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Surgery > Endocrine > Flashcards

Endocrine Flashcards

1
Q

MEN 1

A

Wermer’s Syndrome

Deletion 11q12-13

3 Ps

Parathyroid hyperplasia (90%)
Pancreatic (and duodenal) islet cell tumors (50%)
Pituitary adenomas (25%) - prolactinoma = #1
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2
Q

MEN 2A

A

Sipple’s Syndrome

RET oncogene mutation on chrom 10q11.2

Missense mutations on chrom 1

Medullary thyroid carcinoma (100%) - 20% of all medullary cancers are from MEN

Pheo (33%) - usually b/l

Parathyroid hyperplasia (50%)

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3
Q

MEN 2B

A

Mucosal neuroma may be earliest sign (100%) - hypertrophied lips, thickened eyelids

Medullary thyroid carcinoma (85%)
Pheo (50%)
Marfanoid habitus - skeletal abnormalities of spine (kyphosis), pectus excavatum

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4
Q

Tx for MEN syndromes

A

Perform subtotal or total parathyrodectomy with autotransplantation for parathyroid hyperplasia (MEN 1 and MEN 2A)

Perform total thyroidectomy for medullary thyroid cancer (MEN 2). May require nodal dissection if palpable nodes present

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5
Q

Thyroid development

A

Thyroid develops at base of tongue btw first pair of pharyngeal pouches, in area called foramen cecum

Thyroid gland descends down midline to its final location and develops bilobed organ with isthmus btw lobes

Remains connected to floor of pharynx via thyroglossal duct, which obliterates around month 2. May fail to go away and form a cyst or fistula instead. Usually in kids and should be removed with surg

A pyramidal lobe can be seen in 50-80% of population and represents remnant of distal thyroglossal tract. It extends superiorly from isthmus

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6
Q

Thyroid anatomical relationships

A

Anterior = strap muscles (sternohyoid, sternothyroid, thyrohyoid, omohyoid)

Posterior = trachea

Posterolateral = common carotids, IJ veins, vagus nerves

Parathyroid glands on posterior surface of thyroid and may be IN the capsule

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7
Q

Lymphatic drainage of thyroid

A

To IJ nodes

Intraglandular lymphatics connect both lobes, explaining the high frequency of multifocal tumors in thyroid

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8
Q

Vasculature of thyroid

A

Arteries:

1) Superior thyroid (each side)
- first branch of external carotid at level of carotid bifurc

2) Inferior thyroid (each side)
- from thyrocervical trunk of subclavian artery

3) Ima (sometimes)
- from aortic arch or innominate artery

Venous:

1) Superior thyroid vein (each side)
- drains into IJ

2) Middle thyroid vein (each side)
- Drains into IJ

3) Inferior thyroid vein (each side)
- drains to brachiocephalic vein

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9
Q

Innervation of thyroid

A

***1) R recurrent laryngeal nerve branches from R vagus nerve under R subclavian artery, and ascends (posterior to thyroid) to the larynx btw the trachea and esophagus

L recurrent larygneal nerve branches from L vagus, loops under aortic arch and ascends along tracheoesophageal groove to larynx.

Both innervate muscles of the true vocal cords

2) Sympathetic - superior and middle cervical sympathetic ganglia
3) Parasympathetic - from vagus nerves via branches of laryngeal nerves

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10
Q

Clinical importance of recurrent laryngeal nerve

A

It innervates all the intrinsic muscles of larynx except the cricothyroid (superior laryngeal nerve) and provides sensory innervation to mucous membranes below the vocal cords

It can be damaged during thyroid surgery so surgeon needs to know its course.

Damage produces ipsilateral vocal cord paralysis and results in hoarseness or sometimes SOB due to narrowed airway

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11
Q

TSH function

A

Causes increased formation of TH

Release of TH into circ within 30mins

Increased TH level in blood then feeds back to pit and causes decreased TSH secretion

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12
Q

Assessment and function of thyroid hormones

A

If T4 production goes up, both total T4 and free T4 go up

If T4 production drops, total and free T4 drop

If amount of thyroid-binding globulin changes, only the free T4 changes (not the total)

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13
Q

How much hormone do thyroid follicles store?

