Transcript Parathyroid-2002-03

Parathyroid Disease
Frederick S. Rosen, MD
Anna M. Pou, MD
March 2002
Calcium Regulation
• 99% of body calcium in skeleton
• Miscible Pool: 40% bound to protein, 13%
complexed w/ anions, 47% free ionized
• PTH: Increased Ca, Decreased PO4, Increased
Vitamin D
• Vitamin D: Increased Ca, Increased PO4,
Decreased PTH (slow)
• Kidney, Bones, GI Tract
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Hyperparathyroidism
• 85% solitary adenoma
• 15% hyperplasia or
multiple adenomata
• <1% parathyroid
carcinoma
•Primary HyperPTH: Most
common; postmenopausal
women
•Secondary HyperPTH:
Usually renal failure
•Tertiary HyperPTH: Chronic
Renal Failure; low or normal
Ca, irrepressible PTH
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Hyperparathyroidism
• Usually asymptomatic
• Fatigue and weakness – up to ½ resolve
• Bone and joint pain, stones and hematuria
(Reflect decreased bone density &
nephrolithiasis)
• Osteitis Fibrosa Cystica (Brown tumor) and
Nephrocalcinosis rare
• Calciphylaxis
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Calciphylaxis
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Hyperparathyroidism: Signs &
Symptoms
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Ca=12 mg/dL-14 mg/dL
Constitutional: fatigue, wt loss, anorexia
Musculoskeletal: pain, weakness
Renal: colic, hematuria
GI: Pancreatitis, constipation, PUD, nausea
Neuro: H/A, memory loss, psychosis, insomnia
Skin: pruritus, brittle nails
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Hyperparathyroidism
•Slow progression
•75% of asymptomatic patients remain symptom
free
•Risk factors: XRT, chronic Lasix, chronic
lithium, family h/o MEN
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Hyperparathyroidism: Diagnosis
• Elevated serum Ca X 3
• Elevated PTH
• Other:
– Albumin
– Alkaline Phosphatase
– Phosphorous
– BUN/Cr
– 24-hour urine Ca
– Bone Mineral Density
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Localization
•4 glands in 87% of
patients; range 2-6
glands
•Internal carotid
artery to AP window
•Superior parathyroid
glands within 1 cm of
RLN piercing
cricothyroid
membrane
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Localization
• Location of Ectopic glands:
• Paraesophageal (28%)
• Mediastinum (26%)
• Intrathymic (24%)
• Intrathyroidal (11%)
• Carotid sheath (9%)
• High cervical (2%)
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Localization
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Ultrasound
CT
FNA
MRI
Angiography w/ or w/o selective venous
sampling (Angioablation)
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Localization: Sestamibi
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1989: Cardiac imaging, Technetium derivative
SPECT imaging
False Positives: Thyroid nodules
False Negatives: Small adenomas, hyperplasia
Cheap
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Localization: Sestamibi
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Medical Management
• Q6months
– Ca
– Cr
– U/A
– PTH
•Q12months
–Bone Density
Oral Calcium < 1 g/day
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Medical Management
• Severe Hypercalcemia:
– Saline-furosemide diuresis
– Bisphosphonates (onset of action 24-48h)
– Calcitonin (immediate onset)
– Hemodialysis
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Medical Management
• Estrogen in post-menopausal women
• Chronic Renal Failure (Hypo-Vitamin D,
Hyperphosphatemia, HyperPTH)
– Calcium salts
– Vitamin D or analogs
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Surgical Management
• 95% Success Rate
• 50-85% in renal
failure
• 1% morbidity
• Benign clinical
course of
hyperparathyroidism
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Surgical Management
• NIH Guidelines (1990)
– Symptomatic HyperPTH
– Serum Ca 1-1.6 mg/dL above normal
– H/o life-threatening hypercalcemic event
– ClCr <70% of expected
– Kidney stones on radiograph
– Elevated urine Ca (>400 mg/dL)
– Z-score>2
– Patient requests surgery
– Consistent followup unlikely
– Complicating comorbid condition
– Age<50 yo
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Surgical Management
• Renal failure (Unresponsive to medical tx)
– Renal osteodystrophy/pathologic fractures
– Intractable bone pain/pruritus
– Calciphylaxis
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Surgical Management
• New Tools of the Trade: Minimally Invasive
Surgery
– Pre-operative Sestamibi
– Intraoperative rapid PTH (50%, 80%)
– Hand-held gamma probe
– Methylene blue
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Surgical Management
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Surgical Management
• Adenoma
– Unilateral vs. Bilateral Exploration
– rPTH vs. Frozen Section
• Hyperplasia/Multiple adenomata
– Subtotal – less hypocalcemia
– Subtotal w/ autotransplantation – MEN, Renal
Failure
– Total w/ Cryopreservation – up to 1 year
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Autotransplantation
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Iced saline bath
20-30 mg; 10-20 1-2 mm slices
SCM vs. Brachioradialis
Pockets marked with clips
Up to 50% failure rate
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Surgical Complications
• Failure: missed ectopic adenoma, incomplete
resection in multi-gland dz.
