Ex utero intrapartum treatment (EXIT) in the management of cervical lymphatic malformation

The purpose of this study was to review the outcomes and technical details of EXIT procedures performed in fetuses with large cervical lymphatic malformations.

A retrospective chart review of fetuses with a prenatal diagnosis of cervical lymphatic malformation evaluated at our center between 1995 and 2013 was performed.

We evaluated a total of 112 fetuses with a prenatal diagnosis of cervical lymphatic malformation. Thirteen of the 112 fetuses (11%) were delivered by an EXIT procedure. Criteria to deliver by EXIT were: 1) deviation/compression/obstruction of the airway, and 2) involvement of the floor of the mouth. Two fetuses developed hydrops. Five fetuses developed polyhydramnios. Eleven EXITs were performed electively at term (n=7; 37-38 weeks) or late pre-term (n=4; 34-36/6 weeks), whereas two patients underwent emergency EXIT at 33 and 38 weeks, respectively. The airway was accessed successfully in 12 of 13 cases. Laryngoscopy only was sufficient in 7, rigid bronchoscopy was required in 4, and 1 required a tracheostomy. In one case with a massive lymphatic malformation of the face, neck, and airway, a tracheostomy was not attempted, and the fetus expired. Four patients had invasion of the larynx by the lymphatic malformation. Five patients required a tracheostomy later. Median time from fetal exposure to intubation was 8 (2-29) min. Median total EXIT time was 105.5 (67-142) min. Median maternal blood loss was 800 (300-1000) ml. Median maternal hospital stay was 4 (3-6) days.

The EXIT procedure allows controlled airway access in fetuses with cervical lymphatic malformations and evidence of airway impairment on prenatal images.