Pituitary Pathologies Simplified

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Presentation at the 2014 Philippine Society of Endocrinology & Metabolism annual convention

Presentation at the 2014 Philippine Society of Endocrinology & Metabolism annual convention

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  • 1. “Simple can be harder than complex: You have to work hard to get your thinking clean to make it simple. But it’s worth it in the end because once you get there, you can move mountains.” - Steve Jobs Friday, March 21, 14
  • 2. Iris Thiele Isip Tan MD, MSc Assoc. Prof. 4, UP College of Medicine Chief, Medical Informatics Unit GPs as Pituitary Stalkers Pituitary Pathologies Simplified David and Goliath by Robert Temple Ayres Friday, March 21, 14
  • 3. to be a PituitaryStalker How optical_illusion_tshirt by Kevin https://flic.kr/p/a573df Remember by clsawyer http://www.sxc.hu/photo/659706 Ask Remember Refer Friday, March 21, 14
  • 4. to be a PituitaryStalker How “The world is full of obvious things which nobody by any chance ever observes.” - Sherlock Holmes optical_illusion_tshirt by Kevin https://flic.kr/p/a573df Have a questioning mind! Ask Friday, March 21, 14
  • 5. David and Goliath Who was the real underdog? David and Goliath by Robert Temple Ayres Friday, March 21, 14
  • 6. Did Goliath acromegaly have ? Friday, March 21, 14
  • 7. David and Goliath Painting by Giovan Francesco Nagli Height “6 cubits and a span” (2.5-3 meters) Slow-moving and ponderous Goliath saw “sticks” Stone sunk into forehead Goliath fell forward Did Goliath acromegaly have Friday, March 21, 14
  • 8. 38/F previously healthy 3-month history of blurring of vision headache by beermug http://www.sxc.hu/photo/1413980 Friday, March 21, 14
  • 9. Optometrist or ophthalmologist? Prescribed eye glasses Progressed to dimness of vision frame on eyechart by Brybs http://www.sxc.hu/photo/1152188 Friday, March 21, 14
  • 10. Diffuse throbbing headaches followed by progressive blurring of vision Family physician? Internist? Neurologist? headache by beermug http://www.sxc.hu/photo/1413980 Friday, March 21, 14
  • 11. Menses stopped 6 mos ago followed by the headaches Gynecologist? No galactorrhea Endocrinologist? headache by beermug http://www.sxc.hu/photo/1413980 Friday, March 21, 14
  • 12. Who makes the initial diagnosis of a pituitary lesion? Diagnostic Source Total percentage (n=404)Total percentage (n=404)Total percentage (n=404)Diagnostic Source Prolactinoma Non-functioning Acromegaly Internist/Family Med 16 23 39 Endocrinologist 34 14 25 Gynecologist 34 10 <1 Ophthalmologist/ Optometrist 6 25 6 Relatives/ self 2 1 6 Neurologist 0 3 5 Dentist 0 0 5 Other 8 24 14 Drange et al JCEM 85:168–174, 2000 Friday, March 21, 14
  • 13. Pituitary adenomas come to clinical attention mass effects hormone excess hormone insufficiency Friday, March 21, 14
  • 14. to be a PituitaryStalker How optical_illusion_tshirt by Kevin https://flic.kr/p/a573df Remember by clsawyer http://www.sxc.hu/photo/659706 Ask Remember Illness script Friday, March 21, 14
  • 15. Key Elements of the Clinical Diagnostic Reasoning Process Bowen J. NEJM 2006;355:2217-2225 Patient’s story Data acquisition Accurate “problem representation” Generation of hypothesis Search for and selection of illness script Diagnosis Knowledge Context Experience Friday, March 21, 14
  • 16. Expert clinicians store and recall knowledge as diseases, conditions, or syndromes - “illness scripts” - that are connected to problem representations. These representations trigger clinical memory, permitting the related knowledge to become accessible for reasoning. A room lamp without lampshade by Mattox http://www.sxc.hu/photo/1120345 Bowen J. NEJM 2006;355:2217-2225 Friday, March 21, 14