• Hypocalcemia (20-30%)
– Wait for appearance of symptoms
• TVC paralysis (<1%)
• Hematoma
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Multiple Endocrine Neoplasia
• MEN I
– Moderate-severe hyperPTH in 85%
– Zollinger-Ellison, prolactinomas
– Auto Dominant, MEN1(tumor suppressor),
Chromosome 11
• MEN IIa
– Mild hyperPTH in 70%
– Medullary Carcinoma 100%
– Pheochromocytoma
– Auto Dominant, RET proto-oncogene
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Hyperparathyroidism: Miscellany
• Parathyroid carcinoma
– Hi PTH, palpable neck mass, Hi Ca post-op
– Regional/distant mets in 25-30%, Local recurrence
30%
– Surgery: Ipsilateral thyroid lobe, skeletonization of
RLN, paratracheal nodes
• Hyperparathyroidism-Jaw Tumor Syndrome
– Severe hypercalcemia in teenager
– Multiple parathyroid adenomas; 10% parathyroid
carcinoma
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Hypocalcemia/Hypoparathyroidism
• Neuro: Paresthesias, fasciculations, muscle
spasm, tetany, irritability, movement disorder,
SEIZURE, psychosis
• Visual: Cataracts, optic neuritis, papilledema
• Pulmonary: Bronchospasm
• CV: Prolonged QT, CHF, Hypotension
• GI: Dysphagia, abdominal pain, biliary colic
• GU: Preterm labor
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Hypocalcemia/Hypoparathyroidism
• Acute Hypocalcemia: Parathyroidectomy
– PTH undetectable at 8 hours
– Normalizes by 30 hours
– Calcium nadir at 20 hours, normal Day 2-3
– Hungry bone syndrome – Old, Labs
• Medications: I-131, Cimetidine, ETOH, Cisplatin,
Digoxin, Ampho-B
– Hypomagnesemia
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Hereditary Hypoparathyroidism
• DiGeorge’s syndrome and velocardiofacial
syndrome – agenesis
• Pseudohypoparathyroidism – peripheral
resistance to PTH
– Short stature, round facies, obesity, mild
MR, dental abscesses, short digits, thickened
calvaria, ectopic calcification
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Hypoparathyroidism: Treatment
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Hypoparathyroidism: Treatment
• Acute severe hypocalcemia
– Check ionized Ca
– 100-300 mg (10-30 ml) 10% Ca-gluconate in 150 cc
D5W over 10 minutes
– Continuous infusion at 0.5 mg/kg/hr
– EKG monitoring
– Check Magnesium
– Check Phosphorous
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Conclusion: Impress the
Endocrinologist
• How big is your Chvostek’s? Grade 1-4
• You say Chvostek’s, I say Weiss
• Su-Su-Sudio:
Pseudopseudohypoparathyroidism
• Clean-up: McLean Hastings Nomogram
(Zaloga 1985)
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