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Prenat Diagn. 2019 Mar;39(4):287-292. doi: 10.1002/pd.5428.

Cervical lymphatic malformations: Prenatal characteristics and ex utero intrapartum treatment.

Author information

1
Division of Maternal Fetal Medicine, Department of Obstetrics and Gynecology, Baylor College of Medicine and Texas Children's Hospital, Houston, TX, USA.
2
Department of Obstetrics and Gynecology, Women's Health Hospital, Assiut University, Assiut, Egypt.
3
Division of Neonatology, Department of Pediatrics, Baylor College of Medicine and Texas Children's Hospital, Houston, TX, USA.
4
Department of Radiology, Baylor College of Medicine and Texas Children's Hospital, Houston, TX, USA.
5
Division of Pediatric Cardiology, Department of Pediatrics, Baylor College of Medicine and Texas Children's Hospital, Houston, TX, USA.
6
Division of Pediatric Surgery, Department of Surgery, Baylor College of Medicine and Texas Children's Hospital, Houston, TX, USA.
7
Department of Pathology & Immunology, Baylor College of Medicine and Texas Children's Hospital, Houston, TX, USA.
8
Department of Anesthesiology, Baylor College of Medicine and Texas Children's Hospital, Houston, TX, USA.
9
Department of Otolaryngology-Head and Neck Surgery, Baylor College of Medicine and Texas Children's Hospital, Houston, TX, USA.
10
Division of Pediatric Surgery, Department of Surgery, Cleveland Clinic Foundation, Cleveland, OH, USA.

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.

PMID:
30707444
DOI:
10.1002/pd.5428

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