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Vascular Tumors and Malformations Perinatal Management

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Vascular Tumors and Malformations Perinatal Management Vascular Tumors and Malformations Perinatal Management Christopher Cassady MD Edward B. Singleton Department of Pediatric Radiology Texas Children’s Hospital [email protected] Page 0 xxx00.#####.ppt 12/4/2012 3:05:55 PM Vascular Lesion Management We will discuss both •Diagnostic Options •Therapeutic Options Page 1 xxx00.#####.ppt 12/4/2012 3:05:55 PM Vascular Lesion Management Diagnostic Options Congenital Vascular Neoplasms CONGENITAL HEMANGIOMA KAPOSIFORM HEMANGIOENDOTHELIOMA* TUFTED ANGIOMA* Congenital Vascular Malformations AVM/AVF LYMPHATIC MALFORMATION VENOUS MALFORMATION VENOCAPILLARY MALFORMATION Page 2 xxx00.#####.ppt 12/4/2012 3:05:55 PM Vascular Lesion Management Diagnostic Options Congenital Vascular Neoplasms CONGENITAL HEMANGIOMA KAPOSIFORM HEMANGIOENDOTHELIOMA* Congenital Vascular Malformations AVM/AVF LYMPHATIC MALFORMATION These are the diagnostic options for an imager in the perinatal period Page 3 xxx00.#####.ppt 12/4/2012 3:05:55 PM Vascular Lesion Management Therapeutic Options Why treat? Location: airway, compartment syndrome Shunting/heart failure Shunting/venous hypertension/ischemia Consumptive coagulopathy Hypothyroidism Cosmetic, including ulceration/bleeding Page 4 xxx00.#####.ppt 12/4/2012 3:05:55 PM Vascular Lesion Management Therapeutic Options Medical therapy Steroids Beta blockers Antiangiogenics Chemotherapy Endovascular therapy (embolization)/sclerotherapy Surgery Tincture of Time Page 5 xxx00.#####.ppt 12/4/2012 3:05:55 PM Vascular Lesion Management Diagnostic Options Congenital Vascular Neoplasms CONGENITAL HEMANGIOMA KAPOSIFORM HEMANGIOENDOTHELIOMA* Page 6 xxx00.#####.ppt 12/4/2012 3:05:55 PM Vascular Lesion Management Therapeutic Options HEMANGIOMA Glut 1 negative •RICH: present at birth, involutes by 12-14 months of age •NICH: present at birth, no regression Glut 1 positive •Common Infantile: appears in infancy, proliferative/degenerative •Benign Neonatal Hemangiomatosis •Disseminated NH •PHACES Page 7 xxx00.#####.ppt 12/4/2012 3:05:55 PM Vascular Lesion Management Therapeutic Options HEMANGIOMA Glut 1 negative CONSERVATIVE Treat ulcerations with local wound care STEROIDS if high output symptoms: EMBOLIZE or EXCISE Glut 1 positive CONSERVATIVE PROPRANOLOL STEROIDS THYROXINE as necessary surgery, laser, cryoablation, chemotherapy if desperate Page 8 xxx00.#####.ppt 12/4/2012 3:05:55 PM Vascular Lesion Management Therapeutic Options CONGENITAL HEMANGIOMA Page 9 xxx00.#####.ppt 12/4/2012 3:05:55 PM Vascular Lesion Management Therapeutic Options CONGENITAL HEMANGIOMA Page 10 xxx00.#####.ppt 12/4/2012 3:05:55 PM Vascular Lesion Management Therapeutic Options LIVER LESIONS Lesions can be called solitary hemangioma, diffuse hemangiomatosis, hemangioendothelioma, or hepatic vascular malformation with capillary proliferation. CONFUSING Page 11 xxx00.#####.ppt 12/4/2012 3:05:55 PM Vascular Lesion Management Therapeutic Options LIVER LESIONS BOSTON group: •Focal •Behaves like a RI Congenital Hemangioma (Glut 1 -) •Usually asymptomatic; monitor for shunting •Multifocal or Diffuse •Glut 1 +, behave like Infantile Hemangioma •Multifocal: AV or PV shunting *HO Failure* •Diffuse: Abd Compartment Syndrome, hypothyroid *LO Failure* Page 12 xxx00.#####.ppt 12/4/2012 3:05:55 PM Vascular Lesion Management Therapeutic Options FOCAL HEMANGIOMA Page 13 xxx00.#####.ppt 12/4/2012 3:05:55 PM Vascular Lesion Management Therapeutic Options FOCAL HEMANGIOMA Page 14 xxx00.#####.ppt 12/4/2012 3:05:55 PM Vascular Lesion Management Therapeutic Options FOCAL HEMANGIOMA Page 15 xxx00.#####.ppt 12/4/2012 3:05:55 PM Vascular Lesion Management Therapeutic Options FOCAL HEMANGIOMA Page 16 xxx00.#####.ppt 12/4/2012 3:05:55 PM Vascular Lesion Management Therapeutic Options DIFFUSE HEMANGIOMA Page 17 xxx00.#####.ppt 12/4/2012 3:05:55 PM Vascular Lesion Management Therapeutic Options Algorithm from Boston Page 18 xxx00.