Fetal Cystic Lymphatic Malformations
Systematic Review on Pregnancy and Neonatal Outcomes
Corresponding Author
Diane Nzelu MB ChB, MRCOG, MD
Women's Health, University College London Hospitals NHS Foundation Trust, London, UK
Address correspondence to Diane Nzelu, Consultant in Maternal-Fetal Medicine and Obstetrics, Elizabeth Garrett Anderson Wing, University College Hospital NHS Trust Foundation, 25 Grafton Way, London, WC1E 6DB, UK.
E-mail: [email protected]
Search for more papers by this authorIsmini Panayotidis MBBS
Women's Health, Barking, Havering and Redbridge University Hospitals NHS Trust, London, UK
Search for more papers by this authorGill D. Smith MB BCh, FRCS(Ed), FRCS(Plast)
Plastic Surgery, Great Ormond Street Hospital for Children NHS Trust, London, UK
Search for more papers by this authorPranav Pandya BSc MD, FRCOG
Women's Health, University College London Hospitals NHS Foundation Trust, London, UK
Search for more papers by this authorCorresponding Author
Diane Nzelu MB ChB, MRCOG, MD
Women's Health, University College London Hospitals NHS Foundation Trust, London, UK
Address correspondence to Diane Nzelu, Consultant in Maternal-Fetal Medicine and Obstetrics, Elizabeth Garrett Anderson Wing, University College Hospital NHS Trust Foundation, 25 Grafton Way, London, WC1E 6DB, UK.
E-mail: [email protected]
Search for more papers by this authorIsmini Panayotidis MBBS
Women's Health, Barking, Havering and Redbridge University Hospitals NHS Trust, London, UK
Search for more papers by this authorGill D. Smith MB BCh, FRCS(Ed), FRCS(Plast)
Plastic Surgery, Great Ormond Street Hospital for Children NHS Trust, London, UK
Search for more papers by this authorPranav Pandya BSc MD, FRCOG
Women's Health, University College London Hospitals NHS Foundation Trust, London, UK
Search for more papers by this authorDiane Nzelu and Ismini Panayotidis should be considered joint first authors.
Abstract
Objective
Evaluate pregnancy and neonatal outcomes with fetal cystic lymphatic malformations (LMs), excluding those arising from the posterior neck, to facilitate patient counseling.
Method
A systematic review was performed in accordance with PRISMA guidance. Case series and case reports published between 2000 and 2022 were included.
Results
Sixty-five studies (96 fetuses) met the inclusion criteria. The average gestational age at diagnosis was 25.5 weeks with the commonest location being the anterior neck (28%). All patients were diagnosed with LM using two-dimensional (2D) ultrasound. Prenatal progression in LM size, presence of intralesional bleeding, or fetal hydrops occurred in 70% (41/59), 9% (5/59), and 3% (2/59), respectively. Chromosomal and structural abnormalities were reported in 4% (2/52) and 2% (2/96), respectively. Overall livebirth rate was 94% (79/84); 12/96 resulted in termination and 5/84 in in utero demise.
The average gestational age of delivery was 37.7 weeks. Exactly 19% (15/79) had a vaginal birth, of which shoulder dystocia occurred in one infant. Ex utero intrapartum treatment (EXIT) procedure was performed in 13% (10/79). Postnatal treatment commonly involved surgical excision 38% (30/79), sclerotherapy in 21.5% (17/79), or combination of both in 11.4% (9/79). Of those with reported follow-up, 4 died within 1 year, 1 developed heart failure at 2 years of life, and the remaining 44 had normal developmental outcomes.
Conclusion
Fetal cystic LMs, excluding those in the posterior neck, are not commonly associated with chromosomal, or additional structural abnormalities. They usually increase in size before delivery with only a minority developing complications. The good developmental outcome was reported in all survivors.
Open Research
Data Availability Statement
The data that support the findings of this study are available on request from the corresponding author. The data are not publicly available due to privacy or ethical restrictions.
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