Dr. Enrique Vázquez Quintana

1. HISTORY OF PARATHYROID
SURGERY

2. WORLDWIDE HISTORY

3. In medicine only few things are more
fascinating than the history of parathyroid
disease

4. Oldest recorded case around 7,000
years ago
Early Neolithic period
Skeleton examination revealed osteopenia,
osteodystrophic fibrosa
cystica and salt-pepper appearance
of the cranium

5. Richard Owen 1850
Anatomist at the Natural Museum of
Londres, first described the parathyroids
in a rhinoceros autopsy. He never did a
histological examination.

6. Ivar Victor Sandstrom 1887
• Swedish medical student who described the
number, location, color and blood supply of the
parathyroids on animals and 50 human
autopsies.
• Manuscript- “On a new gland in man and fellow
animals” Publication rejected.
• Later published in the Uppsala Medical Journal.
• Contribution not recognized, committed suicide.

7. Eugene Cley
1891
French physiologist who observed the
relationship between tetany and the
parathyroids.

8. Frederich von Recklinghausen 1891
Pathology professor from Strasbourg who
described seven patients with bone
disease, multiple neurofibromatosis and
café-au-lait spots.

9. Anton von Eiselberg 1892
Disciple of Theodor Billroth-first attempt
to transplant the parathyroids in animals.

10. M. Askanazy 1903
Described a patient with bone disease
associated to a large parathyroid mass,
speculating that it might be a parathyroid
tumor.

11. William J. MacCallum 1903
Pathologist at John Hopkins Hospital
described tumors of the parathyroid
glands and the relief of tetany with a
parathyroid extract in experimental
animals.

12. Pfeiffer and Mayer 1907
Were the first to achieve clinical success
in autotransplanted parathyroid tissue.

13. William Steward Halsted 1907
• At John Hopkins University transplanted
parathyroids in dogs.
• Used calcium gluconate to treat tetany in
experimental animals and patients after
thyroidectomy.
• Emphasized preservation of the
parathyroids during thyroid surgery.

14. Jacob Erdheim 1907
• Pathologist from Vienna who reported the
association of bone disease with
abnormalities of the parathyroid glands.
• But believed the bone changes were
primary, not related to an excess of
parathyroid hormone.

15. W. Halsted and Herbert M. Evans
1907
Evans, medical student at Hopkins
defined the blood supply of the parathyroid
glands using vascular casts injections.
The inferior thyroid artery supplied both the
upper and lower parathyroid glands in over
90% of the patients.

16. Freidrich Schkagenhaufer 1915
• Physician from Vienna first to suggest that
parathyroid tumor was the primary
disease and the bone effects as
secondary.
• He recommended surgery to remove
the enlarged glands to cure the bone
condition.

17. Adolf M. Hanson 1923
• Medical student at the University of
Minnesota prepared a bovine parathyroid
extract to be used to treat tetany in dogs.
• He observed that the extract caused
osteoporosis in animals if used for a long
period of time.

18. James P. Collip 1932
• Biochemist from the University of Alberta
in Canada described independently an
extract identical to that of Hanson.
• He was the first to induce experimental
hypercalcemia and to describe the effects
of severe hypercalcemia.

19. Three most famous patients with
hyperparathyroidism
• Albert Gahne
• Charles Martell
• Elva Dawkings

20. Albert Gahne
• A 34 year street car conductor in Vienna
• Symptoms started in 1919, for 5 years he
had bone pains, x-rays showed
decalcification and cystic fibrosis changes
in the pelvis and both femurs.
• He was treated with parathyroid extract
and four fresh human parathyroid glands
transplantation.

21. Felix Mandl , chairman of surgery
at the University of Vienna in
July 2, 1925 explored the neck of
this patient under local anesthesia.

22. A large LL parathyroid tumor was
removed, three other glands were
preserved. Had an excellent
recovery. The pathology was an
atypical parathyroid adenoma.

23. He had a good clinical improvement
for six years although he was
having kidney stones and bony
pains.

24. Neck reexplored in October 18,
1933, two additional parathyroids
were removed, one inside the
thyroid gland. He continued with
hypercalcemia and died in uremia
in February 26, 1936, eleven years
after his first operation.

25. Autopsy showed extensive cystic
fibrosis of the bones, brown tumors
and decalcification of the spinal
column. No abnormal parathyroid
tissue or tumor recurrence was
found.

26. The pathologic diagnosis of this
patient is not clear.
Parathyroid carcinoma is a
possibility, although no recurrence
or metastasis was detected
postmortem.

