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Current Status of Surgery For
Primary and Secondary Hyperparathyroidism Surgery Grand Rounds 20 Aug., 2014 W. G. Schenk III
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Disclosures
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HYPERPARATHYROIDISM Basic Physiology, Anatomy, Embryology
Primary Hyperpara Clinical Presentations Evolution of surgical approach and pre-op imaging Secondary Hyperpara Clinical Presentation Indications for Surgical Rx Surgical Options
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PTH and CALCIUM HOMEOSTASIS
+ PTH CALCIUM -
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PTH and CALCIUM HOMEOSTASIS
n-PTH c-PTH Renal c-AMP Bone Osteoclast GI Absorption GI, Renal : 1,25 D PTH _ Vit D3 Phos ++ Ca (Albumin) Calcitonin (?) + CALCIUM
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HYPERPARATHYROIDISM Inappropriate excess secretion of
Parathyroid Hormone in Primary Hyperparathyroidism Appropriate Hypersecretion in Secondary Hyperparathyroidism
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HYPERPARATHYROIDISM SURGICAL APPROACES Embryology 3rd Branchial Pouch: Thymus and Lower PTH’s 4th Branchial Pouch: Upper PTH’s Ectopic (5%) and supranumerary (15%)
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DIAGNOSIS OF HYPERPARATHYROIDISM
PTH Ca PO U. Ca Cl / PO Ca x PO4 > 33 Primary Secondary Tertiary FHH Syndrome Malignancy
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Differential Diagnosis of Hypercalcemia
Primary Hyperparathyroidism Malignancy Bone Metastases Paraneoplastic Medication Lithium, Vit. A,D, Thiazides Granulomatous Disease Excess intake,Dehydration, Immobilization Familial Hypocalciuric Hypercalcemia (FHH)
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Symptoms of Primary Hyperparathyroidism
Neurologic Headache, memory loss Neurosis, Psychosis Cardiovascular Hypertension Nephrologic Stones, Renal impairment Dermatologic Pruritis, brittle nails Constitutional Fatigue, Wt loss, Anorexia GI Peptic Ulcer Disease Nausea, Vomiting Musculoskeletal Bone and joint pain Muscle weakness
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Symptoms of Primary Hyperparathyroidism “ASYMPTOMATIC”
Some symptoms can usually be elicited by careful questioning. Rationale for surgical Rx in asyptomatic patient.
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PRIMARY HYPERPARATHYROIDISM
Family History MEN I (Wermer’s Syndrome) Pituitary, Panc Islet Cell MEN II (Sipple’s Syndrome) MCT, Pheo (MEN IIb) FHH (Familial Hypocalciuric Hypercalcemia)
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PRIMARY HYPERPARATHYROIDISM
Physical Exam General : BP, Neuro, Cardiac, Operative risk Neck Exam: Thyroid abnormality adenopathy, palpable mass Vocal cord function, neck mobility Occult Malignancy (Breast, Prostate)
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LABORATORY EVAL IN PRIMARY HYPER-PTH
Calcium Determination(s) Cl, Po4, Alb, Tot Protein, BUN, Creat., Alk Phos. ** Cl/Po4 ratio > 33 24-hr Urine ** Ca > 100 mg / day U-creat., Ca/Cr Clearance ratio, Cyclic AMP Chest X-ray Hand, Bone Xrays (optional) PTH Radioimmunoassay
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PTH Levels in Primary Hyperparathyroidism
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INDICATIONS FOR SURGERY IN PRIMARY HYPERPARATHYROIDISM
(No prior neck surgery) Diagnosis of Hyperparathyroidism Questionable Secure Symptomatic Asymptomatic Significant Calcium Surgical risk Prohibitive Low
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ANATOMIC CORRELATION:
HYPERPARATHYROIDISM SURGICAL APPROACES ANATOMIC CORRELATION: “Sporadic” Primary – single gland (95%) Familial Primary Secondary Multigland Hyperplasia Tertiary
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Sporadic Primary Hyperpara: Single Parathyroid Adenoma
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Hypercellular Parathyroid
800 mg
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Imaging in Primary Hyper-PTH
Rationale for Pre-op Imaging in Primary Hyper-PTH Identify ectopic and supernumerary glands Identify multiple adenomas Provide pre-op anatomic confirmation of biochemical Dx Reduce incidence of persistent/recurrent hyper-para Permit “limited exploration” Reduce complication risk
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locate an experienced neck surgeon”
“The only pre-op localization necessary is to locate an experienced neck surgeon” John Doppman NIH Concensus Conference 1980
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Evolution of Imaging Relevence
Hyperparathyroidism Evolution of Imaging Relevence Improvement in imaging technology Popularity of limited exploration Development of rapid PTH Assay Economic pressures
