Case report Anaesthetic management of a patient with adrenocortical tumour
LAKSHMI VAS MD, SHILPA Y. PANGAM MD AND SAVITA M. SANZGIRI MD Bai Jerbai Wadia Hospital for Children, Acharya Donde Marg, Parel, Bombay 12, India
Summary
Adrenocortical tumours in children are rare. They produce many changes in haemodynamics and blood chemistry due to hormones of the adrenal cortex. The details of perioperative management and the need for perioperative steroid supplementation are discussed.
Keywords: adrenocortical tumour; anaesthetic management
Introduction
Adrenocortical tumours (1-9) are very rare especially in childhood. They form less than 1% of all paediatric neoplasms (1) occurring mainly in children below 5 years with a female preponderance (2). Carcinoma of the adrenal cortex has an incidence of only 0.002% of all childhood malignancies (3). Unlike the inactive or glucocorticoid secreting adrenocortical tumours in adults, 95% of the tumours in children are functioning, secreting varying amounts of sex steriods, glucocorticoids and mineralocorticoids in that order of frequency (4,5). A mixed picture is most common. Surgery forms the effective definitive therapy for adrenal neoplasm (1,2,4-8). The preoperative medical management of the manifestations of these tumours is complex enough, but anaesthesia may cause even more dramatic changes in physiology. The details, not only of anaesthesia and its effect on an already pathological haemodynamic and biochemical situation, but also implications of perioperative steroid sup- plementation form the basis of this report.
Correspondence to: Lakshmi Vas, Bai Jerbai Wadia Hospital for Children, Acharya Donde Marg, Parel, Bombay 12, India.
Case report
A 3-year-old boy weighing 15 kg was admitted to our hospital with complaints of weakness, hirsutism, abnormally enlarged genitals, polyuria and polydipsia for 1 year. On examination, he showed signs of precocious puberty of which he was very sensitive. His physical appearance was otherwise normal. There was a diffuse mass in the left flank. Abdominal CT and ultrasound revealed a left suprarenal mass 7.6 × 10 × 7.6 cm with areas of necrosis and calcified spots. Chest X-ray showed cardiomegaly which was confirmed by 2-D ECHO. There was no concentration of I131 in the adrenal gland in the MIBG scan. The urinary adrenaline levels were normal, ruling out any abnormality of adrenal medulla. Tables 1 and 2 show the details of other relevent investigations. The rest of his blood chemistry was within normal limits including several blood sugar values varying between 4.8-5.3 mmol-1-1 (88 and 96 mg·dl-1).
There was severe hypertension with blood pressure of 240/140 mmHg and his apex beat was shifted outward. He was treated with spironolactone (from 2mg.kg-1.day-1 to 4mg.kg-1.day-1 in two divided doses) and nifedipine (0.5 mg.kg-1.day-1in four divided doses). This achieved blood pressure
| Daily water intake | Urine output | Urine Osm/Ser Osm | Urinary specific gravity | Urinary sodium mmol | Urinary potassium mmol | Serum Na/K | |
|---|---|---|---|---|---|---|---|
| Preoperative | 1900-2600 | 1700-2900 | 250-263 | 1.002-1.010 | 18.7-35.1 | 31.9-45.9 | 144/2.2 |
| 272 | (Normal | (Normal | 133/2.9 | ||||
| 50-150) | 30) | ||||||
| Intraoperative | 2500 | 2000 | 1.008-1.012 | 40 | 38 | Table 3 |
| Testoster- one ng·ml-1 | fSH Miu·ml-1 | LH miu·ml-1 | Prolactin ng.ml-1 | DHEAS ng-ml-1 | Cortisol ng-ml-1 | Aldosterone pg·ml-1 | ||
|---|---|---|---|---|---|---|---|---|
| Morn. | Night | |||||||
| Normal value | 0.2-1 ng | 2-8 | 2-10 | 3-30 | 300-500 | 100-200 | 30-60 | 50-800 |
| Preoperative | 1.4 | 0.4 | 0.5 | 3.8 | <300 | 278 | 239 | 5000 |
| Preanaesthetic | 173 | 5200 | ||||||
| 1st post op day | 180 | 59 | ||||||
| 15th post op day | 176 | 90 | ||||||
readings between 124/98 and 150/108 mmHg, though very labile His serum potassium values remained consistently above 3.5 mmol-1-1. On the day of surgery, the blood pressure was 140/ 100 mmHg and pulse was 120 b·min-1 after his morning dose of spironolactone and nifedipine.
