Perinatal and long-term outcomes in fetuses diagnosed with isolated unilateral ventriculomegaly: systemic review and meta-analysis C. Scala, A. Familiari, A. Pinas, A.T. Papageorghiou, A. Bhide, B. Thilaganathan, A. Khalil Volume 49, Issue 4, Date: April (pages 450–459) Slides prepared by Dr Yael Raz (UOG Editor-for-Trainees) Link to free-access article: http://onlinelibrary.wiley.com/doi/10.1002/uog.15943/full

1. Perinatal and long-term outcomes in fetuses
diagnosed with isolated unilateral ventriculomegaly:
systemic review and meta-analysis
C. Scala, A. Familiari, A. Pinas, A.T. Papageorghiou,
A. Bhide, B. Thilaganathan, A. Khalil
Volume 49, Issue 4, Date: April (pages 450–459)
UOG Journal Club: April 2017
Journal club slides prepared by Dr Yael Raz
(UOG Editor for Trainees)

2. • Definition: atrial width >10 mm, measured at the level of the glomus of
the choroid plexus, perpendicular to the ventricular cavity and positioning
the calipers inside the echoes generated by the lateral walls.
• Can be symmetrical, affecting both lateral ventricles (bilateral), or
unilateral.
• Prevalence: 0.3 and 1.5 per 1000 births, depending on the gestational
age at examination, the technique of measurement and whether one or
both ventricles is/are evaluated.
Outcomes in fetuses with isolated unilateral ventriculomegaly
Scala et al, 2017
Introduction - Fetal ventriculomegaly

3. • Etiology: normal variation, aneuploidy, genetic syndromes, primary brain
abnormalities, congenital infections, cerebrovascular accidents and
intracranial hemorrhage.
• Fetal ventriculomegaly is a common sonographic feature and has been
associated with a poor outcome.
• Even when fetal ventriculomegaly is isolated, there may be an associated
risk of abnormal neurodevelopmental outcome.
• Most relevant studies have assessed the outcomes of bilateral fetal
ventriculomegaly.
Introduction - Fetal ventriculomegaly
Outcomes in fetuses with isolated unilateral ventriculomegaly
Scala et al, 2017

4. To quantify, via systematic review and meta-analysis, the
perinatal and long-term outcomes in fetuses diagnosed with
isolated unilateral ventriculomegaly during the second- or third-
trimester ultrasound examination
Aim of the study
Outcomes in fetuses with isolated unilateral ventriculomegaly
Scala et al, 2017

5. Methods – study selection, data collection
• Studies reporting data on the perinatal outcome of fetuses with isolated
unilateral ventriculomegaly (atrial width >10 mm) detected during the
second or third trimester of pregnancy were included.
• ‘Apparently isolated ventriculomegaly’ – no additional brain or
extracerebral ultrasound abnormalities detected at the time of diagnosis.
• ‘Truly isolated ventriculomegaly’ - no additional brain or extracerebral
abnormalities, chromosomal abnormality or congenital infection were
detected pre- or postnatally.
• Ventriculomegaly was further classified into mild/moderate (atrial width
10–15 mm) and severe (atrial width ≥ 15 mm).
Outcomes in fetuses with isolated unilateral ventriculomegaly
Scala et al, 2017

6. Results –
Included studies
CDSR, The Cochrane Database of Systematic Reviews;
CENTRAL, The Cochrane Central Register of Controlled Trials;
DARE, Database of Abstracts of Reviews of Effects.
Outcomes in fetuses with isolated unilateral ventriculomegaly
Scala et al, 2017

7. Results – Apparently isolated unilateral
ventriculomegaly (108 cases)
• No case of abnormal karyotype
• Prevalence of congenital infection
(n=7: 5 CMV, 2 parvovirus): 8.2%
• Prevalence in fetuses with apparently isolated unilateral ventriculomegaly with
atrial width <15mm (101/108, 93.5%) were similar.
•Prevalence of associated extracerebral
abnormalities detected on US: 6.8%
*size of the boxes is proportional to study sample size
Outcomes in fetuses with isolated unilateral ventriculomegaly
Scala et al, 2017

8. Results – Apparently isolated unilateral
ventriculomegaly (108 cases)
•Prevalence of associated brain abnormalities
detected by prenatal MRI: 5.1%
•Prevalence in fetuses with apparently isolated unilateral ventriculomegaly with atrial
width <15mm (101/108, 93.5%) were similar.
*size of the boxes is proportional to study sample size
•Prevalence of progression of
ventriculomegaly during pregnancy: 5.4%
Outcomes in fetuses with isolated unilateral ventriculomegaly
Scala et al, 2017