A

enough to last 2-3 months

Thus, there is no need to worry about postop hypothyroid patient who is NPO…they can resume taking their synthroid when they begin PO diet

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14
Q

Congenital anomalies of thyroid

A

Persistent sinus tract remnant of developing gland = thyroglossal cyst - may occur anywhere along course as a midline structure with thyroid epithelium, usually between isthmus and hyoid bone

  • # 1 congenital anomaly
  • few symptoms but may be infected
  • Easier to see when tongue is sticking out
  • surgical treatment - excise the duct remnat and central part of hyoid bone (Sistrunk’s operation)

Complete failure to develop

Incomplete descent = lingual thyroid or subhyoid position (if gland enlarges, patient will have earlier respiratory symptoms)
- before surgery to remove it make sure patient has other functioning thyroid tissue (70% of lingual thyroids are the only functioning thyroid)

Excessive descent = substernal thyroid

Malformation of branchial pouch

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15
Q

Causes of hyperthyroidism

A

1) Grave’s
2) Toxic nodular goiter
3) Toxic thyroid adenoma
4) Functional metastatic thyroid cancer
5) Struma ovarii (abnormal thyroid tissue in ovary)

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16
Q

Grave’s disease def

A

1 cause of hyperthyroidism in USA

Autoimmune that causes excess of TH to be produced due to presence of thyroid-stimulating immunoglobulins that stimulate production of TSH

2% of american women; 6x more common in women

Onset 20-40

Families with Graves have higher risk of other AI conditions (diabetes, Addison’s) and other thyroid disorders too

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17
Q

Signs of Graves

A

Nervousness, increased sweating, tachy, goiter, pretibial myxedema, tremor (90%)

Heat intolerance, palpitation, AFib refractory to treatment, weight loss, fatigue, dyspnea, weakness, increased appetite, exophthalmos, thyroid bruit (50-90%)

Amenorrhea, low libidio and fertility

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18
Q

Dx of Graves

A

TFTs - high T3 and/or T4 and low TSH (neg feedback of high hormone levels)

Radioactive iodide uptake test (RAIU) - scan shows diffusely increased uptake

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19
Q

Tx of Graves

A

Antithyroid drugs

Radioiodide ablation with I-131

Subtotal or total thyroidectomy

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20
Q

Choosing a Tx for Graves

A

1) Consider age, severity, size, surg risk, treatment side effects and comorbidities

2) Radioablation is #1 choice
- indicated for small or medium goiters if med therapy has failed, or if other options are contraindicated
- Most patients become euthyroid within 2 months
- Most ultimately require thyroid hormone replacement (Levo)
- Complications include exacerbation of thyroid storm initially
- Contraindicated in pregnancy, women of childbearing age and newborns

3) Surgery is indicated when radioablation is contraindicated or if medical management cannot be used
- Patients should be euthyroid prior to excision
- advantage over radioablation is immediate cure

4) Medical therapy
- B blockers for symptomatic relief
- Antithyroid drugs (PTU, methimazole) inhibit hormone production and peripheral conversion of T4 to T3
- KI reduces hormone production, used to shrink gland prior to surgery
- High recurrence rate with medical tx
- may cause side effects like rash, fever, peripheral neuritis
- Patients relapse if meds are D/C’d
- Check TFTs after any treatment

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21
Q

Risks of thyroid surgery

A

Recurrent laryngeal nerve injury

Hypoparathyroidism

Persistent hyperthyroidism (with subtotal thyroidectomy)

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22
Q

Toxic nodular goiter

A

“Plummer’s Disease”

Causes hyperthyroidism but without the extrathyroidal symptoms

Treatment is surgical since medical therapy and radioablation has a high failure rate

Solitary nodule = lobectomy

Multinodular goiter = subtotal thyroidectomy

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23
Q

Thyroid storm (thyrotoxicosis)

A

Life-threatening extreme exacerbation of hyperthyroidism precipitated by surgery on an inadequately prepared patient (incomplete B blockade and noneuthyroid patient), infections, labor, iodide administration or recent radioablation

Fever, tachy, muscle stiffness, disorientation/AMS

50% with thyroid storm develop CHF

20-40% mortality

Best way to treat is by avoiding it. ppx = achieving euthyroid state preop

Tx = fluids, antithyroid meds, B-block, corticosteroids, sodium iodide or Lugol’s solution (KI) and a cooling blanket