He was sedated with oral diazepam 3 mg. Anaesthesia was induced with 100 mg thiopentone and 15 mg atracurium. Bolus doses of esmolol 7 mg and lignocaine 30 mg were given prior to laryngoscopy and intubation with a 5.5-mm tracheal tube. At the time of induction his blood pressure (NIBP) remained around 130/90-150/100 mmHg. Cannulae were placed in his right radial artery and right internal jugular vein to monitor direct arterial and central venous pressures, respectively. Other monitoring included oximetry, capnography and ECG. A 20-gauge epidural catheter was introduced through an 18-gauge Tuohy needle at L3-4 level and passed up to T8 as measured on the catheter outside. Some 5 ml of 0.25% bupivacaine were injected. Anaesthesia was maintained with nitrous oxide, oxygen, halothane (0.5-2%) and atracurium. Epidural analgesia was repeated every 90 min with 5 ml 0.25% bupivacaine.
The systolic blood pressure was very labile and varied between 70 and 190 mmHg and required constant and continuous control with sodium nitroprusside and esmolol infusions, especially while the tumour was being handled. We initially gave the child Isolyte M but later switched to Ringer’s lactate. His hourly serial serum electrolytes values are shown in Table 3. The anaesthesia and surgery lasted 7h. The tumour was very vascular with grossly dilated vessels on the surface. It had a well defined capsule and weighed 40 g. The other adrenal was found to be of normal size and consistency. The hypotensive drugs were discontinued after removal of the tumour. The blood loss of 150 ml was not replaced. The total IV fluids given was 2500 ml. The urine output was 2000 ml. The child received no steroids in the entire pre, intra and postoperative periods, though hydrocortisone was kept available. During this period the child had no episode of hypotension. At the end of the procedure on reversal the child was awake but comfortable with a pulse of 120 b·min-1, 120/72 mmHg blood pressure, central venous pressure of 10 cmH2O and normal blood gases. 2h postoperatively, the blood pressure rose to 160/ 90 mmHg. This settled at 128/90 mmHg after the
| Pre operative | Intraoperative | Postoperative | |||||||
|---|---|---|---|---|---|---|---|---|---|
| Isolyte M | Ringer s lactate with potassium | R.L isolyte M | |||||||
| 1 h | 2 h | 3 h | 5h | 7h | 2 h | 8 h | 24 h | ||
| Sodium/potassium (mmol·1-2) | 136/3.9 | 133/4.8 | 124/2.9 | 130/4.2 | 134/4.0 | 134/4.1 | 136/3.6 | 134/4 | 136/4 |
| Blood glucose (mg-dl-1) | 5.3 (96) | 16.3 (296) | 27.2 (495) | 11.4 (208) | 8.6 (156) | 8.2 (150) | 7.7 (140) | 9.7 (176) | 10.1 (184) |
evening doses of spironolactone and nifedipine. He received 6 hourly epidural analgesia for 48 h. The next day, 14 h after surgery, his blood pressure was 120/90 mmHg without any hypotensives; 15 days later blood pressure was stabilized around 100/ 70 mmHg. Histopathology showed the tumour to be an adrenal carcinoma. It is now a year since surgery and the child is not on steroids and is normal physically with normal hormone levels.
Discussion
The first paediatric adrenal tumour was reported in 1865 (8). Since then there have been occasional but regular reports of these cases. With present sophisticated imaging modalities, these tumours are being found incidentally (incidentalomas) (2,9). However, there appear to be few reports (10,11) of the anaesthetic management of these children. This is unlike the other adrenal tumour pheochromocytoma, where the anaesthetic norms have been clearly defined.
A major consideration of anaesthetic technique was the type of intravenous fluid to be administered. Most of the available solutions contain sodium, be it lactated Ringer’s solution, 0.9, 0.45 or 0.3 N saline. In a patient such as ours with sodium retention the advisability of using isotonic solutions is debatable though normal intraoperative third space requirements dictate their use. Since 0.3 N saline without any glucose was not available, we decided to use Isolyte M with a sodium content of 56 mmol.1-1 and a potassium content of 24 mmol.1-1 which suited our electrolyte needs. We also had reservations about causing severe hyperglycaemia especially with marginally raised glucocorticoid levels. Our
reservations were justified as hourly monitoring showed rising blood glucose levels (Table 3). There was also a gradual reduction of serum sodium and potassium which necessitated the use of Ringer’s lactate with added potassium. The reduction in serum potassium levels in spite of infusion of Isolyte M with 24 mmol.1-1 of potassium can be explained by the continuing and accentuated loss of potassium in urine intraoperatively. Our patient excreted 2000 ml of urine in 7 h of operative time reflecting the residual polyuric nephropathy.
