TY - JOUR
T1 - Persisting embryonal infundibular Recess (PEIR): Two case reports and systematic literature review
AU - Belotti, Francesco
AU - Lupi, Isabella
AU - Cosottini, Mirco
AU - Ambrosi, Claudia
AU - Gasparotti, Roberto
AU - Bogazzi, Fausto
AU - Fontanella, Marco M.
AU - Fontanella, Marco Maria
AU - Doglietto, Francesco
PY - 2018
Y1 - 2018
N2 - Context The persisting embryonal infundibular recess (PEIR) is a rare anomaly of the floor of the third ventricle with a debated pathogenesis. It can be a cause of misdiagnosis in the case of cystic lesions of the sellar and suprasellar area. Objective To describe two recently evaluated cases and provide a systematic literature review. Evidence Acquisition and Case Descriptions PEIR has been previously reported in six adult patients. Because in some cases it was associated with hydrocephalus and/or empty sella, a possible role of altered intracranial pressure in PEIR formation has been postulated. We evaluated two female patients, aged 34 and 50 years, referred to the Pituitary Surgery Clinic of the University of Brescia with the diagnosis of a sellar cyst and craniopharyngioma, respectively. Endocrine screening and visual field testing were normal. No signs of hydrocephalus or empty sella, as well as other indirect signs of intracranial hypertension, were visible on MRI scans. After a multidisciplinary reevaluation, diagnosis of PEIR was made in both cases. Both patients are followed but have not developed any disturbance related to the PEIR in the following 18 months. Conclusions PEIR is a rare condition, probably unrecognized and the result of dysembriogenesis, which should be included in the differential diagnosis of cystic sellar lesions. Imaging features (funnel pituitary stalk and cyst in the sella) appear pathognomonic. A normal endocrine evaluation might help in the diagnosis and warrants conservative treatment.
AB - Context The persisting embryonal infundibular recess (PEIR) is a rare anomaly of the floor of the third ventricle with a debated pathogenesis. It can be a cause of misdiagnosis in the case of cystic lesions of the sellar and suprasellar area. Objective To describe two recently evaluated cases and provide a systematic literature review. Evidence Acquisition and Case Descriptions PEIR has been previously reported in six adult patients. Because in some cases it was associated with hydrocephalus and/or empty sella, a possible role of altered intracranial pressure in PEIR formation has been postulated. We evaluated two female patients, aged 34 and 50 years, referred to the Pituitary Surgery Clinic of the University of Brescia with the diagnosis of a sellar cyst and craniopharyngioma, respectively. Endocrine screening and visual field testing were normal. No signs of hydrocephalus or empty sella, as well as other indirect signs of intracranial hypertension, were visible on MRI scans. After a multidisciplinary reevaluation, diagnosis of PEIR was made in both cases. Both patients are followed but have not developed any disturbance related to the PEIR in the following 18 months. Conclusions PEIR is a rare condition, probably unrecognized and the result of dysembriogenesis, which should be included in the differential diagnosis of cystic sellar lesions. Imaging features (funnel pituitary stalk and cyst in the sella) appear pathognomonic. A normal endocrine evaluation might help in the diagnosis and warrants conservative treatment.
KW - PITUITARY
KW - PITUITARY
UR - http://hdl.handle.net/10807/206560
U2 - 10.1210/jc.2018-00437
DO - 10.1210/jc.2018-00437
M3 - Article
SN - 0021-972X
VL - 103
SP - 2424
EP - 2429
JO - THE JOURNAL OF CLINICAL ENDOCRINOLOGY AND METABOLISM
JF - THE JOURNAL OF CLINICAL ENDOCRINOLOGY AND METABOLISM
ER -