Initial Treatment of Meningocele and Myelomeningocele Lesion in Children

By Ammar Anwer¹, Naveed Ahmad Khan¹, Obaid Ur Rehman², Javaria Siddiq², Abrar Ullah Khan²

Doi: https://doi.org/10.36283/ziun-pjmd14-1/014

How to cite: Anwer A, Khan NA, Rehman OU, Siddiq J, Khan AU Initial Treatment of Meningocele and Myelomeningocele Lesion in Children. Pak J Med Dent. 2025 Jan ;14(1): 87-91. Doi: https://doi.org/10.36283/ziun-pjmd14-1/014.

Received: Thu, June 13, 2024   Accepted: Mon, December 16, 2024, Published: Fri, January 10, 2025.

Background: Myelomeningocele is a severe form of spina bifida. The disease significantly impacts survival and neurological outcomes in children. The purpose of this study is to investigate the effect of in-time assessment and surgical intervention on functional outcomes in children with MMC.

Methods: A retrospective study was conducted on medical records of 60 children who underwent MMC repair between January 2016 and December 2019. ERC/62/24/06 was categorized into six groups based on the timing of assessment and surgical intervention. The KA Bennett classification system was used for functional outcomes grading. A Kaplan-Meier and log-rank test was used to find survival rates.

Results: Survival analysis revealed that early surgical intervention significantly reduced mortality rates compared to conservative treatment (p<0.05). Children treated within 24 hours (Group C) had higher survival rates (94.4%) compared to those with delayed or no closure (Groups A and B). Functional improvements were noted across all groups, with the highest in groups undergoing early surgery. The presence of hydrocephalus negatively impacted survival, with early closure groups showing lower mortality (26%) compared to those with delayed closure (55.1%). Group E showed fewer cases of hydrocephalus (62%) and smaller thoracic lumbar lesions were excluded from comparisons due to its unique profile assessed on days 3-4 post-birth.

Conclusion: Early surgical intervention within the first 24 hours post-birth significantly improves survival and functional outcomes in children with MMC compared to delayed treatment or conservative management.

Keywords: Myelomeningocele, Spinal Dysraphism, Hydrocephalus, Pediatric Neurosurgery

A form of neural tube defect known as meningoceles occurs when the neural tube protrudes and remains open, resulting in a cystic dilatation of the spinal meninges that contains cerebrospinal fluid without involving any neural tissue. The cervical, thoracic, lumbar, and sacral regions are where they are primarily found ^1,2. Meningoceles mainly extend anteriorly or laterally, and an example of this is an anterolateral meningocele lesion ³. When neural tissue in an open dysraphism is exposed to the outside world, it can result in conditions like myelomeningocele, hemi myelocele, and myeloschisis, which are all linked to Chiari type II malformations. Closed spinal dysraphism, on the other hand, shows signs of intact skin and hidden neural arch deficiencies, it is distinguished based on subcutaneous tumor ⁴. Spinal meningoceles are frequently seen in the posterior side of the thoracic and sacral region and it is more common in females. This disease is caused during embryonic development. The development of the conus medullaris and filum terminale indicates the occurrence of secondary neurulation ⁵. If any complications occur during secondary neurulation, then it can result in lumbar meningoceles ⁶. Anterior sacral meningoceles typically present with symptoms of urological and gastrointestinal complaints in adulthood ⁷. Marfan’s syndrome and neurofibromatosis have been reported about anterolateral meningocele which is a rare lesion. The reason for their occurrence is congenital mesodermal dysplasia and hypoplastic one change ⁸.

The presentation of menigocele in adults usually includes paraparesis and pain ⁹. Other conditions that resemble spinal meningoceles are cystic neoplastic lesions, parasitic foci, and arachnoid cysts ¹⁰. Conventional radiography can assist in observing the spinal canal and the posterior vertebral body ¹¹. Magnetic resonance imaging (MRI) is the primary diagnostic tool for pre-operative surgical planning and post-operative monitoring. However, myelography or computed tomography (CT) can also be used for the evaluation of spinal meningoceles ^12,13. Another noninvasive method for visualizing meningoceles is cine MRI (CMRI). This tool’s primary advantage is its ability to directly observe tissue mobility and offer information in flow ¹⁴.

