Adrenal gland

1. Adrenal gland
part 1
Dr. Khaled Alsharafy
Supervised by
- Ass.Prof Waleed Ghialan
- Assist.Prof Loai Alqobati

2. In this lecture
•Describe the anatomy , embryology and
physiology of adrenal gland.
•Management approach to Conn`s syndrome.
•Management approach to Chusing`s syndrome.
•Management approach to elevated sex harmon.
•Management approach to Pheocytochroma.

3. Brain storming
1
50 year old
patient with
resistant HTN and
elevated serum
aldosterone
CT show 1cm
adrenal mass was
found
2
50 year old patient
with features of
Cushing syndrome
and elevated
salivary cortisol
CT show 1cm
adrenal mass was
found
3
50 year old patient
with resistant HTN
and elevated 24hr
catecholamine
urine level
CT show 1cm
adrenal mass was
found

4. Anatomy and physiology

5. Anatomy
Sabiston textbook of surgery 21st edition

6. Anatomy
Sabiston textbook of surgery 21st edition

7. Anatomy
Sabiston textbook of surgery 21st edition

8. Anatomy
Sabiston textbook of
surgery 21st edition
In up to 20% of
individuals

9. Embryology

10. Extra-adrenal cortical and medullary tissue

11. Histology

12. physiology

13. Primary Hyperaldosteronism

14. Primary Hyperaldosteronism
•Primary hyperaldosteronism,
the unregulated release of
excess aldosterone from one
or both adrenal glands, was
first described by Jerome
Conn in 1954

15. Primary Hyperaldosteronism (epidemiology )
•Age > 50 year
•M>F
•< 7% of patient with
HTN

16. Primary Hyperaldosteronism (clinical pict. )
Hypertension
Hypokalemia

17. Primary Hyperaldosteronism (clinical pict. )
Hypertension
• 2-4 medications
• May responds to spironolactone

18. Primary Hyperaldosteronism (clinical pict. )
Primary Hyperaldosteronism
Stroke
MI
arrythmias
HF

19. Primary Hyperaldosteronism (causes )
Sabiston textbook of surgery 21st edition

20. Primary Hyperaldosteronism (approach )
diagnosis Localization treatment

21. Primary Hyperaldosteronism (approach )

22. Primary Hyperaldosteronism (approach )
•Aldosterone is excreted in urine
•Serum aldosterone is not sufficient for diagnosis
and suppression test should be performed

23. Primary Hyperaldosteronism (approach )

24. Primary Hyperaldosteronism (approach )
• Most common cause is bilateral adrenal hyperplasia 65%.
• 40 years and older are more likely to possess nonfunctioning
adrenal cortical adenomas.
• Adrenal adenomas > 1cm more likely to be functioning

25. Primary Hyperaldosteronism (approach )

26. Primary Hyperaldosteronism (approach )
• successful cannulation
Aldosterone and cortisol level in venous sample / peripheral sample

27. Primary Hyperaldosteronism (approach )
• Lateralization
unbalanced ratio of aldosterone to cortisol
in the left and right adrenal veins

28. Primary Hyperaldosteronism (approach )
• Lateralization ?
Successful study lateralizing strongly to the left adrenal

29. Primary Hyperaldosteronism (approach )

30. Primary Hyperaldosteronism (approach )

31. Primary Hyperaldosteronism (approach )

32. Primary Hyperaldosteronism (approach )
• In patient with +ve PAC/PRA and < 30 year
Consider genetic testing for familial hyperaldosteronism as it is treated
medically

33. Primary Hyperaldosteronism (surgical management )
• Laparoscopic is preferred

34. Primary Hyperaldosteronism (surgical management )
• Cure rate 75% to 95% may observed in 24hrs to several weeks
• Out-come :
1- clinical : blood pressure and antihypertensive medication
2- biochemical : urine aldosterone levels and resolution of hypokalemia

35. Primary Hyperaldosteronism (surgical management )
• Post-operative problem:
1- increase creatinine level post-operative
2- transient hyperkalemia 1-3 week post-operative
3- HTN medications how to stop

36. Primary Hyperaldosteronism (surgical management )
• Reduced benefit in :
1- >45year
2- family hx of HTN
3- long standing HTN
4- use > 2 anti-HTN drugs
5- non-response to spironolactone