27. E.J. Lewis January 6, 1926
• Performed the first parathyroid operation
at Cook County Hospital in Chicago on a
29 year old female patient with
parathyroid carcinoma.
• Operation done six months after Felix
Mandl’s operation and four months before
the first operation of Charles Martell.

28. E. Gold 1927
• Another Viennese surgeon explored the
neck of a 54 year old female with von
Recklinghausen’s disease in July 1927, two
years after Mandl’s operation. He removed
a RU parathyroid adenoma measuring 2.5
X 2.6 cm.
• Gold was the first to use the term
hyperparathyroidism.

29. Mandl analyzed 55 cases and
concluded that surgery was the
only method to cure
hyperparathyroidism.

30. He advised bilateral neck
exploration to identify the normal
and abnormal parathyroid glands.
He established the gold standard
in the treatment of
hyperparathyroidism

31. Charles Martell, the most famous
patient with hyperparathyroidism.
• He was a healthy sea marine captain who
at age 30 years was diagnosed in January
1926 as having hyperparathyroidism by
Eugene F. Dubois at the Belleveu Hospital
in New York.
• In May 1927 he had two neck explorations
at Massachusetts General Hospital by Dr.
EP Richardson, Chief of Surgery. Two
normal parathyroids were removed.

32. In 1929 a third operation was done
in New York by Russell Patterson
with negative results.
Oliver Cope performed three
additional negative explorations on
this patient.
The patient himself insisted on a
mediastinal exploration.

33. On the seventh operation done by
Edward D. Churchill and Oliver
Cope a mediastinal adenoma
measuring 3 X 3 cm was removed.
He developed tetany on the third
postoperative day.
Died six weeks later while
undergoing removal of an
obstructive ureteral stone.

34. Captain Charles Martell

35. The third patient- Elva Dawkings
• A 56 year old female with muscle weakness,
bone fractures and bilateral renal stones.
• Henry A. Dixon, a fourth year medical student at
Barnes Hospital at Washington University
documented a calcium of 17 mgs/dl, phosphorus
1.4 mgs/dl.
• On August 1, 1928, Isaac Y. Olch removed a LL
parathyroid adenoma measuring 3 X 3 cms. She
was treated with parathyroid extract and calcium
IV and orally.

36. James Walton 1931
An English surgeon recommended a wide
exposure during surgery, not only to
explore all the parathyroid glands, but also
to search behind the trachea and the
mediastinum.

37. HYPERPARATHYROIDISM IN PUERTO RICO

38. In God we trust, all others must
bring their data.
Claude H. Organ

39. ENDOCRINOLOGISTS
• MANUEL PANIAGUA CAMARA
• AGUSTIN MARTINEZ DE ANDINO
• LILLIAN HADDOCK SUAREZ

40. Dr. Manuel Paniagua Cámara
Photo courtesy of his daughter
Dr. Carmen T. Paniagua, EdD, RN

41. Dr. Manuel Paniagua Cámara
1914-1983
• Born in San Juan, P.R.
• Medical Degree, Madrid University
• Internal Medicine Specialty-Bayamón
District Hospital.
• Endocrinology Subspecialty-Thomas
Jefferson University
• Captain Medical Corp-WWII

42. Dr. Manuel Paniagua Cámara
• First endocrinologist in Puerto Rico
• 1977 Founding member and First President of
the Sociedad Puertorriqueña de Endocrinología
(SPED)
• Medical Director Rio Piedras Municipal
Hospital
• Director Endocrinology & Diabetes
Section, Dept. of Medicine San Juan City
Hospital until retirement in 1982
• Over 20 published articles

43. Dr. Agustin M. de Andino
Photo courtesy of his son,
Dr. Richard de Andino

44. Dr. Agustín M. de Andino
• Medical School-Jefferson Medical College,
Philadelphia 1944
• Internship and Internal Medicine-JMC
1944-1950
• Fellowship in Endocrinology-JMC 1950-51
• Returned to Puerto Rico-1952
• Bibliography-29 articles

45. Dr. Agustín M. de Andino
• Chief Endocrine and Diabetes Section,
Department of Medicine, San Juan City
Hospital August 1952-June 30, 1968
• Attending in Medicine (Metabolic Disease)
San Patricio VA Hospital, 1952-1964
• Founding member and second president
of the Sociedad de Puertorriqueña de
Endocrinología y Diabetes.

46. Dr. Juan E Rizek
• Trained in Endocrinology in the San Juan
City Hospital in 1959-1960, when the
Chair of Medicine was Dr. Rurico Díaz
Rivera and the Chief of Endocrinology was
Dr.Agustín M. de Andino.

