Magnetic Resonance Imaging as a Predictor of Outcome in Congenital Lymphatic Malformations of the Neck

Abstract

Objectives: To identify factors on magnetic resonance imaging (MRI) that predict the outcomes of children with congenital lymphatic malformations (LMs) of the neck. Design: Retrospective cohort study. Setting: The Royal Children’s Hospital, Melbourne, a quaternary pediatric referral hospital. Patients: All children referred to the vascular anomalies service with LM identified at birth or in the first month of life were born between 1991 and 2015. Outcome measurements: Pattern of involvement on MRI imaging, need for intervention for respiratory difficulty, documented episodes of infection, and aesthetic outcomes. Results: MRI images were available for 27 of 30 patients with LM of the neck present at or soon after birth. Of these, 10 (37%) met the primary outcome of undergoing any intervention whose intention was stated to be for airway compromise, which occurred between 7 days and 4 years of age. The intervention was more likely when MRI showed parapharyngeal involvement (P < .001), although not all cases with parapharyngeal disease required respiratory intervention. Risk increases if other regions of the neck were also involved. Overall, 2 broad patterns of disease were identified, a “posterior pattern” which was typically macrocystic and unilateral, and an “anterior pattern” which was microcystic and bilateral and more likely to have airway compromise. Thirteen patients (48%) had documented episodes of infection. Conclusion: Patterns of disease on MRI are identified which are associated with a greater risk of requiring intervention for airway obstruction. Children with congenital LM of the neck are at high risk of infective episodes.