37. Case 1
1
50 year old
patient with
resistant HTN and
elevated serum
aldosterone
CT show 1cm
adrenal mass was
found

38. Cushing syndrome

39. Cushing syndrome
• The clinical features of
glucocorticoid excess were first
documented by Harvey Cushing
in 1912
• Most common due to
pharmacologic glucocorticoid
use

40. Cushing syndrome ( causes )
• Cushing syndrome is rare
• Cushing disease (pituitary adenoma ) …………( 75%)
• primary adrenal Cushing syndrome ……………( 15%)
• ectopic ACTH syndrome ………………………………( <10%)

41. Cushing syndrome ( causes )
• Primary adrenal Cushing syndrome
• Solitary adrenal adenoma ……………( 90%)
• adrenocortical carcinoma ……………( <10%)
• Bilateral hyperplasia ……………………( 1%)

42. Cushing syndrome ( clinical pict. )

43. Cushing syndrome ( approach )
diagnosis Localization treatment

44. Cushing syndrome ( approach )

45. Cushing syndrome ( approach )
• ACTH is released in circadian rhythm highest at morning.
• Cortisol is excreted in urine and saliva.
• Tow repeated test should be preformed to confirm diagnosis

46. Cushing syndrome ( approach )

47. Cushing syndrome ( approach )
Adrenal Cushing syndrome :
• It cause undetectable plasma ACTH.
• 90% of them are due to adenoma.
• Almost all lesion are seen on CT-scan

48. Cushing syndrome ( approach )
Extra-adrenal Cushing syndrome :
• Ectopic ACTH sources are completely lacking in feedback inhibition.
• 50% of corticotroph microadenomas are visible on pituitary MRI

49. Cushing syndrome ( approach )

50. Cushing syndrome ( approach )

51. Cushing syndrome ( approach )
• Indication for surgery :
1- primary adrenal Cushing syndrome
2- failure of pituitary surgery for Cushing disease
3- unresectable ectopic ACTH secreting tumor for palliation
4- failure of localize ectopic ACTH secreting tumor

52. Cushing syndrome ( approach )
• Peri-operative care :
1- perioperative stress dose steroids (hydrocortisone 100 mg intravenously
every 8 hours for 24 hour)
2- usually be tapered to physiologic replacement levels during the course
of several weeks but may take more than 1year

53. Cushing syndrome ( approach )
• Post operative problem :
1- HPA axis suppression for long duration
2- deleterious physiologic effects are extremely persistent
3- hypercoagulable status ( need prophylaxis )

54. Subclinical Cushing syndrome
•For self-study
- Definition
- Clinical picture
- prognosis
- Diagnosis
- Role of surgical management

55. Case 2
2
50 year old patient
with features of
Cushing syndrome
and elevated
salivary cortisol
CT show 1cm
adrenal mass was
found

56. Sex Steroid Excess

57. ‫الورشة‬ ‫محاور‬
5Ps
Sex Steroid Excess (Adrenal tumors )
Most of these tumors are virilizing
One third of virilizing tumors are malignant
Almost all feminizing tumors are malignant

58. ‫الورشة‬ ‫محاور‬
5Ps
Sex Steroid Excess (Adrenal tumors )
Of adrenocortical carcinomas:
• 20% cause virilization and most of them are in children
• 24% mixed features of Cushing syndrome and virilization

59. ‫الورشة‬ ‫محاور‬
5Ps
Sex Steroid Excess (Adrenal tumors )
Virilizing tumors diagnosed by 24-hour urine
for:
• Testosterone
• DHEA
• DHEA-S

60. ‫الورشة‬ ‫محاور‬
5Ps
Sex Steroid Excess (Adrenal tumors )
Although laparoscopic adrenalectomy remains
the preferred procedure open adrenalectomy
should be performed for obviously malignant
tumors

61. Pheochromocytoma

62. pheochromocytoma
• pheochromocytoma
(dusky-colored tumor,
from the Greek
phaios, dusky(

63. pheochromocytoma
• Men and women are affected equally.
• sporadic cases is between the ages of 40 and 50 years.
• Affects approximately 0.2% of hypertensive individuals.
• Only 0.5% of patients with hypertension and suggestive features will
ultimately prove to have the disease.

