Paeds · endocrinology-diabetes-and-growth
Hypopituitarism and pituitary lesions
Also known as Hypopituitarism · Combined pituitary hormone deficiency · Panhypopituitarism · Congenital hypopituitarism · Septo-optic dysplasia · Pituitary stalk interruption syndrome · Craniopharyngioma · Intracranial germinoma · Central hypothyroidism · Central adrenal insufficiency
Fellowship guide to hypopituitarism and pituitary and hypothalamic lesions in children: the congenital forms (transcription-factor defects, septo-optic dysplasia, pituitary stalk interruption) and the acquired lesions (craniopharyngioma and germinoma), the anterior and posterior hormone deficiencies and the order in which they fail, the dynamic tests that confirm each axis, and the single replacement rule that keeps a child alive — glucocorticoid before thyroxine.
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- A neonate with hypoglycaemia, prolonged jaundice and a micropenis has congenital hypopituitarism with central adrenal insufficiency until proven otherwise — give stress hydrocortisone and check a paired glucose, cortisol and free thyroxine before the values are lost
- Never start levothyroxine before glucocorticoid in a child with suspected panhypopituitarism: replacing thyroxine first accelerates cortisol clearance and can precipitate a fatal adrenal crisis — cortisol always comes first
- A child with short stature, falling height velocity and any visual field loss or new headache has a craniopharyngioma or other suprasellar lesion until an MRI proves otherwise — do not attribute the growth failure to constitutional delay
- Central diabetes insipidus with new anterior pituitary deficiency and a thickened stalk on MRI is a germinoma until markers and imaging exclude it — send serum and cerebrospinal-fluid beta-hCG and alpha-fetoprotein rather than watching
- Central hypothyroidism is missed if only the thyroid-stimulating hormone is checked, because it is low or inappropriately normal — a low free thyroxine with a non-raised thyroid-stimulating hormone is the signature and demands the free thyroxine be measured
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- Hypopituitarism
- Pituitary and hypothalamic disorders
- Congenital and acquired hypopituitarism in children
- Craniopharyngioma and suprasellar tumours
- Pituitary hormone replacement
- Long case: hypopituitarism and pituitary tumour
- Short case: short stature with visual field defect
- Pituitary and hypothalamic lesions
- Craniopharyngioma and germinoma
- Endocrine examination: short stature and pituitary failure
- Pituitary disorders
- Patient Care: pituitary hormone deficiency
- Medical Expert: hypopituitarism
The single idea that organises this topic is that a pituitary problem is really two problems stacked together: a hormone-deficiency problem and, when a lesion is the cause, a mass problem. The deficiency problem is solved axis by axis with paired pituitary-and-target hormones and dynamic tests, and the mass problem is solved by imaging the hypothalamic–pituitary region and treating the lesion. Miss either half and the child comes to harm. [1] [5]
This page covers congenital and acquired hypopituitarism, the pituitary and hypothalamic lesions that cause it in children — craniopharyngioma and germinoma foremost — the anterior and posterior hormone deficiencies, the dynamic tests that confirm each axis, and the replacement strategy. It links to the dedicated diabetes-insipidus and adrenal-insufficiency leaves for their full pathways rather than repeating them. [11] [9]
Overview & Definition
Hypopituitarism means the pituitary gland is not delivering enough of one or more of its hormones. When several anterior hormones fail together the term is combined pituitary hormone deficiency, and when all of them fail it is panhypopituitarism. The deficiency can be of the anterior lobe, the posterior lobe, or both, and it can arise at the level of the hypothalamus, the stalk, or the gland itself. [1]
The distinction that matters most clinically is congenital versus acquired. A congenital cause is present from birth — a mutation in a pituitary transcription factor, a midline malformation such as septo-optic dysplasia, or an interrupted pituitary stalk — and it declares itself in the newborn with hypoglycaemia and prolonged jaundice, or later with short stature and failure to progress through puberty. An acquired cause develops in a previously well child, and in childhood the commonest acquired cause is a tumour of the hypothalamic–pituitary region. [2] [5]
Because the pituitary sits beneath the optic chiasm and beside the hypothalamic centres for appetite, temperature and thirst, a lesion here rarely causes hormone failure alone. Visual field loss, an obesity or growth-arrest syndrome, disturbed sleep and central diabetes insipidus travel alongside the endocrine picture, which is why the evaluation is always both endocrine and neurological. [1] [5]
References11ShowHide
- [1]Higham CE; Johannsson G; Shalet SM Hypopituitarism. Lancet, 2016.PMID 27041067
- [2]Kelberman D; Dattani MT Hypothalamic and pituitary development: novel insights into the aetiology. Eur J Endocrinol, 2007.PMID 17785694
- [3]Webb EA; Dattani MT Septo-optic dysplasia. Eur J Hum Genet, 2010.PMID 19623216
- [4]Cerbone M; Dattani MT Progression from isolated growth hormone deficiency to combined pituitary hormone deficiency. Growth Horm IGF Res, 2017.PMID 29107171
- [5]Müller HL; Merchant TE; Warmuth-Metz M; et al Craniopharyngioma. Nat Rev Dis Primers, 2019.PMID 31699993
- [6]Liu AP; Takami H; Abdelbaki MS; et al Germinoma: Presentation, Management, and Recent Advances. Adv Cancer Res, 2025.PMID 41198339
- [7]Grimberg A; DiVall SA; Polychronakos C; et al Guidelines for Growth Hormone and Insulin-Like Growth Factor-I Treatment in Children and Adolescents: Growth Hormone Deficiency, Idiopathic Short Stature, and Primary Insulin-Like Growth Factor-I Deficiency. Horm Res Paediatr, 2016.PMID 27884013
- [8]Collett-Solberg PF; Ambler G; Backeljauw PF; et al Diagnosis, Genetics, and Therapy of Short Stature in Children: A Growth Hormone Research Society International Perspective. Horm Res Paediatr, 2019.PMID 31514194
- [9]Bornstein SR; Allolio B; Arlt W; et al Diagnosis and Treatment of Primary Adrenal Insufficiency: An Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab, 2016.PMID 26760044
- [10]Fleseriu M; Hashim IA; Karavitaki N; et al Hormonal Replacement in Hypopituitarism in Adults: An Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab, 2016.PMID 27736313
- [11]Di Iorgi N; Napoli F; Allegri AE; et al Diabetes insipidus--diagnosis and management. Horm Res Paediatr, 2012.PMID 22433947