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Phaeochromocytoma
Peri-operative
Anaesthetic
Management
Dr MA Jamkar
Lecturer in Anesthesia
BJ Medical College,Pune
Introduction
Phaeochromocytoma is a functionally active
catecholamine secreting tumor.
90% arises from chromafin cells in adrenal
medula.
10% arise from Para ganglia of sympathetic
nervous system-coeliac, mesenteric, renal,
hypogastric, testicular.
Mostly unilateral, about 9% bilateral.
Occurs in 4th and 5th decade of life, higher
preponderance in the female
10% of the tumors are found in children
Phaeo. Can be a part of autosomal
dominant multiple endocrine
neoplastic syndrome
 Multilple endocrine
adenomatosis type IIB
 Multiple endocrine
adenomatosis type IIA (
Sippel’s syndrome)
 Von Hippel- Lindau
syndrome
Parathyroid
adenoma/hyperplasia,
Medulary carcinoma of
thyroid,Phaeo.
Medularry carcinoma of
thyroid,mucosal
adenoma,Marfanoid app.,
Phaeo.
Haemangioplastoma of
cerebellum,retina or brain
and Phaeo.
Clinical presentation
Hypertension
Headache
Palpitation
Trembling
Sweating
Feeling of panic and
doom
Pallor,wtloss,anorexia
Nausea, vomiting
Phaeochromocytoma-
crisis
‱Hypertensive encephalopathy
‱Neurological deficit
‱Corneal blindness
‱Progressive metabolic acidosis and death
‱Left Ventricular failure
D-D
Thyrotoxicosis,Diabetes mellitus, malignant
hypertension, Carcinoid Syndrome,
Gram negative septicemia,
Cardiomyopathy, Eclampsia of pregnancy
Diagnostic Pointers
Tests Normal Values Range
Urinary
Metanephrine
<1.3mgs/24 hrs 0.3 –1.3 mgs/24
hrs
Urinary HMMA/
VMA
<9mgs/24 hrs 3-9 mgs/24 hrs
Plasma
catecholamine
Adri+Noradri
<1 ng/ml 0.8-1 ng/ml
One single test done on one occasion cannot be considered
diagnostic, It should be done on 2-3 occasions
Localisation
MRI
CT Scan
MIBG scan(Meta-iodobenzyl guanidine)
Selective adrenal vein
sampling for hormone
Catecholamine suppression test
Catecholamine provocation test
False reports may be found if strict dietary and drug restrictions
is not followed
The last three tests on are no longer required.
MRI for
Pheochromocytoma
MIBG scintiscan for
Pheochromocytoma
Clinical
Picture
GANGRENE
OF FINGERs
PAIN DURING
MICTURATION
‱Bending,
‱twisting
‱Change of
‱emotions
‱Post-prandial
‱hypoglycemia
Attacks pre-disposed by
Hyper
Tensive
Attacks
Weakness, hypotension follows
the attack
Severe enough to produce
gangrene – Toes, fingers.
Increased
Catecholamines in
Circulation
Alpha Adrenergic Effect
‱Tachycardia,Excessive
head ache,
‱sweating unrelated to
Temperature
‱Palpitation.Nervousness
,Circumoral Pallor
Beta Adrenergic
Diastolic
hypertension
Bradycardia
Feeling of Doom, Fear
I am going to Die
Preoperative Preparation
Close communication between
anesthetist ,surgeon and physician
should be present.
Once the diagnosis is made surgery is
the only curative procedure.
