P-676 Obstetric and perinatal outcomes in women with Turner Syndrome: A systematic review and meta-analysis
Authors: Nash Z , Walker A , Khattak H , Wattar B , Davies M 
Published: July 2024

Abstract

Study question
What is the pooled risk of adverse obstetric and neonatal outcomes in pregnancies in women with Turner Syndrome (TS)?

Summary answer
TS women experience high rates of adverse outcomes. Risks are increased in egg donation pregnancies and multiple pregnancies compared to singleton spontaneous pregnancies.

What is known already
TS affects 1 in 2,000 live female births. The 2017 ‘Clinical Practice Guidelines for the Care of Girls and Women with Turner Syndrome’ acknowledges women with TS are at increased risk of adverse obstetric and perinatal outcomes. Several retrospective cohort studies reporting obstetric and neonatal outcomes in TS pregnancies have been published in recent years. However, these studies are often limited to a single centre cohort, within one country and thus increasing risk of bias. Overall pooled risk estimates for each outcome have yet to be reported.

Study design, size, duration
A prospectively registered systematic review and meta-analysis was conducted and reported in line with PRISMA guidelines. Overall pooled rates of each outcome Is reported with 95% confidence interval. Pooled risk ratios comparing spontaneous and egg donation pregnancies are also reported. A comparative meta-analysis reports on dichotomous outcomes using summary risk ratio (RR) with 95%CI and on continuous outcomes using weighted mean difference (WMD) with 95%CI. Publication bias was assessed using the Newcastle-Ottawa Scale.

Participants/materials, setting, methods
A comprehensive literature search was undertaken. Eligibility screening was conducted by 2 independent reviewers. Studies were limited to English language with no time limit. Case reports were excluded. 30 studies reporting obstetric or perinatal outcomes in pregnancies of women with TS were included in the analysis. Outcome data was extracted and tabulated using a standard proforma.

Main results and the role of chance
1724 pregnancies were included. Pooled risks: Obstetric outcomes: caesarean section (62.8%)(95%CI:57-76%), pregnancy-associated hypertensive disorders (21%)(95%CI:13-29%), pre-eclampsia (10%)(95%CI:6-14%),gestational diabetes mellitus (6.0%)(95%CI:4-8%), aortic dissection (1.1%)(95%CI:0-2%), intrahepatic cholestasis of pregnancy (4.6%)(95%CI:0-7%), post-partum haemorrhage (17.9%)(95%CI:-3-37%), and maternal mortality (0.3%)(95%CI:0-1%). Perinatal outcomes: prematurity (14.1%)(95%CI:8-18%), low birthweight (25.1%)(95%CI:12-23%), and perinatal mortality (1.2%)(95%CI:0-1%).
Egg-donation pregnancies yield significantly higher rates of: caesarean section (82.8% vs. 55.7%) (RR1.49) (95% CI:1.27-1.76), pregnancy-associated hypertensive disorder (33.0% vs 12.9%: p=0.001), pre-eclampsia (16.9% vs 8.2%: p=0.0023), gestational hypertension (17.9% vs 0.0%: p=0.082), low-birthweight (43.0% vs 1.9%: p&lt;0.001) and preterm birth (17.5% vs 0.0%: p&lt;0.001) than spontaneous pregnancies. Multiple pregnancies resulted in high rates of prematurity (70.0%), low birth-weight (73.3%) and pregnancy-associated hypertensive disorders (60.9%).

Limitations, reasons for caution
Most studies were retrospective and included cases from many years ago so may not be reflective of current obstetric practice and improved care. Most studies were geographically clustered in Europe and North America. Estimations of risk may therefore not be generalisable to other ethnicities and middle and lower income countries

Wider implications of the findings
This study quantifies the risk of adverse outcomes in pregnancies in women with TS. Risk is increased further in egg donation pregnancies and strategies should aim to screen and prevent complications, particularly hypertensive disorders of pregnancy. Multiple pregnancy should be avoided through use of single embryo transfer.

Trial registration number
N/a