#####.ppt 12/4/2012 3:05:55 PM Vascular Lesion Management Therapeutic Options CONGENITAL HEMANGIOMA Page 19 xxx00.#####.ppt 12/4/2012 3:05:55 PM Vascular Lesion Management Therapeutic Options KAPOSIFORM HEMANGIOENDOTHELIOMA Page 20 xxx00.#####.ppt 12/4/2012 3:05:55 PM Vascular Lesion Management Therapeutic Options KAPOSIFORM HEMANGIOENDOTHELIOMA Page 21 xxx00.#####.ppt 12/4/2012 3:05:55 PM Vascular Lesion Management Therapeutic Options KAPOSIFORM HEMANGIOENDOTHELIOMA •Systemic corticosteroids •Alpha interferon •AntiAngiogenics •Multimodal intervention •Surgery: not generally first-line because of infiltration Page 22 xxx00.#####.ppt 12/4/2012 3:05:55 PM Vascular Lesion Management Diagnostic Options Congenital Vascular Neoplasms CONGENITAL HEMANGIOMA KAPOSIFORM HEMANGIOENDOTHELIOMA* Congenital Vascular Malformations AVM/AVF LYMPHATIC MALFORMATION Page 23 xxx00.#####.ppt 12/4/2012 3:05:55 PM Vascular Lesion Management Therapeutic Options AVM/AVF Page 24 xxx00.#####.ppt 12/4/2012 3:05:55 PM Vascular Lesion Management Therapeutic Options AVM/AVF Page 25 xxx00.#####.ppt 12/4/2012 3:05:55 PM Vascular Lesion Management Therapeutic Options PORTOSYSTEMIC FISTULA Congenital arterioportal fistulae •Present as neonates with FTT and abdominal distension from splenomegaly, +/- GI bleeding •enlarged hepatic artery with dilation of the PV at the fistula, pulsatile hepatofugal flow in PV •Treated angiographically with coil/particle embolization Page 26 xxx00.#####.ppt 12/4/2012 3:05:55 PM Vascular Lesion Management Therapeutic Options LIVER LESIONS Page 27 xxx00.#####.ppt 12/4/2012 3:05:55 PM Vascular Lesion Management Therapeutic Options LIVER LESIONS Page 28 xxx00.#####.ppt 12/4/2012 3:05:55 PM Vascular Lesion Management Therapeutic Options Patent Ductus Venosus Page 29 xxx00.#####.ppt 12/4/2012 3:05:55 PM Vascular Lesion Management Therapeutic Options Patent Ductus Venosus Page 30 xxx00.#####.ppt 12/4/2012 3:05:55 PM Vascular Lesion Management Therapeutic Options LYMPHATIC MALFORMATIONS Lesions that require treatment for location typically get a combination of •Surgery (debulking) •Intralesional sclerotherapy with •Doxycycline •Bleomycin •OK432 •STS+ (EtOH or doxycycline) Page 31 xxx00.#####.ppt 12/4/2012 3:05:55 PM Vascular Lesion Management Therapeutic Options LYMPHATIC MALFORMATIONS Page 32 xxx00.#####.ppt 12/4/2012 3:05:55 PM Vascular Lesion Management Therapeutic Options LYMPHATIC MALFORMATIONS Page 33 xxx00.#####.ppt 12/4/2012 3:05:55 PM Vascular Lesion Management Therapeutic Options LYMPHATIC MALFORMATIONS Page 34 xxx00.#####.ppt 12/4/2012 3:05:55 PM Vascular Lesion Management Therapeutic Options LYMPHATIC MALFORMATIONS Page 35 xxx00.#####.ppt 12/4/2012 3:05:55 PM Vascular Lesion Management Therapeutic Options LYMPHATIC MALFORMATIONS Page 36 xxx00.#####.ppt 12/4/2012 3:05:55 PM Vascular Lesion Management Diagnostic with Therapeutic Options Congenital Vascular Neoplasms CONGENITAL HEMANGIOMA Glut 1 - : Nothing, or steroids Glut 1+ : Nothing, or propranolol KAPOSIFORM HEMANGIOENDOTHELIOMA Steroids, Multimodality therapy Congenital Vascular Malformations AVM/AVF Endovascular therapy LYMPHATIC MALFORMATION Sclerotherapy +/- surgery Page 37 xxx00.#####.ppt 12/4/2012 3:05:55 PM Vascular Lesion Management SELECTED REFERENCES Christison-Lagay, ER, Burrows PE, Alomari A, Dubois J, Kozakewich HP, Lane TS, Paltiel HJ, Klement J, Mulliken JB, Fishman SJ. Hepatic hemangiomas: subtype classification and development of a clinical practice algorithm and registry. J Ped Surg 2007; 42: 62-68. Lawley LP, Siegfried E, Todd JL.. Propranolol treatment for hemangioma of infancy: risks and recommendations. Pediatr Dermatol 2009 Sep-Oct;26(5):610-4. Solomon T, Ninnis J, Deming D, Merritt TA, Hopper A. Use of propranolol for treatment of hemangiomas in PHACE syndrome. J Perinatol 2011 Nov;31(11):739-41 Teplisky D, Tincani EU, Lipsich J, Sierre S. Congenital arteriportal fistulas: radiologic treatment and color Doppler US follow up. Pediatrr Radiol 2012 Nov ;42(11): 1326-1332. Page 38 xxx00.#####.ppt 12/4/2012 3:05:55 PM .
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