Pharmacological control of adverse effects of circulating catecholamine
Control of Hypertension
Tachycardia, arrhythmias,
Restoration of blood volume
Control of end organ damage
It is very important to diagnose and treat Pheo before surgery
Mortality of untreated Pheo with any surgery is as high as 50%
Alpha adrenergic antagonists
Phenoxybenzamine - non selective
Adv: long duration of action
Prevents intra op catacholamine surge
Dis Advt: Being non selective: Tachycardia,
arrhythmias , somnolence
Prazocin Doxazocin Terazocin - Selective
Beta adrenergic antagonists
Propranalol
Atenolol
Metoprolol
1.To control the effects of adrenaline
2. To block excessive cardiac sympathatic
drive secondary to alpha blockade
Suppression of Beta rece. mediated cardiac sympathetic activity in
Absence of adequate arteriolar dilation may precipitate
Ac. Pulmonary Oedema
Investigations
ECG
Echocardiography
BSL profile
Renal Function Tests
Renal scan
X-ray Chest
Serial Hematocrit
Serum Calcium
Laperoscopic Adrenalectomy
Sustained hemodynamic changes of
same severity as open surgery
Drugs to avoid
wide choice of drugs Avoid those causing pressor responses
1. Droperidol- inhibits catecholamine reuptake
2 Atropine- inhibits cardiac vagus
Potentiate chronotropic effects of cate.
3 Suxamethonium- involuntary muscle
fasciculation, can provoke cat.release
4. Tubocurarine, atracurium- histamine release
5. Pancuronium – sympathomimetic
6 Halothane – myocardial sensitization
DRUGS WHICH MAY BE USED
SAFELY
Fentanyl, Alfentalyl do not cause histamine
release – can be used freely.
Isoflurane advocated as no arrthymias or
myocardia sensitization.
Vecuronium – no histamine release, no
catecholamine release.
Nitrous oxide – no contraindication for use.
PHARMACOLOGICAL CONTROL
OF CATECHOLAMINE SURGE
DURING SURGERY
Many drugs have been used for this purpose.
Phentolamine
Sodium Nitroprusside
Nicardipine
Sodium nitro glycerin
Magnesium sulphate
Labetolol.
We used SNP + Esmolol infusion
ANAESTHESIA TECHNIQUE
Premedication – previous night. Diazepam,
alpha blocker, beta blocker, H2 blocker
Day of surgery – Inj. Midazolam IV,
Inj. Fentanyl, Inj. Reglan and inj. Ranitidine.
Anaesthesia – almost every anaesthetic
technique is tried. We used either balanced
general anaesthesia or epidural anaesthesia
+ general anaesthesia.
Surgery for
Pheochromocytoma
Open adrenalectomy
1.Lateral Retroperitoneal
2. Transabdominal
Laparoscopic surgery
1.Retroperitoneal
2. Transpertitoneal
Critical Steps in
Perioperative period
Intubation
During tumour manipulation
Immediately following ligation of
the venous drainage of the tumour
Preop. Steroids to be given
if bilateral adrenalectomy is to be planned.
MONITORING
NIBP
Pulse oximetry
ECG
ETCO2
Respiratory Gases
CVP
BSL
Urine output
Peripheral nerve stimulator
Temperature
After tumour excision
After ligation of last major vein: exponential
decrease of BP.
Fill the circulation with colloid solution either
haemaccel or hysteril to bring CVP upto 9
and 10.
Dopamine infusion
Hypotension may be because of removal of
active adrenal gland – opposite adrenal
suppressed.
Intra operative Control of
Catecholamine Release
Combination of regional + G.A. provides
satisfactory condition till tumour exposure
During Manipulation of tumour –brisk
Presser response :
1. Control of hypertension
with potent I V vasodilator eg SNP
2. Control of Tachycardia - with Beta blockers
Rise in BP is more pronounced in NORADR secreting tumour and
Tachycardia more so in ADRI + DOPAMINE secreting tumour
POSTOPERATIVE
PERIOD
Hypotension may persist because of long
acting alpha blockers.
Hypertension – extra adrenal pheo
contralateral adrenal pheo.
Somnolence – sudden withdrawal of
circulating catecholamines
Hypoglycemia – may lead to loss of
consciousness – blood glucose monitoring.
Malignant – non receptive pheo – Residual as
much as possible.
