Fetal surgery for open neural tube defect with severe ventriculomegaly.

OBJECTIVE: Prenatal open neural tube defect (ONTD) repair is performed to decrease the risk of needing treatment for hydrocephalus after birth and to preserve motor function. Some centers may not consider patients to be candidates for surgery if severe ventriculomegaly is present and there is no expected benefit in risk for hydrocephalus treatment. This study sought to compare the postnatal outcome of fetuses with ONTD and severe ventriculomegaly (ventricular width ≥ 15 mm) that underwent prenatal repair with the outcome of fetuses with severe ventriculomegaly that underwent postnatal repair and fetuses without severe ventriculomegaly (< 15 mm) that underwent prenatal repair. METHODS: This was a retrospective study of fetuses with ONTD that underwent prenatal or postnatal repair between 2012 and 2021 at a single institution. The cohort was divided into two groups based on preoperative fetal ventricular size: those with severe ventriculomegaly (ventricular width ≥ 15 mm) and those without severe ventriculomegaly (< 15 mm). Fetal ventricular size was measured by magnetic resonance imaging before surgery using the standardized approach and the mean size of the left and right ventricles was used for analysis. Motor function of the lower extremities was assessed at the time of referral by ultrasound and if flexion-extension movements of the ankle were seen it was considered as preserved S1 motor function. Postnatal outcomes, including motor function of the lower extremities at birth and the need for a diversion procedure for hydrocephalus treatment during the first year after birth, were collected and compared between groups. Multivariate regression analysis was used to adjust for potential confounders. RESULTS: In this study, 154 patients were included: 145 underwent fetal surgery (101 fetoscopic and 44 open hysterotomy) and nine with severe ventriculomegaly underwent postnatal repair. Among the 145 patients who underwent fetal surgery, 22 presented with severe ventriculomegaly. Fetuses with severe ventriculomegaly at referral that underwent prenatal repair were significantly more likely to need hydrocephalus treatment by 12 months after birth than those without severe ventriculomegaly (61.9% vs 28.9%, P < 0.01). However, motor function assessment at birth was similar between both prenatal repair groups (odds ratio, 0.92 (95% CI, 0.33-2.59), P = 0.88), adjusted for the anatomical level of the lesion. The prenatal repair group with severe ventriculomegaly had better preserved motor function at birth compared to the postnatal repair group with severe ventriculomegaly (median level, S1 vs L3, P < 0.01; proportion with S1 motor function, 68.2% vs 11.1%, P < 0.01). Fetuses with severe ventriculomegaly that underwent prenatal repair had an 18.9 (95% CI, 1.2-290.1)-times higher chance of having intact motor function at birth, adjusted for ethnicity, presence of club foot at referral and gestational age at delivery, compared with the postnatal repair group. There was no significant difference in the need for hydrocephalus treatment in the first year after birth between prenatal and postnatal repair groups with severe ventriculomegaly (61.9% vs 87.5%, P = 0.18). CONCLUSIONS: Although fetuses with ONTD and severe ventriculomegaly do not seem to benefit from fetal surgery in terms of postnatal hydrocephalus treatment, there is an increased chance of preserved motor function at birth. Results from this study highlight the benefit of prenatal ONTD repair for cases with severe ventriculomegaly at referral to preserve motor function. © 2024 International Society of Ultrasound in Obstetrics and Gynecology.

Intracranial volumetric evaluation in postnatally repaired myelomeningocele infants.

INTRODUCTION: Most myelomeningocele (MMC) cases present with ventriculomegaly or hydrocephalus, yet a comprehensive volumetric assessment of MMC intracranial structures is lacking. This study aimed to provide baseline data on intracranial structural volumes immediately after birth in MMC infants who underwent repair surgeries after birth (postnatal repair). METHODS: In this retrospective single-center study, we analyzed 52 MMC infants undergoing postnatal repair, utilizing head computed tomography scans at birth for volumetric assessment. Intracranial volume (ICV), lateral ventricles volume (LVV), choroid plexus volume (CPV), and posterior cranial fossa volume (PCFV) were measured. Hydrocephalus was classified into no hydrocephalus, progressive hydrocephalus, and hydrocephalus at birth. Comparative analysis employed the Wilcoxon rank-sum test. Receiver operating characteristic (ROC) analysis discriminated cases with and without ventriculoperitoneal shunt (VPS). RESULTS: The median values were 407.50 mL for ICV, 33.18 mL for LVV, 0.67 mL for CPV, and 21.35 mL for PCFV. Thirty-seven cases (71.15%) underwent VPS. ROC analysis revealed an LVV cut-off value of 6.74 mL for discriminating cases with and without VPS. Progressive hydrocephalus showed no significant difference in ICV but significantly larger LVV compared to no hydrocephalus. Hydrocephalus at birth demonstrated statistically larger ICV and LVV compared to the other two types. CONCLUSION: Baseline volumetric data were provided, and volumetric analysis exhibited statistical differences among three hydrocephalus types. These findings enhance our understanding of intracranial volumetric changes in MMC, facilitating more objective assessments of MMC cases.

