1 INTRODUCTION

Prenatal counseling in cases of severe fetal ventriculomegaly (VM) is often challenging. While it is well established that is confers a significant risk of morbidity and mortality to the neonate, despite a generally poor prognosis, it is often difficult to accurately quantify antenatally the infant outcome as there can be poor correlation between brain imaging and subsequent neurodevelopment antenatally.1 Severe VM is typically classified as Vp > 15 mm2 and is diagnosed via antenatal ultrasound by measuring the atria of the lateral ventricles (Vp).3 Additional structural and chromosomal/genetic abnormalities are commonly reported in cases of SVM.4, 5 Studies have reported high rates of additional abnormalities of 58% - 65%,6-8 with SVM. The incidence of aneuploidies is high >15%9 in the presence of other structural abnormalities, while there is a low incidence of aneuploidy reported in fetuses with isolated SVM.4, 10-12 SVM may also be associated an underlying genetic condition or other intracranial abnormalities including Dandy-walker spectrum, congenital stenosis of the aqueduct of Sylvius (presumed to be an X-linked disorder), agenesis of the corpus callosum, spina bifida, obstruction of cerebrospinal fluid or as the result of a congenital infection.2, 5, 13, 14

While the findings of associated genetic and structural abnormalities negatively impact prognosis, the long-term disability and mortality rate in isolated SVM is also disproportionally high. A 2018 systematic review and meta-analysis reported a 12% mortality rate in isolated SVM15 with mild/moderate disability in 18.6% and severe disability in 39.6%.15

What is perhaps less frequently appreciated and discussed is the increased caesarean delivery (CD) associated with an antenatal finding of SVM. For those who continue the pregnancy, severe VM can progress and in turn cause significant macrocrania, which can result in increased maternal morbidity, as CD is more likely to be indicated secondary to an enlarging head circumference (HC).13, 16 Fetal surgical options including cephalocentesis17, 18 and fetal venticulo-amniotic shunts19, 20 have been reported to decompress the fetal head in order to facilitate delivery. However, their role in the management of this condition is controversial and at present antenatal procedures for ventriculomegaly are not accepted practice due to lack of evidence to support them.17, 20

With regards to optimal timing and mode of delivery there is limited data pertaining to mild and moderate VM and even less guidance to direct management in cases of severe VM. There is a paucity of data on the CD rates in this cohort. To that end, the aim of this study was twofold: to examine the outcomes of fetuses diagnosed with SVM antenatally, and secondly to determine the CD rates in continuing pregnancies. There are minimal data comparing the outcomes of fetuses diagnosed with varying degrees of severe VM. Therefore, the study cohort was subdivided this group into two clinical groups; 1. those with a Vp < 20 mm (Vp < 20) and 2. those with a Vp > 20 mm (Vp > 20) to determine if a difference in perinatal outcome exists between them.

2 METHODS

This was a prospective observational study with Institutional Ethical Approval of patients referred with suspected fetal VM to the Department of Fetal Medicine, National Maternity Hospital, Dublin, Ireland, from 1st January 2011 to 31st July 2020. The department is a tertiary referral center for the Republic of Ireland with onsite fetal MRI facilities and a dedicated national fetal neurosurgical clinic where patients are seen jointly by fetal medicine and pediatric neurosurgery.1 All patients with suspected ventriculomegaly are seen by a fetal medicine consultant within one week of being referred. The patients are offered prenatal genetic diagnosis, fetal MRI and are then counseled regarding the likely prognosis and pregnancy options are discussed including the options to continue the pregnancy and to terminate the pregnancy where appropriate. All patients in this cohort were offered amniocentesis. From 2014 non-invasive pre-natal screening was available in our unit as an option for those who elected not to have invasive testing. A fetal brain MRI is offered after a detailed anatomical and neurosonographic examination by a maternofetal medicine specialist, following discussion with the performing Pediatric Radiologist. Ventriculomegaly is the most common indication for fetal MRI in our institution. Prior to August 2016 fetal MRI facilities were not available in the hospital and all patient referred with ventriculomegaly prior to this may not have been offered the option of fetal MRI. For those after 2016 all patients with severe ventriculomegaly, where aneuploidy was not suspected, were offered a fetal MRI for further investigation. They were then reviewed again in the fetal neurosurgical clinic where a further detailed ultrasound was performed, and the patients were counseled by both a fetal medicine consultant and a pediatric neurosurgeon.