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24
Q

Causes of hypothyroidism

A

Autoimmune thyroiditis

Iatrogenic: s/p thyroidectomy, s/p radioablation, 2/2 antithyroid meds

Iodine deficiency

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25
Q

Signs of hypothyroidism

A

Infants/peds: down’s like facies, failure to thrive, mental retardation immediate tx with thyroid hormone will minimize neuro and intellectual effects

Adolescents/Adults: (particularly when due to AI)

  • 80% female
  • brady, low CO, hypotension, SOB 2/2 effusions
  • fatigue, weight gain, cold intolerance, constipation, menorrhagia, low libido and fertility

Less common = yellowish skin, hair loss, tongue enlargement

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26
Q

Dx of Tx of hypothyroidism

A

H&P

Low T4, T3

High TSH if primary

Low TSH if secondary

Confirm with TRH challenge - TSH will not respond in secondary hypo

Thyroid autoantibodies in AI

Low Hct

ECG may show low voltage or flat/inverted T waves

Tx = thyroxine PO or IV emergently if patient presents in myxedema coma

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27
Q

Thyroiditis (Acute)

A

Infectious etiology = strep pyogenes, staph aureus, pneumococcus (usually via lymphatics from local infection)

Risk = female sex, goiter, thyroglossal duct

Signs = unilateral neck pain and fever, euthyroid state, dysphagia

Tx = IV ABx and surgical drainage

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28
Q

Thyroiditis ( Subacute/de Quervain’s)

A

Post viral- URI

Risk = female

Signs = fatigue, depression, neck pain, fever, unilateral swelling of thyroid with overlying erythema, firm, and tender thyroid, transient hyperthyroidism usually preceding hypothyroid phase

Dx = made by H&P

Tx = usually self-limiting (within 6w)
= manage pain with NSAIDs

10% become permanently hypothyroid

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29
Q

Thyroiditis (Chronic/Hashimoto)

A

Autoimmune

Risk = down’s syndrome, Turner, familial Alzheimer’s, hx of radiation therapy as child

Signs = painless enlargement of thyroid, neck tightness, presence of other AI diseases

Dx = H&P + labs
- Circulating antibodies against microsomal thyroid cell, thyroid hormone, T3, T4, or TSH receptor

Path = firm, symmetrical enlargement; follicular and Hurthle cell hyperplasia; lymphocytic and plasma cell infiltrates

Tx = thyroid hormone (usually results in regression of goiter). With failure of medical tx, partial thyroidectomy is indicated

20% present with hypothyroidism at time of dx. A euthyroid state is more common

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30
Q

Riedel’s Fibrosing thyroiditis

A

Rare

Fibrosis replaces both lobes and isthmus

Risk = other fibrosing conditions like retroperitoneal fibrosis or sclerosing cholangitis

Signs = usually remain euthyroid; neck pain, possible airway compromise; firm, nontender enlarged thyroid

Dx = often bx required to rule out carcinoma or lymphoma

Path = dense, invasive fibrosis of both lobes and isthmus. May also involve adjacent structures

Tx = with airway compromise: Isthmectomy
Without: medical treatment with steroids

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31
Q

Workup of thyroid nodule

A

1) Get TSH/T4
- Low TSH, High T4? Hyperthyroid
- Normal? Euthyroid

2) If hyperthyroid:
- it’s prob a functioning adenoma and NOT cancer
- Get RAIU
- Hot = functioning adenoma (I2 ablation, surgery, medical management)
- Cold = ? (Get US guided FNA)

3) If euthyroid:
- Get US guided FNA

4) the FNA
- Benign
- ? (get RAIU, if functioning then benign. not not then cancer)
- Cancer (surgery and I2 ablation)
- Get a staging workup if cancer

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32
Q

Most common thyroid cancer

A

Papillary (80-85%) - it’s 75% of peds thyroid cancer

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33
Q

Risk factors for the dif thyroid cancers

A

Papillary = radiation

Follicular = dyshormonogenesis

Medullary = MEN II in 30-40%

Anaplastic = Prior diagnosis of well-differentiated thyroid cancer. Iodine deficiency

