Karim Said. 41 year old farmer. Referred from the Uro-surgery Department because of uncontrolled hypertension prior to Lt. partial nephrectomy

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1 Case Presentation Karim Said Cardiology Departement Cairo University 41 year old farmer Referred from the Uro-surgery Department because of uncontrolled hypertension prior to Lt. partial nephrectomy ١

2 At the age of 25 : - Loin pains, dysuria, hematuria - Multiple Rt. & Lt. renal stones - Recurrent attacks of pyelonephritis - Lt. side hydronephrosis - Serum creatinine: mg/dl At the age of 30 - Accidentally discovered HTN, (230/150 mmhg) - Uncontrolled despite multiple antihypertensive regimen - Hypertension was attributed to the renal disease - His older sister (50 y) is also hypertensive ٢

3 Symptoms review Breathlessness on ordinary effort 3 months ago Bony pains Current cigarette smoker Examination Looks healthy; no pallor. Afebrile BP: 180/ mmhg Pulse: 82 bpm & regular & +++ Vascular system: normal & no bruits JVP: not elevated Neck: no goitre, adenopathy, or bruits Chest: NAD Abdomen : only old suprapubic scar. Tender Lt. Renal angle Heart: - forcible LV apex -apical S4 ٣

4 Laboratories Creatinine: 1.9 mg/dl egfr: 52 ml/min (CKD; stage III) Urinalysis: - Granular casts - Pus cells: /HPF Serum K: 4.3 meq/l Hb: 12 gm/dl WBC: /cc FBS: 107 mg/dl ECG: NSR & LVE R: Long acting nifedipine : 80 mg/day Furosemide : 20 mg IV three times daily Metoprolol: 200 mg/day 10 days later: BP: 150/90 mmhg Surgery was scheduled ٤

5 After induction of anesthesia: - Sudden elevation of BP to 280/160 mmhg - Before institution of IV vasodilator, BP went down to 70/30 mmhg warranting the use of saline & IV norepinephrine - This was associated with sinus tachycardia ( 130 bpm) - Surgery was postponed Re-assessment BP: 165/100 mmhg (supine) 140/90 mmhg (standing) Pulse: 80 bpm & regular Clinical examination: NAD ECG: NSR & LVE Revision of previous investigations: An abdominal sonar (performed 1 year ago) was - An abdominal sonar (performed 1 year ago) was suspicious for a mass (2x3 cm) related to the upper pole of the Rt. Kidney (Inflammatory mass) ٥

6 ?? Pheochromocytoma Clues Severe HTN during anesthasia induction Postural hypotension Rt. Suprarenal mass But No symptomps of catecholamine excess Rt. Suprarenal mass may be an incidentaloma Rare - 1/1000 hypertensive patients - in patients suspected for pheochromocytoma, the diagnosis is established in only 1 of 300?? Pheochromocytoma I. Anethasia-induced hypertensive crisis - Well known precipitant for pheo spell -Most deaths are related to failure to consider pheo in patients undergoing surgery,delivery II. Rt. Suprarenal mass - Any adrenal mass should be evaluated dfor hypersecretion, malignancy ٦

7 ?? Pheochromocytoma III. Postural hypotension - 50% of patients with pheo - Hypotension - Contracted intravascular volume - Pheos secreting predominantly epinephrine - Spontaneous necrosis of the tumor - Catecholamine induced cardiomyopathy - VIP secretion severe diarrhea & dehydration?? Pheochromocytoma I. No symptoms of catecholamine excess - 10% of pheo patients lack the triad of: headache sweating- palpitation - Spells are not specific. Most patients with spells do not have a pheochromocytoma II. Rt. Suprarenal mass may be an incidentaloma - 12% of hypertensive patients may have benign nonfunctioning adrenal masses > 1cm ( incidentalomas) - 3 to 10 % of adrenal incidentalomas proved to be pheochromocytomas III. Rare - 1/1000 hypertensive patients - in patients suspected for pheochromocytoma, the diagnosis is established in only 1 of 300 ٧

8 ?? Pheochromocytoma - Morphologically benign, yet physiologically malignant High mortality risk k( (20%) especially during stress (surgery) Cerebral Hge, renal failure, APO Cardiomyopathy, myocarditis, MI, ventricular arrhythmias Aggressive atherosclerosis Malignancy (10%) Familial (10%) Making the diagnosis I. Biochemical tests -Urinary -Plasma II. Tumor localization -CT, MRI - MIBG ٨