Data were entered into the hospital database prospectively by a specialist fetal medicine midwife, and those with severe VM (>15 mm) were identified. Patients were excluded where there was a multiple pregnancy, where the Vp measurement did not meet the above diagnostic criteria for severe ventriculomegaly and those with a subsequent diagnosis of a neural tube defect/spina bifida were also excluded from the analysis. The data were assessed to determine the average gestational age at diagnosis and whether VM was unilateral or bilateral. The rates of chromosomal abnormalities, the survival rates and the CD rates for the overall group was then determined. The data were then further sub-divided into two distinct groups as described above, 1. Vp < 20 and 2. Vp > 20, and the results compared between the two groups. Statistical analysis performed using the Chi-Square test or students t-Test as appropriate.

3 RESULTS

A total of N = 95 singleton pregnancies with confirmed SVM were included for analysis. The maternal demographics are presented in Table 1. The median gestational age at diagnosis was 24 weeks and 1 day (range 16 weeks and 6 days to 38 weeks and 5 days). VM was bilateral in 86/95 (90.5%) of cases and unilateral in 9/95 (9.5%) of cases. Additional structural abnormalities on ultrasound were apparent in 67/95 (70.5%) cases, of which 47.4% had additional intracranial findings only and 28/95 (29.5%) had apparently isolated SVM on ultrasound. Amniocentesis was performed in 46/95 (48.4%) and 10/95 (10.5%) had non-invasive prenatal screening and a further 12/95 (12.6%) had postnatal genetic investigations after neonatal review and genetics input where appropriate. There were 37/95 (38.9%) where no karyotype or chromosomal microarray were performed. In the overall group chromosomal abnormalities were subsequently diagnosed in 15/95 (15.8%) of cases (Table 2), with a rate of 15/58 (25.9%) in those that availed of diagnostic testing. Genetic abnormalities were confirmed in 2/28 (7.1%) of the isolated SVM group versus 13/67 (19.4%) in the non-isolated SVM group. These included trisomy 21 n = 4, trisomy 18 n = 2, triploidy n = 1, X-Linked hydrocephalus (L1 CAM) n = 1, mutation in PP2RIA gene n = 1, chromosome 7q36 mutation n = 1, mutation in ISPD gene n = 1 and other single gene disorders n = 4. There was one case of congenital CMV in this cohort. A total of 59/95 (62.1%) had fetal MRIs performed, with additional intracranial information was provided in 46/59 (78%) of these. Of the n = 36 that did not have a fetal MRI performed the reasons included: maternal spinal rods (n = 1), termination of pregnancy (n = 13), IUD (n = 1), confirmed genetic cause (n = 3), additional significant structural abnormalities (n = 5), no documented reason (n = 11). In the group with no documented reason why an MRI was not performed, 7 were diagnosed with SVM after 33 weeks' gestation (range 33 to 38 weeks and 5 days). Of these 7, one was delivered the next day. Of the remaining six cases, the patients attended the hospital from 2012 to 2016, when MRI was not available on site, and this may have influenced the decision not to perform antenatal MRI in the third trimester in these cases. There was one confirmed case of Fetal Neonatal Alloimmune Thrombocytopenia (FNAIT) in this cohort with underlying intra-ventricular hemorrhage present on antenatal MRI. Table 3 outlines the most likely final diagnosis for all patients after completion of investigations.

