Endocrine Flashcards
MEN 1
Wermer’s Syndrome
Deletion 11q12-13
3 Ps
Parathyroid hyperplasia (90%) Pancreatic (and duodenal) islet cell tumors (50%) Pituitary adenomas (25%) - prolactinoma = #1
MEN 2A
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%)
MEN 2B
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
Tx for MEN syndromes
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
Thyroid development
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
Thyroid anatomical relationships
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
Lymphatic drainage of thyroid
To IJ nodes
Intraglandular lymphatics connect both lobes, explaining the high frequency of multifocal tumors in thyroid
Vasculature of thyroid
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
Innervation of thyroid
***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
Clinical importance of recurrent laryngeal nerve
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
TSH function
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
Assessment and function of thyroid hormones
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)
How much hormone do thyroid follicles store?
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
Congenital anomalies of thyroid
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
Causes of hyperthyroidism
1) Grave’s
2) Toxic nodular goiter
3) Toxic thyroid adenoma
4) Functional metastatic thyroid cancer
5) Struma ovarii (abnormal thyroid tissue in ovary)
Grave’s disease def
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
Signs of Graves
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
Dx of Graves
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
Tx of Graves
Antithyroid drugs
Radioiodide ablation with I-131
Subtotal or total thyroidectomy
Choosing a Tx for Graves
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
Risks of thyroid surgery
Recurrent laryngeal nerve injury
Hypoparathyroidism
Persistent hyperthyroidism (with subtotal thyroidectomy)
Toxic nodular goiter
“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
Thyroid storm (thyrotoxicosis)
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
Causes of hypothyroidism
Autoimmune thyroiditis
Iatrogenic: s/p thyroidectomy, s/p radioablation, 2/2 antithyroid meds
Iodine deficiency