Massive lymphatic malformations of the head, neck, and chest

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.

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.

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.

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.