9. • Median age at neurological assessment: 30.3 (range, 24.2–36.5) months.
Results – Neurodevelopmental delay –
apparently isolated unilateral ventriculomegaly (108 cases)
• Incidence of associated
neurodevelopmental delay: 8.0%
(atrial width <15mm: 5.9%)
*size of the boxes is proportional to study sample size
• Incidence of associated mild
neurodevelopmental delay (a): 2.1% and
severe neurodevelopmental delay (b): 4.1%
Outcomes in fetuses with isolated unilateral ventriculomegaly
Scala et al, 2017

10. • 198/213 (93.0%) cases had postnatal neurodevelopmental assessment.
• Median age at neurological assessment: 38.0 (range, 27.0–49.8) months.
• Incidence of associated neurodevelopmental delay: 7% (in all cases, atrial width was <15mm)
Results – Neurodevelopmental delay –
truly isolated unilateral ventriculomegaly (213 cases)
*size of the boxes is proportional to study sample size
Outcomes in fetuses with isolated unilateral ventriculomegaly
Scala et al, 2017

11. • In cases with apparently isolated unilateral ventriculomegaly detected on
prenatal ultrasound, additional brain abnormalities can be detected on
fetal MRI (pre- or postnatally)
• MRI should remain an important diagnostic tool in such cases.
• The same applies to screening for congenital infections and follow-up
scans to look for progression of the ventriculomegaly.
Discussion - apparently isolated unilateral ventriculomegaly
Outcomes in fetuses with isolated unilateral ventriculomegaly
Scala et al, 2017

12. • Fetuses with truly isolated unilateral ventriculomegaly suffered from a low
incidence of neurodevelopmental impairment postnatally.
• The incidence of neurodevelopmental impairment is similar to that
reported in the general population.
Discussion - truly isolated unilateral ventriculomegaly
Outcomes in fetuses with isolated unilateral ventriculomegaly
Scala et al, 2017

13. • In cases of mild apparently isolated unilateral ventriculomegaly the
prevalence of congenital infection is similar to that in fetuses with bilateral
ventriculomegaly.
• The observed prevalence of chromosomal abnormality (0%), risk of
ventriculomegaly progression (5%) and associated brain abnormalities
detected on fetal brain MRI (5%) seem to be lower than those for fetuses
with bilateral ventriculomegaly.
Discussion - Unilateral vs bilateral ventriculomegaly
Outcomes in fetuses with isolated unilateral ventriculomegaly
Scala et al, 2017

14. Discussion - strengths and weaknesses
Strengths
•Comprehensive search strategy
•Inclusion of a number of key outcomes
identified in only isolated unilateral
ventriculomegaly cases.
•Previous data is limited, findings of
this meta-analysis could be valuable in
parental counseling.
Weaknesses
•Publication bias (small number of
cases in current literature)
•Study heterogeneity:
– Gestational age at time of diagnosis
– Diagnostic interpretation of US and MRI
images is difficult to standardize
– Method and period of follow-up used in
assessing neurodevelopmental outcome.
•Age at neurodevelopmental
assessment may be too early for
some neurodevelopmental delays to
be apparent (but less confounding
factors)
Outcomes in fetuses with isolated unilateral ventriculomegaly
Scala et al, 2017

15. • The prevalence of aneuploidy, congenital infection and progression of
ventriculomegaly in fetuses with a prenatal diagnosis of apparently
isolated unilateral ventriculomegaly is low.
• The incidence of neurodevelopmental delay in cases of both apparently
and truly isolated unilateral ventriculomegaly with an atrial width
<15mm is likely to be similar to that reported in cases of isolated bilateral
ventriculomegaly and to that reported in the general population.
Conclusions
Outcomes in fetuses with isolated unilateral ventriculomegaly
Scala et al, 2017

16. • Should karyotype assessment be offered to women with apparently
isolated unilateral ventriculomegaly?
• Does gestational age at diagnosis correlate with the chance for
neurodevelopmental delay?
Points for discussion
Future perspectives
• Large multicenter prospective studies are needed to ascertain the risk of
neurodevelopmental delay and the possible risk factors that might
influence postnatal prognosis.
Outcomes in fetuses with isolated unilateral ventriculomegaly
Scala et al, 2017