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Encephalocele - Single centre retrospective study
*Corresponding author: Younes Dehneh, Department of Neurosurgery, University Hospital Center Mohammed VIth Oujda, Maroc. younes.jo1@gmail.com
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Received: ,
Accepted: ,
How to cite this article: Dehneh Y, Bakkar N, Dahamou M, Khay H, Khoulali M, Moufid F. Encephalocele - Single centre retrospective study. Karnataka Paediatr J. 2025;40:66-8. doi: 10.25259/KPJ_31_2024
Abstract
Objectives:
Encephalocele management requires long-term follow-up to monitor for potential complications, assess developmental milestones and address any ongoing healthcare needs. Establishing systems for continuity of care and follow-up can be challenging, particularly in resource-constrained settings. In low-income settings especially, access to specialised medical facilities, equipment and trained healthcare professionals may be limited, hindering timely diagnosis and appropriate surgical intervention.
Material and Methods:
A retrospective study spanning 9 years was conducted on all patients who underwent encephalocele surgery at the Neurosurgery Department in our centre. The objective of our study is to highlight the challenges encountered in the management of encephaloceles.
Results:
Eleven patients with encephalocele were identified in our study. Ruptured encephaloceles were treated as emergencies. Histopathological examination of one patient revealed a teratoma.
Conclusion:
Encephalocele surgeries carry risks of complications such as infection, cerebrospinal fluid leakage and neurological deficits. Managing these complications effectively requires close post-operative monitoring and access to intensive care facilities, which may not be readily available in some settings.
Keywords
Encephalocele
Paediatric malformation
Tube neural defect
INTRODUCTION
Encephaloceles are defined as a herniation of brain tissue and/or meninges from the skull through a congenital bony defect due to a defect in the closure of the cranial part of the neural tube. An encephalocele contains either normal brain tissue or gliotic tissue.[1] The incidence of encephaloceles and other neural tube defects is decreasing. This decline is mainly due to the widespread promotion of folic acid consumption among women before conception and the government-sponsored fortification of food staples such as cereal and flour with folic acid.[2,3]
MATERIAL AND METHODS
The study population is represented by all patients hospitalised in the neurosurgery department of our centre over 10 years (from 09/2014 until 02/2024), on all patients who underwent encephalocele surgery.
Type of the study
This is a retrospective study of all patients operated on during 10 years (from 09/2014 until 02/2024).
Aim of the study
Our objective was to examine the demographics, explore the risk factors associated with the development of encephalocele and study the different treatments and their outcomes on survival, quality of life and associated complications in patients with encephalocele.
RESULTS
During the study period, 11 patients underwent surgery in the neurosurgical department for encephalocele. Our centre is responsible for the oriental region in the country. The incidence of congenital encephaloceles is estimated at 0.025/1,000 children over 10 years in our region. The average age of our patients is 20 days, with extremes ranging from 1 day to 3 months; 7 patients were <1 month old. Females were more frequently affected than males, with 6 females and 5 males. In our study, 55% of the patients (6 children) were from rural areas. Seven patients (63.6%) were born in winter, while 3 (27.2%) were born in summer. Maternal age was under 25 years for 6 patients (54.5%), and two of the eleven mothers (18.1%) were over 35 years old. Medical history is discussed in Table 1. Regarding the location of the encephalocele, all our children presented with a posterior encephalocele, with a parietal location being the most frequent in 7 patients. All of the encephaloceles were median; in three cases, there was superior sagittal sinus involvement. Ruptured encephaloceles were observed in 4 patients (36.3%). Three patients (27.2%) presented with microcephaly, and five patients (45.4%) experienced seizures. Brain magnetic resonance imaging (MRI) was obtained in 8 cases; in all the cases, the encephaloceles contained brain parenchyma, hydrocephalus was found in 4 patients (36.3%), Chiari malformation in 3 patients (27.2%) and brain atrophy in 1 patient (9%). All seven newborns hospitalised in the newborn unit were administered antibiotics as per the protocol of the department; they were put on third-generation cephalosporin. Four patients (36.3%) underwent emergency surgery. Two surgeries were performed in collaboration with plastic surgeons. None of the patients required a blood transfusion. Tissue samples were sent for histopathological examination, revealing a teratoma in one case. During the court term period, meningitis occurred in 3 cases (27.2%) and was treated with systemic antibiotics, specifically a regimen of third-generation cephalosporin and vancomycin. One patient died after septic shock. Surgical site infections were noted in 2 cases (18.1%), managed with local antibiotics and regular dressings, with favourable outcomes. In the long term, 4 patients (36.3%) developed active hydrocephalus, which was treated with ventriculoperitoneal shunts.