47. Dr. Lillian Haddock Suárez

48. Dr. Lillian Haddock Suárez
• M.D. degree from Temple University School of
Medicine 1954
• Internship at Bayamón District Hospital 1954-55
• Residency in Internal Medicine 1955-57
at San Juan City Hospital
• Fellowship in Endocrinology and Metabolism at
Johns Hopkins Hospital 1957-59
• Director Division of Endocrinology, Diabetes &
Metabolism 1960-76
• Professor Emeritus 1998

49. ENDOCRINOLOGY PROGRAM
Dr. Mario R. García Palmieri in 1960
designated Dr. Lillian Haddock as
Director of the Endocrinology Section of
the Department of Medicine at the UPR
School of Medicine

50. Doctor Elena Villavicencio
First resident in the endocrinology
program at the University Hospital
from 1960-61

51. Photo of Elena Villavicencio
Courtesy of her daughter Francesca Cuello

52. Doctor Francisco L. Raffucci
Chairman Department of Surgery
Doctor Leo Cuello Mainardi
Program Director of Surgical Residency

53. Dr. Leo Cuello Mainardi
• Born-Santiago de los Caballeros, Dominican Republic
• B.S. Major in Biology-Mayaguez College UPR
• Medical Degree-University of Paris
• Residency-University Hospital Rio Piedras
• Thoracic and CV surgery-Univ. Minnesota
• Master in Science-Univ. Minnesota
• Associate Professor Surgery-Univ. Hospital 1963-67
• Professor Cardio-thoracic Surgery. Univ. of Texas
Medical School 1973-to present

54. Seminar on the differential
diagnosis of hyperparthyroidism
• Sarcoidosis
• Milk-alkali syndrome
• Multiple myeloma
• Hypernephroma
• Metastatic bone disease
• Hyperparathyroidism

55. G. Gordan 1958
The diagnosis of hyperparathyroidism has
gone from bone to stone, to an epigastric
moan to none.

56. FIRST PUBLICATION
ON HYPERPARATHYROIDISM
The clinical , biochemical, operative and
pathological analysis of 14 cases of
primary hyperparathyroidism
Lillian Haddock, Julián Vázquez Plard,
Francisco Aguiló, María del C. Vázquez,
Francisco L. Raffucci, Juan Velázquez
Bull. Puerto Rico Medical Association
62 (3) 76-85, 1970

57. First reported article on
hyperparathyroidism in Puerto Rico
• 14 patients from 1960-1968
• 8 males
• 6 females
• Age from 16-67
• 12 chief cell adenomas
• 1 carcinoma
• 1 normal gland
• One mediastinal tumor and one death

58. My first case
• A.V.C., a 41 year old female who in September
1970 presented with anorexia, loss of weight,
polydipsia, polyuria, constipation, bony pains
and burning of the eyes.
• Calcium 16.4-19.8 mgs/dl
• Phosphorus 1.6-2.9 mgs/dl
• Chest x-rays revealed a mediastinal mass.
• An IVP showed nephrocalcinosis and a pelvic
bone cyst.

59. On physical examination no neck
masses were palpated.
• On September 21, 1970 the neck was explored.
No neck adenomas were identified.
• A large lower neck and retrosternal mass was
found. A median sternotomy was done and a
12x5x3 cm was removed, weighing 113 grams.
• This is the second largest tumor reported in the
literature.
• The pathology was chief cell parathyroid
adenoma.

60. Gross and microscopy

61. Parathyroid adenomas
Weighing over 50 grams
Surgeon Year Location Size(cm) Weight(g)
Dresser 1931 R 6.5x5.0x3.0 53.0
Castleman 1936 R 6.5x5.0x3.5 53.2
Earll 1969 LL 7x5x3 85.0
Snell 1936 RL 6x6x5 101.0
Vázquez 1970 M 12x6x3.5 113.3
Sharpe 1939 LU 7x6.5x4 120.0

62. Lebsche
Sternum Chisel

63. A.V.C.
• Patient alive after 36 years, now age 77
• Her calcium is 10-10.3 mgs/dl, P 3.3
mgs/dl, PTH 72.8 pg/dl.
• Serum creatinine is 1.8 mg/dl
• Creatinine clearance is low 24.93 ml/min.
• She has no stones and continues to work.