Meningocele neck is characterized by an opened sac that is present at the level of the paraspinal muscle ¹⁵. Surgery is the initial course of treatment if neurological symptoms such as paraparesis or bladder problems are present. Surgery is done by removing the lamina and afterward closing the bridge between the sac and meningocele ¹⁶. Myelomeningocele is a more complex condition but it shows the same signs and symptoms as that of meningoceles ¹⁷. The surgery cannot be done with complete independence, especially for subjects with high lesions as they are associated with high risk. Any complication in surgery can have a severe psychosocial and long-term physical impact on a patient ¹⁸. Despite effective surgical treatment for meningoceles, there is a gap in research for myelomeningocele on long-term outcomes, which is a more complex spinal dysraphism. This study aims to investigate the impact of the timing of assessment and treatment initiation on functional outcomes in children with myelomeningocele who underwent surgery.

A retrospective review of medical records of Myelomeningocele repair patients was conducted between January 2016 to December 2019. The ERC/62/24/06 subjects presented with a spectrum of upper or lower motor neuron paralysis. Only patients with complete medical records were included and children with missing records, no surgery at the study hospital, or other birth defects were excluded. A purposive sampling technique was used to collect data for this study. The sample size calculation was conducted using the RiskCalc sample calculator with a minimum difference of 0.3 in proportions set at 0.5, with 80% power at a 5% significance level, with a 1:1 ratio allocation and 0% drop-out rate. Functional grading was based on a classification system developed by KA Bennett et al. in 2014, which assessed muscle power in various lower limb muscle groups ¹⁹. Grades were assigned as follows: Grade 0 indicated complete paralysis, Grade 1 suggested the possibility of walking with extensive support in severe paralysis, Grade 2 referred to children able to walk with aid and moderate paralysis post-surgery, Grade 3 indicated mild paralysis with the ability to walk with aid or independently after surgery, Grade 4 represented almost normal muscle function, and Grade 5 indicated completely normal lower limbs. Patients were categorized into six groups based on the timing of assessment and treatment initiation for analysis: Group A included Children assessed within 3 days of birth and treated either conservatively or with early surgery (n=15). Group B incorporated children assessed on the fourth day of birth and treated either conservatively or with late surgery (n=10). Group C had children with surgery within 24 hours of birth (n=15). Group D included children assessed on the 1st or 2nd day after birth and treated surgically (n=10). Group E included children assessed on the 3rd or 4th day after birth and treated surgically (n=9). Group F incorporated children assessed after the 4th day of life, referred from other hospitals, and treated surgically (n=11). The study data was collected with the informed consent of legal guardians. To summarize patient demographics descriptive statistics such as lesion locations, hydrocephalus rates, and paralysis grades across the groups were taken into account. Kaplan-Meier curves were applied to estimate cumulative survival probabilities, and Chi-square tests analyzed categorical variables like hydrocephalus presence. ANOVA assessed differences in survival rates and paralysis improvement, while logistic regression evaluated factors influencing mortality and functional outcomes. Statistical significance was set at 5%, and analyses were conducted using SPSS.

Table 1.  Vertebral Levels, Proportion of Hydrocephalus, and Demographic Information

Table 1 shows distributions of vertebral lesions and demographic details in all groups which shows high proportions of lesions in the sacral region and also suggests a varied proportion of hydrocephalus from 60% to 80%. The results show that Group E in which children were assessed on the 3^rd and 4^th day of birth has less hydrocephalus and smaller lesions in the thoracic lumbar level of spinal lesion. Due to this group E was excluded from the comparison.

Table 2. Survival Analysis of Open Myelomeningocele Across Different Time Periods for Groups A, B, C, D, and E

Table 2 displays the survival analysis of Group A patients, examining the likelihood of survival across various intervals from birth to the conclusion of the third year. The cumulative survival rate is depicted for all patients surviving beyond the first week. The second analysis aimed to enable a comparison with Group B patients, whose survival data is outlined. The circumstances in Group B were complex, as initial assessments were lacking in the initial days of life. These patients were later referred to the Children’s Hospital, Lahore, at different ages after receiving initial care elsewhere. Despite the administered treatments, open meningocele cases exhibited the highest mortality rate, even though survivors constituted a larger subgroup.

