Special Surgery 1. Diseases of the Adrenal Glands, Surgical Consequences

I. Anatomy and Surgical Logic

• Adrenal glands: Paired retroperitoneal endocrine glands on the upper poles of kidneys
• Cortex: Cortisol, aldosterone, androgens
• Medulla: Catecholamines: epinephrine, norepinephrine
• Main surgical question for every adrenal mass → Functional? malignant? unilateral/bilateral? fit for surgery?
• Wrong preparation can be dangerous

• Pheochromocytoma without alpha-blockade → Hypertensive crisis
• Cortisol-producing tumor after removal → Adrenal insufficiency/crisis
• Conn syndrome without K correction → Arrhythmia risk

II. Functional Adrenal Diseases

Cushing Syndrome

• Cushing syndrome: Chronic glucocorticoid/cortisol excess
• Cushing disease: Pituitary ACTH-producing adenoma → Cushing syndrome

Etiology

1. ACTH-independent → Low ACTH due to negative feedback

• Adrenal adenoma/carcinoma, adrenal hyperplasia
• Iatrogenic glucocorticoid therapy → Common overall cause

2. ACTH-dependent → ACTH stimulates adrenal cortex

• Pituitary adenoma → Cushing disease
• Ectopic ACTH production → Lung/neuroendocrine tumors

Clinical Features

• Central obesity, moon face, buffalo hump
• Hyperglycemia → Type 2 diabetes/impaired glucose tolerance
• Proximal muscle weakness
• Hypertension; severe cortisol excess may cause Na retention + hypokalemia
• Skin → Atrophy, easy bruising, livid striae, acne, hirsutism
• Increased infection risk + poor wound healing
• Peptic ulcer/gastritis, fatty liver, pancreatitis risk
• Irregular menstruation, infertility, decreased libido
• Osteoporosis, glaucoma, cataract
• Psychiatric symptoms → Depression, anxiety, memory loss, insomnia
• Hyperpigmentation → If ACTH is high

Diagnosis

• Screen cortisol excess → 24 h urinary free cortisol, late-night cortisol, or low-dose dexamethasone suppression test

1. Low-dose dexamethasone test

• Cortisol suppressed → Cushing syndrome unlikely
• No suppression → Cushing syndrome likely

2. ACTH level

• Low ACTH → Adrenal/iatrogenic source
• High or inappropriately normal ACTH → Pituitary or ectopic ACTH

3. Localization

• High-dose dexamethasone suppression > 50% → Pituitary source more likely
• Poor suppression → Ectopic ACTH more likely
• Imaging → Adrenal CT/MRI; pituitary MRI/chest imaging if ACTH-dependent

Treatment

• Principle → Remove/control source of cortisol excess
• Pituitary adenoma → Transsphenoidal resection; bilateral adrenalectomy only if refractory/failed selected cases
• Ectopic ACTH → Surgical removal if possible
• Primary adrenal adenoma → Adrenalectomy + perioperative glucocorticoid replacement
• Adrenocortical carcinoma → Open oncological adrenalectomy if resectable

Conn Syndrome / Primary Hyperaldosteronism

• Conn syndrome: Autonomous aldosterone overproduction
• Aldosterone excess → Na retention + K/H loss → Hypertension + hypokalemia + metabolic alkalosis

Etiology

• Aldosterone-producing adrenal adenoma
• Bilateral idiopathic adrenal hyperplasia
• Familial hyperaldosteronism
• Aldosterone-producing adrenocortical carcinoma → Rare

Clinical Features

• Hypertension, often resistant
• Hypokalemia → Muscle weakness, constipation, arrhythmia, QT prolongation
• Metabolic alkalosis

Diagnosis

• Labs → Hypokalemia, suppressed renin, high aldosterone-renin ratio (ARR)
• Confirmatory test if needed → Sodium/saline loading does not suppress aldosterone
• Localization → CT/MRI + adrenal venous sampling (AVS)

• AVS compares adrenal venous aldosterone/cortisol → Unilateral adenoma vs bilateral hyperplasia

Treatment

• Unilateral aldosterone-producing adenoma → Adrenalectomy
• Preoperative spironolactone/eplerenone → Control BP + correct hypokalemia
• Bilateral idiopathic adrenal hyperplasia → Mineralocorticoid receptor antagonist
• MRA examples → Spironolactone, eplerenone