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34
Q

Age groups for each thyroid cancer

A

Papillary = 30-40

Follicular = 5-20

Medullary = 50-60

Anaplastic = 60-70

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35
Q

Sex ratios for each thyroid cancer (F/M)

A

papillary = 2/1

Follicular = 3/1

Medullary = 1.5/1

Anaplastic = 1.5/1

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36
Q

Signs of papillary thyroid cancer

A

Painless mass

Dysphagia

Dyspnea

Hoarseness

Euthyroid

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37
Q

Signs of follicular thyroid cancer

A

Painless mass

Rarely hyperfunctional

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38
Q

Signs of medullary thyroid cancer

A

Painful mass

Palpable LN (15-20%)

Dysphonia

Dyspnea

Hoarseness

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39
Q

Signs of Anaplastic thyroid cancer

A

Rapidly enlarging neck mass (large mass at presentation)

Neck pain

Dysphagia

Hard, fixed LN (50%)

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40
Q

Diagnosis of each thyroid cancer

A

Papillary = FNA, CT/MRI to assess local invasion

Follicular = FNA, CT/MRI to assess local invasion

Medullary = FNA, presence of amyloid is diagnostic. Check for calcitonin

Anaplastic = FNA

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41
Q

Mets for each thyroid cancer

A

Papillary = lymphatic (5% at time of prez)

Follicular = hematogenous

Medullary = lymphatic (local neck and mediastinal nodes). Local (into trachea and esophagus)

Anaplastic = Aggressive local disease. 30-50% have synchronous pulmonary mets at time of dx

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42
Q

Tx of papillary thyroid

A

1) Minimal cancer ( 1.5cm
3) For + LN, modified radical neck dissection
4) I-131 ablation or thyroid suppression (with thyroid hormone) for patients with residual thyroid tissue or LN mets

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43
Q

Tx for follicular thyroid

A

1) Minimal cancer ( 4cm
3) For +LN, modified radical neck dissection
4) I-131 ablation for patients with residual thyroid tissue or LN mets

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44
Q

Tx for medullary thyroid

A

1) Sporadic (80%): total thyroidectomy
2) Familial (20%): Total thyroidectomy and central neck node dissection
3) No value for I-131 ablation
4) Follow patients with calcitonin levels

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45
Q

Tx for anaplastic thyroid

A

1) Debulking resection of thyroid gland and adjacent structures
2) XRT
3) Doxorubicin-based chemo

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46
Q

Prognosis of papillary thyroid

A

worse for older patients and those with distant mets

Presence of +LN not strongly correlated with overall survival

10yr = 74-93%

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47
Q

Prognosis of follicular thyroid

A

Worse for older patients, distant mets, tumor > 4cm, high grade tumors

Presence of +LN not strongly linked with survival

10yr = 60-80%

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48
Q

Prognosis of medullary thyroid

A

10yr = 70-80%

49
Q

Prognosis for anaplastic thyroid

A

Poor

Median = 4-5months

50
Q

Position of parathyroids

A

Superior glands always next to superior lobes of thyroid (from 4th pharyngeal pouch)

Inferior glands more variable (posterior/lateral to thyroid and below inf thyroid artery) - from 3rd pouch

Not uncommon to have a “missing” inferior gland

Most common location for inferiors = thymus. Others could be intravagal, groove in carotid sheath

51
Q

Histology of parathyroids

A

Chief cells - produce PTH

52
Q

Function of parathyroids

A

Regulate calcium and P metabolism by PTH, Vit D, and calcitonin

Primary organ systems involved = GI, bone, kidney

53
Q

PTH

A

Serum calcium levels regulate secretion of cleaved PTH by negative feedback

1) Bone
- stimulates osteoclasts (more bone resorption)
- Inhibits osteoblasts (less bone production)
- This causes release of Ca and P

2) Kidney
- More reabsorption of Ca
- More P excretion

3) GI
- Stimulates hydroxylation of 25-OH D to 1,25 OH D
- 1,25 OH D increases intestinal absorption of dietary calcium (from duodenum mainly) and P
- Promotes mineralization
- Enhances PTH’s effect on bone

54
Q

Hypercalcemic crisis

A

Calcium > 13 and symptomatic

Treat with saline, furosemide, bisphosphonates, and if needed antiarrhythmic agents

