Skip to content
How It Works
Explore
Ask a Question Resources Pricing
Who It's For
Patients
Decode results, prep for appointments, weigh options.
Caregivers & advocates
Carry the research load for someone you love.
Providers & clinicians
Resources for patients in your practice.
Log In Sign Up
PubMed This is a summary of 23 peer-reviewed journal articles Updated
Pediatric Endocrinology

Standard of Care: Replacing What is Missing

At a Glance

Pituitary Stalk Interruption Syndrome cannot be repaired structurally, but children can do well with hormone replacement and regular monitoring. Hydrocortisone comes before thyroid medicine when cortisol is low, and desmopressin requires careful fluid guidance if diabetes insipidus occurs.

While there is no way to “fix” the structural bridge (the pituitary stalk) that did not form correctly, Pituitary Stalk Interruption Syndrome (PSIS) is a highly treatable condition. The goal of treatment is not to cure the anatomy, but to replace exactly what is missing. When hormones are replaced at the right levels, many people with PSIS do well with individualized lifelong replacement [1][2].

The Order of Treatment Matters

If your child has Multiple Pituitary Hormone Deficiency (MPHD), the order in which medications are started is critically important.

The Golden Rule: If your child is deficient in both cortisol and thyroid hormone, Hydrocortisone (cortisol) must be started first [3][4].

Starting thyroid medication (Levothyroxine) first can speed up the body’s metabolism, which makes the body use up its small supply of cortisol even faster. This can trigger a life-threatening adrenal crisis [3]. Your medical team will ensure the adrenal “axis” is stable before adding other treatments like thyroid or growth hormone [5].

Common Hormone Replacements

The specific medications your child needs will depend on which “axes” are affected. Because PSIS can be an evolving condition, your doctor will re-test these levels regularly to see if new replacements are needed as your child grows [6].

  • Hydrocortisone (Cortisol): Replaces the “stress hormone.” It is typically taken as a pill two or three times a day. As covered in the Managing Emergencies page, this medication requires “stress dosing” during illness or injury [7][8].
  • Levothyroxine (Thyroid): Replaces thyroid hormone to maintain energy and metabolism. In PSIS, doctors monitor Free T4 levels rather than just TSH, because the TSH signal from the pituitary may be unreliable [9][10].
  • Recombinant Human Growth Hormone (rhGH): Administered as a small, daily injection under the skin [11]. It is highly effective in PSIS; most children see their greatest “catch-up” growth in the first year of treatment [12][2].
  • Sex Steroids (Testosterone or Estrogen): These are usually started in the teenage years to help your child go through puberty [11]. For boys, this may begin as injections or gels; for girls, it often starts with low-dose estrogen patches or pills [1][13].

Managing Water Balance

The hormone that controls water balance, called Vasopressin (or ADH), is rarely affected in PSIS. In some cohorts, roughly 7-10% of children with PSIS develop a condition called Central Diabetes Insipidus (CDI) (this is different from diabetes mellitus), where the body cannot hold onto water properly [14][15].

If your child has CDI, they will have excessive thirst (polydipsia) and produce large amounts of very pale urine (polyuria) [16]. This is treated with a medication called Desmopressin (DDAVP), which can be taken as a pill, a “melt” under the tongue, or a nasal spray [17][18].
Important Safety Warning: Excessive or unregulated fluid intake while taking desmopressin can cause dilutional hyponatremia (low sodium), which can lead to seizures. You must follow an individualized fluid-intake plan, instructions about dose timing, and watch for warning symptoms such as severe headache, nausea, vomiting, confusion, marked sleepiness, or seizures. Most children with PSIS do not need this medication, even if their MRI shows an ectopic “bright spot” [15][19].

Long-Term Outlook

With consistent treatment, the outlook for children with PSIS can be highly positive.

  • Growth: Many children reach an adult height within the normal range for their family, though results vary [20][11].
  • Development: Hormone replacement supports healthy bone density and brain development [20].
  • Adulthood: While some hormone replacement is usually lifelong (like cortisol or thyroid), growth hormone needs may change. Many adults with PSIS pursue higher education, careers, and even families of their own using specialized fertility treatments when the time comes [21][22].

Your child’s care team will adjust doses as your child grows to ensure they have the individualized support they need for every stage of life [6][23].