  • 17. Illness Script - Structure Bowen J. NEJM 2006;355:2217-2225 Predisposing conditions Pathophysiological insult Clinical consequences Rare 17% of population Equal sex incidence Incidence increases with age Tumor growth Compression of optic nerve Extension to cavernous or sphenoid sinus Compression of normal pituitary Non-functioning pituitary adenoma Friday, March 21, 14
  • 18. By OpenStax College [CC-BY-3.0 (http://creativecommons.org/licenses/by/3.0)], via Wikimedia Commons andThe Pituitary Optic Chiasm Friday, March 21, 14
  • 19. Image from http://www.medicinemcq.com/index.php/journals/sub_details/138/39/FMGE-MARCH-2012-ENT-AND-OPHTHALMOLOGY andThe Pituitary Cavernous Sinus Friday, March 21, 14
  • 20. Illness Script - Structure Bowen J. NEJM 2006;355:2217-2225 Predisposing conditions Pathophysiological insult Clinical consequences Rare 17% of population Equal sex incidence Incidence increases with age Tumor growth Compression of optic nerve Extension to cavernous or sphenoid sinus Compression of normal pituitary Nonspecific headache Visual disturbances (bitemporal hemianopsia) Ophthalmoplegia, diplopia and/or ptosis CSF rhinorrhea Anterior pituitary failure (hypopituitarism) Non-functioning pituitary adenoma Friday, March 21, 14
  • 21. Image from http://www.medicinemcq.com/index.php/journals/sub_details/138/39/FMGE-MARCH-2012-ENT-AND-OPHTHALMOLOGY Bitemporal hemianopsia Friday, March 21, 14
  • 22. Illness Script - Structure Bowen J. NEJM 2006;355:2217-2225 Predisposing conditions Pathophysiological insult Clinical consequences Most common pituitary tumor F:M ratio 10:1 Neoplastic transformation of anterior pituitary lactrotrophs resulting in excess synthesis and secretion of prolactin Prolactinoma Oligo- or amenorrhea Galactorrhea Erectile dysfunction or decreased libido Hypopituitarism (compression from large tumor) Friday, March 21, 14
  • 23. Hypergonadotropic hypogonadism Ovarian failure Hyper- prolactinemia galactorrhea headache amenorrhea Pituitary adenoma visual disturbances Discriminating feature Defining feature Discriminating feature Discriminating feature Defining feature Hypo- gonadotropic hypogonadism Friday, March 21, 14
  • 24. Patient presenting with secondary amenorrhea; negative pregnancy test Check TSH and prolactin levels Both normal Normal TSH Abnormal prolactin Progestogen challenge test No withdrawal bleed Estrogen/progestogen challenge test Withdrawal bleed Normogonadotropic hypogonadism No withdrawal bleed Outflow obstruction Withdrawal bleed Check FSH and LH FSH >20 IU/L LH >40 IU/L Hypergonadotropic hypogonadism FSH & LH <5 IU/L Perform MRI to evaluate for pituitary tumor Normal MRI: hypogonadotropic hypogonadism Normal prolactin Abnormal TSH Thyroid disease Prolactin <100 ng/mL (100 mcg/L) Consider other causes Prolactin >100 ng/mL Perform MRI to evaluate for prolactinoma Negative MRI Friday, March 21, 14
  • 25. What happens when you can’t match an illness script? 59/F with dizziness, vomiting and weakness ... Rounadabout by ColinBroug http://www.sxc.hu/photo/1265027 Friday, March 21, 14
  • 26. Third time admitted for vomiting, dizziness and general weakness Previous admissions also with hypoglycemia and pneumonia or UTI Normal endoscopy end to end ... What to do?! Photo by Ashley Webb https://flic.kr/p/5u36oV Friday, March 21, 14
  • 27. Adrenal insufficiency?! 31 years ago Postpartum bleeding needing BT Delivered twins at home Retained placenta Screaming by ralaenin http://www.sxc.hu/photo/579286 Friday, March 21, 14
  • 28. Illness Script - Structure Bowen J. NEJM 2006;355:2217-2225 Predisposing conditions Pathophysiological insult Clinical consequences 5.1 per 100,000 women Severe postpartum vaginal bleeding Severe hypotension or shock requiring fluid replacement or blood transfusion Sheehan’s syndrome Friday, March 21, 14
  • 29. Friday, March 21, 14
