OBSOLETE: Cervicofacial lymphatic malformation

Cervicofacial lymphatic malformation is a congenital vascular anomaly characterized by abnormal development of lymphatic vessels in the head, neck, and face regions. These malformations consist of fluid-filled cystic spaces lined by lymphatic endothelium and can range from small, localized lesions to large, infiltrative masses that distort facial and cervical anatomy. They are often detected prenatally or at birth and may cause significant cosmetic disfigurement, airway compromise, feeding difficulties, speech problems, and recurrent infections (cellulitis or intralesional bleeding). The malformations are classified as macrocystic, microcystic, or mixed types, which influences treatment decisions. This Orphanet entry (137923) is marked as OBSOLETE, meaning it has been retired or merged into a broader classification of lymphatic malformations. Patients previously categorized under this term may now be classified under more current nomenclature consistent with the International Society for the Study of Vascular Anomalies (ISSVA) classification system. Treatment options include sclerotherapy (injection of sclerosing agents into cystic spaces), surgical excision, and in some cases medical therapies such as sirolimus, which has shown efficacy in reducing the size of lymphatic malformations. Macrocystic lesions tend to respond better to sclerotherapy, while microcystic or mixed lesions may require surgical intervention or combined approaches. Management is typically multidisciplinary, involving specialists in vascular anomalies, otolaryngology, plastic surgery, and interventional radiology.

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