Pituitary Disorders Jo Choudhry, M.D. PGY-1
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Pituitary Disorders Jo Choudhry, M.D. PGY-1

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Pituitary Disorders Jo Choudhry, M.D. PGY-1 Pituitary Disorders Jo Choudhry, M.D. PGY-1 Presentation Transcript

  • Pituitary Disorders Jo Choudhry, M.D. PGY-1
  • The Pituitary Gland
    • Located at the base of the skull
    • Anterior and Posterior lobes
    • Portal connection from the hypothalamus
  • Anterior Lobe Posterior Lobe
    • Growth hormone (GH)
    • Gondadotrophs (LH/FSH)
    • TSH
    • Prolactin
    • Corticotropin (ACTH)
    • Oxytocin
    • Vasopressin
  • Normal Changes in Pregnancy
    • Anterior lobe size doubles-triples due to lactotrophs.
    • Placental estrogens stimulate lactotroph proliferation
    • Decreased response to GnRH, dec. LH/FSH
    • Decrease pituitary GH, inc. placental GH
    • Increase CRH (prob. Placental origin) during 2 & 3 trimesters
    • 2-4 X increase in ACTH, despite inc. in bound and free cortisol.
  • Hyperprolactinemia
    • Causes :
      • 1. disruption of dopamine (tumor, trauma, infiltrative lesions)
      • 2. hypothyroid (increases TRH)
      • 3. estrogen increase (pregnancy)
      • 4. chest wall burns – nueronal effect like suckling
      • 5. chronic renal failure, returns to nml after transplant
      • 6. drugs (verapamil, H2 blockers, estrogens, opiates, dopamine receptor antagonists, reserpine, a-methyldopa)
  • Prolactinomas
    • Most common functional pituitary tumor
    • 10% are lactotroph and somatotroph such as GH producing
    • Presents with amenorrhea and infertility
    • Prolactinomas lose TRH response
    • Microadenomas <10mm on MRI
    • Macroadenomas >10mm
  • Treatment Pregnancy Not Desired
    • Treat only if symptomatic
      • (HA, vision changes)
    • Dopamine agonist (Bromocriptine)
      • 1.25mg qhs 1 wk, then BID
      • If intolerant with nausea, may give vaginally
      • Not recommended for breastfeeding
    • Transspenoidal surgery if unsuccessful
  • Risks of surgery : *4.6% post-op neurologic complication: infarction/hemorrhage *2-10.5% Diabetes Insipidous *8.8% fluid and electrolyte *2% Cerebrospinal fluid rhinorrhea *2% Meningitis *3.2% cranial nerve 3,4,or 6 palsies
  • Treatment Pregnancy Desired
    • If macro , shrink size b/f preg with bromocriptine (36% will develop neurologic symptoms)
    • If causing major visual defect and unresponsive, consider transspenoidal surgery b/f preg.
    • Bromocriptine until
    • preg occurs, then stop.
  • During Pregnancy
    • Visual field check q2-3 mos. and MRI prn
    • If neurologic symptoms occur during preg, usually about 14wga, restart treatment.
      • Class B
    • If severe and unresponsive:
      • 2 nd trimester: consider surgery
        • PTL risk
      • 3 rd trimester: wait until PP
  • Acromegaly
    • 98% GH pituitary adenoma
    • 1/3 of all functional pituitary adenomas
    • Stimulates growth of skin, connective tissue, cartilage, bone, and viscera
    • Nitrogen retention, insulin antagonism, and lipogenesis
  • Risks of Long Term Excess GH
    • Arthropathy
    • Neuropathy
    • Cardiomyopathy
    • Respiratory obstruction
    • Diabetes Mellitus
    • Hypertension: exaterbates cardiomyopathy
      • NOT Reversible
    • increased risk of tumors:
      • leiomyomata
      • colon polyps
    Reduced overall survival by an average of 10 years
  • Diagnosis
    • Somatomedian-C levels and IGF-1 levels
    • If pregnant: special assay to distinguish placental GH
    • 70% pitutary GH responds to TRH, placental variant does not.
  • Treatment
    • Goal: lower the serum insulin-like growth factor to normal for age/gender
    • Surgically accessible micro- or Macroadenomas:
      • Transspenoidal surgery
    • 2 nd Line therapy: Somatostatin analogs or Dopamine agonists
    • 3 rd Line therapy: Somatostatin receptor antagonist
    • Last resort: Radiation
  • Pregnancy and Acromegaly
    • D/C tx with confirmation
    • GH Maternal to Fetal transfer negligible, except for glu intolerance.
    • If severe neurologic sympts, try Bromocriptine
      • May not dec. GH, shrink lactotrophs
    • Somatostatin analogs have been used in 3 pts with no ill effects to fetus, despite transplacental passage.
