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

Affiliations

¹ London Centre for Paediatric Endocrinology and Diabetes, Great Ormond Street Hospital and University College London Hospitals, London, UK; Section of Molecular Basis of Rare Disease, Genetics and Genomic Medicine Programme, Great Ormond Street Hospital Institute of Child Health, University College London, London, UK. Electronic address: manuela.cerbone@ucl.ac.uk.
² Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK.
³ Department of Paediatrics, Royal Devon and Exeter Hospital, Exeter, UK.
⁴ Diagnostic Imaging Department, Neuroradiology Section, Sidra Medicine, Doha, Qatar.
⁵ Whittington Health Trust, London, UK.
⁶ Department of Endocrinology, University of Manchester and Manchester University Foundation Trust, Manchester, UK.
⁷ Department of Paediatric Neurosurgery, Royal Manchester Children's Hospital, Manchester, UK; The Geoffrey Jefferson Brain Research Centre, University of Manchester, Manchester Academic Health Science Centre, Manchester, UK.
⁸ Oxford Centre for Diabetes, Endocrinology and Metabolism, University of Oxford, Oxford, UK; Department of Endocrinology, William Harvey Research Institute, Barts and the London School of Medicine, Queen Mary University of London, London, UK.
⁹ Department of Endocrinology, Queen Elizabeth Hospital, Birmingham, UK.
¹⁰ Department of Endocrinology, William Harvey Research Institute, Barts and the London School of Medicine, Queen Mary University of London, London, UK.
¹¹ Department of Paediatrics, Watford General Hospital, Watford, UK.
¹² Second Paediatric Department, AHEPA University Hospital, Thessaloniki, Greece.
¹³ Developmental Biology and Cancer Programme, Great Ormond Street Hospital Institute of Child Health, University College London, London, UK; Department of Histopathology, Great Ormond Street Hospital NHS Foundation Trust, London, UK.
¹⁴ London Centre for Paediatric Endocrinology and Diabetes, Great Ormond Street Hospital and University College London Hospitals, London, UK; Section of Molecular Basis of Rare Disease, Genetics and Genomic Medicine Programme, Great Ormond Street Hospital Institute of Child Health, University College London, London, UK.

PMID: 34214482
DOI: 10.1016/S2352-4642(21)00088-2

Review

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

Manuela Cerbone et al. Lancet Child Adolesc Health. 2021 Sep.

. 2021 Sep;5(9):662-676.

doi: 10.1016/S2352-4642(21)00088-2. Epub 2021 Jun 30.

Authors

Affiliations

¹ London Centre for Paediatric Endocrinology and Diabetes, Great Ormond Street Hospital and University College London Hospitals, London, UK; Section of Molecular Basis of Rare Disease, Genetics and Genomic Medicine Programme, Great Ormond Street Hospital Institute of Child Health, University College London, London, UK. Electronic address: manuela.cerbone@ucl.ac.uk.
² Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK.
³ Department of Paediatrics, Royal Devon and Exeter Hospital, Exeter, UK.
⁴ Diagnostic Imaging Department, Neuroradiology Section, Sidra Medicine, Doha, Qatar.
⁵ Whittington Health Trust, London, UK.
⁶ Department of Endocrinology, University of Manchester and Manchester University Foundation Trust, Manchester, UK.
⁷ Department of Paediatric Neurosurgery, Royal Manchester Children's Hospital, Manchester, UK; The Geoffrey Jefferson Brain Research Centre, University of Manchester, Manchester Academic Health Science Centre, Manchester, UK.
⁸ Oxford Centre for Diabetes, Endocrinology and Metabolism, University of Oxford, Oxford, UK; Department of Endocrinology, William Harvey Research Institute, Barts and the London School of Medicine, Queen Mary University of London, London, UK.
⁹ Department of Endocrinology, Queen Elizabeth Hospital, Birmingham, UK.
¹⁰ Department of Endocrinology, William Harvey Research Institute, Barts and the London School of Medicine, Queen Mary University of London, London, UK.
¹¹ Department of Paediatrics, Watford General Hospital, Watford, UK.
¹² Second Paediatric Department, AHEPA University Hospital, Thessaloniki, Greece.
¹³ Developmental Biology and Cancer Programme, Great Ormond Street Hospital Institute of Child Health, University College London, London, UK; Department of Histopathology, Great Ormond Street Hospital NHS Foundation Trust, London, UK.
¹⁴ London Centre for Paediatric Endocrinology and Diabetes, Great Ormond Street Hospital and University College London Hospitals, London, UK; Section of Molecular Basis of Rare Disease, Genetics and Genomic Medicine Programme, Great Ormond Street Hospital Institute of Child Health, University College London, London, UK.