55
Q

Calcitonin

A

Secreted by thyroid C cells

Inhibits bone resorption (inhibits calcium release)

Increases urinary excretion of Ca and P

Works as counterregulatory hormone to PTH

56
Q

Hyperparathyroidism (Primary) definition

A

Due to overproduction of PTH, causing increased absorption of calcum from intestines, increased vit d3 production, and decreased renal calcium excretion. This raises overall serum Ca and lowers P

57
Q

Hyperpara (primary) incidence and risk

A

1/4000

MEN I
MEN IIA
history of radiation

58
Q

Signs of hyperpara (primary)

A

Stones, bones, groans, moans

Kidney stones

Bone pain, pathologic fractures, subperiosteal resorption

Groans: nausea, vomit, muscle pain, constipation, pancreatitis

Moans: lethargy, confusion, depression, paranoia

59
Q

Causes of primary hyperpara

A

Solitary adenoma (85-90%)

4-gland hyperplasia (10%)

Cancer (

60
Q

Workup and dx for primary hyperpara

A

Preop imaging = US, sestamibi scan, CT/MRI, operative exploration

Dx:
1) Elevation of PTH with inappropriately high serum Ca (normally a high Ca should lower PTH)

2) Check urine for calcium to rule out diagnos of familial hypocalciuric hypercalcemia (will be low if FHH, high if primary hyperpara)

61
Q

Tx for primary hyperpara

A

1) Solitary adenoma - solitary parathyroidectomy with neck exploration to ID +/- biopsy 3 remaining glands. If preop sestamibi is done to localize area, no need for neck exploration
2) Multiple gland hyperplasia - remove 3 glands, or all 4 with reimplantation of at least 30g of parathyroid tissue in forearm or other site to retain function (makes it easier to resect additional parathyroid gland if hyperparathyroid state persists)

Outcomes:
First op = 90% success rate
Reoperation = 90% success rate if remaining gland is localized preop

62
Q

Secondary hyperpara info

A

Increased PTH due to hypocalcemia that is the result of chronic renal failure (Phosphate retained leads to low calcium), GI malabsorption, osteomalacia, or rickets

63
Q

Signs of secondary hyperpara

A

Bone pain from renal osteodystrophy and pruritis

Patients often asymptomatic

64
Q

What other conditions should you consider in a patient with hypercalcemia?

A

R/O malignancy

ESP colon, lung, breast, prostate, head, neck, MM

Some tumors secrete PTHrP

65
Q

Dx of secondary hyperpara

A

Made by labs in asymptomatic patients

usually due to 4 gland hyperplasia

66
Q

Tx of secondary hyperpara

A

1) Nonsurgical - In renal failure patients, correct Ca and P.
- Restrict P intake, treat with P-binders and Ca/Vit D supplements. Adjust dialysate to maximize Ca and minimize aluminum

2) Surgical - indicated for intractable bone pain or pruritis, or pathologic fractures, with failure of medical therapy
- No role for parathyroid surg in secondary hyperpara
- Maybe renal transplant

67
Q

Tertiary hyperpara

A

Due to persistent hyperparathyroidism after treatment for secondary hyperpara.

Due to autonomously functioning parathyroid glands that are resistant to negative feedback from high Ca levels. Usually s/p renal transplant

Usually a short-lived phenom

If persistent, surgery is indicated (3.5 gland resection)

68
Q

Familial hyperpara

A

MEN 1/2A

High recurrence rate

Total resection with forearm reimplantation is indicated to facilitate potential reoperation if HPTH persists

Patients with sporadic 4 gland hyperplasia may undergo total resection with reimplantation or 3 gland excision

69
Q

Hypopara etiologies

A

Uncommon

Usually from surgery: Following total thyroidectomy, usually transient and treated if symptoms develop

Could be congenital absence of all 4 glands

Could be DiGeorge - no parathyroid and no thymus

Could be functional - chronic hypoMg

70
Q

Hypo para signs

A

Numbness and tingling of circumoral area, fingers, toes

Anxiety, confusion

may progress to tetany, hyperventilation, seizures, heart block

71
Q

Tx for hypo para

A

Supplement with PO Ca and Vit D (to help GI absorption)

