Distensible Venous Malformations (Varices)



Distensible Venous Malformations (Varices)







Key Points



  • Distensible venous malformations (DVMs) are abnormally enlarged and tortuous veins, or less commonly


  • Periocular DVMs are more commonly seen in the orbit than in the eyelid


  • They may develop from a congenital or acquired weakness of the involved vessel wall, obstruction of circulation, compression by a tumor, or trauma or infection that involves the vessel


  • DVM presents as a unilateral, nontender, well-circumscribed, soft, compressible subcutaneous mass that often enlarges with a Valsalva maneuver


  • In most cases, an eyelid DVM can safely be observed


  • Symptomatic DVMs in the head and neck region are preferentially treated surgically


  • Alternative therapy can include embolization with transvenous coiling or n-butyl cyanoacrylate glue or sclerotherapy using 0.75% sodium tetradecyl sulfate


  • Complete surgical excision gives excellent cosmetic results, and recurrences are rare


Low-flow vascular malformations in the orbit are divided into venous malformations (VMs), lymphatic malformations, and combined venous-lymphatic malformations (VLMs).1,2 Pure VMs include distensible and nondistensible venous malformations.1,2 Distensible venous malformations (DVMs) are characterized by the presence of abnormally enlarged and tortuous veins, which may or may not be associated with lymphatic vessels.1,2 The classical example of a nondistensible VM in the orbit is the cavernous venous malformation, which is discussed in Chapter 54. On the other hand, combined VLMs include two types or varieties based on hemodynamics: the distensible venous dominant (VD-VLM), and the nondistensible lymphatic dominant (LD-VLM).2

DVMs were previously referred to as varices, but that term is no longer used in the International Society for the Study of Vascular Anomalies 2018 classification. DVMs are abnormally enlarged and tortuous veins, which may harbor lymphatic or arterial elements.1,2 Periocular DVMs are more commonly seen in the orbit than in the eyelid, and eyelid DVMs frequently represent an extension from the orbit, usually from the anterior feeders and portions of the superior ophthalmic vein.3,4,5,6,7 They can also represent venous abnormalities associated with facial veins that extend to the eyelids, especially the angular vein. In some instances, facial DVM may be seen in genetic disorders such as Klippel-Trenaunay-Weber syndrome. This is a rare congenital condition usually exhibiting excessive growth of bones and soft-tissue vascular anomalies including port wine stains, hemangiomas, and varicose veins that commonly appear in the facial area,8 and associated with additional VMs in other locations such as the legs, arms, or abdomen.9

DVM can be imaged with ultrasound and color Doppler,10 which will show the dilated blood vessel, and the nonpulsatile blood flow confirms the passive or venous nature of the blood flow. Since the majority of eyelid DVMs are associated with orbital vascular anomalies, any new or spontaneous-onset of eyelid DVM should elicit orbital imaging.6,11 Magnetic resonance venography and conventional phlebography can confirm the diagnosis and allow visualization of the location and extent of the lesion, its anatomic relations and boundaries with surrounding tissues, and the inflow and outflow vessels.12


Etiology and Pathogenesis

DVM may develop from a congenital or acquired weakness of the involved vessel wall, or obstruction of circulation, or both.5,6 They can also result from compression by a tumor or an arterial aneurysm over an adjacent vein, an arteriovenous malformation, or any trauma or infection that involves the wall or lumen of the vessel.6,13


Clinical Characteristics

Eyelid DVMs typically present as a unilateral, nontender, well-circumscribed, soft, dark blue to brown or purple, compressible subcutaneous mass with no associated audible bruit or thrill (Figure 60.1). The lesion often enlarges with any activity that increases venous pressure, such as bending over for several minutes, or from a Valsalva maneuver with pressure exerted on the jugular vein, such as coughing, straining, and crying (Figure 60.2).5,13 When subtle, a DVM may not enlarge significantly with Valsalva to be clinically apparent but if such scenario is encountered distensibility with Valsalva can be elicited with radiology.2,14 They usually do not cause eyelid dysfunction, but they can be complicated by inflammation or by thrombus with hemosiderin and dystrophic calcification.15
If thrombosis occurs, the patient usually presents with acute pain, the lesion may be firm, less compressible, and may not enlarge with Valsalva.2 DVM can rupture with minor trauma or vomiting, resulting in hemorrhage and ecchymosis.16 If the eyelid lesion is an extension of an orbital varix, a deep hemorrhage can present with acute pain, proptosis, and motility restriction.7