Pheochromocytoma :
case No 1
CONCLUSION
Pheochromocytoma remains a
great challenge to anaesthetists.
Condition continues to demand
great respect
Management of anaesthesia can
be highly stressful for
inexperienced single handed
anaesthetist.
Development of techniques like
laparoscopy only reduces the
hospital stay.
Successful outcome is a team
work.

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Phaeochromocytoma

  • 2. Introduction Phaeochromocytoma is a functionally active catecholamine secreting tumor. 90% arises from chromafin cells in adrenal medula. 10% arise from Para ganglia of sympathetic nervous system-coeliac, mesenteric, renal, hypogastric, testicular. Mostly unilateral, about 9% bilateral. Occurs in 4th and 5th decade of life, higher preponderance in the female 10% of the tumors are found in children
  • 3. Phaeo. Can be a part of autosomal dominant multiple endocrine neoplastic syndrome  Multilple endocrine adenomatosis type IIB  Multiple endocrine adenomatosis type IIA ( Sippel’s syndrome)  Von Hippel- Lindau syndrome Parathyroid adenoma/hyperplasia, Medulary carcinoma of thyroid,Phaeo. Medularry carcinoma of thyroid,mucosal adenoma,Marfanoid app., Phaeo. Haemangioplastoma of cerebellum,retina or brain and Phaeo.
  • 4. Clinical presentation Hypertension Headache Palpitation Trembling Sweating Feeling of panic and doom Pallor,wtloss,anorexia Nausea, vomiting
  • 5. Phaeochromocytoma- crisis ‱Hypertensive encephalopathy ‱Neurological deficit ‱Corneal blindness ‱Progressive metabolic acidosis and death ‱Left Ventricular failure D-D Thyrotoxicosis,Diabetes mellitus, malignant hypertension, Carcinoid Syndrome, Gram negative septicemia, Cardiomyopathy, Eclampsia of pregnancy
  • 6. Diagnostic Pointers Tests Normal Values Range Urinary Metanephrine <1.3mgs/24 hrs 0.3 –1.3 mgs/24 hrs Urinary HMMA/ VMA <9mgs/24 hrs 3-9 mgs/24 hrs Plasma catecholamine Adri+Noradri <1 ng/ml 0.8-1 ng/ml One single test done on one occasion cannot be considered diagnostic, It should be done on 2-3 occasions
  • 7. Localisation MRI CT Scan MIBG scan(Meta-iodobenzyl guanidine) Selective adrenal vein sampling for hormone Catecholamine suppression test Catecholamine provocation test False reports may be found if strict dietary and drug restrictions is not followed The last three tests on are no longer required.
  • 11. ‱Bending, ‱twisting ‱Change of ‱emotions ‱Post-prandial ‱hypoglycemia Attacks pre-disposed by Hyper Tensive Attacks Weakness, hypotension follows the attack Severe enough to produce gangrene – Toes, fingers. Increased Catecholamines in Circulation Alpha Adrenergic Effect ‱Tachycardia,Excessive head ache, ‱sweating unrelated to Temperature ‱Palpitation.Nervousness ,Circumoral Pallor Beta Adrenergic Diastolic hypertension Bradycardia Feeling of Doom, Fear I am going to Die
  • 12. Preoperative Preparation Close communication between anesthetist ,surgeon and physician should be present. Once the diagnosis is made surgery is the only curative procedure. Pharmacological control of adverse effects of circulating catecholamine Control of Hypertension Tachycardia, arrhythmias, Restoration of blood volume Control of end organ damage It is very important to diagnose and treat Pheo before surgery Mortality of untreated Pheo with any surgery is as high as 50%
  • 13. Alpha adrenergic antagonists Phenoxybenzamine - non selective Adv: long duration of action Prevents intra op catacholamine surge Dis Advt: Being non selective: Tachycardia, arrhythmias , somnolence Prazocin Doxazocin Terazocin - Selective Beta adrenergic antagonists Propranalol Atenolol Metoprolol 1.To control the effects of adrenaline 2. To block excessive cardiac sympathatic drive secondary to alpha blockade Suppression of Beta rece. mediated cardiac sympathetic activity in Absence of adequate arteriolar dilation may precipitate Ac. Pulmonary Oedema