Pseudohypoparathyroidism = familial disease causing resistance of PTH at target tissue. Patient remain hypoCa and hyperP despite bone resorption from elevated PTH. Tx = Ca and Vit Supplements

72
Q

Signs of parathyroid cancer

A

40-50% present with firm, fixed mass that is palpable

Extremely high Ca and PTH. Usually has high levels of hCG (tumor marker)

Neck pain, voice change (due to lesion in recurrent laryngeal nerve)

73
Q

Tx for parathyroid cancer

A

En bloc surg resection of mass and surrounding structures, along with i/l thyroid lobectomy and i/l LN dissection

Postop XRT and chemo not usually helpful

Post op complications = recurrent laryngeal nerve damage, severe hypocalcemia (hungry bone syndrome)

5y survival = 70%

74
Q

Chvostek sign

A

HypoCa

Contraction of facial muscles when tapping on facial nerve

75
Q

Trousseau sign

A

HypoCa

Development of carpal spasm by occluding blood flow to forearm

76
Q

Adrenal anatomy

A

Retroperitoneal

T11

Branches of aorta, inferior phrenic and renal arteries

Venous = R side drains to IVC. L drains to L renal vein

77
Q

Histo of adrenal glands

A

Cortex

Glomerulosa: aldosterone (salt)

Fasiculate: Steroids, cortisol (sugar)

Reticularis: Androgens/estrogen (sex)

78
Q

Adrenal adenoma

A

Most are unilateral

If adrenal gland 6cm, surgically resect due to increased risk of adrenocortical carcinoma

79
Q

Adrenal cortical carcinoma signs

A

Very rare

Women more than men, peak

80
Q

Dx of Adrenal Cortical Carcinoma

A

24h urine for cortisol, aldosterone, catecholamines, metanephrine, VMA, 17-OH corticosteroids, 17-ketosteroids

CT (lesions > 7mm) or MRI (esp to assess IVC invasion)

CXR to r/o pulm mets

81
Q

Tx for Adrenal Cortical Carcinoma

A

1) Radical en block but only 1/3 of adrenal carcinomas are operable
2) If resection not done, debulk to reduce amount of cortisol-releasing tissue
3) Bone mets should be palliated with XRT
4) No role for chemo
5) Monitor steroid hormone levels postop
6) Recurrence also warrants resection

7) recurrence: lungs, LN, liver, peritoneum, bone
- in 10% of b/l cases, patients develop Nelson’s syndome (excess ACTH from pit adenoma. This causes visual issues due to mass effect, hyperpigmentation (more melanocyte-stimulating hormome) and amenorrhea as well)

82
Q

Prognosis for ACC

A

70% present in stage 3 or 4

5yr = 40% for complete resection

If local invasion, median = 2-3y

83
Q

Workup for Cushing’s Syndrome

A

Low THen High

1) 24h urine cortisol elevated then Low dose (single dose) dexa suppression

If this fails, we have Cushing’s syndrome (too much cortisol being made - we need to find where it’s coming from)

Is this cortisol driven (ACTH independent) or driven by ACTH?

2) get ACTH level

If normal/not high then cortisol itself is driving it.

This is a primary adrenal tumor

Get CT to find it then resect

OR they’re eating cortisol

If ACTH is high then it’s ACTH driven

3) Get High Dose (8mg) Dexa suppression (If ACTH was high)

If it suppresses cortisol then it’s Cushing’s Disease (Ant pit issue. MRI and resect)

If it fails then it’s ectopic ACTH - CT or PET everything to find it

The tests do steroid dose at 11PM and measurement at 8AM (low dose)

84
Q

What is the most common cause of ectopic ACTH production?

