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Occipital encephalocele: prognostic factors of psychomotor delay and visual agnosia
Maïla Bounemra1, Khansa Abderrahmen2, Abdelhafidh Slimane2
1Neurosurgery Department, Mongi Ben Hmida National Institute of Neurology of Tunis, Tunis El Manar University, Tunis, Tunisia. bounemramaila@gmail.com.
Insights
Occipital encephalocele, a severe neural tube defect, often leads to psychomotor delay and visual agnosia. Early surgery and multidisciplinary care are crucial for improving outcomes in affected infants.
Area of Science:
- Neuroscience
- Developmental Biology
- Pediatric Surgery
Background:
- Occipital encephalocele is a rare but severe neural tube defect with significant challenges in management and outcomes.
- Despite advances, high morbidity and mortality persist, necessitating better understanding of neurodevelopmental trajectories.
Purpose of the Study:
- To define neurodevelopmental outcomes in occipital encephalocele patients.
- To highlight the incidence of psychomotor delay and visual agnosia.
- To identify key prognostic factors influencing patient outcomes.
Main Methods:
- Retrospective descriptive study of 40 patients treated over 23 years.
- Data collection included demographic, clinical, radiological, surgical, and outcome information.
- Analysis focused on identifying postoperative complications and prognostic indicators.
Main Results:
- A 10% mortality rate was observed, with hydrocephalus being a frequent associated anomaly (40%).
- Psychomotor delay occurred in 67.6% and visual agnosia in 10% of cases.
- Adverse prognostic factors included preoperative hydrocephalus, herniated brain tissue, and delayed surgery.
Conclusions:
- Occipital encephalocele significantly impacts neurodevelopment.
- Delayed surgery, herniated functional brain tissue, and postoperative hydrocephalus worsen outcomes.
- Multidisciplinary management and early follow-up are vital for improving prognosis.
Introduction:
Occipital encephalocele is a rare neural tube defect that presents significant challenges. Despite advances in neurosurgery and prenatal imaging, it remains associated with substantial morbidity and mortality. This study aimed to define the neurodevelopmental outcomes of patients with occipital encephalocele, highlighting the incidence of psychomotor delay and visual agnosia, and identifying key prognostic factors that influence these outcomes.
Methods:
We conducted a retrospective descriptive study including all patients treated for occipital encephalocele at the Mongi Ben Hmida National Institute of Neurology in Tunis over a 23-year period. Demographic, clinical, radiological, surgical, and outcome data were collected from medical records and analyzed to identify postoperative complications and prognostic indicators.
Results:
A total of 40 patients were treated, with a median age at surgery of 11.8 months and a female predominance (sex ratio 0.38). Hydrocephalus was the most frequent associated anomaly (40%). Postoperative complications included meningitis (12.5%) and secondary hydrocephalus (37.5%), with a mortality rate of 10%. Psychomotor delay was observed in 67.6% of cases and visual agnosia in 10%. Univariate analysis identified preoperative hydrocephalus, herniation of functional brain tissue, and delayed surgery as significant adverse prognostic factors.
Conclusion:
Occipital encephalocele remains a severe congenital anomaly with considerable neurodevelopmental impact. Delayed surgical intervention, the presence of herniated functional brain tissue, and postoperative hydrocephalus negatively impact outcomes. A multidisciplinary management approach and early neurodevelopmental follow-up are essential to improve long-term prognosis and quality of life for these patients.
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