  • Cushing’s Disease
    • High ACTH leads to excess glucocorticoid
    • Incidence may be 5-25 per million
    • Women are 3-8X more likely than men
  • Cushing’s disease
    • Centripetal obesity
    • Moon face; buffalo hump
    • Skin atrophy
    • Easily bruised
    • Striae
    • Cutaneous fungal infections
    • Hyperpigmentation
    • Oligo or amenorrhea
    • Hirsutism and Virilization with adrenal tumors
  • Cushing’s Disease
    • Proximal muscle wasting & weakness
    • Osteoporosis
    • Glucose intolerance
    • HTN, hypokalemia
    • Thromboembolism
    • Depression, Psyc
    • Infection
    • Glaucoma
  • Complications if Pregnant
    • Rare due to decreased fertility
    • Premature birth
    • SAB, Stillbirths
    • IUGR
    • Neonatal adrenal insufficiency
    • Maternal: HTN, DM, CHF, Death
  • Diagnosis
    • Cushing’s Syndrome :
    • 24 hr urine cortisol excretion
    • If not 3x nml, measure pm salivary cortisol
    • Cushing’s Disease vs. Syndrome :
    • HIGH dose Dexamethasone suppression test (8mg overnight)
      • Successful if Pituitary origin
  • Treatment
    • Transsphenoidal surgery
    • Pituitary irradiation
    • Adrenalectomy (Surgical, Mitotane)
      • Nelson’s Syndrome : expanding intrasellar tumor and hyperpigmentation
    • Pregnancy:
      • 1 st Trimester: Surgery
      • 2 nd Trimester: Adrenal Enzyme Inhibitors vs. surgery
      • 3 rd Trimester: Early delivery, enzyme inhibitors until lung maturity
  • Thyrotropin-secreting Adenoma
    • <1% of all hyperthyroidism cases
    • 25% of adenomas secrete other hormones
    • Goiter, visual defects, menstral irreg, galatorrhea
    • Lab:
    • Normal or High TSH
    • High total and free T4 and High T3
    • MRI
  • Treatment
    • Transsphenoidal surgery
      • 1/3 Cure
      • 1/3 improvement
      • 1/3 no change
    • Dopamine Agonist
    • Somatostatin Analogue (Octreotide)
      • Works so well, may give before surgery
      • Nausea, diarrhea, bloating, glu intolerance, cholelithiasis
    • Do NOT use antithyroid therapy
  • Gonadotroph adenoma
    • Usually considered non-functioning
      • Secrete inefficiently, variably
    • Presents with nuerologic symptoms
    • Difficult to Diagnose
      • Rule out other adenomas
      • Prepubertal girls= breast devel, vag. Bleeding
      • Premenopausal= amenorrhea, oligo
  • Gonadotroph adenoma vs. menopause and ovarian failure
    • High FSH with low LH
    • High serum free alpha subunit
    • High estridiol, FSH, thickened endometrium and polycystic ovaries
  • Treatment of non-functioning and gonadotrophin macroadenomas
    • Transsphenoidal surgery
    • +/- Radiation
  • Hypopituitarism
    • 76% tumor or treatment of tumor
      • Mass effect of adenoma on other hormones
      • Surgical resection of non-adenomatous tissue
      • Radiation of pituitary
        • Check hormones 6 mos after and then yearly
    • 13% extrapituitary tumor
      • Craniopharyngioma
    • 8% unknown
    • 1% sarcoidosis
    • 0.5% Sheehan’s syndrome
  • Infiltrative Lesions
    • Hereditary Hemochromatosis
      • Fe deposition in pituitary
      • Gonadotropin deficiency most common
      • Tx repeat phlebotomy
    • Pituitary Apoplexy
      • Sudden hemorrhage into pituitary
      • Severe, sudden HA; diplopia; hypopituitarism
      • Sudden ACTH def. is life-threatening hypotension
      • Tx: surgical decompression
  • Sheehan Syndrome
    • Infarction of Pituitary after substantial blood loss during childbirth
    • Incidence: 3.6%
    • No correlation between severity of hemorrage and symptoms
    • Severe: recognized days to weeks PP
      • Lethargy, anorexia, weight loss, unable to BF
  • Sheehan’s Syndrome
    • Typically long interval between obstetric event and diagnosis
    • Of 25 cases studied:
      • 50% permanent amenorrhea
      • The rest had scanty-rare menses
      • Most lactation was poor to absent
    • Dx: MRI empty sella turcica
  • Sheehan’s and Pregnancy
    • TX with hormones
    • 87% live births
    • 13% SAB
    • 0 Stillbirths
    • 0 Maternal deaths
    • Don’t TX
    • 58% live births
    • 42% SAB
    • 1 Stillbirth
    • 3 Maternal deaths
    Labor : HYDRATION!! IV Cortisol: adjusted for pt’s state 25-75mg q6 hr
  • Lymphocytic Hypophysitis
    • 22 y/o female died of circulatory collapse 8 hours after appy. She was 14 mos. PP and had developed 2 nd amenorrhea.
