Lymphatic malformations adjacent to the airway in neonates: Risk factors for outcome

ABSTRACT Purpose: To evaluate imaging, treatment, and outcomes in neonates with a lymphatic malformation (LM) adjacent to the airway and to evaluate risk factors that can predict outcome. Methods: A retrospective case series was conducted of ten patients treated between January 2011 and July 2019. The main outcome measures included airway compromise and clinical response to sclerotherapy ± surgery. Categorical data were compared using the Fisher's exact test. Results: Ex-utero intrapartum therapy was performed in four cases, among whom one died due to sepsis. All patients underwent schlerotherapy, with surgical debulking in two. Four patients showed a good clinical response, and five started experimental systemic treatment. Patients with bilateral disease and patients with an LM with >180 degrees tracheal surrounding were significantly at risk for airway compromise (bilateral: n=6/6 versus n=0/4, p=0.005; >180 degrees: n=5/5 versus n=1/5, p=0.048). The need for LM treatment in the neonatal period was indicative of a poor clinical response (‘non-responders’ 5/6, ‘responders’ 0/4; p=0.048). Conclusions: This study indicates that bilateral disease and >180 degrees tracheal surrounding are risk factors for airway compromise in neonates with an LM adjacent to the airway. In addition, the need for early treatment seems to be indicative of a poor clinical response.