TABLE 1. Maternal demographic Average maternal age (range) 31.7 years Range (19–45 years) BMI (SD) 26.26 (5.6) kg/m2 Nationality Irish 77/95 (81.05%) EU 10/95 (10.53%) Non-EU 8/95 (8.42%) Median gestation at diagnosis (range) 24 + 1 week (16 + 6 weeks to 38 + 5 weeks) Range Median gestation at delivery (range) 38 + 4 weeks (30 + 3 weeks to 42 + 4 weeks) Range Median maternal parity (range) 1 Range (0–7) Bilateral ventriculomegaly 86 Unilateral ventriculomegaly 9 Abbreviations: BMI, Body Mass Index; EU, European Union; SD, Standard Deviation. TABLE 2. Antenatal Findings, Survival and Mode of Delivery Compared between Fetuses with Vp measurements <20 mm and >20 mm Vp < 20 mm (N = 41) Vp > 20 mm (N = 54) p value Overall group (N = 95) Chromosomal abnormalities 7/41 (17.1%) 8/54 (14.8%) p = 0.7 (NS) 15/95 (15.8%) US findingsa Isolated 16/41 (39%) 12/54 (22.2%) p = 0.07 (NS) 28/95 (29.5%) Additional intracranial 13/41 (31.7%) 32/54 (59.3%) p < 0.05 45/95 (47.4%) Extra cranial findings 12/41 (29.3%) 10/54 (18.5%) p = 0.2 (NS) 22/95 (23.2%) Additional intracranial MRI findings 9/28 (32.14%) 37/67 (55.2%) p < 0.05 46/95 (48.4%) HC > 95th 14/41 (34.1%) 35/54 (64.8%) p < 0.05 49/95 (51.6%) TOP 10/41 (24.4%) 12/54 (22.2%) p = 0.6 (NS) 21/95 (24.2%) IUD = 2 1/41 1/54 2/95 (2.1%) NND 4/41 (9.8%) 15/54 (27.8%) p < 0.05 19/95 (20%) Survival (excluding TOP) 26/41 (63.4%) 27/54 (50%) p = 0.19 (NS) 53/74 (71.6%) Delivery information CD 17/30 (56.7%) 30/42 (71.4%) p = 0.19 (NS) 47/72 (65.3%) VD 13/30 (43.3%) 12/42 (28.6%) p = 0.19 (NS) 25/72 (34.7%) Abbreviations: CD, Caesarean delivery; HC, Head circumference; IUD, In utero demise; NND, Neonatal death; NS, Not significant; TOP, Termination of pregnancy; US, Ultrasound; VD, Vaginal delivery; Vp, Measurement of the posterior horn of the lateral ventricle. a US findings relate to the initial ultrasound performed by a fetal medicine specialist reported findings from this detailed assessment. TABLE 3. The Final Diagnosis of this Cohort after Antenatal and Postnatal Assessment Final diagnosis N (%) Isolated VM 16/95 (16.8%) Chromosomal/Genetic abnormalities 15/95 (15.8%) CMV 1/95 (1.05%) Additional findings 63/95 (66.3%) Agenesis of the corpus callosum 12 Hemorrhage 11 VM + dilated third ventricle 10 Aqueduct stenosis 7 Brainstem abnormality 4 Schizencephaly 2 Walker Warburg 1 Arachnoid cyst 1 Cortical migrational anomaly + ACC 1 Encephalocele 1 Lissencephaly 1 Dandy walker malformation 1 Chiari 1 malformation 1 Enlarged CM 1 VM + Sonolucent area in right temporal lobe 1 Multiple anomalies 3 Schizencephaly + HLHS 1 Thantophoric dysplasia 1 VSD 1 Severe hydronephrosis 1 Tetralogy of fallot 1 Note: This Table givens a breakdown of the most likely final diagnosis of each case after US imaging, MRI (as appropriate) and genetic investigations were completed. Abbreviations: ACC, Agenesis of the Corpus Callosum; CM, Cisterna Magna; CMV, Cytomegalovirus; HLHS, Hypoplastic Left Heart Syndrome; VM, Ventriculomegaly; VSD, Ventricular Septal Defect.

The overall survival rate was 53/95 (55.8%). In utero demise was reported in 2 cases and in 21 cases couples opted for termination of pregnancy. However, after exclusion of those who opted for termination of pregnancy (TOP) n = 21/95 (22.1%) the overall survival rate was 53/74 (71.6%), with a rate of 20/23 (86.9%) for those with isolated SVM (Table 2). For those that opted for TOP the median gestation of diagnosis of the VM was 21 weeks and 2 days (range 16 weeks and 6 days to 37 weeks and 2 days). The majority, 14/21 (66.7%), subsequently had TOP after 24 weeks of gestation. Table 4 compares the outcomes by gestational age at diagnosis of VM. Fetuses where severe VM was diagnosed before 24 weeks, had an overall higher rate of TOP and a lower survival rate than those diagnosed from 24 to 32 weeks and those diagnosed after 32 weeks' gestation. The overall CD rate for this group was 47/72 (65.3%). This was significantly higher than the CD for the hospital during the same time period which was 25.4% (P < 0.01). A total of N = 16 pregnancies delivered prior to 37 weeks gestation (16/72, 22.2%). Of these 3/16 (18.75%) were spontaneous vaginal deliveries and 13/16 (81.25%) were iatrogenic and delivered by CD.