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Jim Norman (based on the past 15,000 PTH operations):
“Our patients get the exact same operation regardless of scan findings – we simply do not care if their scans are positive or negative” Norman J, Lopez J, Politz D; Abandoning Unilateral Parathyroidectomy; JACS Mar 2012 ; 214(3) :
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Surgeon-Performed Ultrasound Primary Hyperparathyroidism
Schenk WG, Hanks JB, Smith PW Convenience, Efficiency Surgical / Anatomic Correlation Volume of positive studies Additional relevant findings
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Surgeon-Performed Ultrasound Primary Hyperparathyroidism
Classification of pre-op Neck Ultrasound Class 1: Hi Confidence Class 2: “Possible” Class 0: None seen
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Primary Hyper PTH: Pre-Op Imaging
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Results Class 1: 111/200 (55%) 3 patients deferred surgery
107/108 Intra-op agreement 108/108 Cured
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Results Class 1: 111/200 (55%) 3 patients deferred surgery
107/108 Intra-op agreement 108/108 Cured Class 0 : 4-D CT Scan Class 2 : Surgery w or w/o imaging
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Tc-99m sestamibi scan Coronal slice and 3D reconstruction from a 4DCT
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CURRENT CONTROVERSIES: “SPORADIC” PRIMARY HYPERPARA
“Limited Exploration” vs. Complete Exploration Pre-op Imaging Intra-op PTH Assay Evolving Approaches: Minimally invasive / Robotic Regional Anesthesia Ambulatory/Outpatient
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SECONDARY HYPERPARATHYROIDISM
Appropriate PTH level (Nutritional or Renal). Renal Failure: Numerous influences on Ca homeostasis High proportion of Dialysis pts have elevated PTH. Less than ten percent should require surgical Rx.
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Effects of Renal Failure on PTH Homeostasis
Diminished renal hydroxylation of D3 Decreased GI absorption of Calcium Decreased suppression of PTH by D3 Decreased (total) serum Calcium Decreased renal excretion of phosphorus Direct effect of Hyperphosphatemia Decreased ionized Calcium Elevated “set point” of PTH responsiveness Osteoblast resistance to PTH Autonomous Hypersecretion from Hyperplasia
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DIAGNOSIS OF HYPERPARATHYROIDISM
PTH Ca PO U. Ca Cl / PO Ca x PO4 Primary Secondary Tertiary FHH Syndrome Malignancy + >55
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PTH Levels in Secondary Hyperparathyroidism
The majority of dialysis patients have some elevation in PTH. A level in the pg/ml range is common. Elevated level alone is not an indication for surgical Rx.
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SECONDARY HYPERPARATHYROIDISM Indications for Surgery
Failure of reliable maximal medical Rx. Development of significant symptoms: Musculo- skeletal, pruritis, calcinosis cutis, neuro-psych. Calcium x Phosphorus product above 70. Osteopenia, decreasing measured bone density, bone biopsy. Development of Tertiary Hyper-PTH
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SECONDARY HYPERPARATHYROIDISM
PRE-OP EVALUATION General condition - operative risk. Virtually all are dialysis-dependant Alk Phos Ultrasound (optional); Sestamibi scan not helpful.
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Identification of all Parathyroid Glands
Neck Exploration Identification of all Parathyroid Glands
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SECONDARY HYPERPARATHYROIDISM Surgical Options
Subtotal Parathyroidectomy or Total Parathyroidectomy with PTH Autotransplant Essential Components of Either Procedure: • Complete Neck Exploration • Identification of All PTH’s • Ablation of Hyperplastic Glands • Leave mg Functioning PTH • Consider temporary central line
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FAMILIAL PRIMARY (MEN) SECONDARY AND TERTIARY Special Considerations
SURGERY: FAMILIAL PRIMARY (MEN) SECONDARY AND TERTIARY Subtotal (3 ½ gland) resection Total Parathyroidectomy with Autotransplant. Special Considerations MEN I Syndrome Calcinosis/Calciphylaxis Tertiary after transplant
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Subtotal PTH-x vs Total PTH-x with AutoTx
Long – term Recurrence of Secondary Hyper PTH Patient Compliance Complexity and Duration of Surgery Ease / Cost of Post-op Care Calcinosis – special situation
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Any Questions ?
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