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Myelomeningocele as an anomaly of secondary neurulation

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A Correction to this article was published on 24 November 2022

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Abstract

Purpose

Myelomeningocele (MMC) is the representative entity of open neural tube defects resulting from an error during primary neurulation. However, cases of MMC in the region of the secondary neural tube (below the junction of S1 and S2 vertebrae) are sometimes encountered. We aimed to analyze the clinical features of atypical “low-lying” MMC in comparison to the typical MMC and suggest possible pathoembryogenesis.

Methods

From 1986 to 2020, 95 MMC patients were treated in our institute. A retrospective review of the radiological and clinical information was performed. We defined “low-lying” MMCs as those with fascia or lamina defects below the S1-2 interspinous ligament.

Results

Thirty-one out of the 95 MMC patients were identified as having low-lying MMC. The percentage of low-lying MMC within the entire MMC group increased dramatically (19% from 1990 to 1999 and 48% from 2000 to 2020). Thirty-nine percent of the low-lying MMCs were associated with hydrocephalus, and 36% showed the Chiari malformation. Clean intermittent catheterization was being performed by 52% of the patients and 46% had a motor weakness. The proportions of hydrocephalus, neurological symptoms, and the number of related procedures in the low-lying MMC were substantially lower than the typical MMC in our cohort and the literature.

Conclusions

We present cases of atypical MMC occurring in the region of secondary neurulation. These cases provide clues that secondary neurulation may lead to open neural defects. Future experiments with animal models supporting what we have seen in the clinics will greatly enhance the understanding of the developmental process of neurulation and the corresponding anomalies.

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Availability of data and material

The datasets generated during and/or analyzed during the current study are available from the corresponding author on reasonable request.

Change history

Abbreviations

MMC:

Myelomeningocele

CNS:

Central nervous system

CCM:

Caudal cell mass

MRI:

Magnetic resonance imaging

VP:

Ventriculoperitoneal

FMD:

Foramen magnum decompression

CIC:

Clean intermittent catheterization

UDS:

Urodynamic study

VUR:

Vesicoureteral reflux

DMSA:

Dimercaptosuccinic acid

DSD:

Dyssynergia

EMG:

Electromyography

TMCC:

Terminal myelocystocele

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Funding

This work was supported by the National Research Foundation of Korea (NRF) Grant funded by the Korean Government (MSIP) (NRF-2021M3E5D9021884). This work was also supported by the Education and Research Encouragement Fund of Seoul National University Hospital (J. Y. Lee).

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Author notes

  1. Kyung Hyun Kim

    Present address: Department of Anatomy and Cell Biology, Seoul National University College of Medicine, Seoul, Korea

Authors and Affiliations

  1. Division of Pediatric Neurosurgery, Seoul National University Children’s Hospital, Seoul, Korea

    Ji Yeoun Lee, Joo Whan Kim, Youngbo Shim, Kyung Hyun Kim & Seung-Ki Kim

  2. Department of Anatomy and Cell Biology, Seoul National University College of Medicine, Seoul, Korea

    Ji Yeoun Lee & Saet Pyoul Kim

  3. Medical Research Center, Genomic Medicine Institute, Seoul National University College of Medicine, Seoul, Korea

    Ji Yeoun Lee

  4. Center for Rare Cancers, Neuro-Oncology Clinic, National Cancer Center, 323 Ilsan-ro, Ilsandong-gu, Goyang, Kyounggi-do, 10408, Republic of Korea

    Jeyul Yang & Kyu-Chang Wang

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  1. Ji Yeoun Lee
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Contributions

Ji Yeoun Lee, Kyu-Chang Wang conceptualized and designed the study, collected the data, carried out the initial analyses, drafted the initial manuscript, and reviewed and revised the manuscript. Joo Whan Kim, Youngbo Shim, and Saet Pyoul Kim collected the data and performed the statistical analysis. Kyung Hyun Kim, Jeyul Yang, and Seung-Ki Kim critically reviewed the manuscript for important intellectual content. All authors approved the final manuscript as submitted and agree to be accountable for all aspects of the work.

Corresponding author

Correspondence to Kyu-Chang Wang.

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Ethics approval

The Institutional Review Boards (IRB) of our institution approved this study, and patient consent was not required. The procedures used in this study adhere to the tenets of the Declaration of Helsinki.

Consent for publication

The waiver of consent was granted under IRB review.

Conflict of interest

The authors report no conflict of interest concerning the materials of methods used in this study or the findings specified in this paper.

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The original online version of this article was revised: In this article the references in Table 1 are incorrectly cited. Given here is the corrected table.

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Lee, J.Y., Kim, J.W., Shim, Y. et al. Myelomeningocele as an anomaly of secondary neurulation. Childs Nerv Syst 38, 2091–2099 (2022). https://doi.org/10.1007/s00381-022-05591-8

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