Cervical lymphatic malformations: Prenatal characteristics and ex utero intrapartum treatment

Alireza A. Shamshirsaz*, Kelsey A. Stewart, Hadi Erfani, Ahmed A. Nassr, Nathan C. Sundgren, Amy R. Mehollin-Ray, Shaine A. Morris, Jimmy Espinoza, Magdalena Sanz Cortes, Christopher Cassady, Timothy C. Lee, Eumenia C. Castro, Olutoyin A. Olutoye, Deepak K. Mehta, Darrell Cass, Oluyinka O. Olutoye, Michael A. Belfort

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

14 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)287-292
Number of pages6
JournalPrenatal Diagnosis
Issue number4
StatePublished - Mar 2019

ASJC Scopus subject areas

  • Obstetrics and Gynecology
  • Genetics(clinical)


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