ISSN 1470-3947 (print) | ISSN 1479-6848 (online)

Endocrine Abstracts (2019) 63 P1078 | DOI: 10.1530/endoabs.63.P1078

Pituitary stalk interruption syndrome with late revelation: about 5 cases

Asma Chadli, Siham El Aziz, Lygie sephora & Kibhat Odiki

Department of Endocrinology, Diabetology and Metabolic Diseases. UHC Ibn Rochd, Casablanca, Morocco Laboratory of Neuroscience and Mental Health. Faculty of Medicine and Pharmacy, HASSAN II-Casablanca University, Casablanca, Morocco.

Introduction: Pituitary Stalk Interruption Syndrome (PSIS) is a distinct and rare clinical entity responsible for isolated or combined anterior pituitary insufficiency. The objective of this work is to describe the clinical, hormonal and radiological aspects of PSIS.

Patients and methods: Observational descriptive study of the files of 5 patients including 4 cases of congenital PSIS and one acquired, followed in the endocrinology and diabetology department of Ibn ROCHD University Hospital.

Results: These are male patients with a mean age of 19 years. The reasons for consultation were failure to thrive (3) and micropenis (2). The history of reported head trauma in a patient. The physical examination returned to a statural (-4SD), weight (-3SD) and pubertal (Tanner stages G1P1) delay (3 cases) with 1 case of psychomotor delay. Hormonal exploration revealed an isolated in 2 cases and a complete deficit in 3 cases. Pituitary MRI scan diagnosed ectopia of the post-pituitary gland (4) and pituitary stem hypoplasia without visualization of the anterior or post-pituitary parenchyma (1). Treatment consisted mainly of hormonal substitution.

Conclusion: PSIS is a rare abnormality revealed by a complete or partial hypopituitarism, the diagnosis is often late. Its management involves regular monitoring of the evolving nature of the disease.

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