|Year : 2015 | Volume
| Issue : 2 | Page : 73-77
Laparoscopic adrenalectomy in a pediatric patient
Sheetal Dalal, Ashwini Ukey
Department of Anaesthesia, Government Medical College and Hospital, Nagpur, Maharashtra, India
|Date of Web Publication||16-Jul-2015|
Dr. Sheetal Dalal
13, Pub Cooperative Housing Society, Pratap Nagar, Nagpur, Maharashtra
Source of Support: None, Conflict of Interest: None
A pheochromocytoma is a highly vascular neuroendocrine tumor of the medulla of the adrenal glands, originating from the chromaffin cells or extra-adrenal chromaffin tissue and secreting an excessive amount of catecholamines. Medical management of this tumor includes correction of hypertension and arrhythmias, and assessment of end organ damage. This can be achieved by alpha-receptor blockade and beta-receptor blockade, calcium channel blockers (CCBs), alpha-methyl-tyrosine, etc. Surgical excision of the tumor, either open laparotomy or laparoscopy, is the definitive management. A laparoscopic approach offers several advantages compared with an open laparotomy such as decreasing fluid shifts that may accompany an open procedure, potentially decreasing the surgical stress imposed on the patient, decreasing the need for postoperative analgesia, shortening postoperative convalescence including an intensive care unit stay, and decreasing the overall hospital stay. A 11-year-old male patient with left adrenal mass measuring 2.9 cm × 3.9 cm × 4.7 cm on alpha-blocker and beta-blocker for laparoscopic adrenalectomy was successfully managed perioperatively.
Keywords: Pheochromocytoma, laparoscopic adrenalectomy, vertebrobasilar dolichoectasia
|How to cite this article:|
Dalal S, Ukey A. Laparoscopic adrenalectomy in a pediatric patient. J Health Res Rev 2015;2:73-7
| Introduction|| |
A pheochromocytoma is a highly vascular neuroendocrine tumor of the medulla of the adrenal glands, originating from the chromaffin cells or extra-adrenal chromaffin tissue and secreting excessive amount of catecholamines. In adults, approximately 80% of pheochromocytomas are unilateral and solitary, 10% are bilateral, and 10% are extra-adrenal and these may grow to large size (>3 kg); most weigh <100 g and are <10 cm in diameter. These are seen in two to eight persons in a population of 1,000,000 and mostly occur in young or middle-aged adults, though they present earlier in hereditary cases. Up to 25% of pheochromocytomas may be familial, associated with multiple endocrine neoplasia type 2 A (MEN2A), MEN2B, von Recklinghausen disease, or von Hippel-Lindau syndrome. Most people (90%) with pheochromocytoma have hypertension. 
The other symptoms usually occur in paroxysms lasting from a few minutes to days and include headaches, excess sweating, palpitations, anxiety, nervousness, nausea, and pain in the lower chest or the upper abdomen followed by exhaustion and fatigue. The classical triad of pheochromocytoma comprises headache, perspiration, and palpitations. Weight loss is also seen. Orthostatic hypotension occurs due to extracelluar fluid contraction and loss of postural reflexes in response to prolonged catecholamine stimulation. 
The best confirmatory test is to measure free catecholamines and their metabolites in a 24-h urine collection. These measurements include epinephrine, norepinephrine, dopamine, metanephrine, and vanillylmandelic acid (VMA). Measurement of the plasma catecholamines only reflects that single moment when the blood sample was collected. Radiological imaging studies include ultrasonography (USG), computed tomography (CT) scan, and metaiodobenzylguanidine (MIBG) scan. 
Medical management includes correction of hypertension and arrhythmias, and assessment of end organ damage. This can be achieved by alpha-receptor blockade and beta-receptor blockade, calcium channel blockers (CCBs), alpha-methyl-tyrosine, etc. Surgical excision of the tumor, either open laparotomy or laparoscopy, is the definitive management. 
