Unusual sequel of successful laparoscopic unilateral adrenalectomy in a hypertensive adolescent
G Agarwal1, S Verma1, SB Yadav2,
1 Department of Endocrine Surgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Raebareli Road, Lucknow-226 014, India
2 Department of Endocrinology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Raebareli Road, Lucknow-226 014, India
Department of Endocrine Surgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Raebareli Road, Lucknow-226 014
|How to cite this article:|
Agarwal G, Verma S, Yadav S B. Unusual sequel of successful laparoscopic unilateral adrenalectomy in a hypertensive adolescent.J Postgrad Med 2007;53:52-54
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Agarwal G, Verma S, Yadav S B. Unusual sequel of successful laparoscopic unilateral adrenalectomy in a hypertensive adolescent. J Postgrad Med [serial online] 2007 [cited 2022 Nov 30 ];53:52-54
Available from: https://www.jpgmonline.com/text.asp?2007/53/1/52/30332
An 18-year-old male presented with episodic headache and excessive sweating for two months; and diminished vision in left eye for 15 days. During these episodes, highest blood pressure (BP) recorded was 290/90 mmHg. He didn't have urinary complaints, nor history of weight gain or muscle weakness. There was no contributory family history, nor significant past history. This average-built boy had BP recordings of 120 to 220 mmHg-systolic and 90 to 120 mmHg-diastolic. Cardiovascular examination was suggestive of LVH, no murmurs were heard. Peripheral vascular examination was normal and there were no abdominal bruit. He didn't have any stigmata of multiple endocrine neoplasias (MEN) syndrome Type-2 (marfanoid habitus, mucosal fibromata, thyroid nodules). Hypertensive retinopathy was evident on fundus examination.
What is the differential diagnosis?
The presence of the classical triad of episodic hypertension, headache and diaphoresis make hypercatecholaminism or pheochromocytoma (PCC) as the first differential diagnosis. PCC can have extremely varied presentations, to the extent of being called "the great mimic", yet, the classical triad, when present is highly specific for hypercatecholaminism. Some of these clinical features can overlap with other conditions. In patients with essential hypertension and "hyperadrenergic" features such as tachycardia, sweating and high cardiac output and in patients with anxiety attacks associated with blood pressure elevations, urinary or plasma catecholamines estimation is usually helpful in excluding the diagnosis of PCC. Intracranial tumors and subarachnoid hemorrhage can cause headaches, hypertension and hypercatecholaminism, but are accompanied by other neurological manifestations as well which were absent in our patient. Diencephalic seizures rarely cause paroxysmal hypertension with symptoms of excessive catecholamine secretion that are often preceded by an aura. Cocaine abuse can cause a marked increase in blood pressure, hyperadrenergic symptoms and myocardial damage. Factitious crises may be produced by self-administration of sympathomimetic amines.
Other causes of secondary hypertension-renovascular hypertension, renal arterial stenosis, hyperthyroidism, hypercortisolism, hyperaldosteronism and cardiac/vascular anomalies like aortic co-arctation need to be ruled out. Renovascular hypertension is usually associated with history of renal disease, deranged renal functions and urinary symptoms, renal failure and features of fluid overload and uremia. Hyperthyroidism may be associated with a hyperkinetic, hyperadrenergic state, but these manifestations are typically persistent, not paroxysmal, as was the case in presented patient. Hypercortisolism (Cushing's syndrome) is associated with fluid retention, obesity and muscle weakness. Classical features of Hyperaldosteronism are hypokalemia and periodic paralysis. None of these features were present in our patient.
How to establish the diagnosis ?
The diagnosis of hypercatecholaminism is established biochemically. Patient's 24h urinary total-metanephrines (dual-column resin-exchange assay, Bio-rad, Hercules, USA) were elevated (12.5 mg/24h, normal 131sub -meta-iodo-benzyl-guanidine scintigraphy revealed a solitary focus of radio-tracer concentration in right supra-renal area, consistent with PCC. Patient was thus diagnosed as right adrenal PCC, with left renal and adrenal agenesis.
What associated syndromes are known with PCC ?
PCC are uncommon tumors of adrenal-medulla and sympathetic ganglia and account for How to manage adrenal PCC and what implications would contralateral adrenal agenesis have in this patient ?
