An undiagnosed case An undiagnosed case of Paraganglioma: A of Paraganglioma: A nightmare for the nightmare for the AnesthesiologistAnesthesiologist

Presenters: Dr Amit Sabharwal Authors: Dr Amit Kohli, Dr Sanjay Nihlani, Dr Prashanth, Dr Sunil Thakur, Dr Sonia Wadhawan, Dr Poonam BhadoriaMaulana Azad Medical College and associated LN Hospital, New Delhi.

Patient ProfilePatient ProfileA 75 yr old male,ASA-1, with an intra-abdominal swelling provisionally diagnosed as retroperitoneal tumor was posted for laprotomy under GA • Pain abdomen- 2-3 months, Mass abdomen- 4 to 5 yrs• U/S: large retroperitoneal solid cystic mass 16x9.6x10.4 cm in dimensions CECT: large heterogeneously enhancing solid cystic mass with multiple enhancing septe Diagnosis: ??retroperitoneal mesothelioma, histopath correlation advised

Pre Anaesthetic Pre Anaesthetic EvaluationEvaluation• No significant past history- no h/o of any

ailment, previous operation,drug intake, & palpitations/chest pain ,family history

• O/E –BP 124/80, Pulse 64/min, R/R 16bpm,

Pallor present.–Cardiac , GI, Renal & Pul System NAD– Inv included Xray chest, ECG, Hemogram,

KFT, SE, & LFT , Urine R,M were all WNL except Hb which was 9 gm%

Optimization: 1 unit packed cell transfusedPlan: General anesthesia with epidural

analgesia

Perioperative EventsPerioperative EventsStandard anesthesia protocol:

On manipulation of mass Vitals, Ecg changes

Inj Xylocard, Inj propofol Vent Bigeminy

repeat dose of Inj Xylocard Vent Tachycardia,Palpable

pulses,hypertension manipulation stopped,inj amiadarone

bolus,infusion NTG infusion

vitals under control Sx continued ,mass resected!

Perioperative EventsPerioperative EventsSudden hypotensionFluid resuscitation

Stable hemodynamicsMonitored over 30 min,regular respiration

Extubatiion trial attemptedShifted to ICU

Uneventful postoperative period

•Epidural care• Histopathological report- paraganglioma with concurrent tuberculosis!

Differential DiagnosisDifferential Diagnosis?Intraoperative hypertension..?Hypertensive episode precipitating an arythymia?Undiagnosed catecholamine secreting tumour•Intraoperative events in retrospect point to the mass being a silent paraganglioma.•Though Clinical suspicion is primal in aiding diagnosis, lack of any signs and symptoms preoperatively caught us off guard• Retroperitoneum, a common site of occurrence for paragangliomas.

Plausible scenario for the anesthesiologist to encounter..

Pheochromocytoma,paraganPheochromocytoma,paragangliomasgliomas tumors arising from catecholamine producing

chromaffin cells (adrenal /extra adrenal). Not under neurogenic control Account for less than 0.1% of all cases of

hypertension in adults. Eitiology: isolated (90%), inherited

(10%),both sexes of any age affected 10% can turn malignant - liver,bone,spinal

cord,lungs,brain and lymph nodes. Most tumors secrete NE either alone or more

commonly combined with a smaller amount of EP (85:15). 15% secrete EP alone.

Topographic Topographic sitessites

Signs and symptomsSigns and symptoms

Catecholamine pathway and sites of action of various drugs

Preoperative managementPreoperative managementDetailed history,physical

examination,laboratory and cardiac evaluation.

Optimization with pharmacological agentsPreoperative blood pressure goals: no

standard guidelines: 130/80 mm Hg (while sitting),100 mm Hg systolic (while standing, not less than 80/45 mm Hg)

Heart rate: 60-70/min while sitting, 70-80/min while standing

Ref: Preoperative management of the pheochromocytoma patient. Karel Pacak. J Clin Endocrinol Metab. Nov 2007,92(11):4069-4079

Drugs used:Drugs used: Alpha adrenoceptor antagonists: phenoxybenzamine,prazosin,terazosin,doxazocin

Beta adrenoceptor antagonists: atenolol, metoprolol, propranolol

Combined alpha and beta receptor blockers: labetalol,carvedilol

Calcium channel blockers: amlodipine, nicardipine, nifedipine,varapamil

Catecholamine synthesis inhibitors

Intraoperative managementIntraoperative managementConcerns during induction, intubation, surgical

incision, abdominal exploration, manipulation, vein ligation,reversal.

Monitoring- noninvasive and invasiveAvoid morphine, atracurium, atropine,

pancuronium, succinylcholineSafe-

thiopentone,etomidate,benzodiazepines,fentanyl,

sufentanil,alfentanil,enflurane,isoflurane,nitrous oxide,vecuronium and rocuronium

Drugs useful prior to ligation SNP,phentolamine,niroglycerin,labetalol,magnesium sulphate,lidocaine,esmolol,amiadarone,diltiazem

Hypotension-most common cause of death(intra and post op) fluids,depth of anesthesia, vasopressors ( phenylephrine, norepinephrine) ,inotropes (dopamine)

Post operative:Vasopressor supportSteroid supplementationHypoglycemiaVentilatory support