  • 30. Illness Script - Structure Bowen J. NEJM 2006;355:2217-2225 Predisposing conditions Pathophysiological insult Clinical consequences 5.1 per 100,000 women (Iceland) Severe postpartum vaginal bleeding Severe hypotension or shock requiring fluid replacement or blood transfusion Pituitary enlargement Lactotroph hyperplasia Pituitary gland susceptible to ischemia Postpartum pituitary necrosis Failure of postpartum lactation Failure to resume regular menses Varying degrees of hypopituitarism Empty sella on CT scan or MRI Sheehan’s syndrome Friday, March 21, 14
  • 31. Sheehan’s Syndrome Presentation At ER following a stressful event Coma of hypothyroidism Adrenal insufficiency Hypoglycemia Hyponatremia http://www.sxc.hu/photo/65898Ozkan& Kolac, Neuroendocrinol Lett 2005;26(3):257-60 Friday, March 21, 14
  • 32. GH deficiency Reduced energy and vitality Reduced muscle mass and strength Decreased sweating and impaired thermogenesis Increased central adiposity Increased CV risk Decreased BMD Prabhakar & Shalet, Postgrad Med J 2006;82:259–66 Friday, March 21, 14
  • 33. ACTH deficiency Fatigue, weakness, anorexia, weight loss Nausea, vomiting, abdominal pain Hypoglycemia Circulatory collapse if acute onset Loss of axillary and pubic hair in women Prabhakar & Shalet, Postgrad Med J 2006;82:259–66 Friday, March 21, 14
  • 34. Gonadotrophin deficiency Men: erectile dysfunction, soft testes, reduced muscle mass and energy Women: oligo/amenorrhea, dyspareunia, breast atrophy Both: loss of libido, flushes, infertility, regression of sexual characteristics, reduced BMD Prabhakar & Shalet, Postgrad Med J 2006;82:259–66 Friday, March 21, 14
  • 35. TSH deficiency Fatigue, apathy, psychomotor retardation Cold intolerance, dry skin Constipation Weight gain Prabhakar & Shalet, Postgrad Med J 2006;82:259–66 Friday, March 21, 14
  • 36. 45/M referred to surgeon for colon CA Referred by surgeon to me for coarse facial features http://www.cinemainsomnia.com/lobo- loves-rondo-does-rondo-love-lobo/ Friday, March 21, 14
  • 37. Illness Script - Structure Bowen J. NEJM 2006;355:2217-2225 Predisposing conditions Pathophysiological insult Clinical consequences 40-70 cases per million population Mean age of onset 30 y Onset to diagnosis 5-15 y Mean age at diagnosis: 40 y in men and 45 y in women GH excess GH-secreting pituitary adenomas Inc incidence of colonic polyps & colon adenoCA, diabetes, hypertension and CVD Soft tissue swelling & enlargement of extremities Increase in ring and/or shoe size Hyperhidrosis Coarsening of facial features Prognathism, macroglossia Acromegaly Friday, March 21, 14
  • 38. Rondo Hatton American Actor Acromegaly attributed to exposure to German mustard gas attack in World War I http://i1139.photobucket.com/albums/n545/travislouie37/Rondoisgoodwiththeladies.jpg Friday, March 21, 14
  • 39. Sheehan’s syndrome Non-functioning adenoma hypopituitarism Functioning Pituitary adenoma Discriminating feature Defining feature Discriminating feature Discriminating feature mass effects ACTH TSH Excess secretion PRL GH Hormone deficiency Postpartum hemorrhage Defining feature Friday, March 21, 14
  • 40. At rounds more than a decade ago with MALA ... How much does a human pituitary gland weigh? Seagull in front of sea by cobrasoft http://www.sxc.hu/photo/1437851 Friday, March 21, 14
  • 41. “But what physician has not had patients who don’t make any sense at all? To tell the truth, they’re our stock-in-trade. We talk and write about the ones we can make sense of.” - Walker Percy Dr. House MD Caricature Hugh Laurie by Nelson Santos https://flic.kr/p/46tXvx Friday, March 21, 14
  • 42. to be a PituitaryStalker How optical_illusion_tshirt by Kevin https://flic.kr/p/a573df Remember by clsawyer http://www.sxc.hu/photo/659706 Ask Remember Refer Friday, March 21, 14
  • 43. Thank You @endocrine_witch Friday, March 21, 14