The hemodynamic paradox: Synchronous robotic surgery for normotensive pheochromocytoma in VHL
https://doi.org/10.15605/jafes.041.S1
- Author:
Thiru Murugaan Balakrishnan
1
;
Nurbadriah Jasmiad
1
;
Ng Wei Wei
1
;
Anilah Abdul Rahim
1
;
Ijaz Hallaj Rahmatullah
1
;
Subashini Rajoo
2
Author Information
1. Hospital Raja Permaisuri Bainun
2. Hospital Kuala Lumpur
- Publication Type:Journal Article
- From:
Journal of the ASEAN Federation of Endocrine Societies
2026;41(S1):23-
- CountryPhilippines
- Language:English
-
Abstract:
Introduction:Normotensive pheochromocytomas in Von Hippel-Lindau
(VHL) syndrome present unique perioperative challenges.
Standard alpha-blockade may induce intolerable orthostatic
hypotension, making calcium channel blockers (CCB)
a practical alternative. Furthermore, the primary intraoperative danger in these specific phenotypes may not be
a hypertensive crisis, but profound vasoplegia. We report
a VHL patient undergoing synchronous robotic surgery
exhibiting this paradoxical hemodynamic response.
Case:A 37-year-old female with VHL syndrome presented
with an incidental 3.5-cm left adrenal mass and bilateral
renal masses. Biochemistry confirmed a normotensive,
noradrenergic pheochromocytoma (24-hour urine
normetanephrines 4.2x upper limit of normal). Renal biopsy
revealed a clear cell papillary renal cell tumor. Due to prior
severe intolerance to Prazosin (hypotension/dizziness with
low-dose Prazosin 0.5 mg ON), we utilized amlodipine for
preoperative optimization. She was only able to tolerate
low-dose 2.5 mg OD alongside oral sodium chloride and
ample oral fluid loading. She underwent a synchronous
robotic-assisted left adrenalectomy and left midpole renal
tumor excision. Strikingly, tumor manipulation did not
precipitate a hypertensive crisis. Instead, she developed
hypotension requiring an intravenous noradrenaline infusion prior to adrenal vein ligation and tumor removal.
Vasopressor support was successfully weaned 12 hours
postoperatively, and she was discharged well.
Conclusion:Normotensive, noradrenergic pheochromocytomas in VHL
are hemodynamically fragile. Chronic catecholamine excess
induces homologous desensitization and downregulation
of alpha-1 adrenergic receptors. This physiological adaptation explains the normotensive presentation and highlights
the intraoperative vasoplegia experienced once sympathetic
tone is altered by anesthesia. While CCB monotherapy with
volume expansion safely facilitates prolonged, synchronous
robotic surgeries, clinicians must anticipate and combat
refractory hypotension rather than classical hypertensive
spikes.
- Full text:2026072813225306931EP_A009.pdf
