BACKGROUND: The persisting embryonal infundibular recess (PEIR) is a rare anomaly of the floor of the third ventricle, whose pathogenesis is still not well comprehended.
AIM THE STUDY: To describe two recently evaluated cases and provide an updated literature review.
RESULTS AND DISCUSSION: PEIR has been previously reported in 8 adult patients. In the earliest cases, this anomalous condition was associated to hydrocephalus and empty sella syndrome. These associations led to the hypothesis that altered intracranial pressure was at the base of the pathogenic process. Nevertheless, Steno et al. recently described one case not associated to hydrocephalus, suggesting a dysembryogenic hypothesis.
We evaluated two female patients, aged 34 and 50, referred to the Pituitary Surgery Clinic of Brescia with the diagnosis of a sellar cyst and craniopharyngioma, respectively. Endocrine screening and visual field testing were normal. No signs of hydrocephalus were visible on MRI scans. After a multidisciplinary re-evaluation, diagnosis of PEIR was made in both cases.
CONCLUSIONS: PEIR is a rare condition, probably often unrecognized, which should be included in the differential diagnosis of cystic sellar lesions.