Table 3 Summary for treatment algorithm of lymphatic malformations presenting at common locations
From: Lymphatic malformations: a 9-year experience at the vascular anomaly clinic
Region | Subtypes | Pathology | Complications/presentation | Treatment |
|---|---|---|---|---|
Neck | Type 1: posterior triangle | Mostly macrocystic; usually unilateral | Mostly disfigurement Infection, pain | 1st line: injection sclerotherapy 2nd line: surgical excision |
Type 2: submandibular | Significant microcystic component; may be bilateral + tongue involvement | Potential risk of airway obstruction due to midline extension Disfigurement, infection, pain | May need emergency procedure to secure airway (Fig. 3) 1st line: injection sclerotherapy ± sirolimus 2nd line: surgical debulking | |
Head | Parotid | Macrocystic / mixed | Mostly disfigurement Infection, pain | Injection sclerotherapy |
Face (cheek, forehead) | Microcystic | Mostly disfigurement | Combined treatment: injection sclerotherapy, sirolimus, surgery through hidden scars (Fig. 6) | |
Tongue (Fig. 9) | Microcystic | Localized painful and bleeding mucosal lesions/vesicles Generalized macroglossia | Sirolimus can rapidly control pain and bleeding Injection usually has poor response Surgery for localised lesions or reduction glosso-plasty | |
Retro-orbital | Medium sized cysts | Proptosis | Sirolimus Injection sclerotherapy | |
Lips | Microcystic | Disfigurement | Injection sclerotherapy Surgery to reduce the size | |
Limbs and trunk | Axilla, chest wall, groin | Mostly macrocystic | Mostly disfigurement Infection, pain | Injection sclerotherapy Surgery (debulking) |
Gluteal | Combined macro-/micro cystic | Mostly disfigurement Infection, pain (Fig. 8) | Surgical excision/ injection sclerotherapy | |
Arms, forearms, legs | Macrocystic | Mostly disfigurement Infection, pain | Surgical excision/ injection sclerotherapy |