La cute e le anomalie vascolari

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1 La cute e le anomalie vascolari Maya El Hachem Responsabile UOC Dermatologia Dipartimento di Medicina Pediatrica, Direttore: Prof. A. G. Ugazio

2 2 Enjolras O, Mulliken JB. Vascular tumors and vascular malformations (new issues). Adv Dermatol 1997

3 3 Hemangiomas Congenital Hermangiomas: NICH RICH IH

4 IH, RICH and NICH IH RICH NICH F:M 3-5/1 1:1 1:1 dimensions variable 6 cm 5 cm Clincal aspect and history GLUT-1 Imaging Bright red cutaneous or subcutaneous Spontaneous regressione; possible residual atrophy and/or telangectasias + (but - in segmental hemangiomas) Minimal or moderate arteries and prominent veins, no intravscular thrombi Dome-shaped, central depression, telangectasia, pale margin. Ulcer, scar or central depression; regression within 14 mo - - Abnormal and large draining veins, intravscular thrombi 4 Slightly elevated, red purple, well demarcated, central and peripheral telangectasia and pale margin. Proportional growth with the child and no regression AV F and prominent arteries and veins, intravascular thrombi

5 5 Infantile hemangiomas Nodular: the most common form (75% of cases) Single lesion Multifocal with or without organ involvement(> the liver) Segmental: less common (25% of cases), possible association with other abnormalities Complications: Ulceration Infection Bleeding

6 6 Hemangioma phases Caspasi PROLIFERATIVE STABiLiZATION INVOLUTIVE VEGF bfgf VEGF bfgf birth 3-12 months 9-36 months 2-7 years

7 7

8 Laryngeal localization 882 Eventual laryngeal localization should be considered in all patients with suspected PHACE and respiratory symptoms (86% of cases in this group) The subglottic localization is more frequent than the transglottic, uperglottic and tracheal Durr ML et Al. Airway hemangiomas in PHACE syndrome. Laryngoscope

9 PHACES syndrome The diagnostic approach should also take into consideration the possible associated anomalies: 9 Posterior fossa anomalies Hemangioma Arterial anomalies Coartation aortic arc and cardiac defect Eye abnormalities Sternal malformation or supraumbelical rafe Siegel, Dawn H. et al. Stroke in Children With Posterior Fossa Brain Malformations, Hemangiomas, Arterial Anomalies, Coarctation of the Aorta and Cardiac Defects, and Eye Abnormalities (PHACE) Syndrome: A Systematic Review of the Literature. Stroke

10 10 PELVIS/SACRAL/LUMBAR Perineal or lumbosacral hemangioma Abnormalities of the anorectal or urogenital regions Lipomyelomeningocele Skin tag Imaging of spine and pelvis is necessary Frade F et al. PELVIS or LUMBAR syndrome: the same entity. Two case reports. Arch Pediatr. 2012

11 11 IH: Diagnostic approach Deep lesion OR Cutaneous lesion Clinical diagnosis and differential diagnosis Vascular tumor Gray scale US Doppler US Non vascular tumor

12 12 A A B High resolution gray scale and Doppler US allow excellent visualization of superficial mass tumor. Doppler US is the easiest modality to assess the hemodynamics of a vascular lesion and to clarify a doubtful diagnosis MRI: best technique to evaluate the extent of the lesions and their relationship to adjacent structures A: 7 mo girl. Iso-hypoechoic softtissue mass in the zygomatic region. Numerous vessels, high velocity arterial flow with low resistance B B: 14 mo old girl. Small hyperechoic soft-tissue mass very poor vascularization high speed arterial flow with low resistance Pictorial review: Vascular anomalies of the head and neck. T.A.Warren, et al. Journal of Medical Imaging and Radiation Oncology 2012 Vascular anomalies: what a radiologist needs to know. J.Dubois et Al.Pediatr Radiol 2010

13 Differential diagnosis DFSP Giant cell reparative granuloma 13 Bilateral ectropion Pilomatricoma Vascular malformation Miofibroma

14 Kaposiform hemangioendothelioma (KH) and tufted angioma: Diagnostic approach Clinical aspect and history Imaging: Doppler US, MRI, angio-mri Histology PLT count, D-dimer, Fibrinogen Possible Kasabach-Merritt phenomenon in both

15 KH: Histology Tufted Angioma (Angioblastoma of Nakagawa) A B C D Fibrous tissue into the dermis (H&E 10X) with irregular nodules (A) and nests of epithelioid cells (B) (C) Positive staining for CD31 and CD2-40 (D). Cannon ball

16 MRI and angio-mri in a newborn with KH Marked hypertrophy of all the components of the right thigh and an infiltrated mass highly vascularized Vascular abnormality of iliac and femoral vessels A B

17 17 Treatment of vascular tumors No sequelae Possible esthetical damage (> face) Function and/or life threatening, ulceration, pain No treatment Only 10 to 15% of hemangiomas requires treatment Medical treatment: Propranolol Corticosteroids Vincristine Interferon Pulsed dyelaser Surgery Mixed treatment in some cases

18 18

19 19

20 20 Mecchanism of action: Not yet completely clear 1.Vasoconstriction 2.Blocking of proangiogenic signals 3.Induction of apoptosis in proliferating endothelial cells

21 21 Management of propranolol Heart screening Dose: 1-3mg/Kg/day 65% of pts has been treated with 2 mg/kg/day 25% had 3 mg/kg/day Increase progressively in order to evaluate the subjective response Administration in 2 or 3 doses/day The monitoring modality of side effects depends on the age of the patient, socio-cultural level, comorbidity Propranolol and Infantile Hemangiomas Four Years Later: A Systematic Review. Ann L. Marqueling et Al. Pediatric Dermatology 2013

22 22 Duration of the treatment: still discussed 9/2/10 3/9/10 18/02/11 Rebound after stoppping treatment stopped at 11 months of age

23 23 Safety and side effects high rate of efficacy and a low rate of serious adverse events. all studies examining the response of IHs to propranolol between 2008 and % of the studies don t report side effects Major side effects: Hypoglycemia Bronchospasm (especially in concomitant viral infection) Bradycardia and hypotension rare and > asymptomatic Hyperkalaemia (cell lysis) Beth A. Drolet et al. Initiation and Use of Propranolol for Infantile Hemangioma: Report of a Consensus Confernce. Pediatrics 2013

24 2 days Prednisone+Ticlodipine Kaposiform hemangioendothelioma 1 mo Propranolol,prednisone 2 mo Vincristine and propanolol Treatment: Corticosteroids, vincristine, (interferon), ticlodipine, aspirin 3 weeks after vincristine After 12 weeks of Vincristine 20 weeks later

25 Surgery: indication 25

26 CUP Gestione delle anomalie vascolari in OPBG 26 Ambulatorio dermatologico *** approccio multidisciplinare: dermatologo: case-manager, radiologo interventista e chirurgo plastico Indagini diagnostiche in AG : DH senza AG ambulatoriale APA per dye laser approccio terapeutico medico dermatologo, pediatra, cardiologo, endocrinologo, etc (laboratorio analisi) Caposala UOC di Dermatologia APA per asportazione di piccole lesioni; coinvolgimento patologo approccio interventistico (ricovero ordinario per trattamento) radiologia interventistica o chirurgia d'organo in funzione della localizzazione della lesione Ambulatorio altre specialità Pazienti non inviati dall ambulatorio dedicato *** raramente coinvolti: oculista, genetista, ORL, broncopneumologo, neurologo, neurochirurgo, ortopedico, etc

27 27

28 Thank you 28

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