used in 1958 for these lesions oral, topical or injected response rate t ~ 70% long lived status as first line therapy anti-angiogenic effect decreases endothelial cell proliferation causes endothelial cell apoptosis › › › › › › › Cushingoid facies personality changes gastric irritation weight gain diminished gain of height and weight immunosuppression non-systemic fungal infections (all complications usually resolve with discontinuation of therapy) 9 hypertension suppression of hypothalamic-pituitary adrenal function hyperglycemia myositis osteoporosis cataracts close monitoring of height and weight BP checks urine checks stool checks physical exam every 1-2 weeks until on a stable dose NO live immunizations MD visit if temp > 38.5 Caution if varicella exposure (Call MD immediately) initial dose of 2-3 mg/kg/day given QD in the am most common preparations › Prelone 15 mg/5cc › Pediapred 1mg/1cc (always give with Ranitidine) 10 first described for use in hemangiomas in 1989 mechanism of action: anti-angiogenic agent, down regulates bFGF response in about 60% of patients alpha-2a or alpha-2b subcutaneous injection neurotoxicity in about 30% of patients › spastic diplegia (may be permanent) › other developmental delays other side effects: flu flu-like like syndrome, anemia, neutropenia alterations in liver enzymes mood changes neurological exam weekly › if neurological changes occur—consider discontinuing drug baseline CBC and LFTs, then every other week thyroid function physician experience 11 Non selective -blocker › › › › Inhibits B1 and B2 adrenergic receptors Pure antagonist without partial agonistic effects Lipophilic properties Membrane stabilizing characteristics Eff Effect › 2 days decrease in color › Long term use needed › Effective for older patients with hemangiomas Early: Vasoconstriction, decreased release of nitric oxide Intermediate: Blocking of pro-angiogenic signals Long term: Induction of apoptosis 12 chemotherapy agent › used in the treatment of many malignant and non- malignant disorders mechanisms of action: › induces apoptosis in endothelial cells › interferes with mitotic spindle microtubules central access Early: › › › › peripheral neuropathy constipation jaw pain rare hematological toxicity *very limited long term effects (usually tolerated well) An appreciation of the complexities of categorizing vascular anomalies Clinical recognition of “at risk” capillary hemangiomas Basic understanding of the use of medical interventions in the treatment of Proliferative Capillary Hemangiomsa 13 look carefully know when to investigate further refer f early l provide support …You are NOT alone!!!!! 14