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Introduction <p>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 <b>psychomotor delay</b> and <b>visual agnosia</b>, and identifying <b>key prognostic factors</b> that influence these outcomes.</p> Methods <p>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.</p> Results <p>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.</p> Conclusion <p>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.</p>

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Occipital encephalocele: prognostic factors of psychomotor delay and visual agnosia

  • 期刊论文

  • 发表日期：

    2026年1月2日

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  • Maïla Bounemra,
  • Khansa Abderrahmen,
  • Abdelhafidh Slimane,
  • Nesrine Chaouch,
  • Ala Belhaj,
  • Sofiane Bouali,
  • Khalil Ghedira,
  • Imed Ben Said,
  • Jalel Kallel

摘要

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.