Management of giant cystic hygromas in infants

Abstract

Giant cystic lymphangioma is a rare lesion that presents major therapeutic challenges. Four infants presented in the first weeks of life with diffuse cysts in the neck. The tongue was involved in three, the mediastinum in one, and the pharynx and supraglottic larynx in two. These lesions are characterized by hundreds of cysts that infiltrate in and around muscles, nerves, and vessels. Complete excision is not possible without damaging the normal structures. All four infants initially had partial removal with unroofing and drainage of the remaining cysts and temporary tracheostomy to protect the airway. One infant died from a thrombosed sagittal sinus. She had severe venous congestion of the head and neck postoperatively which may have been caused by an overzealous attempt to remove all of the cysts. The other three have required nine additional operations for removal of recurrent neck cysts (one operation), removal of mediastinal cysts (one operation), partial glossectomy (three operations), and laser excision of lymphangiomas of the pharynx and supraglottic larynx (four operations). Two patients have required partial glossectomy which should be performed early to protect the airway, to allow normal speech development, and to prevent malocclusion and prognathism from constant pressure of the tongue against the teeth. Removal of wedges of tissue from each side and from the center of the dorsum of the tongue reduces bulk with minimal risk to the lingual arteries and nerves. Recurrent tongue enlargement is common and can be treated by repeated partial glossectomy. Lymphangiomatosis of the pharynx and supraglottic larynx is difficult to treat. The carbon dioxide laser allows very precise excision of individual cysts with minimal drainage to adjacent mucosa, but in our limited experience it has not prevented recurrence. Despite the extensive nature of these lesions, the impossibility of complete excision, and the need for multiple operations, the long-term results in the three surviving patients have been satisfactory. Asymmetry of the neck and face due to lymphedema tends to improve with time. All three children have an acceptable appearance and normal speech for their ages.