Most of the reports of adrenocortical carcinoma (4-8,10,11,13). have mentioned hypercortisolism with obvious Cushingoid features requiring perioperative supplementation of steroids which extends into months postperatively. However, we had a confusing situation in our case with only marginally raised cortisol, concomitant with obvious hypersecretion of the remaining hormones of the adrenal cortex. The raised night value of cortisol and the loss of diurnal variation would normally signify hypercortisolism with an indication for perioperative steroids. However, this child showed neither clinical nor biochemical features of hypercortisolism, despite the 1 year history, while the features of hyperaldosteronism (12-17) and virilization were unequivocal. In addition to this, there was diastolic hypertension and a low plasma potassium level. Hypertension in our case probaby refelected aldosterone induced sodium retention, and an isotonically increased extracellular fluid volume associated with normal serum sodium. The renal secretion of potassium to retain sodium resulted in muscle weakness and hypokalaemic nephropathy with polyuria and associated polydipsia, with an inability to concentrate urine optimally. As is
common in cases of hyperaldosteronism, there was no dependent oedema. We were not convinced the levels of cortisol in this child were sufficiently excessive to suppress his normal adrenal gland because he had no features of glucocorticoid excess, such as central obesity, buffalo hump, muscle wasting, thin skin prone to bruising, or levels of hyperglycaemia commonly associated with hypercortisolism. His blood sugar was in the normal range with repeated consistency. The glucocorticoid level at night was certainly raised -200 ng·ml-1 (normal 40 ng·ml-1), but the morning value was only marginally raised (250 against a normal of 200). This disparity between normal levels and clinical findings led to a dilemma whether the estimated values did indeed indicate a significant excess of glucocorticoid which could potentially suppress the contralateral normal adrenal gland.
On inquiry, the venepuncture and withdrawal of blood sample at night had taken 30 min due to the struggling of the child in the absence of EMLA cream. Our patient was very sensitive about his virilization and was very apprehensive about medical personnel. Though the sample was collected half an hour later, it was likely that the stress in a child who had been suddenly woken up from deep sleep, led to higher values of cortisol than might be expected. We could have repeated the test but its cost was borne by the parents who expressed their inability to do so.
The level of endogenous cortisol necessary to suppress the HPA axis is not elucidated, although the levels of exogenous steriods that do so are clearly established. There are practically no data about adrenal suppression by endogenous cortisol from adrenocortical tumours. This is taken for granted and steroid supplementation is advocated perioperatively as a matter of routine (4-8,10,11,13). This requirement is clear cut in an established Cushing’s syndrome but in a case like ours where the hypertension was due to an obviously high aldosterone, the ambiguously raised glucocorticoid with hardly any of its clinical manifestations made the decision difficult for us. Steroids have a role in maintenance of normal arterial systemic pressure and volume through their support of vascular responsiveness to vasoactive substances. Glucocorticoids enhance catecholamine synthesis in adrenal medulla, and also their effects on the peripheral vasculature as vasopressors. In the absence of cortisol the vasopressor action of
catecholamines is diminished and hypotension ensues (10,15,18). In a patient with adrenocortical tumour, there will be a total cessation of cortisol supply from the tumour after its removal. If the remaining adrenal is suppressed there may be a reduced amount of cortisol. The cortisol is highly protein bound 90% (75% to transcortin and 15% to albumin) only 10% is the physiologically active available form. As a result it has a transient action and it is likely that once the source of cortisol is removed a lack of it is likely to be felt if the normal adrenal is suppressed.
This forms the basis of the apprehension that in the absence of supplemetary steroids, a patient with adrenal suppression will have a cardiovascular collapse. However, it has been consistently proven that there has been very little demonstrated association between steroid therapy and induced adrenal suppression and perioperative collapse (15, 18-21). With this background information we decided to withhold perioperative steriods unless it became necessary. This decision was based on a wish to avoid unnecessary medication with potentially harmful drugs while there was ambiguity regarding the suppression of the normal adrenal gland. We anticipated that if the normal adrenal was indeed suppressed, then we were likely to see hypotension in the period after excision of the tumour and we had hydrocortisone ready in the presently advocated doses if required (22,23). We continued the same watchfulness into the postoperative period, with the steroid kept ready and monitoring of direct arterial blood pressure. But as seen from the details, our patient had a steady blood pressure in the higher range without any additional support for 14 h after removal of the tumour. In fact he required hypotensive agents 3h postoperatively but settled down after that. Probably at that time he was still responding to a slow release of the cortisol from its binding sites and also to the circulating aldosterone left over from the tumour secretions. The normal cortisol and aldosterone levels in the immediate postoperative period were also convincing.
In conclusion, patients may not fit into a particular category of illness and as such treatment needs to be individualized. The clinical indicators of hormone excess may be more reliable than their biochemical estimations. We did take a calculated risk by
withholding steroids, but this paid off by avoiding long-term steroids in our patient.
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Accepted 2 September 1998