ISSN 1662-4009 (online)

ESPE Yearbook of Paediatric Endocrinology (2022) 19 1.13 | DOI: 10.1530/ey.19.1.13

ESPEYB19 1. Pituitary and Neuroendocrinology Clinical Papers (5 abstracts)

1.13. Management of children and young people with idiopathic pituitary stalk thickening, central diabetes insipidus, or both: a national clinical practice consensus guideline.

Cerbone M , Visser J , Bulwer C , Ederies A , Vallabhaneni K , Ball S , Kamaly-Asl I , Grossman A , Gleeson H , Korbonits M , Nanduri V , Tziaferi V , Jacques T & Spoudeas HA



Lancet Child Adolesc Health. 2021 Sep;5(9):662-676. doi: 10.1016/S2352-4642(21)00088-2. Epub 2021 Jun 30; PMID: 34214482.

Brief Summary: This comprehensive guideline defines the role of cerebrospinal fluid tumor markers, whole-body imaging, and the indications, timing and risks of stalk biopsy, as well as criteria for discharge in children and young people with idiopathic pituitary stalk thickening, central diabetes insipidus, or both.

Unexplained pituitary stalk thickening and/or central diabetes insipidus (DI) in children and adolescents may be due to occult neoplasia, congenital defects, or may remain idiopathic in a third of the patients even after careful work-up. Differential diagnostics is often problematic.

This paper, by an expert UK national guideline development group, describes a systematically developed management flowchart and clinical practice guideline to inform specialist care and improve outcomes in children and young people (aged <19 years) with idiopathic pituitary stalk thickening, central diabetes insipidus, or both.

The authors recommend dynamic pituitary function testing, specialist pituitary imaging, measurement of serum β-human chorionic gonadotropin and alpha-fetoprotein concentrations, chest x-ray, abdominal ultrasonography, optometry, and skeletal survey for all such patients. Stalk thickening of 4 mm or more at the optic chiasm, 3 mm or more at pituitary insertion, or both, is potentially pathological, particularly if an endocrinopathy or visual impairment coexists. The authors also make recommendations regarding surveillance, the use of cerebrospinal fluid tumor markers, whole-body imaging, indications, timing and risks of stalk biopsy, and give criteria for discharge. Finally, they also encourage to set up registry studies to validate its recommendations.

This guideline serves as a valuable reference for pediatric endocrinologists and oncology colleagues who care for children with pituitary stalk thickening and/or DI.

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