Transcript Primary Hyperpara

Current Status of Surgery For
Primary and Secondary
Hyperparathyroidism
Surgery Grand Rounds
20 Aug., 2014
W. G. Schenk III
Disclosures
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
PTH and CALCIUM HOMEOSTASIS
+
PTH
-
CALCIUM
PTH and CALCIUM HOMEOSTASIS
n-PTH
c-PTH
Renal c-AMP
PTH
Vit D3
_
Bone Osteoclast
Phos
GI, Renal : 1,25 D
Ca++
Calcitonin
(?)
GI Absorption
(Albumin)
CALCIUM
+
HYPERPARATHYROIDISM
Inappropriate excess secretion of
Parathyroid Hormone
in Primary Hyperparathyroidism
Appropriate Hypersecretion in
Secondary Hyperparathyroidism
HYPERPARATHYROIDISM
SURGICAL APPROACES
Embryology
3rd Branchial Pouch: Thymus and
Lower PTH’s
4th Branchial Pouch: Upper PTH’s
Ectopic (5%) and supranumerary (15%)
DIAGNOSIS OF HYPERPARATHYROIDISM
PTH
Primary
Secondary
Tertiary
FHH
Syndrome
Malignancy
Ca++
PO4
U. Ca++
Cl / PO4
> 33
Ca x PO4
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)
Symptoms of Primary Hyperparathyroidism
Constitutional
Fatigue, Wt loss, Anorexia
Neurologic
Headache, memory loss
Neurosis, Psychosis
GI
Peptic Ulcer Disease
Nausea, Vomiting
Cardiovascular
Hypertension
Musculoskeletal
Bone and joint pain
Muscle weakness
Nephrologic
Stones, Renal impairment
Dermatologic
Pruritis, brittle nails
Symptoms of Primary Hyperparathyroidism
“ASYMPTOMATIC”
Some symptoms can usually be elicited by careful questioning.
Rationale for surgical Rx in asyptomatic patient.
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)
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)
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
PTH Levels in Primary Hyperparathyroidism
INDICATIONS FOR SURGERY IN
PRIMARY HYPERPARATHYROIDISM
(No prior neck surgery)
Diagnosis of
Hyperparathyroidism
Questionable
Secure
Symptomatic
Asymptomatic
Significant
Calcium
Surgical risk
10.5
Prohibitive
11.0
Low
HYPERPARATHYROIDISM
SURGICAL APPROACES
ANATOMIC CORRELATION:
“Sporadic” Primary – single gland (95%)
Familial Primary
Secondary
Tertiary
Multigland Hyperplasia
Sporadic Primary Hyperpara: Single Parathyroid Adenoma
Hypercellular Parathyroid
800 mg
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
“The only pre-op localization necessary is to
locate an experienced neck surgeon”
John Doppman
NIH Concensus Conference
1980
Hyperparathyroidism
Evolution of Imaging Relevence
 Improvement in imaging technology
 Popularity of limited exploration
 Development of rapid PTH Assay
 Economic pressures
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) : 260-269
Surgeon-Performed Ultrasound
for
Primary Hyperparathyroidism
Schenk WG, Hanks JB, Smith PW
 Convenience, Efficiency
 Surgical / Anatomic Correlation
 Volume of positive studies
 Additional relevant findings
Surgeon-Performed Ultrasound
for
Primary Hyperparathyroidism
Classification of pre-op Neck Ultrasound
 Class 1:
Hi Confidence
 Class 2:
“Possible”
 Class 0:
None seen
Primary Hyper PTH: Pre-Op Imaging
Results
Class 1:
111/200 (55%)
3 patients deferred surgery
107/108 Intra-op agreement
108/108 Cured
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
Tc-99m sestamibi scan
Coronal slice and 3D reconstruction from a 4DCT
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
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.
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
DIAGNOSIS OF HYPERPARATHYROIDISM
PTH
Ca++
PO4
U. Ca++
Cl / PO4
Ca x PO4
Primary
Secondary
Tertiary
FHH
Syndrome
Malignancy
+
>55
PTH Levels in Secondary Hyperparathyroidism
The majority of
dialysis patients have
some elevation in PTH.
A level in the 100- 300
pg/ml range is common.
Elevated level alone
is not an indication
for surgical Rx.
SECONDARY HYPERPARATHYROIDISM
Indications for Surgery
 Failure of reliable maximal medical Rx.
 Development of significant symptoms: Musculoskeletal, pruritis, calcinosis cutis, neuro-psych.
 Calcium x Phosphorus product above 70.
 Osteopenia, decreasing measured bone density,
bone biopsy.
 Development of Tertiary Hyper-PTH
SECONDARY HYPERPARATHYROIDISM
PRE-OP EVALUATION
 General condition - operative risk.
 Virtually all are dialysis-dependant
 Alk Phos
 Ultrasound (optional); Sestamibi scan not helpful.
Neck Exploration
Identification of all Parathyroid Glands
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 40-70 mg Functioning PTH
•
Consider temporary central line
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
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
Any
Questions
?