Cervical lymphatic malformations: Prenatal characteristics and ex utero intrapartum treatment

Abstract

Background The ex utero intrapartum treatment (EXIT) is utilized to transition fetuses with prenatally diagnosed airway obstruction to postnatal life. We describe the unique clinical course, diagnosis, treatment, and outcomes of patients with cervical lymphatic malformation (CLM) managed with EXIT. Methods Review of fetuses with diagnosed CLM was delivered by EXIT (2001‐2018) in a tertiary referral fetal center. Outcomes included survival, tracheostomy at discharge, neonatal course after delivery, and pulmonary hypoplasia. Data are reported as median [range] and rate (%). Results Out of 45 patients delivered by EXIT, 10 were delivered for CLM: seven had polyhydramnios, one had nonimmune hydrops, five delivered preterm, and three were emergency EXITs. The EXIT time and estimated blood loss were 125 minutes (95, 158) and 900 mL (500, 1500), respectively. Airway was secured in all. There was one neonatal death (day 8) with prematurity, sepsis, and pulmonary hypoplasia. Three out of nine were discharged with a tracheostomy. Conclusion In CLM, close monitoring for structural neck involvement and development of polyhydramnios are important and may be an indication for EXIT as the optimal delivery mode. An experienced multidisciplinary team is a key factor for an effective approach to the obstructed airway in CLM.

Ex utero intrapartum treatment (EXIT) procedure: Indications and procedural considerations

“…Shamshirsaz et al 14 described ten cases of lymphatic malformation treated with EXIT procedure over an 18-year period. An airway was able to be secured in all ten cases with one case requiring drainage of a cyst prior to delivery 14 . Shamshirsaz et al 14 did not disclose the types of airways secured at the time of procedure but went on to explain that three of the infants were discharged home with a tracheostomy.…”

“…Fetal and placental position must also be assessed prior to commencement of the procedure. Breech position may require a modified EXIT as performed by Duek et al 4 , or external cephalic version and anterior placental position may require an upper segment uterine incision 14 .…”

“…Following administration of general anaesthesia, the obstetrician will commence the procedure. Ideally, a lower segment uterine incision is used; however, in the case of anterior placenta, such as that described by Shamshirsaz et al 14 , an upper segment hysterotomy may be necessary. Prior to uterine incision, the tone should be checked to ensure the uterus is adequately relaxed 8 .…”

“…Lymphatic malformations are another type of benign tumour, accounting for one case in every 20 000 births 14 and five per cent of benign tumours in infants 3 . Two thirds of cases are found in the head or neck 3 .…”

“…Two thirds of cases are found in the head or neck 3 . Pucher et al 3 explain that lymphatic malformations affect the airway slightly differently to teratomas with Shamshirsaz et al 14 confirming they tend to cause airway deviation rather than compression as they are softer than teratomas.…”