Capillary malformation
Presentation: “port wine stain”. Flat, pink, blanching lesion
Imaging: only required for facial lesions or other finding suspicious for Sturge–Weber suspected. Head MRI: leptomeningeal vascular malformation, calcification, retinal angiomatosis
Treatment: First line: pulsed dye laser; typically requires multiple sessions. Alternatives: intense pulse light; photodynamic therapy
Prognosis: highly variable: 10% complete response; 20–30% are completely resistant
Venous malformation
Presentation: blue-tinted lesions that expand with Valsalva and compression. Associated with frequent bleeding, adjacent skin discoloration and pain
Imaging: T2weighted short tau inversion–recovery: preferred MR sequence to evaluate lesion size and vascular flow rate. Typically demonstrates hyperintense, septated, cystic lesions, often with phleboliths. T1 weighted MR with contrast demonstrates heterogeneous enhancement
Treatment: First line: compression devices for benign lesions. Sclerotherapy is first line for painful lesions and those causing disfigurement. Common agents include: concentrated ethanol, foamed sodium tetradecyl sulfate (STS), foamed polidocanol
Prognosis: Sclerotherapy is often curative for focal venous malformations (VMs), whereas diffuse VMs at increased risk of recurrence and require multiple treatments
Microcystic lymphatic malformations (<1 cm)
Presentation: present at birth, most commonly on the head and neck. Skin-coloured masses ballotable to palpation, with red or yellow blisters when involving the mucous membrane
Imaging: T1 weighted MRI: diffuse hypointensity. T2 weighted MRI: diffuse hyperintensity
Treatment: surgical resection. Reserved for lesions at risk of significant complication
Prognosis: Curative treatment rare and recurrence common
Macrocystic lymphatic malformations (>1 cm)
Presentation: less common, appear later in life and are more prone to haemorrhage and infection
Imaging: T1weighted MRI: septated cystic mass with hypo- to isointense heterogeneity that do not enhance with contrast. T2weighted MRI: well-defined, lobulated, hyperintense mass
Treatment: Sclerotherapy: bleomycin, ethanol, STS and doxycycline. Reserved for lesions at risk of significant complication
Prognosis: varies with lesion characteristics and sclerosing agent used. Typically good response, but rarely achieve complete resolution
Arteriovenous malformation
Presentation: warm pink-bluish lesions with a pulsatile thrill, can progress to dystrophic skin changes, bleeding, ulceration and necrosis. Associated heart failure in severe cases
Imaging: T1and T2weighted MRI: mesh of flow voids often with visible shunt. CT angiography and MR angiography: both enable arteriovenous malformation (AVM) flow analysis by determining the size and location of the AVM nidus, arterial feeders and shunt volume. Angiography: dilated arteries with early filling of enlarged draining veins beginning at the nidus
Treatment: embolization with or without surgical resection. Embolic agents: ethanol, n-butyl cyanoacrylate glue, onyx, coils. No definitive consensus exists regarding the most effective embolic agent
Prognosis: dependent on size, location and flow rate of lesion. Embolization followed by surgical resection provides best chance for curative treatment