Vascular malformations of head and neck

Classification

Clinical features

• Painless swelling or facial asymmetry
• Cosmetic deformity
• Pain and thrombosis in VM
• Recurrent infection, sudden enlargement, dysphagia, dysphonia or airway obstruction in LM
• Ulceration, bleeding, pulsation, bruit and high-output cardiac failure in extensive AVM
• Oral cavity lesions may cause macroglossia, speech and feeding difficulty.

Investigation flowchart

Suspected vascular malformation
            ↓
History + examination
- Present since birth?
- Compressibility, colour, bruit/thrill
- Airway, feeding, speech, bleeding symptoms
            ↓
Assess urgency
            ↓
Airway compromise / major bleeding?
     ┌───────────────┴───────────────┐
     ↓                               ↓
Yes                             No
Secure airway, control          Doppler ultrasonography
bleeding, urgent                to distinguish low-flow
multidisciplinary review        from high-flow lesion
                                     ↓
                              MRI with contrast
                         defines extent and tissue planes
                                     ↓
                  MR angiography / catheter angiography
                    if AVM or embolization is planned

Management flowchart

Vascular malformation confirmed
            ↓
Multidisciplinary vascular-anomaly team
(ENT, interventional radiology, plastic surgery,
dermatology, anesthesia)
            ↓
Asymptomatic, small, non-progressive?
     ┌───────────────┴────────────────┐
     ↓                                ↓
Yes                              Symptomatic / progressive
Observe, counsel,               pain, disfigurement, bleeding,
photographs and follow-up       functional or airway problem
                                         ↓
                          Identify flow type
     ┌───────────────────┬─────────────────────┬───────────────────┐
     ↓                   ↓                     ↓
Capillary            Venous / LM               AVM
Laser therapy        Image-guided              Staged embolization
for superficial      sclerotherapy             followed by complete
lesions              ± laser / surgery         surgical excision if feasible

Treatment according to type

1. Capillary malformation
   • Observation if mild.
   • Pulsed-dye laser is useful for port-wine stains.
   • Assess facial port-wine stain in trigeminal distribution for associated glaucoma/Sturge-Weber syndrome.
2. Venous malformation
   • Conservative: reassurance, analgesia, compression where feasible.
   • Image-guided sclerotherapy is first-line for symptomatic lesions.
   • Surgical excision for small, localized, well-defined lesions or residual deformity after sclerotherapy.
   • Laser can treat superficial mucosal or cutaneous components.
3. Lymphatic malformation
   • Observe uncomplicated localized macrocystic lesions.
   • Treat infection-related swelling with antibiotics, with corticosteroids in selected severe inflammatory episodes.
   • Macrocystic LM: aspiration and sclerotherapy, often effective.
   • Microcystic/mixed or extensive disease: repeated sclerotherapy, debulking surgery, laser for mucosal disease; systemic sirolimus may be considered in difficult extensive lesions.
   • Secure airway first if tongue, floor-of-mouth, pharyngeal or laryngeal involvement causes obstruction.
4. Arteriovenous malformation
   • Avoid incomplete excision or simple ligation because recurrence/progression can occur.
   • Angiographic embolization to reduce flow, usually followed by definitive complete surgical excision where possible.
   • Repeated staged procedures may be necessary.

Key exam point

Do not confuse vascular malformation with infantile haemangioma.
Haemangioma is a vascular tumour with a proliferative phase followed by involution. Vascular malformation is present from birth, grows with the patient, and persists.