Giant occipital encephalocele: anatomical characteristics and functional evaluation. A case report
DOI:
https://doi.org/10.59156/revista.v40i01.788Keywords:
AngioMRI, Giant encephalocele, Occipital lobe, Visual evoked potentialsAbstract
Background: encephalocele is a congenital dysraphic malformation of the midline skull, characterized by herniation of intracranial contents through a cranial defect. The occipital region is the most common location and is associated with increased morbidity due to the possible inclusion of functional brain tissue and neurovascular structures, as well as potential involvement of the visual pathway.
Objectives: to describe a case of giant occipital encephalocele treated surgically, incorporating vascular and visual functional assessment through complementary studies.
Case description: a full-term male newborn presented at birth with a large occipital mass of soft consistency, with solid and cystic components, covered by a violaceous membrane and implanted at the occipital skull base. Neuroimaging studies confirmed the diagnosis of a giant occipital encephalocele, without evidence of hydrocephalus. Initial clinical examination revealed ocular abnormalities with disconjugate eye movements.
Surgery: early surgical correction was performed, consisting of complete excision of the herniated sac, multilayer dural reconstruction, and definitive cutaneous closure using a rotational flap. Histopathological examination confirmed a meningoencephalocele. The postoperative course was complicated by wound dehiscence associated with surgical site infection, which was successfully treated with targeted antibiotic therapy. Follow-up studies included magnetic resonance imaging, MR angiography, and visual evoked potentials, revealing complex structural brain abnormalities and prolonged P100 latency in the right eye.
Conclusion: giant occipital encephalocele represents a significant surgical and functional challenge. An integrated anatomical, vascular, and visual functional assessment provides valuable information for postoperative follow-up and for estimating neurological and visual prognosis in these patients.
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