9 I. Biochemical tests Making the diagnosis - Urinary hf fractionated t metanephrine hi h fractionated catecholamine h total metanephrinre h total catecholamine h VMA -Plasma 1. Fractionated metanephrine 2. Fractionated catecholamine 3. Total metanephrinre 4. Total catecholamine Making the diagnosis I. Biochemical tests Results Normal 24 h total catecholamine (ug) h VMA (mg) ٩

10 II. MRI III. MIBG ١٠

11 A Link? Pheochromocytoma Multiple l renal stones Bony pains Hypercalcaemia * Total serum calcium : 11.4 & 11.7 mg /dl Pheos and hypercalcaemia Value of hypercalcaemia in pheos 1. Renal stones & obstructive uropathy 2. Hypercalcemia HTN 3. MEN IIA: * Pheochromocytoma (50%) Pheochromocytoma (50%) * Hyperparathyroidism (15%) * Medullary thyroid carcinoma (90%) ١١

12 Pheos and hypercalcaemia Value of MEN IIA in pheos 1. Thyroid carcinoma (90%) 2. Family screening (autosomal dominance with 100% penetrance for medullary thyroid carcinoma) 3. Bilateral pheochromocytoma (50%) Pheos and MEN AII Serum PTH : normal (13 pg/ml) Thyroid exam: free Family history: free ١٢

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14 Diagnosis: 1. Sporadic Rt. Adrenal Pheochromocytoma 2. Hypercalcaemia (Humoral hypercalcaemia of benignancy) 3. Obstructive uropathy 4. CKD (stage III) R: Doxazosine : 6 mg/day Metoprolol: 200 mg/day Blood pressure still uncontrolled. - Long acting nifedipine 40 mg/day was added - BP: /85-95 mmhg No postural hypotension ١٤

15 Preopera Perioperative management Full adrenergic receptor blocker (α,ß) Volume expansion (2L saline) Hypertensive crisis: 1. IV phentolamine (of choice) 2. IV nitroprusside ( if resistant) 3. IV esmolol (if arrythmias) After Pheo resection: aggressive volume replacement blood sugar monitoring (hypoglycemia) Outcome Smooth postoperative course 2 weeks postoperatively: - BP: 140/85 mmhg (off medications) - Serum creatinine: 1.5 mg/dl - Total serum calcium: 8.7 mg/dl ١٥

16 Take Home Messages A paradoxical blood pressure response to anesthesia is a well- documented observation in unrecognized pheochromocytoma; one should prompt consideration of its possibility, even in asymptomatic individuals. Take Home Messages A paradoxical blood pressure response to anesthesia is a well- documented observation in unrecognized pheochromocytoma; one should prompt consideration of its possibility, even in asymptomatic individuals. Patients with pheos who are not under adequate BP control have high surgical mortality (20%) ١٦

17 Take Home Messages A paradoxical blood pressure response to anesthesia is a well- documented observation in unrecognized pheochromocytoma; one should prompt consideration of its possibility, even in asymptomatic individuals. Patients with pheos who are not under adequate BP control have high surgical mortality (20%) Lack of typical symptoms of pheochromocytoma does not preclude the diagnosis. Take Home Messages A paradoxical blood pressure response to anesthesia is a well- documented observation in unrecognized pheochromocytoma; one should prompt consideation of its possibility, even in asymptomatic individuals. Patients with pheos who are not under adequate BP control have high surgical mortality (20%) Lack of typical symptoms of pheochromocytoma does not preclude the diagnosis. Plasma calcium should be measured in patients with Pheos. ١٧

18 Take Home Messages A paradoxical blood pressure response to anesthesia is a well- documented observation in unrecognized pheochromocytoma; one should prompt consideration of its possibility, even in asymptomatic individuals. Patients with pheos who are not under adequate BP control have high surgical mortality (20%) Lack of typical symptoms of pheochromocytoma does not preclude the diagnosis. Plasma calcium should be measured in patients with Pheos. In patients with pheos, components of MEN IIA should be ruled out or in. ١٨

Pheochromocytoma AMERICAN ASSOCIATION OF CLINICAL ENDOCRINOLOGY ILLINOIS CHAPTER OCTOBER 13, 2018

Pheochromocytoma AMERICAN ASSOCIATION OF CLINICAL ENDOCRINOLOGY ILLINOIS CHAPTER OCTOBER 13, 2018 Steven A. De Jong, M.D., FACS, FACE Professor and Vice Chair of Surgery Chief, Division of General Surgery