  • 14. Investigations ECG Echocardiography BSL profile Renal Function Tests Renal scan X-ray Chest Serial Hematocrit Serum Calcium
  • 15. Laperoscopic Adrenalectomy Sustained hemodynamic changes of same severity as open surgery
  • 16. Drugs to avoid wide choice of drugs Avoid those causing pressor responses 1. Droperidol- inhibits catecholamine reuptake 2 Atropine- inhibits cardiac vagus Potentiate chronotropic effects of cate. 3 Suxamethonium- involuntary muscle fasciculation, can provoke cat.release 4. Tubocurarine, atracurium- histamine release 5. Pancuronium – sympathomimetic 6 Halothane – myocardial sensitization
  • 17. DRUGS WHICH MAY BE USED SAFELY Fentanyl, Alfentalyl do not cause histamine release – can be used freely. Isoflurane advocated as no arrthymias or myocardia sensitization. Vecuronium – no histamine release, no catecholamine release. Nitrous oxide – no contraindication for use.
  • 18. PHARMACOLOGICAL CONTROL OF CATECHOLAMINE SURGE DURING SURGERY Many drugs have been used for this purpose. Phentolamine Sodium Nitroprusside Nicardipine Sodium nitro glycerin Magnesium sulphate Labetolol. We used SNP + Esmolol infusion
  • 19. ANAESTHESIA TECHNIQUE Premedication – previous night. Diazepam, alpha blocker, beta blocker, H2 blocker Day of surgery – Inj. Midazolam IV, Inj. Fentanyl, Inj. Reglan and inj. Ranitidine. Anaesthesia – almost every anaesthetic technique is tried. We used either balanced general anaesthesia or epidural anaesthesia + general anaesthesia.
  • 20. Surgery for Pheochromocytoma Open adrenalectomy 1.Lateral Retroperitoneal 2. Transabdominal Laparoscopic surgery 1.Retroperitoneal 2. Transpertitoneal
  • 21. Critical Steps in Perioperative period Intubation During tumour manipulation Immediately following ligation of the venous drainage of the tumour Preop. Steroids to be given if bilateral adrenalectomy is to be planned.
  • 22. MONITORING NIBP Pulse oximetry ECG ETCO2 Respiratory Gases CVP BSL Urine output Peripheral nerve stimulator Temperature
  • 23. After tumour excision After ligation of last major vein: exponential decrease of BP. Fill the circulation with colloid solution either haemaccel or hysteril to bring CVP upto 9 and 10. Dopamine infusion Hypotension may be because of removal of active adrenal gland – opposite adrenal suppressed.
  • 24. Intra operative Control of Catecholamine Release Combination of regional + G.A. provides satisfactory condition till tumour exposure During Manipulation of tumour –brisk Presser response : 1. Control of hypertension with potent I V vasodilator eg SNP 2. Control of Tachycardia - with Beta blockers Rise in BP is more pronounced in NORADR secreting tumour and Tachycardia more so in ADRI + DOPAMINE secreting tumour
  • 25. POSTOPERATIVE PERIOD Hypotension may persist because of long acting alpha blockers. Hypertension – extra adrenal pheo contralateral adrenal pheo. Somnolence – sudden withdrawal of circulating catecholamines Hypoglycemia – may lead to loss of consciousness – blood glucose monitoring. Malignant – non receptive pheo – Residual as much as possible.
  • 27. CONCLUSION Pheochromocytoma remains a great challenge to anaesthetists. Condition continues to demand great respect Management of anaesthesia can be highly stressful for inexperienced single handed anaesthetist. Development of techniques like laparoscopy only reduces the hospital stay. Successful outcome is a team work.