64. A. V. C. photo

65. Evaluation of hypercalcemia
• SMA 23, now Comprehensive Metabolic
Profile
• PTH measurements
• Localization of abnormal parathyroids

66. Localization Tests
• Toluidine blue
• Thyroid lymphography
• Seleno methionine
• PTH determination with massage
• Barium swallow
• Thallium-technetium substraction scan
• Ultrasonography
• CT scanning
• MRI
• Arteriography
• Selective cannulation of neck veins for PTH levels
• Sestamibi parathyroid scan

67. Localization of Parathyroid Lesions:
Blind Study for Surgeons
Enrique Vázquez-Quintana, M.D., Cid
Quintana, M.D.,Francisco Aguiló, M.D.,
Heriberto Pagán-Sáez, M.D., Frieda Silva,
M.D.
Puerto Rico Medical Association Bull.
Sept. 1989, Vol. 81:343-344

68. Fifteen consecutive patients with
hyperparathyroidism were studied.
CT scans and Thallium-Technetium
substraction Scan were used to
localize the affected gland.
The results were unknown to the
surgeons.

69. In all fifteen patients an adenoma
was identified and removed.
• Only four parathyroid glands were
identified by CT.
• The Thallium-Technetium substraction
scan identified seven of the 15 affected
glands.
• It was documented that the utilization of
those two localizing tests were not
indicated.

70. Unusual Cases of
Hyperparathyroidism
Enrique Vázquez-Quintana, M.D., Lillian
Haddock, M.D., Francisco Aguiló, Ramón
Sotomayor, M.D. Margarita Ramírez, M.D.
Julián Vázquez-Plard, M.D., Vilma Rabell,
M.D., Heriberto Pagán Sáez, M.D.
Puerto Rico Health Sciences Journal,
Vol. 14, No.3, September 1995

71. Unusual Cases of
Hyperparathyroidism
• Carcinoma of the parathyroid
• Large Mediastinal tumor
• Reoperation for hyperplasia 20 years later
• Advanced bone disease
• Tertiary hyperparathyroidism secondary to
hypophosphatemic ricketts

72. Multiple Cysts in the Glenoid Area

73. Bone Cyst of the Pelvis

76. Surgical Management of
Hyperparathyroidism: a 24 Year
Experience
Enrique Vázquez Quintana,
M.D,FACS
Puerto Rico Medical Association
Bulletin
Vol. 87, No.709, September 1995

77. A total of 130 patients from 1971
to 1994.
• Females 100
• Males 30
• Average age 56.7 years
• Range of age 19-83 years
• Renal lithiasis and bone changes 66.6%
• Asymptomatic 28.1%

78. Primary Hyperparathyroidism
1971-1994 130 cases
• Adenoma 121---------93.1%
• Hyperplasia 7---------5.4%
• Chief cell 6
• Clear cell 1
• Carcinoma 1
• No lesion 1
• Associated thyroid lesions 12---9.2%

79. Primary Hyperparathyroidism
1971-1994 130 cases
Complications:
• Transient hypocalcemia-----3
• Permanent hypocalcemia---1
• Persistent hypercalcemia---3
• Recurrent hypercalcemia---1
• Renal colic----------------------1
• Reoperation--------------------1
• Mortality-------------------------0

80. Radiologic Manifestations of
Hyperparathyroidism
Enrique Vázquez-Quintana, M.D., H.
Pagán-Sáez, M.D., F. Bayrón-Vélez,M.D.,
Marian de Jesús, M.D.
Puerto Rico Medical Association Bulletin
Vol. 87, Vol. 7-9, September 1995

81. Parathyroid Carcinoma:
Diagnosis and Management
Enrique Vázquez-Quintana, M.D.
The American Surgeon
Vol.63, No11, Pag.954-957
November 1997

82. Barium swallow

83. Gross pathology

84. Microscopic examination

85. Criteria for Malignancy
• Invasion of surrounding tissues
• Capsular invasion
• Dense fibrous septa
• Vascular or lymphatic metastasis
• Presence of mitotic figures
• Visceral metastasis

86. Clinical Profile of 128 Subjects Operated for
Primary Hyperparathyroidism
Lillian Haddock, M.D.,Francisco Aguiló,
M.D., Enrique Vázquez Quintana, M. D.,
María del C. Vázquez, BS, MT,Vilma
Rabell, M.D., Myriam Allende, M.D.
Puerto Rico Health Science Journal
Vol. 17 No. 4, 309-316, Dec. 1998

87. SUMMARY
• 128 patients with hyperparathyroidism
from 1960-1990
• 95 females, 33 males
• Only 7 patients were asymptomatic
• 93% cure rate
• 7 patients with MEN-1 in three families
• 4 patients with MEN-2 in one family

88. PATHOLOGICAL FINDING
• Single adenoma 93 73.8%
• Hyperplasia 18 14.2
• Intrathyroidal adenoma 2 3.6
• Two adenomas 4 3.2
• Huge mediastinal adenoma 1 0.8
• Cystic adenoma 1 0.8
• Carcinoma 2 1.6
• Not found 3 2.2
• Intrathyroidal hyperplastic 2 1.6

