adrenal hypertension
TRANSCRIPT
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ADRENAL AND EXTRA-ADRENAL
HORMONE SECRETING TUMOURS
A SEVEN YEARS EXPERIENCE AT RGH
Dr.Nadir Mehmood
Assistant ProfessorSurgery
Rawalpindi Medical College
Rawalpindi
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INTRODUCTION
Small in size but powerful in influence , the Adrenal glands and theirhormones are necessary to sustain life and maintain homeostasis.
Adrenal glands were overlooked by early anatomist and were firstaccurately described in 1563 by Bartholomeos Eustachius.
More than 300 years passed before there was some understandingof the functions of these glands.
Adrenal and extra adrenal tumors are rare and often present laterwhen physiological derangement is well established .
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INTRODUCTION
Despite the rarity of such tumors there have beensignificant advances in the understanding andmanagement of these tumors in the recent years.
Disorders of Adrenals and the related structure maypresent in a variety of ways depending on the effects ofhormones they produce.
Even such tumors may produce mass effect or be an
incidental finding in the course of investigating some
other problem. So such findings do not always warrant treatment.
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PHYSIOLOGY
Hyperfunction of adrenal gland is divided into threecategories depending upon the main hormone ofsecretion
1: Hypercotisolism
Cushing syndrome ( primary adrenal pathology
Cushings disease (secondary to raised ACTH)
2: Hyperaldosteronsim
Conns syndrome ( primary adrenal pathology)
Secondary hyperaldosteronism ( chronic renalfailure
3: Hypercathecholaminemia
Adrenal glands tumors
Extra adrenal chromoffin tissue tumors
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PHSYIOLOGY
As these adrenal hormones regulate a wide range ofphysiological functions,it is not surprising that hyperplastic orneoplastic conditions of adrenal and extra-adrenal tissues cancause excessive secretion of various hormones resulting in avariety of clinical syndrome
The presentation and diagnosis of adrenal and extra-adrenaltumors secreting hormones is complex and the discriminatingfactors for each are necessary to minimize the time and costin diagnosis.
Because of the rarity of such tumors, most of the studies in
the literature are retrospective in nature.
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PURPOSE OF THE STUDY
To evaluate a broad range of features ,including
Clinical Features
Evolution of disease/syndrome
Hormone secretion
Imaging technique
Problems during anesthesia
Approaches to the exposure of the related
structures Technique used
Intra and postoperative morbidity and mortality
Histopathological diagnosis
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METHOD
A detailed history, especially pertaining to headache,sweating,palpitation,flushing,mass or pain abdomen,uncontrolled hypertension, breathlessness, episodicweakness of limbs or tremors in hand, nausea,vomiting or diarrhea and weight loss/gain wasrecorded on data sheets.
History of any medical treatment for the presentingsymptoms/complaints and their efficacy in relievingthe complaints was recorded.
Previous medical and surgical history,family H/O anydisease like hypertension,diabetes or obesity or tumorof any site was recorded.
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METHOD
A detailed clinical examination of each patient includingweight and height, vital signs, skin, face, neck, hairdistribution, extremities and abdomen was performed.
A record of blood pressure was analyzed, especiallyafter the abdominal examination/palpitation in patientswith pheochrmocytoma.
Patients with visual complains and headaches weretested for visual acuity and in patients with severe
uncontrolled hypertension, fundoscopy was alsoperformed.
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INVESTIGATIONSRoutine investigations on all patients
C.P. E.S.R
R/E urine
B.S.R
Urea and creatinine
Serum electrolyte Blood grouping
E.C.G
Chest X-ray P.A view
Special tests were selectively performed including,
L.F.Ts
I.V.U
Echocardiography
Serum hormones level - cortisol, aldosterone, renin andurinary VMA
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INVESTIGATIONS
Routine imaging included
- Ultrasound examination
- C.T scan on all patients Results of all these investigations
were listed in tabulated form and
the findings of ultrasound, C.T scanand operative findings werecomparatively analyzed.
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OPERATIONS A total 14 operations were performed in 13 patients. One patient with bilateral adrenal pheochromocytoma was operated
twice , once at a time.