A

1 = Small cell lung cancer (oat cell carcinoma)

85
Q

Addison’s Disease def

A

Adrenal insufficiency

Primary = Due to destruction of adrenal cortex with sparing of medulla

Secondary = Failure due to hypothalamic or pituitary abnormalities

86
Q

Causes of Primary Addison’s Disease

A

1) Post-adrenalectomy
2) AI
3) TB
4) Fungal infx
5) AIDS
6) metastatic cancer
7) Familial glucocorticoid deficiency

87
Q

Causes of secondary Addison’s

A

1) Exogenous steroids (#1 - longterm for medical use or for illicit use)
2) Craniopharyngioma
3) Pituitary surgery or irradiation
4) Empty sella syndrome

Abruptly stopping longterm steroid use may cause adrenal insufficiency too. 6 months may be required for intrinsic controls to come back online

88
Q

Addisonian crisis

A

Acute situation due to some extrinsic stressor like infection or surgery

89
Q

Signs of Addison’s

A

Nause, vomiting

Abdominal pain

Tachy

Weight loss

Fatigue

Lethargy

Hyperpigmentation (low levels of cortisol cause increased pit production of proopiomelanocortin - POMC - which is a precursor to ACTH and MSH)

Fever and hypovolemic shock in crisis

90
Q

Dx of Addison’s

A

1) HypoNa, HyperK (due to low aldosterone which is normally produced by adrenals)
2) ACTH stimulation test - give ACTH and measure cortisol level after 30mins. If adrenal failure is present, there will be no increase in cortisol
3) Baseline ACTH level is elevated in patients with primary failure due to absence of negative feedback

91
Q

Tx of Addison’s

A

1) Glucocorticoid therapy for primary and secondary causes
2) Additional mineralocorticoid therapy for primary cause
3) Addisonian crisis - Volume (D5NS) and IV glucocorticoids

92
Q

Stress dose steroids perioperatively?

A

Patients with Addison’s OR anyone who has been on steroids for more than 6 months prior to surgery should get periop stress dose steroids

93
Q

Causes of hyperaldosteronism

A

Primary (too much aldosterone secretion) = Conn’s Syndrome

  • Aldosterone-secreting tumor (66%)
  • Idiopathic adrenocortical hyperplasia (30%)

Secondary (due to high renin leading to high aldo)

  • Renal artery stenosis
  • Cirrhosis
  • CHF
  • Normal pregnancy
94
Q

Signs of hyperaldo

A

HTN

Muscle weakness and cramping

HA

Polyuria

Polydipsia

HypoK

95
Q

Dx for hyperaldo

A

Primary/Conn’s

1) Diastolic HTN without edema
2) High plasma aldo
3) Normal or low plasma renin
4) HypoK, high urineK (off antihypertensive meds)
5) Post-captopril plasma aldo:
- normally results in low aldo
- diagnostic of hyperaldo if ratio > 50
6) Imaging
- CT picks it up if >1cm. If there is an aldosteronoma, opposite adrenal appears atrophied
- Iodocholesterol scan - picks up 90% of aldosteronomas and shows how functional they are. Hyperplasia will present as b/l hyperfunction versus unilateral for tumor
- if all imaging inconclusive, maybe sample adrenal vein for aldo and cortisol pre and post ACTH
- U/l elevation of aldo or ald/cortisol ratio indicated aldo-secreting adenoma
- B/l elevation of aldo is consistent with hyperplasia

96
Q

Caveat to eval for Conn’s

A

Make sure patient isn’t just an uncontrolled hypertensive on K wasting diuretics

97
Q

Tx for hyperaldo

A

Primary

1) Hyperplasia - medical treatment with spironolactone, nifedipine, amiloride and/or other antihypertensive. NO SURG
2) Adenoma - Lap adrenalectomy

Outcomes: most patients become normotensive and normokalemic with tx. 20-30% have recurrent HTN in 2-3 years

Secondary
1) Treat underlying cause

98
Q

Hypoaldo

A

Decreased aldo without a change in cortisol production

Causes = Congenital error of aldo synthesis, failure of glomerulosa (AI), s/p adrenalectomy, drug inhibition

Signs = postural hypotension, persistent and severe hyperK, muscle weakness, arrhythmias

Tx = mineralocorticoid replacement

99
Q

Neuroblastoma def

A

A medullary tumor of adrenals

  • Embryonal neural crest tumor mainly in children (small round blue cell tumor)
  • # 4 most common peds malignancy
  • Can occur anywhere along sympathetic chain (50% in adrenal, 25% paraspinal ganglia, 20% thorax, 5% pelvis)
  • May spontaneously differentiate and regress
  • Aggressive tumor that commonly presents with distant mets. In 50% of infants and 66% of older children (to LN, bone, liver, subQ)
100
Q