89. MORTALITY
• 2 DEATHS
• 1 FROM BILATERAL PNEUMOTHORAX
• 1 FROM RESPIRATORY FAILURE

90. Parathyroid Surgery Without
Instant PTH or Radioguided
Sestamibi Scan
Enrique Vázquez-Quintana, M.D.
Daniel E. Vázquez-Torres M.D.
Presented at the Southeastern
Surgical Congress Poster Session
Feb. 2007

91. A total of 56 patients in one
year
• Primary hyperparathyroidism----52
• Seconday hyperparathyroidism---3
• Tertiary hyperparathyroidism-----1
• 54 of 56 patients are normocalcemic
with normal PTH levels
• 96% success rate

92. Table I
Total number of patients 56
Primary hyperparathyroidism 52
Single adenoma 51
Two adenomas 1
Secondary hyperparathyroidism 3
Tertiary hyperparathyroidism 1

93. Table II
Associated conditions:
Papillary carcinoma of thyroid 3
Multinodular goiter 3
Single thyroid nodule 4
Adrenal tumor 1
Reoperations 3
Total 14

94. Table III
Sestamibi parathyroid scan:
Positive 35
Negative 8
Equivocal 9
Total 52

95. NUMBER OF OPERATIONS BY
YEARS
• 1970-1994 130
• 1995 24
• 1996 27
• 1997 32
• 1998 31
• 1999 13
• 2000 25

96. NUMBER OF OPERATIONS BY YEAR
• 2001 37
• 2002 40
• 2003 36
• 2004 41
• 2005 45
• 2006 50
• 2007 34
• 2008 8

97. GRAND TOTAL
583
No deaths

98. Mediastinal Lesions

101. NIH CONSENSUS CONFERENCE
1990
Indications for surgery
1. Serum calcium above 1-1.5 mgs/dl above the
normal values
2. Urinary calcium above 400 mgs/24 hours
3. Creatinine clearance reduced by 30%
4. Bone density below 2 sd of normal
5. Patients under 50 years of age
6. Medical surveillance not possible

102. The indications were revised in
another meeting in April 2002
to include asymptomatic patients
and only minor changes were
made.

103. Long Term Outcome of Patients
with Elevated PTH following
Intraoperative Parathormone
Guided Parathyroidectomy
Wiliam Méndez-Latalladi, M.D. et als
Endocrine Surgery Div.,Surgery Dept.
University of Miami School of Medicine

104. Summary of article:
• Presented at Western Surgical Association
meeting in 2007
• 522 patients
• 97% had a successful procedure
• Follow up of 43 months
• 505 remain with normal PTH and calcium
• 107 or 33% developed elevated PTH levels
• 7% developed recurrent hyperparathyroidism
warranting reoperation

105. In Puerto Rico
• There is no hospital with instant PTH
capability.
• Sestamibi radioactive guided probe at
the Oncologic Hospital in the Puerto Rico
Medical Center.

106. !Congratulations!
The surgeon who did the first
parathyroid operation in Puerto
Rico was Dr. Francisco L. Raffucci.

107. First patient operated for
Hyperparathyroidism in Puerto Rico

108. First of the fourteen patients
• R.A.M. a 60 year old male patient with
serum calcium of 12.9-15 mgs/dl.
• An adenoma from the LL parathyroid
gland measuring 4 X 3 cm and weighing
12 grams was removed in 1960.
• He developed tetany postoperatively.
• The pathology was a chief cell adenoma.

109. Guess what?
Surprisingly, two of the authors of
the first publication suffered from
hyperparathyroidism
and underwent surgery in 2004

110. In life there is only chance,
change and death.
Dr. Gumersindo Blanco

111. Enigmatic or Preplexing Disease
• Generally we have four parathyroids
• External stimulus
• Single parathyroid adenoma most frequently
seen
• Double adenomas are very rare
• Recurrent disease is also very rare
• Normocalcemic hyperparathyroidism
• Three patients with non-functioning adenomas
• Why the external stimulus does not repeat itself
more frequently?

112. Humbleness
We surgeons ought to have:
Intellectual and technical ability
Correct diagnosis
Surgical assistance
Peace of mind
Altruism
Empathy
Spirituality
• There is always someone who knows more than you do.
Another surgeon or …….

116. Acknowledgments:
• Dr. Sylvia Barceló - Director Outpatient
Clinics, Puerto Rico Medical Center
• Mrs. Lisandra Brenes Torre – Manager,
Health Information Division, Puerto Rico
Medical Center