Cervicofacial lymphatic malformation: clinical course, surgical intervention, and pathogenesis of skeletal hypertrophy.

Abstract

This is a retrospective review of the clinical course and long-term soft-tissue/skeletal problems in 17 patients with large cervicofacial lymphatic malformations. Morbidity included infection (71 percent), airway compromise requiring tracheostomy (65 percent), poor dental health with aggressive caries (53 percent), abnormal articulatory patterns (47 percent), and episodic bleeding (35 percent). All patients underwent soft-tissue excision (mean four procedures per patient). Damage to facial nerve (76 percent) and hypoglossal nerve (24 percent) were common postoperative sequelae. Contour resection did not alter the progression of skeletal hypertrophy. Overgrowth most commonly occurred in the mandibular body, manifesting as anterior open bite deformity and class III occlusion (65 percent). Early mandibular body ostectomy was done in four children with grotesque hypertrophy. Jaw osteotomy was required in 71 percent of the patients to improve the maxillary/mandibular relationship. Histologic examination revealed intraosseous lymphatic malformation in areas of skeletal overgrowth in two-thirds of surgical specimens. The complexity of managing cervicofacial lymphatic malformation underscores the need for an interdisciplinary program in every major referral center.