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Differences between Myeloschisis and Myelomeningocele in Patients Undergoing Prenatal Repair of Open Spina Bifida
José Miguel Müller1, Edgardo Corral Sereño2, Aura Jimenez3
1Neurosurgery Department, Hospital Regional Libertador Bernardo O'Higgins, Rancagua, Chile.
Introduction:
Open spina bifida (OSB) manifests as myelomeningocele (MMC) or myeloschisis (MS). Both lesions theoretically leak cerebrospinal fluid (CSF) and produce different degrees of Chiari II malformation (CHMII). However, it is not entirely clear whether these forms of OSB have different clinical manifestations. This study aimed to evaluate the clinical and/or radiological differences between MS and MMC in patients who underwent prenatal OSB repair.
Methods:
A total of 71 prenatal repairs were performed with the open technique at the Public Hospital of Rancagua, Chile, between 2012 and 2022. We performed follow-up magnetic resonance imaging (MRI) of fetuses that qualified for prenatal OSB repair surgery. We examined the correlations between various anthropomorphic measurements and clinical and imaging variables, such as the type of lesion and dimensions such as ventricle atrium diameter, degree of severity of CHMII, need for CSF shunt at 12 months, and walking at 30 months.
Results:
This study included 71 fetuses with OSB for which 38 MRI examinations were analyzed; 61% (43/71) of lesions were MMC and 39% (28/71) were MS. Grade 3 (severe) Chiari II malformations were found in 80% (12/15) of MS and 43% (10/23) of MMC (p < 0.05). Fetuses with an atrial diameter less than 13.48 mm had a lower probability of requiring a CSF shunt at 12 months (p < 0.05). MMC was associated with a significantly higher frequency of clubfoot at birth (p < 0.05), whereas MS was significantly associated with more severe CHMII (p < 0.05). Although the correlations were not significant, we observed clear trends that more children with MS required shunts at 12 months and could walk at 30 months compared to children with MMC.
Conclusions:
MS and MMC are distinct subtypes of OSB. Further studies of larger cohorts that include biomolecular and histological analysis are required to better understand the differences between these lesions. The findings of this study may enable healthcare providers to better advise parents and prepare healthcare teams earlier for the management of patients undergoing prenatal repair of OSB.
Insights
Myelomeningocele (MMC) and myeloschisis (MS) are distinct open spina bifida (OSB) subtypes. MS is linked to more severe Chiari II malformations, while MMC is associated with clubfoot, impacting prenatal repair management.
Area of Science:
- Medical research
- Pediatric surgery
- Fetal medicine
Background:
- Open spina bifida (OSB) presents as myelomeningocele (MMC) or myeloschisis (MS), both potentially causing cerebrospinal fluid (CSF) leakage and Chiari II malformation (CHMII).
- Clinical distinctions between MMC and MS in fetuses undergoing prenatal repair remain unclear.
Purpose of the Study:
- To investigate clinical and radiological differences between myeloschisis (MS) and myelomeningocele (MMC) in fetuses treated with prenatal open spina bifida (OSB) repair.
Main Methods:
- Analysis of 71 prenatal OSB repairs (2012-2022) using open technique.
- Follow-up magnetic resonance imaging (MRI) and correlation of anthropometric measurements with clinical and imaging variables.
Main Results:
- Myeloschisis (MS) was associated with a higher incidence of severe Chiari II malformations (CHMII) (80%) compared to myelomeningocele (MMC) (43%).
- MMC correlated with a higher frequency of clubfoot at birth.
- Fetuses with smaller atrial diameters (<13.48 mm) showed a reduced need for CSF shunts at 12 months.
Conclusions:
- Myeloschisis (MS) and myelomeningocele (MMC) represent distinct subtypes of open spina bifida (OSB).
- Further research with larger cohorts, including biomolecular and histological analyses, is warranted to elucidate differences.
- Findings can aid healthcare providers in counseling parents and preparing for prenatal OSB repair management.
