Congenital Vascular Malformations

1. Congenital Vascular Malformations Chelsey Tinder, MD USF Department of vascular and endovascular surgery

2. Congenital Vascular Malformations • Malformed vessels secondary to arrested development during various stages of embryogenesis, most occur between the 4th and 10th week of embryogenesis • Grow as the child grows, do not regress • Incidence up to 15% of live births • Wide range of presentation with an unpredictable course • 88% asymptomatic • Can involve any organ system, most commonly pelvis, extremities, intracranial circulation

3. Mulliken Classification • Fast flow lesions • Low flow lesions

4. Hamburg Classification • Predominately arterial • Truncular • Hypo-, a-, hyperplasia, obstruction, membrane, spur, dilatation • Extratruncular • Infiltrating or limited • Predominately venous • Truncular • Hypo-, a-, hyperplasia, obstruction, membrane, spur, dilatation • Extratruncular • Infiltrating or limited • Predominately AV shunting defects • Truncular • Deep AVF, superficial AVF • Extratruncular • Infiltrating or limited

5. Hamburg Classification • Combined vascular defects • Truncular • Arterial and venous, hemolymphatic • Extratruncular • Infiltrating hemolymphatic, limited hemolymphatic • Predominately lymphatic defects • Truncular • Hypo-, a-, hyperplasia, obstruction, membrane, spur, dilatation • Extratruncular • Infiltrating or limited

6. Classification • Extratruncular Lesions • Arrested development early in the embryo • Arise and are composed of mesenchymal cells • Growth influenced by hormones, trauma • Higher rate of recurrence • Compress surrounding structures • Truncular lesions • Occur later in embryogenesis, cells differentiated • More hemodynamically significant

7. Types of CVM • Capillary malformation • Dilated capillary vessels in the dermis, asymmetric overgrowth of the involved limbs, and sometimes multiple soft tissue tumors • Venous Malformation • Bluish swelling under the skin • Compressible, enlarge with dependence • Increased incidence of thrombosis/PE • Low flow lesions • Lymphatic Malformation • Diffuse (lymphedema) or localized (lymphangioma) swelling

8. Types of CVM • Arteriovenous malformation • Usually extratruncular • Initially present as local swelling +- thrill, bruits, local hyperthermia • Develop symptoms of shunting – skin necrosis, distal gangrene, high output cardiac failure • Nidus present – central area of AV connection (no capillaries) • High flow lesions • Develop dilated, thickened, tortuous vessels, arterialized veins (medial thickening and fibrosis) • Most morbid, highest rate of recurrence

10. Noninvasive imaging • MRI • Duplex • Air plethysysmography • Volumetry • Whole-body blood pool scintigraphy • Uses tagged RBCs to detect abnormal blood collections • Radioisotope lymphoscintigraphy • Transarterial lung perfusion scintigraphy • Uses albumin to calculate shunting percentage • CT

11. Uterine AVM

12. Invasive imaging • Angiography • Venography • Lymphangiography

13. Indications for therapy • Absolute • Hemorrhage • High output cardiac failure • Ischemia • Chronic venous hypertension • Threat to life or vital function • Relative • Disabling pain • Functional impairment • Cosmetically severe deformity • Vascular – bone syndrome • High risk of complications • Lymph leak

14. Therapy • High flow lesions – Endovascular therapy • Direct puncture, transarterial, or transvenous approach • Superselective catheterization of feeding vessels until identification of nidus • Embolization with polymerizing agents, ethanol (toxic,) detachable balloons, coils can be used definitively • Nitinol mesh plug successful with large AVMs • Temporary occlusion with foams or collagen can be used for preoperative devascularization • Complications include end organ ischemia, embolization, tissue destruction by ethanol, severe swelling with progression to compartment syndrome, PE, arterial emboli, MI from ethanol

15. AVM embolization

16. Coil embolization of pulmonary AVM

17. Therapy • High flow lesions - open surgical therapy • Often not possible or horribly morbid operations due to complexity and location of lesions • Most suitable for well circumscribed lesions affecting the • Should involve ligation of all feeding vessels and entire malformation unless doing so would result in end organ ischemia, the only potential for cure is complete eradication of the nidus • Incomplete resection can lead to recurrence or block access from an endovascular approach

18. Therapy • Low flow lesions • Sclerotherapy with ethanol (toxic,) sodium tetradecyl sulfate, polidocanol, ethanolamine oleate, or foam sclerotherapy • Induces direct damage to endothelium resulting in fibrosis and thrombosis • Large lesions require multiple treatments • Complications include tissue destruction by ethanol, nerve damage from slerosing agents, severe swelling with progression to compartment syndrome

19. Congenital Vascular Malformations • Complex problem with a wide array of manifestations • Therapy often ineffective or palliative, many interventions may be required - complete lesion eradication in only 15% of patients