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<p>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.</p>

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

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2026年3月22日

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  • Wouter J. Dronkers,
  • Irene M. J. Mathijssen,
  • Attie Go,
  • Victor Volovici,
  • Jochem K. H. Spoor

摘要

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.