lymphangioma

Lymphangioma is a benign malformation of the lymphatic system characterized by abnormal dilatation of lymphatic channels. Most lesions are congenital, presenting at birth or in early childhood; some may become apparent after infection or trauma. They are typically slow-growing and noninvasive, but can cause functional or cosmetic problems depending on location and size.

Lymphangiomas are traditionally categorized by the size and location of cystic spaces: cystic lymphangioma (cystic hygroma) comprises large macrocystic spaces; cavernous lymphangioma contains larger lymphatic channels within the deep tissues; capillary lymphangioma features small, superficial channels. Lymphangiomatosis denotes multifocal or diffuse involvement.

Most arise in the head and neck region (about 60-75%), followed by axilla, mediastinum, and retroperitoneum. Lesions are usually soft, compressible, and transilluminate. In the neck, airway compromise or dysphagia can occur. Intra-abdominal or extensive disease may present with abdominal distention, pain, or cosmetic concerns. Infections can complicate lymphangiomas.

Ultrasound is often the first imaging modality; it shows an anechoic or hypoechoic multicystic lesion. MRI provides better delineation of extent and relation to surrounding structures, while CT is used when MRI is contraindicated. Definitive diagnosis is by histopathology showing dilated lymphatic vessels lined by endothelium, with lymphatic fluid.

Management is individualized. Small, asymptomatic lesions may be observed. Symptomatic, progressive, or cosmetically disturbing lesions are commonly treated with sclerotherapy (eg, agents such as doxycycline, bleomycin, or OK-432) or surgical excision. Laser therapy or combined modalities is used in select cases. Recurrence is common after incomplete removal.

Overall prognosis is favorable for isolated lesions; prognosis worsens with diffuse lymphangiomatosis or multisystem involvement. Potential complications include infection, hemorrhage, rupture, and chylous leakage.