Diseases associated with neuroblastoma

A

Neurofibromatosis

Beckwith-Wiedermann Syndrome

Trisomy 18

101
Q

Signs of neuroblastoma

A

Abdominal or flank mass

Respiratory distress

SubQ blue tumor nodules (blueberry muffin sign)

102
Q

Dx of neuroblastoma

A

Imaging CT for staging. MRI

Urinary tumor markers - high 24h levels of homovanillic acid, vanillylmandelic acid and metanephrines

103
Q

Tx of neuroblastoma

A

Localized (stage 1/2) - surg resection

Nonlocalized disease (stage 2) - surg resection with chemo +/- radiation

Mets (4) - surg resection with chemo +/- radiation

LN mets warrants XRT

104
Q

prognosis of neuroblastoma

A

Mortality lower when diagnosed within first year of life

5y survival = 90% for disease confined to primary site, 20-40% for disseminated disease

105
Q

10% rule for pheocrhomocytoma

A 
10% malignant
10% familial
10% extra-adrenal (morel likely to be malignant. Often at organ of Zuckerkandl to left of aortic bifurcation at IMA)
10% bilateral (more likely familial)
10% in children
106
Q

Pheo definition

A

Chromaffin cell tumor usually in adrenal medulla (90%) but can be anywhere along sympathetic chain.

Erroneous production of Epi

107
Q

Pheo risk factors

A

MEN IIA/2B (usually cause bilateral)

VHL

Neurofibromatosis

FHx

108
Q

5 Ps of Pheo

A

Paroxysms (comes and goes - since Epi is released in pulses)

Pressure (HTN)

Pain (HA)

Palpitations (sympathetic overdrive)

Perspire

109
Q

Dx of Pheo

A

24h urine VMA and metanephrine (elevated)

110
Q

Tx of Pheo

A

Alpha block first

THEN B block

THEN resect

A before B bc we don’t want the B block to cause unopposed alpha activity which would raise BP even more

If you resect first you’ll release catecholamines everywhere and make patient stroke out and die ( for same reason, ligate veins before resection)

111
Q

parts of pit gland

A

Anterior = Adenohypophysis

Posterior = Neurohypophysis

112
Q

Sheehans signs

A

Postpartum infarction and necrosis of pit leading to hormonal failure

Pit ischemia due to hemorrhage, hypovolemic shock, pit portal venous thrombosis

Failure of lactation
Amenorrhea
Progressive decreased adrenal function and thyroid function

113
Q

Posterior pit disorders

A

SIADH and DI

114
Q

SIADH

A

15% of hospitalized patients

Impaired water secretion

Hypersecretion of ADH results in increased urinary sodium with high urine osmolality

Causes = CNS injury, cancer, trauma, drugs

115
Q

DI

A

Decreased ADH secretion

Impaired water conservation; large volumes of urine, leads to increased plasma osmolality and thirst

1/3 idiopathic
2/3 due to tumor or trauma

116
Q

Signs of pit macroadenoma

A

> 1cm

Visual loss (b/l hemianopsia), hypopituitarism, HA, hyperprolactinema due to compression of stalk

  • compression prevents dopamine from going from hypothal to post pit where it would normally inhibit prolactin production

This causes hyperprolactinemia and nipple discharge

117
Q

Signs of pit microadenoma

A

Prolactinoma (#1) = secondary amenorrhea, galactorrhea

GH - Gigantism or acromegaly depending on age; course facial features, thick finger and heel pads, cardiomegaly, hepatomegaly, enlarged mandible, increased teeth spaces, neuropathy, arthropathy, osteoporosis, HTN, DM, goiter

ACTH - Cushings

118
Q

Complications of pit surgery

A

Death (direct hypothal injury

119
Q

Treatment for pit adenomas

A

PreOp: complete endocrine assessment, lytes to look for borderline DI

Surg:

  • Transphenoidal vs transcranial vs perioperative
  • PostOp XRT for large lesions

Primary Radiation:
- consider when surg contraindicated for other reasons in nonfunctioning tumor as primary therapy may worsen preexisting hypopituitarism

Medical

  • For prolactin - Bromocriptine
  • For GH - somatostatin which shrinks tumor size in 20-50%, normalizes GH in 50%, and normalizes IGF-1 in 40-80%

Surgery (27 decks)

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