TABLE 4. Comparison of outcomes by the gestation at diagnosis Gestation at diagnosis (weeks + days) Number Gestation at delivery (median) TOP Survival <24 N = 47 37 weeks +2 days 18/47 (38.3%) 14/47 (29.8%) 24 + 0 to 32 + 0 N = 22 37 weeks +1 day 2/22 (9.1%) 16/22 (72.7%) >32 N = 26 38 weeks +6 days 1/26 (3.8%) 23/26 (88.5%) p value NS P < 0.05 P < 0.05 Note: This table provides the outcomes in terms of gestation at delivery, rate of TOP and the survival rates when subdivided by gestation at diagnosis of the ventriculomegaly. Abbreviations: NS, Not significant; TOP, Termination of pregnancy. P < 0.05 was considered statistically significant.

In our series only one patient required cephalocentesis at the time of CD to facilitate delivery of the fetal head through a lower transverse abdominal incision. This patient had CD at 36 + 1 week' gestation with an increasing HC, measuring 406 mm on ultrasound the week prior. A lower uterine segment incision was performed and as it was not possible to deliver the fetal head through the lower transverse incision 180 ml was aspirated under direct vision with a 16 gauge needle and the fetal head was then successfully delivered though the standard transverse uterine incision. The HC at birth was 476 mm. The baby was born alive and died at 3 h of life. In this case there was a diagnosis of severe hydrocephalus secondary to an intracranial tumor and an intracranial teratoma was confirmed on postmortem examination. The were no cases of cephalocentesis performed during a vaginal birth in this series. In one other case a patient had a CD at 34 weeks' gestation after MDT discussion with fetal medicine, pediatric neurosurgeons and the neonatal team. The HC in this case was 400 mm on US and a decision was made to perform a CD at this gestation in an attempt to avoid cephalocentesis as the parents wished for the baby to be born alive and also to avoid an extended uterine incision at a later gestation. In this case the patient had had a prior CD and the fetus was expected to have a poor prognosis. CD was performed through a standard lower segment incision; the fetal head was delivered easily, and the infant demised shortly after birth. The HC at birth was measured at 418 mm and this was the largest HC in our cohort successfully delivered by lower uterine segment incision that did not require additional peri-CD drainage.

The data were then subdivided into those with Vp < 20 and those with Vp > 20 and the results compared between the two groups. These results are presented in Table 2. No significant differences were observed in the rate of chromosomal abnormalities between the groups. Those with a Vp > 20 had a higher rates of additional intracranial findings on ultrasound, Vp > 20 32/54 (59.3%) versus Vp < 20 13/41 (31.7%), P < 0.05. They also had higher rates of macrocrania, Vp > 20 35/54 (64.8%) than those in the Vp < 20 group 14/41 (34.1%), P < 0.05. Those with Vp > 20 mm also had a higher rate of neonatal death than those with Vp < 20 mm, P < 0.05 (Table 2). There was no significant difference observed in the CD rates between the two groups.

The data were further analyzed to assess the survival rates and CD rates in those with macrocrania (defined as HC > 95th centile) and those without (defined as HC < 95th centile) and these findings are presented in Table 5. Fetuses with a HC > 95th centile had a lower survival rate than those with a normal HC (P < 0.05). They also had significantly higher rates of CD at 86.1%, while those with a HC<95% had a CD rate of 44.4% (P < 0.05).

TABLE 5. Survival and Mode of Delivery Compared between Fetuses with macrocrania and those with a normal head circumference on antenatal ultrasound HC < 95th centile n = 46 HC > 95th centile N = 49 p value Vp > 20 mm 16/46 (34.8%) 35/49 (71.4%) p < 0.05 Average Vp 20.5 mm 28.5 mm p < 0.05 Range (15–37.9 mm) (15–48.4 mm) Survival (Ex-TOP) 33/38 (86.8%) 20/36 (55.6%) p < 0.05 TOP 8/46 (17.4%) 13/49 (26.5%) IUD 2/46 (4.3%) 0/49 (0%) NND 3/46 (8.3%) 16/49 (32.6%) p < 0.05 Median parity 1 1 p = NS Range (0–5) (0–7) Primiparous 17/46 (34%) 24/49 (49%) Multiparous 29/46 (63%) 25/49 (51%) Median gestation at delivery 37 weeks + 5 days 37 weeks + 4 days p = NS Range (weeks + days) (30 + 3 to 41 + 5) (34 + 4 to 42 + 4) CD 16/36 (44.4%) 31/36 (86.1%) p < 0.05 VD 20/36 (55.6%) 5/36 (13.9%) p < 0.05 Abbreviations: CD, Caesarean delivery; HC, Head circumference; IUD, In utero demise; NND, Neonatal death; NS, Not significant; TOP, Termination of pregnancy; US, Ultrasound; VD, Vaginal delivery. 4 DISCUSSION