    • Autopsy: lymphocytic infiltration of pituitary and thyroid
    • Symptoms: HA, lethargy, weight loss, hyperprolactinemia
  • Lymphocytic Hypophysis
    • Scheithauer et al, ’90
      • 69 women that died during preg or PP
      • 5 had the disease, 4/5 died at 38-41 wga
    • Consider especially if no hemorrhage
    • TX: HRT (thyroid, cortisol)
  • Pituitary Necrosis
    • Pregnant Diabetic Patients
      • Due to vascular changes
    • DX: severe, midline HA and vomitting in 3 rd trimester followed by decrease of insulin requirements
    • 3/8 cases reported: assoc. with fetal and then maternal death
  • Central Diabetes Insipidus
    • Polydipsia and Polyuria (2-15 Liters/day)
    • Abrupt onset
    • 30-50% are idiopathic
      • Dec. production by
      • hypothalamus
    • Surgery or Trauma
    • Rare with Sheehan’s
      • Mild, undetectable degree
    Hypothalamus Pituitary Kidney
  • Dx of Central DI
    • Water Deprivation test:
      • Restrict p.o. fluids or administer hypertonic saline to increase serum osmolality to 295-300 mosmol/kg (nml: 275-290)
      • Central DI: urine osmolality still low and returns to normal after administer vasopressin
      • Nephrogenic DI: exogenous vasopressin does not alter urine osmolality much
  • Pregnancy and Central D.I.
    • Transient D.I. during pregnancy due to acquired or hereditary D.I.
      • Latent: Unable to sustain during pregnancy
    • Transient Arginine Vasopressin resistant, but L-Deamino, 8-D-arginine vasopressin (DDAVP=Desmopressin) responsive
      • High amounts of placental vasopressinase
    • D.I. antedates pregnancy. Most deteriorate due to vasopressinase
  • Treatment of Central D.I.
    • DDAVP (Desmopressin Acetate)
      • Synthetic analog
      • Not catabolized by vasopressinase
      • No vasopressor action
      • Administered intranasally (rec.) or p.o.
      • Titrate 10-20ug qd or bid
      • Safe in pregnancy and breastfeeding
  • References
    • Saunders; Maternal-Fetal Medicine 5 th Edition; Chapter 51 ppg. 1083-1094.
    • Weiss, R; Refetoff, S; Thyrotropin Secreting Pituitary Adenomas ; Up To Date online Jan. 2005; www.uptodate.com
    • Synder,P.; Clinical Manifestations and diagnosis of gonadotroph and other clinically nonfunctioning adenomas ; Up To Date online; Jan. 2005; www.uptodate.com
    • Barker,F; Klibanski,A; Swearingin,B; Transsphenoidal Surgery for Pituitary Tumors in the United States, 1996-2000: Mortality, Morbidity, and the Effects of Hospital and Surgeon Volume; Journal of Clinical Endocrinology and Metabolism Vol. 88, No. 10, ppg. 4709-4719.
    • Nieman, L; Orth, D; Clinical manifestations of Cushing’s Syndrome; Up To Date online; Jan. 2005; www.uptodate.com
    • Nieman, L; Orth, D; Treatment of Cushing’s Syndrome: Diminishing adrenal cortisol synthesis. Up To Date online; Jan. 2005; www.uptodate.com
    • Synder, P; Abrahamson, M; Management of lactotroph adenoma (prolactinoma) during pregnancy; Up To Date online; Jan. 2005; www.uptodate.com
    • Melmed, S; Treatment of Acromegaly; Up To Date online; Jan. 2005; www.uptodate.com
    • Melmed, S; Clinical manifestations of acromegaly; Up To Date online; Jan. 2005; www.uptodate.com
    • Synder, P; Treatment of Hypopituitarism; Up To Date online; Jan. 2005; www.uptodate.com
    • Abrahamson, M; Synder, P; Causes of hypopituitarism; Up To Date online; Jan. 2005; www.uptodate.com
    • Garner, P. Pituitary Disorders of Pregnancy; Endotext.com; Chapter 2A; March 2002.
    • Rose, B.; Causes of Central Diabetes Insipidous; Up To Date online; Jan. 2005; www.uptodate.com
    • Rose, B.; Treatment of Central Diabetes Insipidous; Up To Date online; Jan. 2005; www.uptodate.com
    • Rose, B; Diagnosis of polyuria and Diabetes insipidus; Up To Date online; Jan. 2005; www.uptodate.com
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