Nelson Syndrome Follow-up

  • Author: Thomas A Wilson, MD; Chief Editor: Stephen Kemp, MD, PhD   more...
 
Updated: Mar 5, 2012
 

Further Outpatient Care

  • Monitor patients at 3-month to 4-month intervals with measurement of growth parameters, blood pressure, and assessment of thyroid function and pubertal development.
    • Loss of pituitary function after pituitary radiotherapy is inevitable, although the time taken for this to occur varies and depends on the dose and number of fractions of radiation that are administered.
    • Growth failure may precede biochemical evidence of growth hormone deficiency with pharmacologic testing. Overnight growth hormone sampling is thought to be more sensitive than pharmacologic testing because the hypothalamus is more radiosensitive than the pituitary and neurosecretory dysfunction may precede actual growth hormone (GH) deficiency. For this reason, patients who have received pituitary radiation need ongoing follow-up of their growth and thyroid function at 3-month and 6-month intervals by an endocrinologist, and patients may require pubertal induction or treatment of hypogonadism in addition to their ongoing replacement with hydrocortisone and fludrocortisone.
  • Thyroid function should be measured at least every 6 months or more frequently if growth velocity is declining and deficiency is evolving.
    • Thyroxin supplementation is necessary when the free T4 levels are below the reference range.
    • Thyroid-stimulating hormone (TSH) is not helpful in determining adequacy of thyroxin replacement in patients with central hypothyroidism.
  • Patients with central diabetes insipidus require treatment with desmopressin acetate (DDAVP).
    • The dosage required varies among individuals and can range from 50-400 mcg/d administered as a single or twice daily dose orally. It may also be administered as a nasal spray, in which case the dose is 5-40 mcg/d usually in 2 divided doses.
    • The evening dose is usually larger to reduce problems with nocturia.
    • Children who receive DDAVP need to have regular sodium measurements, with the frequency determined by the stability of the child. More frequent measurements are needed if the child develops increased fluid losses due to intercurrent illness, hot ambient temperatures, or GI losses.
    • Patients with a normal sense of thirst should be allowed to drink ad libitum because by doing so they regulate their own osmolality.
    • Fluid management is difficult in patients with diabetes insipidus who lack normal thirst sensation. Regular weighing on accurate scales is important, and prescribed fluid intake must be administered based upon the size of the child and the environmental conditions. Greater vigilance and more frequent measurements of sodium are needed in these children.
    • Some physicians believe that children receiving DDAVP should be allowed to "break through" and become polyuric periodically to avoid the risk of hyponatremia. Mixed opinion exists about the frequency with which breakthrough is required.
  • Patients with Nelson syndrome who have undergone radiotherapy and have multiple pituitary hormone deficiencies require lifelong endocrine follow-up. Transition is ideally achieved in the late adolescent years in a clinic that has both adult and pediatric endocrinologists.
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Inpatient & Outpatient Medications

Replacement of hormones depends upon those that are found to be deficient. Regular clinical and biochemical assessment identifies these deficiencies.

  • These may include the need for pubertal induction and sex hormone replacement and growth hormone replacement in addition to adrenal hormone replacement.
  • Patients with central diabetes insipidus also require DDAVP, available as a nasal spray or tablet.
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Deterrence/Prevention

  • Nelson syndrome has become increasingly uncommon because of the significant improvements in all aspects of biochemical and radiological diagnosis and surgical and medical management of Cushing syndrome.
  • Hyperpigmentation with adrenocorticotropin (ACTH) levels above 154 pg/mL have been shown to have a high positive predictive value of Nelson syndrome or ectopic ACTH syndrome.
  • These patients should have an MRI of the pituitary. If no tumor is detected, and ectopic ACTH syndrome is ruled out, MRI of the pituitary should be periodically repeated.
  • Appropriate workup should enable accurate early identification of nearly all cases of ACTH-secreting pituitary adenoma, enabling prevention of virtually all cases; thus, Nelson syndrome should become a disease of the past.
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Complications

The following are the potential complications in patients with pituitary macroadenomas:

  • Related to the primary disease - Visual loss, hypopituitarism, and cranial nerve complications
  • Related to surgical intervention - Infection, stroke, hypothalamic damage, hypopituitarism, and visual loss
  • Related to radiotherapy - Hypopituitarism, damage to vision, difficulties with learning or memory, and the risk of developing second tumors
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Prognosis

  • The prognosis for patients with Nelson syndrome is good with early recognition, prompt coordinated treatment with surgeons and radiotherapists, and appropriate hormone replacement.
  • The falling incidence and lack of longitudinal studies suggest that good recent epidemiological data are lacking.
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Patient Education