A laparoscopic approach offers several advantages compared with an open laparotomy such as decreasing fluid shifts that may accompany an open procedure, potentially decreasing the surgical stress imposed on the patient, decreasing the need for postoperative analgesia, shortening postoperative convalescence including an intensive care unit stay, and decreasing the overall hospital stay. Contraindication for laparoscopy are large tumor(s) of size >6 cm and malignant tumor(s) with extensive adhesions. The anesthetic management for laparoscopic adrenalectomy is different from an open procedure due to the creation of a pneumoperitoneum, which causes an increase in the intra-abdominal pressure and can induce catecholamine release by the pheochromocytoma. A 11-year-old male patient with left adrenal mass measuring 2.9 cm × 3.9 cm × 4.7 cm on alpha-blocker and beta-blocker for laparoscopic adrenalectomy was successfully managed perioperatively. ,
| Case Report|| |
A 11-year-old male patient weighing 25 kg presented with repeated history of headache, increased sweating, polyuria, polydipsia, and epistaxis. He was operated for antrochoanal polyp under general anesthesia 15 days ago. Though the intraoperative and immediate postopertative periods were uneventful, he presented with bleeding from the nose 2 days after the surgery and nasal packing was done. He was diagnosed to have uncontrolled hypertension (200/120 mmHg). He was, then, referred to our institute for further management. In the past, he had one episode of convulsion for which he had been taking carbamazepine tablet since the last 1.5 years.
Routine laboratory and radiological investigations were done, along with Urinary Vanilly mandelic Acid U. VMA in 24 h - 35.4 mg (n < 7 mg); CT of the abdomen revealed the left adrenal mass approximately measuring 2.9 cm × 3.9 cm × 4.7 cm. Records of pulse rate (PR) noted daily, blood pressure (BP), and input/output charts were maintained. The patient had persistent tachycardia and hypertension. A diagnosis of phechromocytoma was done. Other investigations like those concerning Sr. prolactin 3.38 ng/mL (n 2.1-17.7 ng/mL), Sr. parathyroid hormone (PTH) 27.56 pg/mL (n 15-65 pg/mL), Sr. calcium 9.4 mg% (n 9-11.5 mg%), Sr. osmolality 274.4 mmol/L (n 295-350 mmmol/L), U. osmolality 448.39 mOsm/kg H 2 O (n 500-850 mOsm/kg H 2 O), urine specific gravity test value 1.015, and a normal thyroid profile test were done; his negative family history ruled out association with the MEN syndrome. Magnetic resonance imaging (MRI) head revealed vertebrobasilar dolichoectasia.
He was started with prazosin tablet of dosage 6.25 mg four times a day (q.i.d.) (25 μg/kg), propranolol tablet of dosage 12.5 mg twice daily (b.d.) (1 mg/kg/day), and Tegretol tablet of dosage 200 mg b.d. With this regimen, the patient had adequate heart rate (HR) and BP control (HR 80-100 bpm and BP 130-140/80-90 mmHg). Pediatric surgeons planned laparoscopic left adrenalectomy. In due course of his hospital stay, he developed lower respiratory tract infection that was treated with antibiotics and nebulization.
In the preanesthetic checkup, detailed history was noted. On examination, his HR was noted to be 120 bpm, respiratory rate (RR) 20 breaths/min, and BP 130/90 mmHg and Bilateral Basal crepititions were noted. No airway difficulties were noted. Routine investigations were within normal. X-ray of the posterioranterior (PA) chest view was within normal limits (WNL). Electrocardiogram (ECG) showed sinus tachycardia. Two-dimensional echocardiography was ordered that revealed mild left ventricle dilatation, good left ventricular (LV) function, and left ventricular ejection fraction (LVEF) to be 60%. On fundus examination, he had Grade IV hypertensive retinopathy.
Surgery was planned under general anesthesia and thoracic epidural anesthesia. The equipment for invasive BP and central venous pressure (CVP) monitoring were kept ready, along with the routine monitors. Antihypertensive drugs like nitroglycerin (NTG), Sodium Nitroprusside ( SNP), phentolamine, esmolol, metoprolol, etc. Inotropes such as dopamine, dobutamine, noradrenaline, isoprenaline, phenylephrine, insulin, and 25% dextrose, along with routine resuscitative equipment and medications were kept ready.
