Cushing syndrome

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Case Presentation:

Case Presentation Presenter Dr.Md.Monowarul Islam

Particulars of patient:

Particulars of patient Name:Nurizzaman Age:28years Sex:Male Occupation: Salesman Address: Lalbagh, Dhaka

Presentation:

Presentation Progressive central obesity for 1year Weakness, skin pigmentation and insomnia for same duration

History of present illness:

History of present illness Patient states that about 1 yr back he noticed abnormal increase in size of his abdomen in respect to the limbs. His face also gradually become swollen. He noticed swelling in his nape of the neck. He felt progressive weakness specially in the limbs. He also complains of weight gain in this period, the amount he can not quantify. He developed irritability and difficulties in sleeping. With these complaints he consulted a physician, who adviced some investigations which subsequently revealed hypokalamia and a mass lesion in his right suprarenal gland. He has no history of headache,swetting, palpitation, and postural hypotention. He has no history of cough, jaundice or chest pain.

History of past illness:

History of past illness Nothing contributory Previous operation/accident: None

Drug history:

Drug history Previous treatment: Some medication he had taken as per advice of his physician but he can’t mention the details Drug Allergy: not known

Family history:

Family history None of his family member has suffered from similar illness

Personal History:

Personal History Non-alcoholic, non-smoker

General Physical Examination:

General Physical Examination Appearance: Moon face with facial plethora Central obesity & buffalo hump Behaviour: Irritable Skin condition: Striae over abdomen & thigh, Acne present and easily bruisable Hair distribution: Male pattern Pulse:80 bpm BP:150/100mm of Hg Respiration: 18 breaths/min Oedema: absent Dehydration: absent JVP: not raised Lymph nodes: not enlarged Heart: S1S2 normal, no added sound Lungs: clear

Abdominal Examination:

Abdominal Examination Inspection: S triae over lower abdomen No visible lump, engorged vein or any scar mark Palpation: No palpable lump Liver, spleen and kidneys are not palpable Percussion No ascites Auscultation Bowel sound present

Salient Features:

Salient Features Mr. Nuruzzaman, 28-yr-old salesman presented with central obesity, moon face buffalo hump, striae over abdomen and thigh. He felt progressive weakness specially in the limbs. He also complains of weight gain. He is irritable and feel difficulties in sleeping. He has no history of headache, swetting, palpitation, and postural hypotention. He has no history of cough, jaundice or chest pain.

Investigation:

Investigation

Operation:

Operation Preoperative medication: CV Line placement: Incision: Exposure: Findings: Procedure: Complication: Post operative convalescence:

Discussion:

Discussion

Cushing syndrome :

Cushing syndrome Hypercortisolism secondary to excessive production of glucocorticoids by the adrenal cortex is defined as Cushing syndrome

Clinical Characteristics:

Clinical Characteristics

HPA Axis:

HPA Axis

Causes of Cushing syndrome:

Causes of Cushing syndrome Three main groups: Exogenous ACTH-dependent ACTH-independent

Exogenous Cushing Syndrome.:

Exogenous Cushing Syndrome. Exogenous Cushing syndrome is a result of iatrogenic glucocorticoid administration.

ACTH-Dependent Cushing Syndrome:

ACTH-Dependent Cushing Syndrome 80% is due to primary pituitary pathology and is known as Cushing disease . Ectopic ACTH production is another main cause Ectopic CRH syndrome is a third cause

ACTH-INDEPENDENT CUSHING SYNDROME.:

ACTH-INDEPENDENT CUSHING SYNDROME. Uncontrolled hypersecretion of cortisol by adrenal tissues is classified as ACTH-independent Cushing syndrome. Adrenal neoplasms and rare forms of bilateral adrenal disease are responsible for this group of conditions .

ADRENAL TUMORS.:

ADRENAL TUMORS.

PowerPoint Presentation:

Cortisol-secreting benign adenomas of the adrenal gland ,10% of Cushing syndrome result from a dominant unilateral hyperplastic nodule

ACTH-INDEPENDENT MACRONODULAR ADRENAL HYPERPLASIA (AIMAH).:

ACTH-INDEPENDENT MACRONODULAR ADRENAL HYPERPLASIA (AIMAH). The condition is characterized by multiple large (up to 4 cm) nodules replacing the glands with each adrenal weighing over 60 g . Weights in excess of 200 g also have been reported for each gland affected by AIMAH .

