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Evidence-based Practice and Quality Improvement

Von-Hippel Lindau Syndrome (VHL) suspicion on pheochromocytoma surgery - anesthetic management


Araújo, R.; Freitas, S.; Santa, Bárbara R.; Marques, C.; Neves, I.

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European Journal of Anaesthesiology (EJA): June 2013 - Volume 30 - Issue - p 28-28
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Background: VHL is characterized by retinal or CNS hemangioblastomas, as well as renal and pancreatic cysts, erythrocytosis and pheochromocytoma1. We present a patient submitted to pheochromocytoma exeresis, with suspicion of VHL.

Case report: 68 year old female with adrenal incindentaloma, proposed to adrenalectomy. Co-morbidities were hypertension, DM2, dyslipidemia, CRF with chronic anemia. Skin hemangioma raised the VHL presumption. Neurological examination and MRI of the brain and eye were normal.

The preparation was conducted for 15 days with iv fluid therapy, phenoxybenzamine, nifedipine and propranolol. On the OR, BP was 160/90mmHg. Besides the classic non-invasive monitoring, invasive BP was monitored and lumbar epidural catheter was placed. Anesthesia was performed with fentanyl, propofol and rocuronium and maintained with desflurane. Prior to tumor removal, the patient reached MAP's of 70-90mmHg. After its extraction MAP reached 45 mmHg and a Noradrenaline infusion was started and maintained until the D2 PO. The patient was discharged after 5 days.

Discussion: When presented with pheochromocytoma and VHL presumption, one must screen other benign/malignant tumors, and the preparation with alpha-blockers and beta-blockers should be ensured as well as the screening of cardiomyopathy and CHF. It is recommended general anesthesia combined with epidural2. The fluid replacement is the first-line treatment for hypotension intraoperatively. If vasopressors are needed, is preferable to use noradrenalin2.

As VHL can display different clinical presentations, which are mainly unspecific, it is of great importance that clinicians remain vigilant in order to identify cases early in its stages of development and arrange the proper management.


1. JCTVA;02,16:359-369 2.M.E.J. ANESTH 20(1),09.
    2. Warner MA, van Heerden JA: Pheochromocytoma: anesthetic and surgical management employed at the Mayo Clinic, in Manager WM, Gifford Jr RW (eds). Clinical and Experimental Pheochromocytoma. Cambridge, MA, Blackwell Science, 1996, 388-407.

    Learning points: Highlighting the importance of the early presumptive diagnosis of VHL, based on signs, allowing the control of perioperative morbidity; Control/ treatment of disease manifestations without neglecting the necessary preoperative preparation.

    © 2013 European Society of Anaesthesiology