All the antihypertensive medications, antibiotics, and nebulization were continued till the day of surgery. Adequate hydration was maintained and adequate blood was kept ready if required in the intraoperative period. Records of PR noted daily, BP, and input/output charts were maintained. Fresh Sr. electrolytes and ECG were ordered on the day of surgery, which was WNL.
With written informed high-risk consent, intravenous (IV) administration of midazolam of dosage 0.5 mg was given outside the operating room (OR); the patient was taken inside the OR on a wheelchair wheel chair. Monitors like those to study noninvasive blood pressure (NIBP), peripheral capillary oxygen saturation (SpO 2 ), and ECG were attached. Following basal parameters were noted: PR 100 bpm, BP 150/100 mmHg, and random blood sugar (RBS) 70 mg%. Five percent dextrose normal saline (approximately 225 mL) was infused. He was, then, premedicated with IV glycopyrrolate of dosage 0.1 mg and IV Rantac of dosage 25 mg. Preoxygenation with 100% oxygen was done. IV fentanyl of dosage 60 μg and IV propofol of dosage 50 mg were given for induction, IV lidocaine of dosage 37.5 mg was given and after confirming mask ventilation and IV vecuronium of dosage 2.5 mg was given as muscle relaxant. The patient was ventilated with oxygen and inhalational sevoflurane on Bain circuit for 3-4 min. His trachea was intubated with Cuffed Endotracheal tubeb no. 5.5. Correct placement of the endotracheal tube was confirmed by the end-tidal CO 2 (ETCO 2 ) monitor and B/L chest auscultation. Anesthesia was maintained with O 2 , N 2 O, and inhalational sevoflurane with IV Vec of dosage 0.25 mg top-ups for muscle relaxation. Urinary catheterization was done and urinary output (U/O) was 200 mL. Intra-arterial and central venous catheterization was done. An 18-G epidural catheter was inserted in the T9-T10 interspace and fixed at 8-cm mark. Epidural top-up with 6-cc 0.25% bupivacaine was given after test dose. IV hydrocortisone of dosage 50 mg and IV Deriphyllin 1 cc were given in view of the respiratory status.
Surgery proceeded in the radiotherapy (RT) lateral decubitus position and pneumoperitoneum was created. Vitals including HR, invasive blood pressure (IBP), CVP, SpO 2 , ETCO 2 , and urine output were monitored. Blood sugar was monitored hourly with glucometer. Infusion of glyceryl trinitrate (25 mg/50 mL) was started at 7.5 mL/h, i.e. 3.75 mg/h (0.5-5 μg/kg/min). With the creation of pneumoperitoneums, BP approximately rose to 170/100 mmHg, with tumor handling BP levels rising further. An infusion of sodium nitroprusside (50 mg/50 mL) was started at 3.8 mL/h, i.e., 3.8 mg/h (0.5-5 μg/kg/min) when BP rose to 200/120 mmHg. IV esmolol of dosage 5 mg boluses were given on two occasions to counteract tachycardia. The BP was maintained in the range 120-130/80-90 mmHg. The adrenal gland was separated from the surrounding structures and both the infusions were stopped after venous ligation of the gland [Figure 1] and [Figure 2]. Maintenance of fluid was given with Ringer's lactate (RL) (200 mL). Fluid boluses were given with hydroxyethyl starch 500 mL and 250 mL of whole blood after ligation of the adrenal veins. On one occasion, the patient had hypotension, his BP was 80/64 mmHg that was treated with fluids and IV mephentermine 3 mg bolus. There was no further requirement of vasopressors. The adrenal gland was removed with a small incision over the abdominal wall as it was not possible to remove the gland through the ports. Hemostasis was achieved; the ports were closed after desufflation. The patient was made supine. Epidural top-up with 0.125% bupivacaine was given. IV ondansetron of dosage 2 mg was given as antiemetic. The patient was hemodynamically stable with PR 100 bpm, BP 116/80 mmHg, RBS 132 mg%, no pallor, blood loss of approximately 200 mL, and urine output of 1,000 mL. He was reversed with IV glycopyrrolate of dosage 0.3 mg and IV neostigmine of dosage 1.5 mg. He was, then, extubated after thorough oral suctioning and return of reflexes.