  • The main educational requirements for patients with Nelson syndrome relate to their need to increase replacement glucocorticoid doses with intercurrent illness, physical stress, or anesthesia and the need for parenteral steroids before anesthesia or with vomiting or severe diarrhea.
  • Patients receiving other treatment also require education specific to that treatment (eg, administration of growth hormone injections or DDAVP).
  • All patients on steroid replacement should wear a Medic Alert bracelet or medallion.
  • If patients have hypogonadotropic hypogonadism, they also require counseling at an appropriate age about the availability of assisted reproduction.
  • For excellent patient education resources, visit eMedicine's Endocrine System Center. Also, see eMedicine's patient education articles Hypopituitarism in Children and Anatomy of the Endocrine System.
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Contributor Information and Disclosures
Author

Thomas A Wilson, MD  Professor of Clinical Pediatrics, Chief and Program Director, Division of Pediatric Endocrinology, Department of Pediatrics, The School of Medicine at Stony Brook University Medical Center

Thomas A Wilson, MD is a member of the following medical societies: Endocrine Society, Pediatric Endocrine Society, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Coauthor(s)

Vardhini Desikan, MBBS, MPH  Pediatric Endocrinology Fellow, Department of Pediatrics, State University of New York at Stony Brook

Vardhini Desikan, MBBS, MPH is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Nothing to disclose.

George P Chrousos, MD, FAAP, MACP, MACE, FRCP(London)  Professor and Chair, First Department of Pediatrics, Athens University Medical School, Aghia Sophia Children's Hospital, Greece; UNESCO Chair on Adolescent Health Care, University of Athens, Greece

George P Chrousos, MD, FAAP, MACP, MACE, FRCP(London) is a member of the following medical societies: American Academy of Pediatrics, American College of Endocrinology, American College of Physicians, American Pediatric Society, American Society for Clinical Investigation, Association of American Physicians, Endocrine Society, Pediatric Endocrine Society, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Antony Lafferty, MB ChB, FRACP  Senior Lecturer of Pediatric Endocrinology, Monash University Department of Pediatrics, National Institutes of Health, Bethesda, MD, and Princess Margaret Hospital for Children, Perth, Western Australia

Antony Lafferty, MB ChB, FRACP is a member of the following medical societies: Endocrine Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Angelo P Giardino, MD, PhD, MPH  Associate Professor, Baylor College of Medicine; Chief Medical Officer, Texas Children's Health Plan; Chief Quality Officer, Medicine, Texas Children's Hospital

Angelo P Giardino, MD, PhD, MPH is a member of the following medical societies: Academic Pediatric Association, American Academy of Pediatrics, American Professional Society on the Abuse of Children, Harris County Medical Society, Helfer Society, and International Society for Prevention of Child Abuse and Neglect

Disclosure: Bayer Honoraria Review panel membership; Pfizer Grant/research funds Independent contractor; MedImmune Honoraria Review panel membership; Teva Pharmacutical travel & honoraria Managed Care Advisory Panel; CIGNA Honoraria Physician Advisory Council

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Lynne Lipton Levitsky, MD  Chief, Pediatric Endocrine Unit, Massachusetts General Hospital; Associate Professor of Pediatrics, Harvard Medical School

Lynne Lipton Levitsky, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Diabetes Association, American Pediatric Society, Endocrine Society, Pediatric Endocrine Society, and Society for Pediatric Research

Disclosure: Pfizer Grant/research funds P.I.; Tercica Grant/research funds Other; Eli Lily Grant/research funds PI; NovoNordisk Grant/research funds PI; NovoNordisk Consulting fee Consulting; Onyx Heart Valve Consulting fee Consulting

Merrily P M Poth, MD  Professor, Department of Pediatrics and Neuroscience, Uniformed Services University of the Health Sciences

Merrily P M Poth, MD is a member of the following medical societies: American Academy of Pediatrics, Endocrine Society, and Pediatric Endocrine Society

Disclosure: Nothing to disclose.

Chief Editor

Stephen Kemp, MD, PhD  Professor, Department of Pediatrics, Section of Pediatric Endocrinology, University of Arkansas for Medical Sciences College of Medicine, Arkansas Children's Hospital

Stephen Kemp, MD, PhD is a member of the following medical societies: American Academy of Pediatrics, American Association of Clinical Endocrinologists, American Pediatric Society, Endocrine Society, Phi Beta Kappa, Southern Medical Association, and Southern Society for Pediatric Research

Disclosure: Nothing to disclose.

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