Progressive hydrocephalus despite early complete reversal of hindbrain herniation after prenatal open myelomeningocele repair

Lu, Victor M.; Snyder, Kendall; Ibirogba, Eniola R.; Ruano, Rodrigo; Daniels, David J.; Ahn, Edward S.

doi:10.3171/2019.7.focus19434

Cited by 8 publications

(5 citation statements)

References 26 publications

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“…This is demonstrated in our study, in which 17% of fetuses without significant ventriculomegaly at the last prenatal assessment still required postnatal CSF diversion treatment. Previous research has shown that even fetuses undergoing in‐utero closure and demonstrating an optimal prenatal course and regression of hindbrain herniation may sometimes require CSF diversion surgery, albeit often later in life 14 .…”

Section: Discussionmentioning

confidence: 99%

Correlation of fetal ventricular size and need for postnatal cerebrospinal fluid diversion surgery in open spina bifida

Agrawal

AlRefai

Abbasi

et al. 2022

Ultrasound in Obstet & Gyne

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What are the novel findings of this work?This study on fetuses with open spina bifida supports the previous finding of an association between the timing of closure (prenatal or postnatal) and the need for postnatal cerebrospinal fluid (CSF) diversion. Our findings also demonstrate that ventriculomegaly is associated independently with the need for postnatal CSF diversion, irrespective of timing of closure. Fetal ventriculomegaly progresses at a similar rate in fetuses with open spina bifida that undergo prenatal closure and those that undergo postnatal closure. What are the clinical implications of this work?The findings of this study should help in clinical counseling regarding the need for postnatal CSF diversion in patients undergoing fetal or postnatal spina bifida closure.

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Section: Discussionmentioning

confidence: 99%

Correlation of fetal ventricular size and need for postnatal cerebrospinal fluid diversion surgery in open spina bifida

Agrawal

AlRefai

Abbasi

et al. 2022

Ultrasound in Obstet & Gyne

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“…Although uncommon, this occurrence has been previously documented. 13 Our patient underwent endoscopic third ventriculostomy with choroid plexus cauterization. The goal with this procedure was to prevent the need for a shunt, because shunting in spina bifida has been shown to be inversely associated with ambulatory status.…”

Section: Discussionmentioning

confidence: 92%

Postnatal myelomeningocele closure in smallest reported neonate: illustrative case

Mohis,

Cordeiro,

Larson

et al. 2024

Journal of Neurosurgery: Case Lessons

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BACKGROUND Myelomeningocele (MMC) is the most serious form of spina bifida, a congenital defect in neural tube development. Defect closure in a patient with an extremely low birth weight presents unique challenges and risks; lower birth weight is associated with multiple organ system concerns, homeostasis is difficult, and local tissue is underdeveloped. To the authors’ knowledge, the present case is the lowest reported weight (490 g) for a neonate with postnatal MMC repair. OBSERVATIONS A preterm male with a prenatally diagnosed lumbosacral MMC and associated Chiari malformation type II was born at 23 weeks 1 day to a 29-year-old mother, gravidity 6 parity 4. The patient was medically stabilized and underwent MMC closure on day of life 5. His weight was 490 g at the time of this repair, and he did not have any surgical complications. At age 16 months, he underwent endoscopic third ventriculostomy with choroid plexus cauterization; he has not required any further hydrocephalus treatments since the last follow-up at 30 months of age. LESSONS To the authors’ knowledge, this case is the lowest birth weight ex utero MMC closure reported in the literature. Challenges of prematurity and size required appropriate preoperative stabilization, careful hemostasis and temperature regulation, and meticulous surgical technique.

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“…Nevertheless, this information is not available at the time of referral, so it cannot be used as part of parental counseling prior to repair. Residual HBH after prenatal ONTD repair has been linked to an increased risk for postnatal hydrocephalus treatment 4,5 ; however, some cases with complete in-utero HBH reversal can also develop progressive hydrocephalus and require postnatal diversion treatment 21 . Gradual-onset hydrocephalus may persist as a sequela of successful HBH reversal with prenatal repair.…”

Section: Discussionmentioning

confidence: 99%

“…It is possible that a combination of different predisposing features, including persistent ventriculomegaly, other ventricular changes after ONTD repair, a more cephalad defect and higher intracranial pressure compromising CSP integrity, could affect long-term CSF hydrodynamics, leading to a higher risk for VP shunting after birth 6,21,22 .…”