In the postoperative recovery room, he was kept in a propped-up position and oxygen was supplemented via a simple oxygen mask. Maintenance fluids were given at 85 mL/kg. Administration of antibiotics and nebulization were continued. Invasive monitoring was continued in the postoperative period for 24 h. The parameters monitored were PR, SpO 2 , IBP, CVP, U/O, and RBS that were essentially normal. There was no need of vasopressors in the postoperative period. Analgesia was achieved with epidural top-ups with 0.125% bupivacaine. At his 2-week postoperative follow-up, he had normal BP and HR. He made an uneventful recovery.
| Discussion|| |
Surgical resection of the tumor is the only curative procedure. Prior to surgery, it is imperative to control arterial pressure, HR, and arrhythmias and to restore the blood volume to normal. Any anesthetic, surgical, or pharmacological stimulus to catecholamine release must be avoided. The anesthesiologist should take relevant history, assess the severity of hypertension, and look for any end organ damage, especially catecholamine-induced cardiomyopathy and cardiac failure, which is associated with a high mortality. ,,
Dolichoectasia of the vertebrobasilar system, a finding of uncertain pathogenesis, is an elongation and dilatation of the major arteries of the posterior fossa.  Mostly congenital, it may also be a result of long-standing hypertension. Patients may present with cranial nerve dysfunction, transient ischemic attack, hydrocephalus, and subarachnoid hemorrhage and unusually with vertebral artery dissection. 
Preoperative preparation is conventionally done with alpha-blocker (selective or nonselective) over a period of 10-14 days and subsequently, additional beta-blocker is required to treat any associated tachycardia. During alpha-receptor blockade, tachycardia and arrhythmias can occur due to resultant unopposed beta receptor activity.  Phenoxybenzamine, a noncompetitive and nonselective alpha-adrenergic antagonist, is usually used and is associated with reflex tachycardia and long duration of action; hence, there is a need to stop the drug 24 h prior to surgery to prevent postoperative hypotension.  In our case, selective competitive alpha-1-adrenergic antagonist prazosin was used because it does not produce reflex tachycardia, has a shorter duration of action, can be rapidly adjusted before surgery, and the duration of postoperative hypotension is decreased. However, surgical management of the pheochromocytomas is inadequate with prazosin alone and intraoperative medications are required to suppress the vasopressor surges generated by the tumor during surgical manipulation and excision. , Preoperative alpha-receptor blockade also reexpands the intravascular volume in 2-3 weeks, as evidenced by the decrease in the hematocrit levels. Correction of hypovolemia is also important in the reduction of morbidity. If tachycardia, arrhythmia, or myocardial infarction is present despite adequate alpha-recpetor blockade, use of beta blockers is recommended. Beta-blockers improve the ejection fraction (EF) and decrease ventricular remodeling.
Laparoscopic adrenalectomy has been demonstrated to be a safe and effective approach to treat benign adrenal lesions. As surgical skills improve, it will most likely be comparable with open adrenalectomy in terms of overall cost. Although it is technically demanding and takes slightly longer than open surgery, the benefits to the patient in terms of hospital stay, convalescence, and cosmetic results have been conclusively reported. Laparoscopic adrenalectomy has become the gold standard for treating benign adrenal lesions. ,,
Sympathetic blockade up to T4 by epidural anesthesia has been shown to inhibit sympathetic innervations in the adrenal medulla, although the release of catecholamines in response to tumor handling is not prevented. We opted for general anesthesia with thoracic epidural placement for better postoperative analgesia as well as decreased the need for opioids in the postoperative period, thereby decreasing the risk of respiratory depression, especially in pediatric patients. 
The proposed timing for initiation if invasive monitoring is done is before the induction so as to accurately diagnose catecholamine surges during the induction and intubation. Since in our case the patient was a pediatric one, we preferred to put the lines after intubation for the fear of anxiety and release of catecholamines.
