Version 1
: Received: 20 December 2023 / Approved: 20 December 2023 / Online: 20 December 2023 (12:03:01 CET)
How to cite:
Spezzani, C.; Di Rita, A.; Noris, A.; Peraio, S.; Lenge, M.; Appleton, T.B.; Menegatti, M.; Mantovani, G.; D’Incerti, L.; Taverna, M.; Giordano, F. Understanding Syringomyelia in Myelomeningocele: A Clinical Observational Study.. Preprints2023, 2023121548. https://doi.org/10.20944/preprints202312.1548.v1
Spezzani, C.; Di Rita, A.; Noris, A.; Peraio, S.; Lenge, M.; Appleton, T.B.; Menegatti, M.; Mantovani, G.; D’Incerti, L.; Taverna, M.; Giordano, F. Understanding Syringomyelia in Myelomeningocele: A Clinical Observational Study.. Preprints 2023, 2023121548. https://doi.org/10.20944/preprints202312.1548.v1
Spezzani, C.; Di Rita, A.; Noris, A.; Peraio, S.; Lenge, M.; Appleton, T.B.; Menegatti, M.; Mantovani, G.; D’Incerti, L.; Taverna, M.; Giordano, F. Understanding Syringomyelia in Myelomeningocele: A Clinical Observational Study.. Preprints2023, 2023121548. https://doi.org/10.20944/preprints202312.1548.v1
APA Style
Spezzani, C., Di Rita, A., Noris, A., Peraio, S., Lenge, M., Appleton, T.B., Menegatti, M., Mantovani, G., D’Incerti, L., Taverna, M., & Giordano, F. (2023). Understanding Syringomyelia in Myelomeningocele: A Clinical Observational Study.. Preprints. https://doi.org/10.20944/preprints202312.1548.v1
Chicago/Turabian Style
Spezzani, C., Maria Taverna and Flavio Giordano. 2023 "Understanding Syringomyelia in Myelomeningocele: A Clinical Observational Study." Preprints. https://doi.org/10.20944/preprints202312.1548.v1
Abstract
Background: Myelomeningocele (MMC) is the most common open spinal dysraphism (OSD) and is typically associated with other clinical and/or radiological abnormalities. Chiari type 2 malformation (CM-II) is observed in 80% to 96.7% of patients, and approximately 80% of cases involve posterior fossa abnormalities leading to hydrocephalus. MMC patients can present with syringomyelia, which usually improves by treating hydrocephalus. However, a minority of patients develop a progressive form of syringomyelia requiring more invasive treatments. Through this retrospective observational study, our objective is to elucidate the incidence of syringomyelia, its symptomatic manifestations, and the associated radiological and clinical characteristics in pediatric patients diagnosed with myelomeningocele
Methods: A retrospective analysis was conducted on the medical records of 39 pediatric patients diagnosed with myelomeningocele at a tertiary referral institute. The study encompassed individuals born between 1997 and 2022. Descriptive statistics were employed to explore epidemiological aspects and clinical features. Additionally, incidence rates of syringomyelia and its progressive variants were calculated, with a focus on their correlation with diagnoses of hydrocephalus, Chiari type 2 malformation (CM-II), and spinal cord tethering.
Results: Over the last twenty-five years, the incidence of syringomyelia in pediatric patients affected by MMC was 46.2%. The incidence of its progressive forms was 16.7%. Two patients improved after the management of hydrocephalus with secondary Endoscopic Third Ventriculostomy (ETV), and only one patient required syrinx-shunting.
Conclusions: The occurrence of syringomyelia among patients with myelomeningocele (MMC) in our center aligns with reported rates in existing literature. Our findings indicate that active hydrocephalus, leading to a deterioration of hindbrain herniation, appears to be a significant factor in the development of progressive symptomatic syringomyelia within this population.
Medicine and Pharmacology, Neuroscience and Neurology
Copyright:
This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
