Prenatally Diagnosed Lymphatic Malformations of the Head and Neck

Abstract

Objectives: To describe prenatal diagnosis and perinatal management of patients with head and neck lymphatic malformations (LMs) at a tertiary fetal diagnostic and vascular anomalies center. To identify prenatal characteristics correlating with the need for airway intervention at birth. To describe postnatal clinical course in patients with prenatally diagnosed head and neck LMs. Methods: A retrospective review of all patients with head and neck LMs diagnosed in utero at a tertiary prenatal diagnostic center from 1996 to 2020 was performed. Prenatal records of pregnant patients and postnatal records of patients were reviewed. Univariate and multivariate logistic regression analyses were conducted to identify correlates of need for extrauterine intrapartum treatment (EXIT) delivery and correlates of clinical outcomes. Results: Over the study period, 26 fetuses were diagnosed with LM of the head and neck, 15 of which survived the perinatal period. Outcomes in these survivors are reported including birth modality, DeSerres’ stage, duration and modality of treatment, complications, hospitalizations, and functional outcomes including need for tracheostomy and gastrostomy tube. The majority of patients (n = 8) were born via the EXIT modality. Almost half (n = 7) were DeSerres’ stage V. Prenatal imaging findings of neck vessel compression (p = 0.023) and tracheal deviation (p = 0.006) correlated with EXIT delivery. The most common treatment modalities were sclerotherapy (n = 14), surgery (n = 7), and sirolimus (n = 8). Conclusions: This study identified prenatal anatomic findings of tracheal deviation and neck vessel compression as factors associated with EXIT delivery. None of the patients in our series required tracheostomy during the EXIT procedure, although 20% eventually underwent tracheostomy. The majority of infants with prenatally diagnosed head and LMs required multimodality treatment with some combination of sclerotherapy, surgery, and medical management, as well as multidisciplinary care from our vascular anomalies team throughout the first years of life and beyond.