PMID: 34214482
DOI: 10.1016/S2352-4642(21)00088-2

Erratum in

Correction to Lancet Child Adolesc Health 2021; 5: 662-76.
[No authors listed] [No authors listed] Lancet Child Adolesc Health. 2022 Dec;6(12):e28. doi: 10.1016/S2352-4642(21)00205-4. Epub 2021 Jul 10. Lancet Child Adolesc Health. 2022. PMID: 34252389 No abstract available.

Abstract

Unexplained or idiopathic pituitary stalk thickening or central diabetes insipidus not only harbours rare occult malignancies in 40% of cases but can also reflect benign congenital defects. Between 2014 and 2019, a multidisciplinary, expert national guideline development group in the UK systematically developed a 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. All such cases of idiopathic pituitary stalk thickening and central diabetes insipidus require 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 occult disease. 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. In this guideline, we define the role of surveillance, cerebrospinal fluid tumour markers, whole-body imaging, indications, timing and risks of stalk biopsy, and criteria for discharge. We encourage a registry of outcomes to validate the systematic approach described in this guideline and research to establish typical paediatric stalk sizes and the possible role of novel biomarkers, imaging techniques, or both, in diagnosis.

PubMed Disclaimer

Conflict of interest statement

Declaration of interests IK-A has been a member of the National Health Service England Paediatric Neurosciences Clinical Reference Group from 2013 onwards, member of Council of the Society of British Neurological Surgeons from 2018 onwards, and was chairman of the British Paediatric Neurosurgical Group from 2016 to 2018. TJ reports grants from Brain Tumour Charity, Children with Cancer UK, Cancer Research UK, Great Ormond Street Hospital Children's Charity, Olivia Hodson Cancer Fund, and National Institute of Health Research; and personal fees from Bayer, outside of the submitted work. TJ is also a director and shareholder in Repath and Neuropath; the company secretary at Repath; the editor in chief of Neuropathology and Applied Neurobiology; and the lead for the childhood solid tumour domain Genomes Clinical Interpretation Partnership for Genomes England and the 100 000 genomes projects. HAS is chair of the Project Board for all eight paediatric endocrine tumours in simultaneous development; Project Board Lead for pituitary adenomas, craniopharyngiomas, and idiopathic PST, CDI, or both; responsible for raising grants to fund wider endeavour; initiator and founder of group with wider involvement across all guidelines; non-renumerated, voluntary trustee, founder, and chair of Success Charity–Life After Cure (RCN 1188298); and is a stakeholder in this guideline and donated (like other charity stakeholders Association of Multiple Endocrine Neoplastic Disorders, Pituitary Foundation, BSPED, British Neurological Surgeons, RCPCH, and CCLG) between £1000 and £3000 either in kind or in a restricted grant to the CCLG charity to support its administrative expenses only for this and seven other endocrine tumour childhood guidelines. All other authors declare no competing interests.

Publication types

Actions

MeSH terms

Actions
Actions
Actions
Actions
Actions
Actions
Actions
Actions
Actions
Actions
Actions
Actions
Actions
Actions

LinkOut - more resources

Full Text Sources
- Elsevier Science

Save citation to file

Email citation

Add to Collections

Add to My Bibliography

Your saved search

Create a file for external citation management software

Your RSS Feed

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

Affiliations

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

Authors

Affiliations

Erratum in

Abstract

Conflict of interest statement

Publication types

MeSH terms

LinkOut - more resources

Full Text Sources