Section: Discussionmentioning

confidence: 99%

Perforation of cavum septi pellucidi in open spina bifida and need for hydrocephalus treatment by 1 year of age

Sanz Cortes,

Johnson,

Sangi‐Haghpeykar

et al. 2024

Ultrasound in Obstet & Gyne

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ObjectiveTo measure the association of detecting a perforated cavum septi pellucidi (CSP) seen on brain imaging of fetuses referred to a large Fetal Center for open neural tube defect (ONTD) repair at the time of referral and six weeks postoperatively, with the eventual need for hydrocephalus treatment by 1 year of age. In utero repair of ONTD reduces the risk of developing severe hydrocephalus requiring treatment by 1 year of age. Additional studies have shown that the presence of severe fetal lateral ventriculomegaly (> 15 mm) before in utero repair significantly increases the risk of requiring postnatal treatment for hydrocephalus. Perforation of the CSP (pCSP) is encountered on fetal imaging and can reflect increased intraventricular pressure in the fetal brain. We sought to evaluate the association of pCSP seen on fetal imaging before or after in utero ONTD repair with the eventual need for hydrocephalus treatment.MethodsRetrospective cohort study of 110 patients who underwent laparotomy‐assisted fetoscopic ONTD repair between 2014 and 2021 at a single center. Eligibility criteria for surgery were based on MOMS trial criteria although maternal BMI up to 40 kg/m2 was allowed. Fetal brain imaging was assessed with ultrasound and MRI at initial referral and six weeks postoperatively. Retrospective review to assess CSP integrity was performed using stored US and MRI scans. Information on the need for hydrocephalus treatment by 12 months was obtained by medical record review. Parametric tests and non‐parametric tests were used as appropriate to compare outcomes between pCSP vs intact CSP cases as assessed by ultrasound at referral. Logistic regression analyses were performed to assess predictive values for the need for a hydrocephalus treatment.ResultsA total of 20.6% and 21.8% cases were identified with a preoperative pCSP by US and MRI, respectively, and 26.6% and 24.2% on post‐operative US and MRI, respectively. Ventricular size increased after surgery [preoperatively: 11 (5.89‐21.45) mm vs. postoperatively:16 (7‐43.5) mm; p<0.01] as well as the proportion of cases with severe ventriculomegaly [12.7% vs 57.8%; p<0.01].Assessment of 107 cases with complete CSP evaluation by US at referral were included for comparison: 22 with pCSP and 85 with intact CSP. The pCSP group presented with larger ventricles (14.32 +3.45 mm vs. 10.37+2.37 mm); p<0.01) and more severe ventriculomegaly (40.9% vs. 5.9%; p<0.01). The same associations were observed at 6 weeks postoperatively [ventricular size: 21 mm (13‐43.5 mm) vs. 14.25 mm (7‐29 mm); p<0.01], and [severe ventriculomegaly: 95% vs. 46.8%; p<0.01]. Cases with pCSP had a lower rate of hindbrain herniation (HBH) reversal postoperatively (65% vs. 88.6%; p=0.01).Cases with pCSP required treatment for hydrocephalus more frequently than those without that feature [17/19 (89.5%) vs. 17/75 (22.7%); p<0.01]. The strongest predictor for this outcome was the lack of HBH reversal (OR 36.2, 95% CI 5.96‐219.12; p<0.01) followed by pCSP at referral (OR 23.4, 95% CI 5.42‐100.98, p<0.01) and by pCSP at 6 weeks postop (OR 19.48, 95% CI 5.68‐66.68, p<0.01).ConclusionsThe detection of a pCSP in fetuses with ONTD can reliably identify those cases at highest risk for needing hydrocephalus treatment by 1 year of age. The evaluation of this brain structure can greatly improve our counseling to families considering fetal surgery for ONTD, in order to set expectations about postnatal outcome.This article is protected by copyright. All rights reserved.

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Progressive hydrocephalus despite early complete reversal of hindbrain herniation after prenatal open myelomeningocele repair

Cited by 8 publications

References 26 publications

Correlation of fetal ventricular size and need for postnatal cerebrospinal fluid diversion surgery in open spina bifida

Correlation of fetal ventricular size and need for postnatal cerebrospinal fluid diversion surgery in open spina bifida

Postnatal myelomeningocele closure in smallest reported neonate: illustrative case

Perforation of cavum septi pellucidi in open spina bifida and need for hydrocephalus treatment by 1 year of age

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