Usually significant hemodynamic fluctuations occur at the induction of anesthesia, laryngoscopy and intubation, creation of pneumoperitoneum, tumor manipulation, and after tumor removal. Catecholamine surge can be decreased with various drugs like opioids, beta-blockers, CCBs, alpha-adrenergic agonist, vasodilators, etc. in small doses just before laryngoscopy. In our case, we used IV lidocaine of dosage 1.5 mg/kg before laryngoscopy for this purpose. With CO 2 insufflation and tumor handling, there is significant release of catecholamines in the circulation as evidenced by the rise in BP levels and associated tachycardia in spite of adequate preoperative alpha-receptor blockade.  A similar response was observed in our case. We used infusion of nitroglycerin and sodium nitroprusside allowed us to markedly attenuate the hemodynamic changes secondary to these catecholamine releases and to keep the BP levels below 130/90 mmHg.  Ultrashort-acting esmolol can be used as bolus of 5-10 mg or a continuous infusion of 50-200 μg/kg/min can be used to tackle tachycardia and tachyarrythmias. , Long-acting beta-blockers like labetalol (1-3 mg/kg/h), Metoprolol (0.1 mg/kg up to 5 mg), etc. can also be used. Other drugs used for intraoperative hypertension management are phentolamine, enalaprilat, urapidil, milrinone, and CCBs like nicardipine.  James et al. have demonstrated that MgSO 4 in a loading dose of 40-60 mg/kg followed by infusion of 1-2 g/h achieved good hemodynamic stability. ,
The pneumoperitoneum with CO 2 may lead to hypercapnia and acidosis, which in turn are known stimuli of catecholamine secretion and hypertension. Rocha et al. found a more than 10-fold elevation in catecholamines during abdominal insufflation to 12 mmHg with CO 2 , with about 50% of patients experiencing hypertensive episodes.  We could successfully manage this catecholamine response with NTG and SNP infusions.
Another catastrophic event that can occur is hypotension after the ligation of adrenal veins due to decrease in the circulating catecholamine levels, residual effects of antihypertensive drugs in the pre- and intraoperative periods, decreased effective circulating blood volume due to alpha adrenoceptor action, and blood loss during surgery.  This can be managed by adequate hydration in the preoperative period and additional fluid boluses with crystalloids or colloids after the ligation of tumor vessels. Excessive blood loss should be replaced with blood. Residual hypotension can be managed with vasopressors like phenylephrine (2-10 μg/kg/min then 1-5 μg/kg/min), norepinephrine (0.05 μg/kg/min), dopamine (2-10 μg/kg/min), and dobutamine (2-20 μg/kg/min). , At times, these drugs are continued in the postoperative period till systemic vascular resistance (SVR) adapts to the decreased levels of alpha-adrenergic agonist stimulation. In our case, hypotension was tackled with fluid boluses and a bolus of mephentermine without use of any other vasopressor. No residual hypotension was observed probably due to adequate preoperative hydration, use of short acting antihypertensive drugs. and judicious replacement of intraoperative fluids.
Increased adrenoceptor activity suppresses insulin release; hence, patients are prone to hyperglycemia during the preoperative period. After vessel ligation, the inhibition of insulin is released that may lead to hypoglycemia. Therefore, monitoring and correction of blood glucose levels is crucial in the intraoperative period. 
Geetha et al. in 2014 reported a case of a 12-year-old boy with bilateral pheochromocytoma for bilateral cortical, sparing laparoscopic adrenalectomy. Induction and intubation using the same drugs as ours was done but they maintained anesthesia on isoflurane. They also preferred to put epidural and invasive monitoring after induction. They used SNP infusion and esmolol for intraoperative control of hypertension but due to the residual tumor, they required postoperative SNP initially followed by prazocin, atenolol, and nifedipine for the control of hypertension in spite of good analgesia. Repeat surgery required only nitroglycerin drip during handling of the tumor. 
| Conclusion|| |
Management of patients with pheochromocytoma remains a challenge to anesthesiologists. A close communication is required among the operating surgeon, endocrinologist, and anesthesiologist for better patient care. This case report emphasizes the safety of laparoscopic procedure for adrenalectomy under General anaesthesia and that epidural anesthesia in pediatric patients with pheochromocytoma if safe anesthesia is administered with adequate preoperative preparation, careful monitoring, and prompt management of crises during the perioperative period.
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[Figure 1], [Figure 2]