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Page 0 xxx00.#####.ppt 12/4/2012 3:05:55 PM Vascular Tumors and Malformations Perinatal Management Christopher Cassady MD Edward B. Singleton Department of Pediatric Radiology Texas Children’s Hospital [email protected]
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Page 1: Vascular Tumors and Malformations - pedrad.org · xxx00.#####.ppt 12/4/2012 3:05:55 PM Vascular Lesion Management Diagnostic Options Congenital Vascular Neoplasms CONGENITAL HEMANGIOMA

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Vascular Tumors and

Malformations Perinatal Management

Christopher Cassady MD

Edward B. Singleton Department of Pediatric Radiology Texas Children’s Hospital

[email protected]

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Vascular Lesion Management

We will discuss both

•Diagnostic Options

•Therapeutic Options

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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

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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

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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

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Vascular Lesion Management

Therapeutic Options

Medical therapy Steroids Beta blockers Antiangiogenics Chemotherapy Endovascular therapy (embolization)/sclerotherapy Surgery Tincture of Time

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Vascular Lesion Management

Diagnostic Options

Congenital Vascular Neoplasms CONGENITAL HEMANGIOMA KAPOSIFORM HEMANGIOENDOTHELIOMA*

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Vascular Lesion Management

Therapeutic Options

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

HEMANGIOMA

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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

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Vascular Lesion Management

Therapeutic Options

CONGENITAL HEMANGIOMA

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Vascular Lesion Management

Therapeutic Options

CONGENITAL HEMANGIOMA

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Vascular Lesion Management

Therapeutic Options

LIVER LESIONS

Lesions can be called solitary hemangioma, diffuse hemangiomatosis, hemangioendothelioma, or hepatic vascular malformation with capillary proliferation.

CONFUSING

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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*

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Vascular Lesion Management

Therapeutic Options

FOCAL HEMANGIOMA

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Vascular Lesion Management

Therapeutic Options

FOCAL HEMANGIOMA

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Vascular Lesion Management

Therapeutic Options

FOCAL HEMANGIOMA

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Vascular Lesion Management

Therapeutic Options

FOCAL HEMANGIOMA

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Vascular Lesion Management

Therapeutic Options

DIFFUSE HEMANGIOMA

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Vascular Lesion Management

Therapeutic Options

Algorithm from Boston

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Vascular Lesion Management

Therapeutic Options

CONGENITAL HEMANGIOMA

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Vascular Lesion Management

Therapeutic Options

KAPOSIFORM HEMANGIOENDOTHELIOMA

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Vascular Lesion Management

Therapeutic Options

KAPOSIFORM HEMANGIOENDOTHELIOMA

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Vascular Lesion Management

Therapeutic Options

KAPOSIFORM HEMANGIOENDOTHELIOMA

•Systemic corticosteroids

•Alpha interferon

•AntiAngiogenics

•Multimodal intervention

•Surgery: not generally first-line because of infiltration

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Vascular Lesion Management

Diagnostic Options

Congenital Vascular Neoplasms CONGENITAL HEMANGIOMA KAPOSIFORM HEMANGIOENDOTHELIOMA*

Congenital Vascular Malformations AVM/AVF LYMPHATIC MALFORMATION

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Vascular Lesion Management

Therapeutic Options

AVM/AVF

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Vascular Lesion Management

Therapeutic Options

AVM/AVF

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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

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Vascular Lesion Management

Therapeutic Options

LIVER LESIONS

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Vascular Lesion Management

Therapeutic Options

LIVER LESIONS

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Vascular Lesion Management

Therapeutic Options

Patent Ductus Venosus

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Vascular Lesion Management

Therapeutic Options

Patent Ductus Venosus

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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)

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Vascular Lesion Management

Therapeutic Options

LYMPHATIC MALFORMATIONS

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Vascular Lesion Management

Therapeutic Options

LYMPHATIC MALFORMATIONS

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Vascular Lesion Management

Therapeutic Options

LYMPHATIC MALFORMATIONS

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Vascular Lesion Management

Therapeutic Options

LYMPHATIC MALFORMATIONS

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Vascular Lesion Management

Therapeutic Options

LYMPHATIC MALFORMATIONS

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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

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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.

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

Lawley LP, Siegfried E, Todd JL.. Propranolol treatment for hemangioma of infancy: risks and recommendations. Pediatr Dermatol 2009 Sep-Oct;26(5):610-4.

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.


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