The finding of antenatal SVM poses a number of challenges, both in terms of obstetric management and counseling the parents regarding an often guarded and uncertain prognosis. As alluded to above, SVM has long been associated with adverse neonatal outcomes with a 21% mortality rate.3 We found an overall survival rate in this cohort of 71.6% (after exclusion of TOP), although it was significantly higher 20/23 (87%) in the isolated severe VM group versus those with additional abnormalities 33/51 (64.7%) (P < 0.05). It is frequently described in the literature that cases of SVM associated with extracranial abnormalities results in a poor outcome, with livebirth rates as low as 30%–40%.3, 11, 15 No difference was observed in the overall survival rates between those with Vp < 20 versus Vp > 20, though those in the Vp > 20 group had a higher neonatal death (NND) rate of 27.8% (Table 2).

There were chromosomal abnormality rates in the SVM population of 15.8%, with 7.1% observed in the isolated SVM group versus 19.4% in the non-isolated SVM group, consistent with previously reported studies.3, 10, 11, 15 However, it must be acknowledged that the rate was higher, 25.9%, in those that availed of diagnostic testing. The reasons for this variation are multifactorial. In some cases, women choose not to have diagnostic testing, some opted for NIPS, some opted for TOP prior to embarking on any additional genetic testing, and in some cases additional genetic testing was not recommended postnatally after detailed neonatal assessment.

Our study confirms previous reports of high rates of additional structural abnormalities on ultrasound of 70.6% associated with SVM, of which 47.4% had additional intracranial findings only. Other studies reported additional structural findings in SVM patients of 58%–65%.6, 7, 15 Those in the Vp > 20 group had a higher rate of intracranial findings than those in the Vp < 20 group at 59.3% versus 31.7% respectively, P < 0.05. MRI has enhanced antenatal diagnosis of fetal abnormalities in recent years.2 US lacks the ability to detect subtle cortical brain anomalies and MRI provides additional information in these scenarios.16 In this study we found fetal MRI provided additional diagnostic information in 78% of cases. MRI results were useful to guide counseling in several respects. In 8 cases, parents opted for termination of pregnancy after the MRI reported additional findings, which included agenesis of the corpus callosum. In 4 other cases the parents chose to continue the pregnancy but with a plan for palliative care after the birth. These included in cases of schizencephaly, severe hemorrhage, severe Aqueduct stenosis and one case where there were additional cerebellar findings on MRI. Furthermore, additional MRI findings were useful to counsel patients regarding the potential surgical treatment options that may be required after birth, to allow families to prepare for this. Moreover, when the MRI found no additional intracranial findings this was also helpful for parents to make decision regarding continuing or terminating the pregnancy.

Aside from the significant fetal morbidity and mortality observed in this cohort, this study also highlights the increased CD associated with an antenatal diagnosis of SVM in continuing pregnancies. We found women with an antenatal diagnosis of SVM had a disproportionally high CD rate of 65.3%, which was significantly higher than the background rate in the unit during this time period of 25.4% (P < 0.01). When comparing those with a Vp < 20 and Vp > 20, no significant difference was observed between the groups in perinatal outcomes. This high rate of CD is important to consider in counseling regarding the maternal risks associated with a diagnosis of SVM and continuation of the pregnancy.21