Adrenal PCC are managed surgically, after adequate alpha and if needed beta adreno-ceptors blockade., As preoperative preparation, the patient was treated with alpha-adenoceptor blocker Prazosin, starting with 6mg/day, gradually increasing to maximum 18 mg/day when adequate BP control (10 mmHg, nasal stuffiness, weight gain, falling hematocrit) were achieved. Appropriate fluids, salt replacement and plasma infusion were administered. Anticipating postadrenalectomy hypocortisolism in view of a nonvisualized left adrenal gland, Hydrocortisone infusion was started the morning of the operation. Lateral transperitoneal laparoscopy-assisted right adrenalectomy was performed. Per-operative BP fluctuations were controlled with sodium-nitroprusside. He didn't require peri-operative blood transfusions. Post-PCC-excision hypotension, an anticipated sequel, was successfully managed with colloids and vasopressors.
The 5 cm circumscribed right adrenal tumor removed was reported as benign PCC on histopathology, in view of absence of metastases, local invasion and histological features of malignancy. Following an uneventful postoperative recovery, the patient was discharged on postoperative day 4 on corticosteroid supplements (prednisolone 15 mg/day). He was normotensive, off anti-hypertensives, had normal kidney functions and free of any complications.
In follow-up at one, three, six and 12 months, the patient has remained asymptomatic, normotensive without any anti-hypertensive medications and had normal 24h urinary total-metanephrines. Serum-cortisol and short-ACTH stimulation tests performed have been suggestive of continued hypocortisolism, confirming the contralateral adrenal agenesis.
When is postadrenalectomy corticosteroid replacement/supplementation required?
Peri-operative corticosteroid replacement or supplementation is required in patients who have or are expected to develop hypocortisolism postoperatively. This usually occurs following bilateral adrenalectomy or unilateral adrenalectomy for cortisol-producing adenomas which cause suppression of the contralateral adrenal. In the present case, unilateral laparoscopic adrenalectomy for adrenal PCC resulted in long-term hypocortisolism because the contralateral adrenal was congenitally absent. He has remained dependent on glucocorticoids and mineralo-corticoids supplements for over one year of follow-up.
How do patients undergoing unilateral adrenalectomy for PCC fare ?
With appropriate peri-operative management, operative mortality in PCC patients in most endocrine centers is What is the appropriate surgical approach to adrenal pheochromocytoma?
For most benign adrenal pathologies, laparoscopic adrenalectomy is currently favored for its superiority over conventional-open adrenalectomy with regard to lesser postoperative pain and shorter convalescence, while being equally effective. Laparoscopic adrenalectomy, specially for PCC is technically demanding and currently offered in India by only a few surgeons at centers of excellence.
Can adreno -cortical function be preserved following bilateral adrenalectomy?
Adrenal-Cortex-preserving adrenalectomy or adrenal-sparing adenomectomy has been proposed for small adrenal adenomas or PCCs in recent years,,,, which can be performed laparoscopically also. These procedures are of unproven efficacy for PCC and have invited widespread criticism owing to high recurrence rates, especially as most recurrences of benign-curable pathologies (at first instance) surface as unresectable-incurable disease. Moreover, not all patients undergoing the cortex-preserving adrenalectomy derive the benefits of avoidance of corticosteroid replacement., Because of these concerns, the authors do not practice this for PCC, reserving this option for small Conn's adenomas alone. In the presented patient with large PCC and no discernable cortical tissue, cortex-preserving option was not entertained. Another option with doubtful efficacy is orthotopic auto-transplantation of adrenal-cortical tissue.
Are PCC and URA known to coexist commonly?
URA is a common congenital abnormality, reported in 1:1000 autopsies., Ipsilateral adrenal agenesis is more rarely encountered, seen in ~10% of URA subjects at autopsy and in 17% on CT scan. URA may occur in isolation or may be associated with other congenital syndromes. Coexistence of URA and PCC is not a known association and in the absence of a common embryological basis for the two conditions, such an association in our patient seems merely a chance coexistence. To the best of the authors' information, there are no reports of coexistent URA and adrenal tumors in the scientific literature. The known associated renal affections in PCC patients are renal cell carcinoma and cysts seen in VHL patients. Two VHL patients rendered anephric after bilateral nephrectomy for multiple renal cell carcinomas have been reported to have laparoscopic removal of PCC. Renal arterial stenosis may be associated with PCC, more commonly in childhood, with about 100 reported cases.
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