Introduction: Intracranial neurenteric cysts are very infrequent congenital lesions. Within the nervous system, they are most commonly located in the rachis. Another frequent site is the craniocervical junction. Few cases of supratentorial location have been reported. A differential diagnosis is required to distinguish them from other cysts. They are not often diagnosed in the paediatric age. The clinical features they give rise to are due to the mass effect or episodes of chemical meningitis. Magnetic resonance imaging is the best diagnostic method although on many occasions they cannot be distinguished from arachnoid cysts. Treatment consists in surgery with complete resection of the membranes in order to prevent recurrences.

Case Reports: We report two cases of infants who, in the last weeks of gestation, presented supratentorial intracerebral cystic lesions that resembled arachnoid cysts. A neonatal magnetic resonance scan confirmed the existence of such supratentorial cysts with septae inside them. After presenting an increase in the cranial perimeter, surgical treatment was undertaken with a neuronavigation-guided craniotomy and debridement of the cysts. The cysts contain mucus and have thick membranes. Pathological study results are consistent with a neurenteric cyst. One of the patients presented complete resolution after the intervention with good cerebral expansion. In the second case, there was persistence of an adjoining arachnoid cyst, in which placement of a cyst-peritoneal shunt was necessary with full resolution.

Conclusions: Most intracranial cysts are arachnoid cysts, but there are other cystic lesions that must be treated by surgical means so that they can be completely excised and sent for pathological analyses.

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