2 patients were not operated, one with incidentaloma, and the otherwith right adrenal pheochromcytoma with multiple metastasis in theliver ( Pt.refused operation )
Preoperative blood pressure was controlled using a combination ofdrugs which included
- Diovan(Valsartan)
- Prazocin
- Nifidipine
- Atenolol
- Frusemide
for patients with pheochromocytoma and Cushing syndrome and- Spinolactone for patients with Conns synd.
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TECHNIQUE
Antibiotic prophylaxis as 1 gm of 1st generationCephalosporin I/V was given to all the patientsfollowed by two doses postoperatively.
General anesthesia by a senior anesthetist. Posterolateral approach through the bed of 12th rib
in 11patients.
Anterior midline in 2 patients with paraganglionoma
and pheochromocytoma of organ Zukerkandle.
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Peroperative tachyarrhthmia during themanipulation of pheochromocytoma wascontrolled by infusion of lignocaine, and rise inB.P was controlled by nitrocine infusion.
Postoperative hypotension of varying degreeoccurred in almost all patients withpheochromocytoma, even the one with bilateraladrenal pheochromocytoma which wascontrolled by infusion of:
0.9% N/Saline
Gelafundin
Blood transfusion
Infusion of dopamine
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Replacement of fluid ranged from 3.5 litres to 7 litres
Average 5.25 litres as
-N/saline 2000ml
-Gelafundin 100ml
-5% D/W 1000ml-2 units of blood.
Coticosteriod replacement was given to all patientsafter surgery for Cushings syndrome and after secondoperation on patient with bilateral pheochromocytoma.
The patients were followed up for an average of 2.5years, ranging from 12 months to 5 years
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PRESENTATION /CLINICAL
FEATURES
PHEOCHROMOCYTOMA
Recurrent episodes of severe headache,sweating palpitation and flushing ( face, handsand chest) 7
Triad of headache, palpitation and sweating withmass in Rt.H.C and Rt.L.R 1
Pain in Rt.L.R and Rt.H.C.associated with triad1
PARAGANGIONOMA
Mass in Rt.H.C 1
Hypertension not controlled by ordinary dosageof antihypertensive drugs
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PRESENTATION /CLINICAL
FEATURES
CONN SYNDROME
Episodic weakness of limbs and tremors in the hand 3
Adding headache,palpitation and breathlessness 1
Other symptoms of nausea, vomiting and diarrhea 1
CUSHING DISEASE
Obesity,breathlessness on exertion, mood changes and easybruisibility. 2
Off and on complain of backache, joint pain, increased appetite, weightgain, sweating, vertigo and headache
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Age
Most of the patients were young
Age Range 15 - 45 years
Average age 31.066 years
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ON CLINICAL EXAMINATION
Pulse rate Range 80-96/min
Av 80/min
B.P Systolic range 140-240mmhg
Av 196
Diastolic range 95-150mmHg
Av 128.57mmHg
R/R Range 18-28/min
Av 20/ min
Weight 125kg=2 with cushing Incident 130kg
Ht 5 . 1, 5 . 2 and 5 . 4
Range 46-96kg with pheo and conn Av 62.09kg
Height 4 . 10 - 5 . 8Av 5 . 2
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ABDOMINAL EXAMINATION
Mass abdomen in3 cases - all pheo (20% of all cases )
Visible bulge and palpable mass that was ballotable andmoved with respiration.
After completing the abdominal examination patientstypically experienced episodes of headache, sweatingand flushing -- all pheo cases
So medical students and junior residents were refrainedfrom examination of patients with suspectedpheochromocytoma.
Striae distentie on abdomen, chest and limb in boththe cases of Cushings and incedendaloma.
