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Occipital encephalocele: a retrospective analysis and assessment of post-surgical neurodevelopmental outcome
Soumen Kanjilal1, Pawan Kumar Verma2, Shreyash Rai3
1Department of Neurosurgery, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, UP, India.
Insights
Large encephalocele sacs (≥5 cm) and mature neural tissue within the sac are predictors of poor neurological development in children. These findings aid in understanding developmental outcomes following surgical excision.
Area of Science:
- Pediatric Neurosurgery
- Developmental Neuroscience
- Congenital Malformations
Background:
- Encephalocele involves extracranial herniation of brain structures through cranial defects, often with associated anomalies impacting neurological development.
- Understanding predictors of neurological outcomes is crucial for managing patients with encephalocele.
Purpose of the Study:
- To identify predictors of neurological development in patients who underwent surgical excision of occipital encephalocele.
- To correlate clinical and histopathological findings with neurodevelopmental outcomes.
Main Methods:
- Retrospective analysis of 35 patients with occipital encephaloceles operated between 2012-2022.
- Evaluation of sac size, hydrocephalus, associated anomalies, and neural tissue presence on biopsy.
- Assessment of neurological outcomes using social, language, cognitive, and motor milestones, stratified into developmental delay categories.
Main Results:
- A large sac size (≥5 cm) was significantly associated with neurodevelopmental delay (AOR=33.5, p=0.003).
- Presence of mature neural tissue in the sac also predicted significant neurodevelopmental delay (AOR=13.32, p=0.041).
- Nearly half (49.6%) of the patients experienced moderate to severe developmental delay.
Conclusions:
- Large occipital encephalocele sac size and mature neural tissue on histopathology are indicators of potential poor neurological development.
- These factors are critical for prognostication and management planning in pediatric encephalocele cases.
Background:
Encephalocele represent a group of disorders which is characterised by extracranial herniation of the leptomeninges, brain, and CSF through a structural defect in the cranium. They are usually associated with other intracranial anomalies which may impact the neurological development.
Aim:
This study aimed to assess the predictors of neurological development of patients undergone surgical excision of occipital encephalocele.
Methods:
All patients with occipital encephaloceles operated over the last decade (2012-2022). The sac size, presence of hydrocephalous, and associated anomalies were noted. The biopsy of these patients were reviewed and categorised as those which contains mature neural tissue and those without. The neurological outcomes were assessed by social, language, cognitive, and motor milestone and has been stratified into no delay, mild (1 of 4), moderate (2 or 3 of 4), and severe development delay (4 of 4).
Results:
Total of 35 patients were included with median age of 10 months (IQR = 5-20 months). Fifteen (42.9%) patients had sac size of ≥ 5 cm, and 23 (65.7%) patients had mature neural tissues on biopsy. The median follow-up period was 6.4 years (IQR = 4.38-10.65) years. Seventeen (49.6%) patients had moderate to severe developmental delay. The sac size of ≥ 5 cm (AOR = 33.5; 95%CI = 3.35-334.8) (p = 0.003) and presence of mature neural content in the sac (AOR = 13.32; 95%CI = 1.1-160.36) (p = 0.041) were associated with significant neurodevelopmental delay.
Conclusion:
The presence of a large sac of ≥ 5 cm and the presence of mature neural tissues on histopathological specimen of patients with encephalocele point towards the possibility of poor neurological development.
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