Long-term neuroimaging and neurological outcome of fetal spina bifida aperta after postnatal surgical repair.

OBJECTIVE: Parents faced with the choice between postnatal management and prenatal surgery for spina bifida need to have up-to-date information on the expected outcomes. The aim of this study was to report the long-term physical and neurological outcomes of infants with prenatally diagnosed isolated spina bifida that underwent postnatal surgical repair and were managed by a multidisciplinary team from a large tertiary center. METHODS: This was a retrospective cohort study of all cases of fetal spina bifida managed in a tertiary unit between October 1999 and January 2018. All cases of fetal spina bifida from the local health region were routinely referred to the tertiary unit for further perinatal management. Details on surgical procedures and neonatal neurological outcomes were obtained from institutional case records. Ambulatory status, bladder and bowel continence and neurodevelopment were assessed at a minimum of 3 years. RESULTS: During the study period, 241 pregnancies with isolated spina bifida were seen in the unit. Of these, 84 (34.9%) women opted to continue with the pregnancy after multidisciplinary counseling by clinicians. Sixty-seven infants underwent postnatal repair of spina bifida aperta and were included in the analysis. After birth, hindbrain herniation was observed in 91.5% of infants with only seven requiring surgical decompression. Ventriculoperitoneal shunt placement was needed in 64.2% of infants, while normal cognitive development or mild impairment was demonstrated in 85.4% of cases with data for this outcome available, at a mean age of 8 years. Cumulatively, 40% of infants were walking independently or using minor support, and normal or mild impairment of bladder and bowel function was reported in 45.5% and 44.4% of infants, respectively. CONCLUSIONS: Neurodevelopmental and neurological outcomes between prenatal and postnatal repair are similar. As with fetal surgery, conventional postnatal surgery is associated with the reversal of hindbrain herniation. Similarly, postnatal ventriculoperitoneal shunt placement appears to be required mainly in fetuses without evidence of significant fetal ventriculomegaly. Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd.

The natural history of neural tube defects in the setting of an Irish tertiary referral foetal medicine unit.

Neural tube defects (NTDs) carry a heavy burden for affected individuals and their family. Physical and neurological outcome measures may help in counselling couples. The aim of this audit was to review all cases of NTDs seen at a tertiary referral foetal medicine unit. Cases were identified from obstetric, neonatal and neurosurgical records. Thirty-six cases of NTDs were identified. Of the 36, 25% (n = 9, one trisomy 18) opted for termination of pregnancy abroad. Of the remaining 27, 19% (n = 5) died in the antepartum period. 81% (n = 22) were liveborn with four neonatal deaths (one trisomy 18). Of 15 cases, 14 had neurosurgical repair within a median time of 3 days and 9 of these also had a ventriculoperitoneal shunt inserted. Antenatal ultrasound accurately diagnosed lesion level in the majority of cases. The survival rate for babies diagnosed with non-lethal neural tubes defects is high when multidisciplinary care is initiated early.

Postnatal Repair of Open Neural Tube Defects: A Single Center with 90-Month Interdisciplinary Follow-Up.

After publication of the Management of Myelomeningocele Study (MOMS) there is confusion regarding which treatment of open neural tube defects (NTD) is best. We report our results of postnatally repaired open NTDs born between 2007-2018 (n = 36) in critical reflection of the MOMS study. Neurosurgical, orthopedic, and urologic data were assessed. We also introduce a new entity: "status post prenatal repair". FU ranged from 29 to 161 months (mean: 89.1 m) in 7 cases of myeloschisis and 24 myelomeningoceles in the final collective n = 31. The shunt rate was 41.9%, and the endoscopic third ventriculostomy rate was 16.1%. Hydrocephalus requiring treatment was not associated with the anatomical level, but with premature birth (p = 0.048). Myeloschisis was associated with shunt placement (p = 0.008). ROC analysis revealed birth <38.5th week predicts the necessity for hydrocephalus treatment (sensitivity: 89%; specificity: 77%; AUC= 0.71; p = 0.055). Eight (25.8%), patients are wheelchair-bound, 2 (6.5%) ambulate with a posterior walker, 10 (32.3%) with orthosis and 11 (35.5%) independently. One (3.2%) patient underwent detethering at 5.5 years. A total of three patients underwent five Chiari decompressions (9.6%). Further, nineteen orthopedic procedures were performed in nine patients (29.0%). A total of 17 (54.8%) patients self-catheterize, which was associated with an anatomical lesion at L3 or below (p = 0.032) and 23 (74.2%) take anticholinergic medication. In conclusion, shunt dependency is associated with myeloschisis, not with the anatomical defect level. Hydrocephalus treatment is associated with premature birth. In this postnatal cohort with significantly longer follow-up data than the MOMs study, the ambulation rate is better, the shunt rate lower and the secondary tethered cord rate better compared to the MOMS study.