Pheochromocytoma

• Pheochromocytoma: Rare adrenal medulla chromaffin-cell tumor
• Produces catecholamines, usually norepinephrine > epinephrine
• Can cause life-threatening hypertensive crisis during anesthesia, surgery, biopsy or tumor manipulation

Clinical Features

• Classic triad → Headache + sweating + palpitations/tachycardia
• Episodic or sustained severe hypertension
• Anxiety, tremor, pallor, hyperglycemia
• Complications → Malignant arrhythmia, heart failure, angina/MI, stroke, aortic dissection

Diagnosis

• Biochemistry first → Plasma free metanephrines or 24 h urinary fractionated metanephrines
• VMA and chromogranin A may be increased but are less central
• Localization → Adrenal CT/MRI; scintigraphy/PET if selected complex case
• Do not biopsy adrenal mass until pheochromocytoma is excluded

Treatment

1. Preoperative alpha-blockade → Phenoxybenzamine or selective alpha-1 blocker: doxazosin/prazosin/urapidil
2. Beta-blocker → Only after alpha-blockade if tachycardia persists
3. Adrenalectomy → Careful BP control, no-touch technique, early adrenal vein ligation

III. Adrenal Tumors and Incidentaloma

Adrenocortical Carcinoma

• Rare, aggressive cancer of adrenal cortex
• Can be functional or non-functional
• Functional symptoms → Hypercortisolism, hyperaldosteronism, androgen/estrogen excess
• Suspicion → Large, irregular, invasive or rapidly growing adrenal mass
• Treatment → Complete open en bloc R0 resection if possible
• Mitotane/chemotherapy → Adjuvant or advanced disease depending on stage
• Debulking → Only selected advanced functional/symptomatic cases; cure requires complete resection

Adrenal Incidentaloma

• Adrenal incidentaloma: Adrenal mass > 1 cm found incidentally on imaging
• Workup questions

1. Is it hormonally active?
2. Is it malignant/suspicious?
3. Does it need surgery or observation?

• Hormonal workup → Cortisol excess, pheochromocytoma, aldosterone excess if HT/hypokalemia
• Imaging → Size + benign vs suspicious features
• Surgery → Functioning tumor, suspicious imaging, significant growth, or large indeterminate mass commonly > 4-6 cm
• Small homogeneous non-functioning benign-appearing mass → Clinical/radiological follow-up

Adrenal Metastases

• Common primaries → Lung, breast, kidney, melanoma
• Usually non-functional
• Surgery → Only selected isolated/symptomatic metastasis or diagnostic uncertainty when result changes management
• Biopsy only after pheochromocytoma is excluded

IV. Adrenalectomy and Surgical Consequences

Indications

• Functional adrenal mass → Cortisol, aldosterone, catecholamines, sex steroids
• Suspicion of adrenocortical carcinoma
• Selected incidentaloma → Functioning, suspicious, large/growing
• Selected isolated adrenal metastasis

Approaches

• Laparoscopic adrenalectomy: Preferred for benign small/moderate tumors

• Often used for small functional tumors and obese patients; lower morbidity than open surgery

• Retroperitoneal / posterior retroperitoneoscopic: Direct retroperitoneal access

• Useful for small tumors, bilateral adrenalectomy or previous abdominal surgery in experienced centers
• Position → Prone/jackknife; incision/access near 11th-12th ribs

• Open transabdominal: Large tumor, suspected carcinoma, invasion, pediatric/complex cases

• Incisions → Transverse, subcostal, chevron, midline
• Advantage → En bloc resection + vascular control

Perioperative Consequences

• Bleeding → Adrenal vein/IVC/renal vein; gland is vascular
• Adjacent organ injury → Liver, spleen, pancreas, kidney, colon, diaphragm
• Pheochromocytoma → Alpha-block before surgery; intraoperative BP control essential
• Cortisol-producing tumor → Perioperative steroid coverage; avoid sudden steroid withdrawal
• Bilateral adrenalectomy → Lifelong glucocorticoid + mineralocorticoid replacement
• Adrenal crisis → Hypotension/shock, hyponatremia, hyperkalemia, hypoglycemia → Hydrocortisone + fluids
• Conn syndrome → Correct hypokalemia preoperatively; monitor BP and potassium after surgery