Figure 1. Survival Analysis for Different Time Periods of All Groups

Table 3: Cumulative Survival Analysis of Open Myelomeningocele for Groups A, B, C, D, and E

Table 4: Survival of Open Myelomeningocele

Tables 3 and 4 show the survival data of surgery subjects done within four days after birth. This table shows the mortality and cumulative survival of children managed with conservative treatment or delayed surgery with the children treated with immediate surgery.

Figure 2. Cumulative Survival Analysis of all Groups

Figure 3.  Survival Curve of Open Myelomeningocele

Among cases with no closure or delayed closure (Group A + B), patients without hydrocephalus exhibited a mortality rate of 45.3%, whereas those with hydrocephalus showed a higher mortality rate of 55.1%. In contrast, in cases with early closure (Group C + D + E), patients without hydrocephalus demonstrated a notably lower mortality rate of 26.0%, whereas those with hydrocephalus had a mortality rate of 46.8%.

Paralysis did not significantly improve during the first week in Group A. 12 children survived in the severely paralyzed group and 3 children died. Group B’s analysis was limited due to fewer infants being evaluated immediately after birth. Similarly, Groups C and D showed minimal changes in paralysis levels within the first week, contrasting with Group E, where some improvement was noted. Although surgeries performed on the 3rd and 4th day exhibited less improvement, they still outperformed conservative treatments. Out of 60 children, 15 died, while the remaining showed varying degrees of improvement.

Significant variations in the distribution of vertebral lesions, hydrocephalus proportions, and survival outcomes across the groups were found in this study. Group E included subjects who were assessed on the 3rd and 4th days of birth, and showed reduced hydrocephalus and smaller thoracolumbar lesions. That led to its exclusion from paralysis and improvement comparisons. Early surgical intervention showed improved survival rates in both delayed and conservative treatment. Mortality was found to be notably higher in hydrocephalus cases across all groups.
Myelomeningocele is a severe form of spina bifida and it is a congenital condition rarely present in adults²⁰. It is characterized by the incomplete closure of the backbone and membranes around the spinal cord ²¹. A comprehensive understanding is required of survival rates to improve patient care. Survival rates and long-term outcomes for children with Meningocele and Myelomeningocele can vary widely ²². It can be influenced by factors such as the level of the spinal lesion, the presence of hydrocephalus, and the quality of medical and surgical interventions ²³.

The variability in survival outcomes across different cohorts underscores the complexity of MMC and the multifactorial nature of its management ²⁴. This study underscores a significant reduction in mortality rates within the first four days after birth. This aligns with previous 2014 research which highlighted the negligible impact of treatment modality on patient mortality ¹⁹. Surgical intervention within the first 24 hours yields optimal outcomes. A 2018 study demonstrated promising outcomes including reduced mortality rates and improved muscle function, albeit in a smaller sample size ²⁵. However, another 2022 study did not provide specific mortality rates for closed myelomeningocele cases ²⁶. Another 2022 study reported a rate of 90%, compared to the 65% observed in this study, possibly influenced by differences in lesion location and patient demographics²⁷. The sample size was relatively small which may limit the generalizability of our findings. Another limitation was relying on existing medical records, which may have introduced selection bias and limited the control over variables.

Our study emphasizes the crucial role of early surgical intervention in managing myelomeningocele. Timely surgery significantly improves survival and lower limb function compared to conservative approaches.

MRI – Magnetic Resonance Imaging

CT – Computed Tomography

CMRI – Cine Magnetic Resonance Imaging

ERC – Ethical Review Committee

SPSS – Statistical Package for the Social Sciences

With heartfelt gratitude to all who supported this work.

The authors declare no conflict of interest.

Ethical guidelines and approved by the Ethical Review Board/Committee, numbered ERC/62/24/06.

AA and NAK conceptualized the study and supervised the research. OUR and JS collected and analyzed the data. AUK contributed to data interpretation and manuscript preparation. All authors reviewed and approved the final manuscript.

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