Abstract 1367

A 28 yo G1 woman presented to perinatology clinic with a 19 week fetal ultrasound showing a septated, anterior nuchal mass. Chromosomes were 46 XY. The mass slowly increased in size over subsequent weeks, then doubled in size between 31 to 32 weeks. There was extension into the mediastinum, deviation of the trachea and polyhydramnios. A multidisciplinary conference regarding management was held with perinatology, neonatology, obstetrical anesthesia and pediatric ENT. Plans were made for delivery using the EXIT procedure (Mychaliska, et al, 1997), or Ex-Utero Intrapartum Treatment. This method of deep maternal anesthesia and full relaxation of the uterus provides time for the airway of the neonate to be secured while uteroplacental gas exchange is preserved. Vecuronium can be given IM to the neonate while procedures are performed. This infant was born at 35 weeks, and uteroplacental gas exchange was maintained for 16 min while ENT performed rigid bronchoscopy and then placed the endotracheal tube. The mass was a cystic hygroma with involvement of the mandible and soft tissues of the left face, neck, and extending posteriorly to the right side of the neck. The trachea was displaced to the right and the left jugular was 1 cm in diameter. Hygroma tissue extended deeply into the mediastinum. Excision of the mass and tracheostomy were done in the first week of life. Two more drainage procedures were required during the hospitalization. He was discharged on oral feedings on day of life 56. At one year of age, the infant has had normal development. Plans are in progress to use the OK-432 (Picibanil) streptococcal antigen as a sclerosing agent, to eradicate the remaining hygroma tissue.

This case is an illustration of the use of a highly specialized, multidisciplinary team approach to a complicated and potentially life-threatening neonatal disease. The efficacy of the EXIT procedure is dependent on the development of this team approach.