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INVESTIGATIONS
H.b Range 9.6 - 15gm
Av. 11.6533gm
T.L.C Range 4400-8900
Av. 6200
E.S.R Range 10-90mn
Av. 23.6mn
Urine R/E Albumin +/++3 cases
B.S.R
S.Urea/ creatinine
L.F.Ts Select
H.B.V and C Screen all
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INVESTIGATIONS
S.Electrolytes
Na Range N Av N
K Range 1.3-2.6 Av 2(conns only)
Cl Range N Av N
Ca Range N Av N
S.Renin Range 0.6-2.62
Av 1.32666
S.Aldosterone Range 35.9-130
Av 91.8666
S.Prolactin 789.2 (N-61-512.m.I.u/ml)
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Three measurements of 24-hr urinary VMA
in 10 patients of pheochromocytoma and
incidentaloma
Range 4 - 15.6mg / 24hr
Average9.1972mg / 24hr
Normal upto 8 mg / 24 hrNo relation with the size of the tumor
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Tumor N Male Female Rt Lt B/L
Total 15 5 10 7 5 1
Adrenal 13 5 8 7 5 0
Pheo 7 4 3 4 2 1
Conn 3 1 2 2 1 0
Cush 2 0 2 1 1 0
Incid 1 0 1 0 1 0
Pheo OrgZ 1 0 1 0 0 0
Paragang 1 0 1 0 0 0
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PREOP-PREPARATION Patients were admitted on an average of 5 days preoperatively B.P control with
Cap Diovan ( Valsartan) 80mg OD
Tab minipres ( prazocin ) 1 mg - 2mg T.D.S
Cap Adalet ( nifidipine) 10 mg-20 mg T.D.S
Tab Atenolol 50mg-100mg O.D/ B.D
Tab Frusemide 20mg-40mg O.D or B.DTab Spinoloctone 100mg TDS
Tab Voltaral( diclfenac) 50mg BD for pain
Correction of iron deficiency with hematanics
Fluids
C.V.P was passed in Rt.Subclavian vein via infraclavicular route in O.T.
preoperativly.
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DURATION OF SURGERY
Range 1.75 hrs - 5.5hrs
Average 2.5 hours
Postoperative stay
Range 7 - 15 days Average 9.3 days
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MORBIDITY
No wound infection
Chest infection in 1 patient
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MORTALITY
One patient with paraganglianoma
suffered from cardiac arrestperoperatively.
Died on 3rd postoperative day due toC.V.A. and respiratroy distress.
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FOLLOW UP
After two months of follow up all the
patients were symptomatically improvedwithout any antihypertensive therapy.
Serum electrolyte and other laboratory
data became normal after the first week ofoperation.
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S. No. U/S C.T Scan V.M.A Perop Finding
1. 83 X 68mm 80 X60mm 4.7mg/ 24hr 100 X 90mm2. Normal 30 X 20mm 7.5mg/ 24hr 30 X 20mm3. 40 X 16mm 24 X 25mm 9.5mg/ 24hr Not operated4. Normal 6.0mg/ 24hr 5. 55 X 52 X 40mm 45 X 38 X 48mm 15.6mg/ 24hr 45 X 35 X 45mm6. 120 X 80 X 50mm 150 X 90X 60mm 13.2mg/ 24hr 150 X 90 X 60mm7. (Rt) 35 X 39mm
(Lt) 36 X 24mm41.27 X 35.63
36 X 24mm40 X 50mm30 X 40mm
8. 65 X 80mm 58 X 75mm 9.5mg/ 24hr 55 X 75mm9. 30 X 38mm 35 X 40mm 11.2mg/ 24hr 35X 40mm10. 40 X 29mm 40 X 33mm 9.0mg/ 24hr 40 X 30mm11. Huge mass>150mm Huge mass>150mm 7.5mg/ 24hr Not operated12. 15 X 12mm 15 X 12mm 15 X 10mm13. 35 X 40mm 35 X 40mm 7.6mg/ 24hr 35 X 40mm14. 25 X 30mm 26 X 32mm 25 X 30mm15. 50 X 40mm 51 X 40mm 9.5mg/ 24hr 50 X 40mm
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ConclusionYoung hypertensive patients should be
investigated thoroughly.
Pre-op preparation is essential to succesful
management of such patients.
Comparing theresult of U/S, C.T. and operativefindings, U/S had a sensitivity of 86.6% in
diagnosing tumors compared with 100%
sensitivity of C.T. scan.Urinary VMA excretion was not related to size of
the tumor.
Over-night dexamethasone test has good
diagnostic yield in hypercathecholaminia.
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Cortical sparing adrenalectomy is
a good option in patients with
bilateral tumors.
Sequential operation is better
choice to simultaneous surgery in
patients with bilateral tumors.