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A rare case of occipital encephalocele presenting as the largest congenital head mass in an infant

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Abstract

Background

The prevalence of encephalocele is estimated to be 0.8–5.0 per 10,000 live births. The most frequent encephalocele is the occipital encephalocele. It is a congenital neural tube defect characterized by the protrusion or herniation of intracranial contents through a cranial defect. The term “giant/massive/large encephalocele” is used to describe an encephalocele that is significantly larger than the size of the head.

Case description

A 2-month-old male infant presented in the neurosurgery outpatient department with one of the largest head masses over the posterior aspect since birth. The swelling was gradually progressive and developed ulceration over the swelling with intermittent cerebrospinal fluid (CSF) discharge but no associated weakness in limbs. Magnetic Resonance Imaging (MRI) brain showed a large occipital meningoencephalocele containing predominantly cyst with part of the cerebellar and occipital lobe. The surgery was planned. The sac contained CSF with the gliotic occipital lobe. The sac and gliotic brain tissue was excised. He had an uneventful postoperative course.

Conclusion

Surgery serves several functions, including reducing the torque and weight of the head to allow for more normal motor development, removal of the thin, leaking scalp and dural closure to prevent CSF leak and subsequent infection, and improving the cosmetic and social issues that the child and family may have to endure.

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Acknowledgements

The authors acknowledge all professors and consultants in the Department of Neurosurgery for the guidance and assistance.

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Authors and Affiliations

  1. Department of Neurosurgery, All India Institute of Medical Sciences, Patna, Bihar, 801507, India

    Anand Kumar Das & Saraj Kumar Singh

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  1. Anand Kumar Das
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  2. Saraj Kumar Singh
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Contributions

Anand Kumar Das and Saraj Kumar Singh contributed equally to the manuscript preparation. The first draft of the manuscript was written by Anand Kumar Das. Both authors commented on previous versions of the manuscript. Both authors read and approved the final manuscript.

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Correspondence to Saraj Kumar Singh.

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Das, A.K., Singh, S.K. A rare case of occipital encephalocele presenting as the largest congenital head mass in an infant. Childs Nerv Syst 40, 253–256 (2024). https://doi.org/10.1007/s00381-023-06085-x

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  • DOI: https://doi.org/10.1007/s00381-023-06085-x

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