Fetuses with severe macrocrania require individualized management to facilitate safe delivery in continuing pregnancies and to minimize maternal morbidity where possible. The options for delivery for a fetus with significant macrocrania may include consideration for preterm delivery to allow for safe delivery of the fetal head, use of cephalocentesis to decompress the fetal head prior to or during delivery or consideration of a classical CD. In our cohort 86.1% of fetuses with a HC > 95th centile had a CD, which was significantly higher than the background rate of 25.4%. There is no evidence to suggest that preterm or CD improves maternal or neonatal outcome in mild or moderate VM2 although little is known about the effects of preterm delivery on SVM fetuses, particularly where a poor prognosis is anticipated. In select cases preterm delivery may be considered with worsening macrocrania in an attempt to avoid a potentially more difficult or complex CD at term.22, 23 We acknowledge that preterm delivery confers significant risks of prematurity13, 24 and it should only be considered in cases where there is an anticipated very poor neonatal outcome, in an effort to decrease maternal morbidity. As alluded to above, a CD was performed in one case at 34 weeks' gestation due to progressively worsening macrocrania. Preterm delivery in this context decreased the need for cephalocentesis or an extended uterine incision/classical incision being performed at a later gestation and allowed the parents time with their baby.

While there have been significant advances in the diagnoses of fetal malformations in recent years, the same improvements have not been seen in the surgical treatments for fetal ventriculomegaly.19 Fetal surgical options, including cephalocentesis17, 18 and venticulo-amniotic shunts,19, 20 have been reported to decompress the fetal head in order to facilitate delivery. Due to the high risk of fetal demise with cephalocentesis, it is now rarely performed and is typically only used to cause decompression of the fetal head to aid delivery and to prevent surrounding structures being compressed in the fetal cranium.25 If done successfully, cephalocentesis can facilitate vaginal delivery or a lower segment uterine incision at the time of CD and as such result in decreased maternal morbidity related to delivery. In our cohort only one case with severe VM and macrocrania required cephalocentesis peri-CD to facilitate delivery of the fetal head through a lower transverse uterine incision, and thus reducing maternal morbidity in this context. Despite unfavorable outcomes during the early experience with in utero CSF diversion, improvements in prenatal diagnosis and fetal surgery have led to a renewed interest in fetal ventriculo-amniotic fluid (VA) shunting.23 Several reasons have been cited to explain the historic suboptimal outcomes and include poor patient selection and lower quality imaging modalities.23, 26 Advance in neurosonography and fetal MRI have improved detection of pathologies which are more likely to be amenable to in utero shunting, such as Aqueduct Stenosis.23, 27, 28 Ventriculo-amniotic shunting may improve neurologic outcomes in selected patients and decrease maternal morbidity at the time of delivery.28 There is a need for further evaluation on the topic20 and a research agenda has been constructed, and investigation is underway to determine in use in routine clinical practice.20, 28

Extended or classical CD procedures have also been considered as a means to facilitate delivery of the large fetal head, where it is not possible to deliver the fetus through a low transverse incision and are associated with increased maternal morbidity.29 Classical CD procedures are associated with risks of complications at both at the time of surgery, including a risk of infection and blood loss,28-30 and are also a cause of morbidity in subsequent pregnancies, particularly in relation to an increased risk of uterine rupture.28, 29 In our series we did not perform any classical CD procedures to facilitate delivery in the context of fetal macrocrania, nor were there any J, T incisions or any significant uterine angle tears reported in this cohort. Other studies however have used classical CD as a means to facilitate delivery. A 2009 retrospective study reported the delivery of 13 out of 23 fetuses with fetal macrocrania by classical CD from 1st March 2003 to the 30th June 2007.13 From this series 22% of infants died in the neonatal period, 70% went to the neonatal intensive care unit and 8% were transferred for further surgical management and subsequently died.13 Given the increased maternal morbidity, without a significant improvement in fetal outcome, the authors are therefore of the option that classical CD should only be considered where other means of delivery are either unsuccessful or unacceptable.

Finally, it must be remembered there is an increased level of psychological stress experienced by parents with the ultrasonographic detection of a fetal anomaly in pregnancy.31 The severity of the fetal malformation and ambiguity concerning diagnosis and prognosis, as is often the case with SVM, also increases parental psychological distress, social dysfunction and health perception.32 As it has also been reported that there appears to be no correlation between the severity of the neurologic outcomes and the prenatal ventricular dilation on antenatal ultrasound imaging in the SVM group,33 this further adds to the complexities of antenatal counseling and support should be offered to couples when faced with this complex diagnosis.

The strengths of this study include that as this is a large tertiary referral centre with a national fetal neurosurgical