Common questions in this guide

Which hormone should be started first when a child has PSIS?
If a child with Pituitary Stalk Interruption Syndrome has low cortisol and low thyroid hormone, hydrocortisone should be started before levothyroxine. Thyroid medicine can speed metabolism and use up cortisol more quickly, increasing the risk of a life-threatening adrenal crisis. The child's medical team should confirm that cortisol support is in place before adding thyroid or growth hormone.
Why is free T4 checked instead of relying on TSH in PSIS?
In Pituitary Stalk Interruption Syndrome, the pituitary signal called TSH may not reliably show whether the body has enough thyroid hormone. Doctors therefore use free T4, along with the child's symptoms and overall health, to guide levothyroxine dosing. Testing is repeated as the child grows.
What hormone replacements might a child with PSIS need?
Treatment depends on which pituitary hormones are missing. It may include hydrocortisone for cortisol, levothyroxine for thyroid hormone, daily growth hormone injections, and testosterone or estrogen to support puberty. Children with central diabetes insipidus may also need desmopressin, and hormone needs can change over time.
How does growth hormone treatment work for children with PSIS?
Recombinant human growth hormone is usually given as a small daily injection under the skin. Many children have their greatest catch-up growth during the first year, while the care team tracks growth and adjusts the dose. Results vary from child to child.
How can I tell if my child has diabetes insipidus, and is desmopressin safe?
Central diabetes insipidus can cause unusual thirst and large amounts of very pale urine, but most children with PSIS do not need desmopressin. When it is prescribed, drinking too much or taking it without an individualized plan can dangerously lower blood sodium and cause seizures. Follow the prescribed fluid and dosing instructions and seek urgent help for severe headache, vomiting, confusion, marked sleepiness, or a seizure.
Will hormone treatment for PSIS continue into adulthood?
Some hormone replacement, such as cortisol or thyroid hormone, is often lifelong, while the need for growth hormone may change and requires reassessment. Sex-steroid treatment helps support puberty, and specialized fertility treatment may help some adults build families. Doses and monitoring should be adjusted as the child grows.
What is the long-term outlook for a child with PSIS?
With consistent, individualized treatment, many children with Pituitary Stalk Interruption Syndrome reach an adult height within the normal range for their family. Hormone replacement supports bone density and brain development, and many adults pursue education, careers, and families. Outcomes vary, so ongoing follow-up is important.

Get notified when new evidence is published on Pituitary stalk interruption syndrome.

We monitor PubMed for new peer-reviewed studies on this topic and email a short summary when something meaningful changes.

References

References (23)
  1. 1

    Pituitary stalk interruption syndrome: A rare case report and literature review.

    Zhang W, Qian F, Lu G, et al.

    Medicine 2020; (99(50)):e23266 doi:10.1097/MD.0000000000023266.

    PMID: 33327247
  2. 2

    Growth Hormone Therapy Benefits Pituitary Stalk Interruption Syndrome Patients with Short Stature: A Retrospective Study of 75 Han Chinese.

    Wang CZ, Guo LL, Han BY, et al.

    International journal of endocrinology 2016; (2016()):1896285 doi:10.1155/2016/1896285.

    PMID: 27190512
  3. 3

    Hypopituitarism.

    Higham CE, Johannsson G, Shalet SM

    Lancet (London, England) 2016; (388(10058)):2403-2415 doi:10.1016/S0140-6736(16)30053-8.

    PMID: 27041067
  4. 4

    Pituitary stalk interruption syndrome: cause, clinical manifestations, diagnosis, and management.

    Voutetakis A, Sertedaki A, Dacou-Voutetakis C

    Current opinion in pediatrics 2016; (28(4)):545-50 doi:10.1097/MOP.0000000000000378.

    PMID: 27386973
  5. 5

    A case of an infant with congenital combined pituitary hormone deficiency and normalized liver histology of infantile cholestasis after hormone replacement therapy.

    Wada K, Kobayashi H, Moriyama A, et al.

    Clinical pediatric endocrinology : case reports and clinical investigations : official journal of the Japanese Society for Pediatric Endocrinology 2017; (26(4)):251-257 doi:10.1297/cpe.26.251.

    PMID: 29026274
  6. 6

    Pituitary Magnetic Resonance Imaging as a Prognostic Factor of Pituitary Insufficiency-The Follow-Up Analysis of a Cohort of Children With Pituitary Stalk Interruption Syndrome.

    Chrzanowska J, Jacków-Nowicka J, Bodetko A, et al.

    Clinical endocrinology 2026; (105(2)):231-236 doi:10.1111/cen.70129.

    PMID: 41844520
  7. 7

    Pituitary Stalk Interruption Syndrome with Excessive Height Growth Combined with Congenital Absence of the Uterus and Ovaries: A Rare Case Report and Review of the Literature.

    Wu R, Xu J

    Diabetes, metabolic syndrome and obesity : targets and therapy 2024; (17()):1739-1747 doi:10.2147/DMSO.S456678.

    PMID: 38645656
  8. 8

    Emergency management of adrenal insufficiency in children: advocating for treatment options in outpatient and field settings.

    Miller BS, Spencer SP, Geffner ME, et al.

    Journal of investigative medicine : the official publication of the American Federation for Clinical Research 2020; (68(1)):16-25 doi:10.1136/jim-2019-000999.

    PMID: 30819831
  9. 9

    Thyrotrophic status in patients with pituitary stalk interruption syndrome.

    Zhang Q, Zang L, Li YJ, et al.

    Medicine 2018; (97(2)):e9084 doi:10.1097/MD.0000000000009084.