64. pheochromocytoma
malignant
familial
children
bilateral
Extra-
adrenal
recur
incidental

65. pheochromocytoma
familial
MEN1
MEN2A
MEN2B
Von Hippel-lindau disease
Neurofibromatosis type 1
Other neuroectodermal disorder
Carney`s syndrome

66. Pheochromocytoma( clinical pict.)

67. pheochromocytoma( approach )
diagnosis Localization treatment

68. pheochromocytoma( approach )
• Catecholamines have many metabolites.
• Plasma free metanephrine testing is for screening not diagnosis.
• Catecholamines and their metabolites are excreted in urine and its
measurement is the best in diagnosis.
• Tests performed during episodes of acute pain, critical illness, or
urgent hospitalization may be misleading.

69. pheochromocytoma( approach )

70. pheochromocytoma( approach )

71. pheochromocytoma( approach )

72. pheochromocytoma( approach )
• CT scans are 85% to 95% sensitive and 70% to 100% specific.
• MRI scans are 95% sensitive and almost 100% specific.

73. pheochromocytoma( approach )

74. pheochromocytoma( approach )

75. pheochromocytoma( approach )

76. pheochromocytoma( approach )
Peri-operative care
• Pre-operative
• Intra-operative
• Post-operative

77. pheochromocytoma( approach )
Pre-operative

78. pheochromocytoma( approach )
Pre-operative

79. pheochromocytoma( approach )
Pre-operative
1- Alpha Blockers: Phenoxybenzamine
phenoxybenzamine 10 mg twice daily. The dosage can be titrated
upward every 2 to 3 days to a maximum of 40 mg three times daily for
at least 2 weeks

80. pheochromocytoma( approach )
Pre-operative
2- Beta blockers:
a) For tachycardia
b) To control supraventricular and ventricular arrhythmias
Always after starting alpha blocker

81. pheochromocytoma( approach )
Pre-operative
3- Calcium channel blockers:
a) For paroxysms of hypertension
b) patients with secondary cardiovascular complications

82. pheochromocytoma( approach )
Pre-operative
4- a-Methyl para tyrosine:
Pre treatment for 3 days depletes tumor catecholamine stores by 50%.

83. pheochromocytoma( approach )
Pre-operative
Adequacy of blockade is assessed using Roizen’s criteria:

84. pheochromocytoma( approach )
Intra-operative
1- Anesthesia :
premedication , type of anesthesia and invasive monitoring of
hemodynamic condition .

85. pheochromocytoma( approach )
Intra-operative
2- hypertensive crisis : risk factors
a) Large tumor
b) high plasma NE concentrations
c) more postural hypotension after a blockade
d) MAP >100 mm Hg

86. pheochromocytoma( approach )
Intra-operative
2- hypertensive crisis : management
a) Diltiazem (5-10 mg)
b) labetalol (5-15 mg)
c) Nicardipine
d) SNP
e) NTG infusions

87. pheochromocytoma( approach )
Post-operative
1- Post tumor excision hypotension : due to
a) residual a-blockade
b) residual action of vasodilators
c) hypovolemia
d) catecholamine withdrawal
e) a adrenoceptor downregulation

88. pheochromocytoma( approach )
Post-operative
1- Post tumor excision hypotension : management
a) volume replacement to restore CVP to 10-12 mm Hg
b) If ineffective, norepinephrine is the pressor of choice.
c) Vasopressin in refractory hypotension.

89. pheochromocytoma( approach )
Post-operative
1- hypothermia
2- Hypoglycemia

90. pheochromocytoma( approach )
Peri-operative
Laparoscopic adrenalectomy, and more recently, robotic-assisted
adrenalectomy is associated with less severe hemodynamic
fluctuations, reduced blood loss and vasodilator use.

91. pheochromocytoma( out-come )
•Mortality decrease from 50% to about 1% .
•Surgery is curative in more than 90%

92. pheochromocytoma
•For farther reading
pheochromocytoma in pregnancy

93. Case 3
3
50 year old patient
with resistant HTN
and elevated 24hr
catecholamine
urine level
CT show 1cm
adrenal mass was
found

94. Adrenal gland part 2
•Adrenal gland cancer
•Adrenal gland incidentaloma
•Adrenal insufficiency
•Adrenal gland surgery

95. Reference
• Sabiston Textbook of Surgery 21st ed
• Schwartz’s Principles of Surgery 10th ed
• Baily and love short practice of surgery 28th ed
• Rush university medical center review of surgery 6th ed