Management of Combined Vascular Malformations

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Capillary-lymphatico-venous malformation

The first reports of patients with a slow-flow capillary-lymphatico-venous malformation (CLVM) were published in the nineteenth century by Hilaire, Trélat, and Monod.2, 3 It was not until 1900 that this constellation of findings was considered more than mere coincidence. French physicians, Maurice Klippel and Paul Trénaunay,4 were the first to recognize Klippel-Trénaunay syndrome as a distinct entity.5 They proposed the main characteristics of the syndrome were a localized vascular nevus,

Capillary-arteriovenous malformation and capillary-arteriovenous fistulas

Capillary-arteriovenous malformation (CAVM) and capillary-arteriovenous fistulas (CAVFs) correspond to the old eponym Parkes Weber syndrome. This syndrome is characterized by the presence of a confluent or patchy CM with underlying multiple microarteriovenous fistulas in association with soft tissue and skeletal hypertrophy of the affected limb (Fig. 10).7, 34 There is often an associated lymphatic component. Like other vascular malformations, the diagnosis may be suspected antenatally but

CLOVES syndrome

Congenital lipomatous overgrowth, vascular malformations, epidermal nevi, and skeletal anomalies (CLOVES) syndrome is a newly recognized syndrome.37, 38 Its main features are truncal lipomatous masses, vascular malformations, and acral/musculoskeletal anomalies (Fig. 11). Not all features must be present to make the diagnosis of CLOVES syndrome. Until recently, many patients with this syndrome were misdiagnosed as either having CLVM or Proteus syndrome. Like CLVM and Parkes Weber syndrome,

Summary

Proper diagnosis of patients affected by complex combined vascular malformations is essential. These patients benefit from an interdisciplinary approach involving many medical and surgical specialists. Interventions must be tailored to the specific needs and symptoms of the patient. Outcomes are optimized with careful preoperative planning, identification of comorbidities, and realistic expectations on behalf of both the patient and surgeon.

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