Giant parieto-occipital encephaloceles: always a dismal prognosis? A case report and review of the literature

The presented case involves an infant with a prenatally diagnosed giant encephalocele including parts of the occipital and superior parietal lobes, as well as the superior cerebellum. Imaging revealed a complex encephalocele with absence or hypoplastic aspects of venous structures, including the vein of Galen and transverse and sigmoid sinus. Clinically, despite the anticipated poor prognosis, the patient remained stable after delivery, leading to a reassessment of the initial palliative care plan. Surgical reconstruction of the encephalocele was performed at 4 weeks of age. The most recent follow-up was conducted at 4 years of age. The patient demonstrated significant developmental progress including speech, language, and motor skills without complaints of headache or nausea. The presented case highlights the medical and ethical challenges physicians face while dealing with pediatric patients with severe congenital anomalies. The case emphasizes the importance of personalized care and reconsideration of long-term expectations for these conditions.

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Publication Details

Journal
Child s Nervous System
Published
2026-03-22
DOI
https://doi.org/10.1007/s00381-026-07212-0
Primary Topic
Head and Neck Surgical Oncology
Type
article
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article

Giant parieto-occipital encephaloceles: always a dismal prognosis? A case report and review of the literature

I. M. Mathijssen, Wouter J. Dronkers, Jochem K. H. Spoor, Attie T. J. I. Go et al.
Child s Nervous System
Head and Neck Surgical Oncology
article

Giant parieto-occipital encephaloceles: always a dismal prognosis? A case report and review of the literature

I. M. Mathijssen, Wouter J. Dronkers, Jochem K. H. Spoor, Attie T. J. I. Go, Victor Volovici
article en

Abstract

The presented case involves an infant with a prenatally diagnosed giant encephalocele including parts of the occipital and superior parietal lobes, as well as the superior cerebellum. Imaging revealed a complex encephalocele with absence or hypoplastic aspects of venous structures, including the vein of Galen and transverse and sigmoid sinus. Clinically, despite the anticipated poor prognosis, the patient remained stable after delivery, leading to a reassessment of the initial palliative care plan. Surgical reconstruction of the encephalocele was performed at 4 weeks of age. The most recent follow-up was conducted at 4 years of age. The patient demonstrated significant developmental progress including speech, language, and motor skills without complaints of headache or nausea. The presented case highlights the medical and ethical challenges physicians face while dealing with pediatric patients with severe congenital anomalies. The case emphasizes the importance of personalized care and reconsideration of long-term expectations for these conditions.

Child s Nervous SystemVol. 42(1)
Erasmus MC (NL), Erasmus MC - Sophia Children’s Hospital (NL), Erasmus University Rotterdam (NL)
Openalex Percentile: Top 14%
Head and Neck Surgical Oncology
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Giant parieto-occipital encephaloceles: always a dismal prognosis? A case report and review of the literature — I. M. Mathijssen, Wouter J. Dronkers, et al. · Child s Nervous System (2026) | TGRS Research Map | TGRS