| Variable | Patients number (11) | Percentage |
|---|---|---|
| Consanguineous marriage | 5 | 45.4 |
| Multiparity | 4 | 36.3 |
| Use of herbal medicinal products | 1 | 9.09 |
| Use of folic acid | 10 | 90.9 |
| Use of iron | 10 | 90.9 |
| History of birth defect | 1 | 9.09 |
| History of maternal illness | 3 | 27.2 |
| History of drug use | 2 | 18.1 |
| Exposure to passive cigarette smoke | 2 | 18.1 |
DISCUSSION
Encephaloceles are characterised by the protrusion of brain tissue and/or meninges from the skull through a congenital bony defect, resulting from incomplete closure of the cranial portion of the neural tube. Encephalocele is classified as primary or secondary.[4,5] A primary encephalocele is congenital, whereas a secondary encephalocele is acquired.[1] Encephaloceles are typically solitary. In the literature, double encephalocele is a very rare condition, with only a few reported cases.[6] The precise developmental anomaly leading to the occurrence of occipital encephaloceles is not fully understood. Since encephaloceles are covered by skin, a defect in neurulation alone cannot entirely explain their aetiology, as such a defect would result in the absence of skin coverage.[7] It has been suggested that encephaloceles are a multifactorial disorder, with environmental factors playing a significant role.[8] Over 80% of encephalocele cases are not linked to a specific syndrome.[8] Numerous studies have subsequently confirmed that fenugreek ingestion during pregnancy can contribute to neural tube anomalies.[9,10] In the prenatal stage, obstetric ultrasound is an effective screening examination, and polyhydramnios may be the first presenting feature.[11] MRI is used to provide a clear view of the contents of the encephalocele and to diagnose other cranial anomalies, such as hydrocephalus.[12,13] Between 36% and 60% of encephaloceles often have associated malformations.[14,15] Caesarean section is preferred over vaginal delivery to minimise the risk of infection. In the case of a ruptured encephalocele with cerebrospinal fluid (CSF) leak, surgical intervention is urgent.[1] The surgical treatment principles for encephaloceles involve reducing the herniation while preserving as much viable brain tissue as possible, ensuring watertight closure of the dura with sufficient skin coverage, addressing any cosmetic deformity and considering cranial or craniofacial reconstruction as necessary.[7] Post-operative CSF leakage is a complication that increases the risk of post-operative meningitis.[13] Hydrocephalus is the most post-operative complication.[16] The presence of hydrocephalus, along with associated intracranial and extracranial anomalies alongside any form of encephalocele is indicative of a poorer prognosis.[14]
CONCLUSION
Managing encephaloceles in low-income countries poses significant challenges due to limited resources and infrastructure. Despite these obstacles, efforts must be made to improve access to essential healthcare services, including prenatal care and surgical interventions. By addressing these challenges comprehensively, we can strive to improve the prognosis and quality of life for individuals affected by encephaloceles in low-income countries.
Ethical approval:
The study/research was approved by the University. The study committee of the university hospital center approves the favorable opinion to publish this work.
Declaration of patient consent:
The authors certify that they have obtained all appropriate patient consent.
Conflicts of interest:
There are no conflicts of interest.
Use of artificial intelligence (AI)-assisted technology for manuscript preparation:
The authors confirm that there was no use of artificial intelligence (AI)-assisted technology for assisting in the writing or editing of the manuscript and no images were manipulated using AI.
Financial support and sponsorship: Nil.
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