PRIMARY PIGMENTED NODULAR ADRENOCORTICAL DISEASE (PPNAD).:

PRIMARY PIGMENTED NODULAR ADRENOCORTICAL DISEASE (PPNAD). adrenal glands in this condition remain normal in size and exhibit black or brown cortical nodules

PowerPoint Presentation:

Up to 50% of patients with Cushing syndrome exhibit urolithiasis; therefore stone formers with cushinoid features also deserve a hypercortisolemia evaluation.

Diagnostic Tests.:

Diagnostic Tests.

Presence of at least 3 strongly suggest it :

Presence of at least 3 strongly suggest it Redistribution of fat central obesity moon face buffalo hump Striae over abdomen & thigh Hypertention Proximal myopathy Emotional lability, irritability, insomnia, sometimes psychotic personality Osteoporosis Blood glucose: postprandial hyperglycemia 80% Elevated fasting blood glucose 20% Women with cushing’s syndrome sometimes features of adrenal androgen excess present recession of hairline, hiesutism, small breast & deepening of voice

CBC:

CBC Polycythemia present > half of the cases HG 14-16 gm/dl Anaemia – malignant tumour with ectopic ACTH TLC: 12000-20000 Lymphocyte < 20% Eosinophil : absent or few

Electrolytes:

Electrolytes Increase Sodium and CO2 Decrease potassium

Differentiation of cushing’s syndrome with an anxious individual with elevated plasma cortisol level:

Differentiation of cushing’s syndrome with an anxious individual with elevated plasma cortisol level 24 hour urinary cortisol estimation Normal: 10-50 microgm/24h Elevated > twofold- cushing’s syndrome False positive : Acute illness, Alcoholism, Depression

Suppression of ACTH and cortisol by dexamethasone :

Suppression of ACTH and cortisol by dexamethasone Assess feedback suppression of ACTH DXM- is 30 times potent ACTH suppressant than cortisol Procedure: 1 mg oral DXM at 11 PM Blood sample draw 8-9 AM Interpretation: Normal: 5-20mg/dl <5 mg/dl- Cushing’s syndrome ruled out > 10mg/dl Cushing’s syndrome present 5-10 mg/dl – equivocal, repeat/ measure urine cortisol

Tests for ACTH dependent/independent:

Tests for ACTH dependent/independent When cushing’s syndrome dx Plasma ACTH Level: Normal : 10-50 pg/ml Cushing’s disease: 10- 200pg/ml Ectopic ACTH syndrome: > 200 pg/ml Adrenal tumor: <5 pg/ml

Localizing source of ACTH:

Localizing source of ACTH Aim:1. Majority of patients have Cushing’ disease ( pituitary adenoma) 2. Ectopic ACTH sundromes have obvious malignancy MRI of pituitary- 50-60% positive Remainder should – venous sample of ant. Pituitary If both exclude pituitary lesion- CT scan of Chest and Abdomen to detect tumour

PowerPoint Presentation:

When suppressed ACTH Level- CT Scan of Abdomen with 3 mm section through Adrenals Adenomas are usually 3-6 cm in diameter Carcinomas are usually >5 cm in diameter Contralateral adrenal is atrophic / normal In Cushing’s disease or ectopic ACTH syndrome – bilateral adrenal enlargement is typical.

Complications:

Complications Hypertension-cardiac failure, stroke Secondary Diabetes Intractable skin/systemic infection Compression fracture of osteoporotic vertibra and ribs Renal stone due to bone resorption Psychosis

Treatment:

Treatment Cushing’s disease: Transsphenoid resection of pituitary adenoma, Success rate 80% Bilateral adrenal hyperplasia-no Tx needed Ectopic ACTH Syndrome: Removal of primary is the therapy of choice If not possible-bilateral adrenalectomy

Total bilateral Adrenalectomy:

Total bilateral Adrenalectomy Indication: Non-resectable pituitary tumour / ectopic ACTH secreting tumour with failed medical therapy to control cortisol excess

Peri-operative management:

Peri-operative management Danger: Temporary or permanent adrenal insufficency Start cortisol preoperatively and continue post-operatively On the day of operation 200mg, then reduce gradually on successive days 150, 100, 80, 60 and 40mg then maintenance dose 20-30 mg daily Hydrocortisone 10mg orally 3 times daily then reduce to 10 mg at 7-8 am daily within 2-3 weeks Substitution may be necessary up to 6 months to 2 yrs depending upon rate of recovery

Medical therapy:

Medical therapy Indication: Unfit for surgery Unsuccessful resection of Pituitary Ectopic Adrenal tumour Drugs: Ketoconazole, Metyrapone, Mitotane ( Cytotoxic)

PowerPoint Presentation:

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