Review Airway compromise in the fetus and neonate: Prenatal assessment and perinatal management Greg Ryan a, b, * , Stig Somme c , Timothy M. Crombleholme c a Fetal Medicine Program, Mount Sinai Hospital, Toronto, Canada b Departments of Obstetrics & Gynaecology and Medical Imaging, Division of MaternaleFetal Medicine, University of Toronto, Toronto, ON, Canada c Colorado Fetal Care Center, Colorado Institute for Maternal and Fetal Health, Division of Pediatric General, Thoracic and Fetal Surgery, University of Colorado School of Medicine, Children's Hospital Colorado, Aurora, CO, USA Keywords: Ex-utero intrapartum treatment (EXIT) Cervical teratoma Laryngeal atresia Congenital high airway obstruction syndrome (CHAOS) Micrognathia Pierre Robin sequence summary The fetus with a potentially obstructed airway can be identified on routine antenatal imaging. These cases should be referred to fetal care centers, which have the necessary expertise to fully evaluate and manage these fetuses and neonates appropriately. Complete airway obstruction may result in fetal hydrops and intrauterine demise. If a newborn infant has a compromised airway at delivery, the inability to secure its airway quickly may result in a hypoxic cerebral insult or death. In the most severely affected cases, prenatal, perinatal, or postnatal surgical intervention may be necessary. The timing of such an intervention will depend on the exact cause of the airway obstruction, other associated findings and the anticipated difficulty in establishing an airway at delivery. Fetal ultrasound and magnetic resonance imaging can differentiate between intrinsic and extrinsic airway obstruction, which allows for the optimal planning and management of the delivery and neonatal resuscitation. © 2016 Elsevier Ltd. All rights reserved. 1. Introduction Fetal airway obstruction may be categorized into two groups: those that are extrinsic or intrinsic to the airway (Box 1). 2. Extrinsic airway obstruction Any cause of extrinsic airway obstruction, from the lips down to the main-stem bronchi, may result in problems during fetal development, and difficulties in establishing an airway at delivery. Extrinsic airway obstructions may be in an oral, cervical, or thoracic location and the most frequent causes are cervical teratomas or epignathus, cervical lymphangiomas, and micrognathia. 2.1. Oral 2.1.1. Oropharyngeal teratoma: epignathus Epignathus is a rare oropharyngeal teratoma arising from the sphenoid bone, palate or pharynx, with an estimated incidence of 1:35,000 to 1:200,000 [1]. It usually presents as a large mass pro- truding through the mouth and obstructing the upper airway. Im- mediate removal is often necessary at the time of delivery to allow an airway to be established by orotracheal intubation. A large epignathus tumor may present in the second trimester with poly- hydramnios and airway obstruction [2]. If pharyngeal obstruction is complete, the ensuing polyhydramnios may require serial amnioreductions to minimize the risks of premature labor and delivery [3]. Imaging with ultrasound and ultrafast magnetic resonance imaging (MRI) can provide important information about the mass (Fig. 1). Management of the fetus with epignathus is dictated by the degree of airway obstruction, gestational age at presentation and any other physiologic impact that the mass may have on the fetus. Some masses are small and do not completely obstruct the fetal airway and therefore can be managed by conventional intubation. By contrast, an epignathus which grows very rapidly to large exo- phytic proportions may completely obstruct the fetal airway and may even cause fetal hydrops from the increased blood flow through the tumor, resulting in a high output failure state. All such cases warrant urgent consultation at a fetal care center. Treatment involves resection of the mass, which, despite its large size, usually arises from a narrow pedicle on the fetal palate. Depending on gestation and severity, this might theoretically warrant * Corresponding author. Address: University of Toronto, 3201, 700 University Ave., Toronto, ON, M5G 1X5, Canada. Tel.: þ1 (416) 586 8415; fax: þ1 (416) 586 8617. E-mail address: gryan@mtsinai.on.ca (G. Ryan). Contents lists available at ScienceDirect Seminars in Fetal & Neonatal Medicine journal homepage: www.elsevier.com/locate/siny http://dx.doi.org/10.1016/j.siny.2016.03.002 1744-165X/© 2016 Elsevier Ltd. All rights reserved. Seminars in Fetal & Neonatal Medicine xxx (2016) 1e10 Please cite this article in press as: Ryan G, et al., Airway compromise in the fetus and neonate: Prenatal assessment and perinatal management, Seminars in Fetal & Neonatal Medicine (2016), http://dx.doi.org/10.1016/j.siny.2016.03.002