    PMID: 29480822
  10. 10

    Congenital Hypopituitarism.

    Parks JS

    Clinics in perinatology 2018; (45(1)):75-91 doi:10.1016/j.clp.2017.11.001.

    PMID: 29406008
  11. 11

    Growth and Adult Height during Human Growth Hormone Treatment in Chinese Children with Multiple Pituitary Hormone Deficiency Caused by Pituitary Stalk Interruption Syndrome: A Single Centre Study

    Wang F, Han J, Wang Z, et al.

    Journal of clinical research in pediatric endocrinology 2020; (12(1)):71-78 doi:10.4274/jcrpe.galenos.2019.2019.0086.

    PMID: 31475508
  12. 12

    Pituitary Stalk Interruption Syndrome: Analysis of Response to Growth Hormone Therapy.

    Ravichandran R, Saikia UK, Bhuyan AK, Baro A

    Indian pediatrics 2024; (61(2)):154-157.

    PMID: 38321728
  13. 13

    Pituitary stalk interruption syndrome: phenotype, predictors, and pathophysiology of perinatal events.

    Diwaker C, Thadani P, Memon SS, et al.

    Pituitary 2022; (25(4)):645-652 doi:10.1007/s11102-022-01243-x.

    PMID: 35749012
  14. 14

    PITUITARY STALK INTERRUPTION SYNDROME: REPORT OF TWO CASES AND LITERATURE REVIEW.

    Lichiardopol C, Albulescu DM

    Acta endocrinologica (Bucharest, Romania : 2005) 2017; (13(1)):96-105 doi:10.4183/aeb.2017.96.

    PMID: 31149155
  15. 15

    Clinico-radiological correlation of pituitary stalk interruption syndrome in children with growth hormone deficiency.

    Sridhar S, Raja BR, Priyanka R, et al.

    Pituitary 2023; (26(5)):622-628 doi:10.1007/s11102-023-01351-2.

    PMID: 37695468
  16. 16

    Causes and Follow-Up of Central Diabetes Insipidus in Children.

    Liu W, Hou J, Liu X, et al.

    International journal of endocrinology 2019; (2019()):5303765 doi:10.1155/2019/5303765.

    PMID: 31049061
  17. 17

    Desmopressin Suppresses Gonadotropin-Induced Spermatogenesis in Patients With Pituitary Stalk Interruption Syndrome: A Retrospective, Single-Center Cohort Study.

    Wen J, Jiangfeng M, Min N, et al.

    Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists 2021; (27(2)):124-130 doi:10.1016/j.eprac.2020.08.001.

    PMID: 33563411
  18. 18

    Beyond Classical Diabetes Insipidus: A Retrospective Case Series Illustrating the Diagnostic Complexity and Diverse Etiologies of Polyuria-Polydipsia Syndrome.

    Jena D, Mishra S, Palai S, et al.

    Cureus 2026; (18(7)):e112989 doi:10.7759/cureus.112989.

    PMID: 42621583
  19. 19

    Clinical Features and Analysis in Pituitary Stalk Interruption Syndrome.

    Guo Q, Zhao J, Yu S

    International journal of endocrinology 2024; (2024()):2493083 doi:10.1155/2024/2493083.

    PMID: 38828392
  20. 20

    Bone Mineral Density in Pituitary Stalk Interruption Syndrome: The Role of Insulin-Like Growth Factor-1 and Testosterone at Different Skeletal Sites.

    Zhu Y, Nie M, Wang X, et al.

    Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists 2022; (28(11)):1118-1124 doi:10.1016/j.eprac.2022.07.011.

    PMID: 35940470
  21. 21

    Gonadotropic status in adult women with pituitary stalk interruption syndrome.

    Terray A, Baussart B, Zins M, et al.

    European journal of endocrinology 2024; (190(6)):501-508 doi:10.1093/ejendo/lvae064.

    PMID: 38857190
  22. 22

    Efficacy of Pulsatile Gonadotropin-Releasing Hormone Therapy in Male Patients: Comparison between Pituitary Stalk Interruption Syndrome and Congenital Hypogonadotropic Hypogonadism.

    Huang Q, Mao J, Wang X, et al.

    Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists 2022; (28(5)):521-527 doi:10.1016/j.eprac.2022.02.008.

    PMID: 35218954
  23. 23

    Normal or elevated prolactin is a good indicator to show pituitary stalk interruption syndrome in patients with multiple pituitary hormone deficiency.

    Eren E, Ongen YD, Ozgur T, et al.

    Journal of pediatric endocrinology & metabolism : JPEM 2022; (35(11)):1394-1400 doi:10.1515/jpem-2022-0366.

    PMID: 36136319

This page explains hormone replacement and safety considerations for children with PSIS for informational purposes only and does not constitute medical advice. Your child's endocrinology team should tailor medicines, doses, fluid plans, and emergency instructions to their needs.

Guide contents Pituitary stalk interruption syndrome
Research & Literature
04 Understanding the Tests 06 The Road Ahead