Lymphatic malformation of the lingual base and oral floor

Background: Lymphatic malformation of the tongue and floor of the mouth is associated with chronic airway problems, recurrent infection, and functional issues related to speech, oral hygiene, and malocclusion. There are no accepted anatomic guidelines or treatment protocols. Methods: This retrospective review focused on anatomic extent, treatment, complications, and airway management in 31 patients with lymphatic malformation of the lingual base and oral floor. Results: Involved adjacent structures included the neck (77 percent), mandible (41 percent), face (42 percent), lips (10 percent), pharynx (45 percent), and larynx (26 percent). Fifty-eight percent of patients required tracheostomy during infancy; decannulation was possible in two-thirds of these patients. Management included resection alone (42 percent), resection and sclerotherapy (26 percent), resection and laser coagulation (16 percent), sclerotherapy and laser coagulation (16 percent), and resection and radiofrequency ablation (3 percent). Resection involved the neck (58 percent), floor of the mouth (52 percent), and tongue (42 percent); there were often multiple procedures. Aspiration was tried with little success in 10 percent of patients. Virtually all patients had residual abnormal lymphatic tissue. Complications and post-therapeutic problems included infection (81 percent), neural damage (27 percent), difficulty in speech (23 percent), feeding problems (10 percent), and seroma or hematoma (6 percent). Associated dental/orthognathic conditions, particularly prognathism and anterior open bite, were documented in one-third of patients. Conclusions: The initial step in the protocol is control of the neonatal airway. Staged cervical resection is undertaken in late infancy to early childhood; resection should also include abnormal tissue in the oral floor. Sclerotherapy is primarily for macrocystic disease or secondarily for recurrent cysts following partial extirpation. Vesicles of the mucous membranes and dorsal tongue are treated either by sclerotherapy, laser (carbon dioxide, yttrium-aluminum-garnet, or potassium-titanylphosphate), or radiofrequency ablation. Reduction for macroglossia is indicated for persistent protrusion or to allow correction of malocclusion. Embolization controls lingual bleeding. Orthognathic procedures are undertaken at the appropriate age, only after lingual size and position are acceptable.