Massive lymphatic malformations of the head, neck, and chest.

OBJECTIVE: To provide clinicians with an overview of the complications and functional and clinical outcomes of children affected by massive lymphatic malformations affecting multiple sites in the head, neck, and/or chest.

METHODS: We reviewed the medical records of all patients who received treatment for massive cervicofacial lymphatic malformations at our tertiary care pediatric institution during a 17-year period. Massive lesions were defined as (1) unilateral lesions that infiltrated at least 50% of two anatomic regions, including the head, neck, or chest; (2) unilateral lesions in which there was involvement of the midline structures of the head or neck; or (3) bilateral lesions in which 50% of each side was affected. Data consisted of critical clinical parameters, including extent of anatomic involvement, treatment interventions, pre- and post-treatment complications, lesion resolution, and functional outcomes.

RESULTS: Sixteen patients were treated for massive head, neck, and chest lymphatic malformations. Ten of 16 had massive unilateral lesions, whereas 6 had bilateral lesions. Eleven patients experienced airway compromise resulting from airway involvement; eight of these patients required placement of a tracheostomy tube. Six patients, all of whom had airway compromise, suffered with orodigestive complications; four of these six required gastrostomy tube placement. Eleven of 16 patients were managed exclusively with surgery, and 3 of 11 showed marked (60-80%) disease resolution; however, all 3 of these patients required preoperative placement of a tracheostomy tube, and only 1 was decannulated following resection. The severity of the airway symptoms resulted in the death of three patients, all of whom had tracheostomies.

CONCLUSIONS: Children with massive head, neck, and chest lymphatic malformations suffer from high morbidity and significant mortality. Airway involvement is an important prognostic factor in these patients.