Evolution of posterior fossa and brain morphology after in utero repair of open neural tube defects assessed by MRI

Rethmann, Christin; Scheer, Ianina; Meuli, Martin; Mazzone, Luca; Moehrlen, Ueli; Kellenberger, Christian J.

doi:10.1007/s00330-017-4807-y

Cited by 31 publications

(53 citation statements)

References 33 publications

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“…Our sonographic data regarding HH reversal in 96 % of cases after fMMC repair is underlined by the study published on MR imaging by Retmann et al with the first 27 cases that underwent fMMC repair at the Zurich Centre for fetal Diagnosis and Therapy, also showing a resolution of the HH in 96 % of cases [18]. Other authors have shown MRI data demonstrating that HH improves or reverses in 80-96 % of cases after open fMMC repair [5,7,19,20].…”

Section: Discussionsupporting

confidence: 67%

Hindbrain Herniation and Banana and Lemon Sign After Open Fetal Myelomeningocele Repair – When Do These Signs Disappear and is Shunting Predictable?

et al. 2020

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Purpose The aim was to describe the sonographic follow-up of hindbrain herniation (HH), the banana and lemon sign after fetal myelomeningocele (fMMC) repair, and the time of disappearance of these signs after the intervention, and to investigate any predictive value for the necessity of shunting during the infant’s first year of life. Additionally, the sonographic evolution of the transcerebellar diameter (TCD) before and after fetal intervention was assessed. Patients and Methods The first 50 patients that underwent fMMC repair at Zurich Center for Fetal Diagnosis and Therapy (www.swissfetus.ch) were included in this study. Sonographic scans performed weekly after fMMC repair focusing on HH and banana and lemon signs were analyzed and compared between the shunted and the non-shunted group. ROC curves were generated for the time intervals of resolution of the signs in order to show their predictive accuracy for the need for shunting until 1 year of age. Results HH resolved in 48 fetuses (96 %) before delivery. The sonographic disappearance of HH within the first two weeks after fMMC repair was associated with a significantly lower incidence of shunt placement (OR 0.19; 95 % CI 0.4–0.9) during the first year of life (p = 0.03). All fetuses with persistent HH before delivery received a shunt. TCD growth was observed in all fetuses. Conclusion The reversibility of HH within two weeks after fMMC repair is associated with an 80 % lower incidence of shunt placement during the infant’s first year of life. Moreover, it allows the cerebellum to grow and to normalize its configuration.

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Section: Discussionsupporting

confidence: 67%

Hindbrain Herniation and Banana and Lemon Sign After Open Fetal Myelomeningocele Repair – When Do These Signs Disappear and is Shunting Predictable?

et al. 2020

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“…37,38,57,58 For instance, in our own current experience of 65 cases, we had only one patient with mild pulmonary edema (1.5%, MOMS 6%) and no patient requiring transfusion at delivery (MOMS 9%). Further, in our cohort, a higher rate of complete resolution of hindbrain herniation (over 90%) 59 (MOMS 36%), and a higher median gestational age at birth have been noted. 60 In addition, a different tocolysis management has led to markedly less side effects without compromising uterine efficacy.…”

Section: Diagnostic Workup and Eligibility For Prenatal Osb Repairsupporting

confidence: 52%

Intrauterine Treatment of Spina Bifida

Mazzone¹,

Meuli²

2016

Donald School Journal of Ultrasound in Obstetrics and Gynecology

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Prenatal repair for open spina bifida (OSB) represents nowadays a valid therapeutic option that must be considered whenever a fetus is diagnosed with this severe congenital malformation. However, a judicious weighing of the benefits obtained by fetal surgery against the risks is necessary for every individual case. This article provides the background information that is needed to accomplish that. It describes the evolution of fetal surgery for spina bifida, its benefits and risks, and the different techniques used today.

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“…Occlusion of CSF flow outlets of the fourth ventricle and impairment of the CSF circulation at the craniocervical junction can result in obstructive hydrocephalus [12]. Dysplastic tectal plate, commonly demonstrated in Chiari II patients, may be associated with cerebral aqueduct stenosis [3]. Hypoplastic posterior cranial fossa and cerebellar herniation can also decrease cerebral venous outflow, which might thus impair CSF absorption and result in a component of communicating hydrocephalus [12].…”

Section: Discussionmentioning

confidence: 99%

“…Consequently, the rhombencephalic vesicle including the developing fourth ventricle fails to expand and a small posterior fossa evolves. Indeed, intrauterine repair of the open spinal dysraphism has been shown to result in the improvement of the posterior fossa deformity [2,3]. Although the hallmark of Chiari II malformation is a small posterior fossa, supratentorial abnormalities, such as periventricular nodular heterotopia and callosal dysgenesis, are not uncommon [4], possibly related to the impairment of neuronal migration and axonal guidance by altered CSF hydrodynamics [5].…”

Section: Introductionmentioning

confidence: 99%

Cerebral Diffusivity Changes in Fetuses with Chiari II Malformation

2018

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Objectives: Chiari II malformation is a congenital abnormality characterized by a small posterior fossa with downward displacement of the hindbrain into the foramen magnum. Diffusion-weighted imaging (DWI) can be used to quantitatively characterize brain injury and physiological maturation. We aim to evaluate DWI parameters of the infra- and supratentorial brain in fetuses with Chiari II malformation. Methods: MRI and US studies of 26 fetuses with Chiari II malformation and 15 controls were evaluated for the presence/severity of hydrocephalus, myelomeningocele level, and brain apparent diffusion coefficient (ADC) values. Cerebral flow impedance parameters (resistance and pulsatile indexes, resistance index [RI], and pulsatility index [PI] respectively) from concurrently preformed fetal US were also evaluated. Results: Of the Chiari II group, 16 fetuses had hydrocephalus. There was increasing severity of lateral ventriculomegaly in the high compared to low spinal dysraphism subgroups. There were significant lower ADC values in the frontal (p = 0.01) and temporal lobes (p = 0.05) in Chiari II group compared with normal, regardless of the presence or severity of hydrocephalus. Fetuses with Chiari II showed significantly lower RI and PI values. Conclusions: Abnormal ADC values indicate supratentorial microstructural changes in fetuses with Chiari II malformation. Further investigation of the role of diffusion imaging metrics in evaluating abnormal brain development, parenchymal damage and efficacy of fetal surgery is needed.

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Evolution of posterior fossa and brain morphology after in utero repair of open neural tube defects assessed by MRI

Abstract: • Hindbrain herniation is significantly more pronounced in myeloschisis than in myelomeningocele • Resolution of hindbrain herniation 4 weeks after in utero closure of ONTD • MRI is valuable for preoperative assessment and postoperative evaluation following in utero repair.

Cited by 31 publications

References 33 publications

Hindbrain Herniation and Banana and Lemon Sign After Open Fetal Myelomeningocele Repair – When Do These Signs Disappear and is Shunting Predictable?

Hindbrain Herniation and Banana and Lemon Sign After Open Fetal Myelomeningocele Repair – When Do These Signs Disappear and is Shunting Predictable?

Intrauterine Treatment of Spina Bifida

Cerebral Diffusivity Changes in Fetuses with Chiari II Malformation

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