Panhypopituitarism is a topic covered in the Select 5-Minute Pediatrics Topics.

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Basics

Description

Technically, “panhypopituitarism” (pan meaning “all”) requires deficiency of all 8 pituitary hormones; however, the term generally is used for deficiencies of >1 pituitary hormone.

Epidemiology

  • Congenital forms affect both sexes equally and are diagnosed early in childhood.
  • The epidemiology of acquired or secondary forms depends on the underlying cause.

Risk Factors

Genetics

Most cases are not thought to be genetic; however, there are rare autosomal recessive, autosomal dominant, and X-linked forms.

Pathophysiology

  • Pathology is based on specific deficiency or deficiencies.
  • Growth hormone (GH): hypoglycemia in newborns and poor growth in patients older than 6–12 months
  • Adrenocorticotropic hormone: hypocortisolism
  • Thyroid-stimulating hormone (TSH): hypothyroidism
  • Luteinizing hormone (LH)/follicle-stimulating hormone (FSH): hypogonadism
  • Antidiuretic hormone: diabetes insipidus
  • Prolactin: Hyperprolactinemia can accompany hypothalamic causes of hypopituitarism.

Etiology

  • Idiopathic (some may be due to hypophysitis, [inflammation of the pituitary gland])
  • Congenital
    • Absence of the pituitary (empty sella syndrome is a risk)
    • Pituitary malformations (ectopic posterior pituitary, hypoplastic infundibular stalk, hypoplastic pituitary)
    • Genetic disorders due to mutations in genes or transcription factors (POUF1, HESX1, LHX3, LHX4, OTX2, SOX2, SOX3, PTX2, PROP1, etc.)
    • Familial panhypopituitarism
    • Rathke cleft cyst
  • Acquired
    • Birth trauma or perinatal insult
    • Surgical resection of the gland or damage to the stalk
    • Traumatic brain injury
    • Hypophysitis
    • Iron deposition secondary to chronic transfusion therapy (e.g., β-thalassemia)
  • Infection
    • Viral encephalitis
    • Bacterial or fungal infection
    • Tuberculosis
  • Vascular
    • Pituitary infarction
    • Pituitary aneurysm
  • Cranial irradiation
  • Tumors:
    • Craniopharyngioma
    • Germinoma
    • Glioma
    • Pinealoma
    • Primitive neuroectodermal tumor (medulloblastoma)
  • Histiocytosis
  • Sarcoidosis

Commonly Associated Conditions

  • Midline defects such as cleft lip/palate, hypotelorism, single central maxillary incisor
  • Septo-optic dysplasia (de Morsier syndrome)
  • Holoprosencephaly

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Citation

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TY - ELEC T1 - Panhypopituitarism ID - 14130 Y1 - 2015 PB - Select 5-Minute Pediatrics Topics UR - https://im.unboundmedicine.com/medicine/view/Select-5-Minute-Pediatric-Consult/14130/all/Panhypopituitarism ER -