Most cases are sporadic, although a few cases reported as AD
Deep vein hypoplasia & absence of valves, with resultant obstruction of venous flow, lead to venous hypertension, development of varices, and limb hypertrophy
Mesodermal defect during fetal development causes maintenance of microscopic arteriovenous communications
Clinical
Generally affects a single extremity, although cases of multiple affected limbs reported
Leg most common site (then arms > trunk, rarely head/ neck)
90% limbs
10% head & neck involvement
Haemangioma is often noted on the lateral aspect of the limb
May be limited to skin or extend deeper to subcutaneous tissue, including muscle & bone
Visceral organs, such as pleura, spleen, liver, bladder, and colon may also be affected.
Visceral organ involvement portends greater morbidity secondary to internal haemorrhage
The Klippel-Trenaunay vein is a large lateral (i.e. atypical for a varicosity), superficial vein sometimes seen at birth
Vein begins in the foot or the lower leg and travels proximally until it enters thigh or gluteal area
Varicosities may be extensive, though often spare saphenous distribution
Varicosities may affect superficial, deep, and perforating veins
Varicosities may remain stable or gradually expand
Pain and lymphoedema common.
Symptoms may worsen during pregnancy
Pathology
Deep veins often anomalous or absent
So saphenous vein stripping = Hazardous!
Investigations
Evaluation of the deep venous system can be completed with duplex scanning contrast venography, USS, contrast venography and arteriography and nuclear MRI studies
Arteriography is especially helpful in the diagnosis of AV fistula
Management
Conservative if asymptomatic
Compression garments (usually class II) for chronic venous insufficiency, lymphoedema, recurrent cellulitis, and recurrent bleeding from capillary or venous malformations
However, if absent or hypoplastic deep venous system, elastic compression may increase venous stasis and cause discomfort
Referral to a pain clinic
Cellulitis and thrombophlebitis - analgesics, elevation, antibiotics & corticosteroids
Anticoagulant therapy - acute thrombosis/ prophylactically prior to surgical procedures
Increased risk of thrombotic events, avoid using OCP
Laser treatment for haemangioma
Can be effective in lightening colour of port-wine stain
Indicated in case of ulceration
Surgical intervention for varicosities & venous malformations controversial
Venous stripping, ligation, excision or sclerotherapy are contraindicated unless the surgery involves the superficial system and the underlying deep system is normal or demonstrates only mild-to-moderate reflux
Inadequate evaluation prior to excision increases surgical complications
Radiotherapy has been reported to be of help
Induce regression of haemangiomas; however results can be slow to develop
Endovenous laser therapy of the greater saphenous vein gaining support