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The outcome of surgical management for the patient with giant occipital encephalocele: a case illustration and systematic review

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Abstract

Purpose

Occipital encephalocele (OE) is one of congenital malformation of the central nervous system. However, giant OE, mostly defines as bigger-than-head OE, is extremely rare and carries a worse prognosis. Here, we presented our systematic review of the management of the giant OE and illustrated our case.

Method

The systematic review was carried out under PRISMA guidelines. Publications were searched under “occipital encephalocele” from 1959 to April 2021. Our primary area of interest was the outcome of patients who have undergone surgery for giant OE. Variables of interest included age, sex, size of the sac, presentations, associated anomalies, management, outcome, and follow-up period were collected.

Result

We collected 35 articles, consisting of 74 cases (including 1 case from our illustrative case), to perform a systematic review. The mean age at the time of surgery was 3.53 ± 8.22 months. The mean circumference of the sac was 52.41 ± 18.6 cm. The three most common associated anomalies were microcephaly, corpus callosal agenesis/dysgenesis, and Chiari malformation. After the surgery, survival was reported in 64 (90.1%) patients. Postoperative complications were reported in 14 cases (16 events). Age above 1 month at the time of surgery was significantly associated with survival (p = 0.02) but not with complication (p = 0.22). In contrast, the type of surgery was not associated with survival (p = 0.18) or complications (p = 0.41).

Conclusion

Despite a rare condition with a poor prognosis, our reported case and systematic review revealed promising outcomes of surgery regardless of surgical strategies, especially in patients older than 1 month. Thus, appropriate planning is essential for the treatment of this condition.

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Acknowledgements

We thank you Dr. Ariya Mongkolporn, M.D. for her great work in illustration.

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Authors and Affiliations

  1. Division of Neurosurgery, Department of Surgery, Faculty of Medicine, Thammasat University Hospital, Thammasat University, 95 Phahonyothin Rd., Klong1, Klongluang, Pathumthani, 12120, Thailand

    Vich Yindeedej, Ruksakul Sungpapan, Gahn Duangprasert & Raywat Noiphithak

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  1. Vich Yindeedej
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  2. Ruksakul Sungpapan
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  3. Gahn Duangprasert
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  4. Raywat Noiphithak
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Contributions

Conception and design, V.Y., R.S.; methodology and analysis, V.Y.,R.N. writing—original draft preparation, V.Y.; writing—review and editing, V.Y., R.N.; project administration, G.D.; supervision, R.N. All authors reviewed the manuscript.

Corresponding author

Correspondence to Raywat Noiphithak.

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Ethics approval

This study was approved by the Institutional Review Board at the Human Research Ethics Committee of Thammasat University (Medicine) with reference number MTU-EC-SU-0–361/64. This study was performed in line with the principles of the Declaration of Helsinki.

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Informed consent for publication was obtained from the involved patient’s parents (underage patient).

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All authors declare no competing interest.

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Yindeedej, V., Sungpapan, R., Duangprasert, G. et al. The outcome of surgical management for the patient with giant occipital encephalocele: a case illustration and systematic review. Childs Nerv Syst 39, 2161–2167 (2023). https://doi.org/10.1007/s00381-023-05934-z

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