Summary
Knowledge of the broad spectrum of clinical signs and symptoms associated with pheochromocytoma is a pre-requisite for diagnosis and the avoidance of a wrongly added beta-blocker medication prior to sufficient alpha-blockade. The diagnosis is made biochemically by measuring urine catecholamines and metanephrines. After the pheochromozytoma has been localized, the appropriate preoperative pharmacological therapy is crucial for the prevention of serious vascular complications. With respect to preoperative treatment, an initial alpha-blockade by the non-specific, non-competitive and irreversible alpha-blocker phenoxybenzamine is highly recommended. After a stepwise elevation of phenoxybenzamine up to an individual and maximal tolerable dose, a non-cardioselective betablocker should be added. With strict obedience of these guidelines and since monitoring and anesthesiological procedures have been improved continuously, the incidence of perioperative morbidity and mortality could be reduced dramatically. With respect to emergency therapy, phentolamine is the medication of choice. Due to a limited availability of phentolamine in most countries, urapidil represents a reasonable alternative. If sodium nitroprusside is used, extensive pharmacological and toxicological effects have to be considered.
Zusammenfassung
Die Kenntnis des Spektrums der Symptomatologie des Phäochromozytoms und der Phäochromozytomkrise sind unabdingbare Voraussetzungen für das Erkennen der Erkrankung, der Vermeidung einer kontraindizierten und deletären Betablocker-Applikation ohne initiale Alpha-Blockade sowie der Bahnung der richtigen Diagnostik. Die biochemische Sicherung der Verdachtsdiagnose erfolgt heute unkompliziert über die Bestimmung der Katecholamine und der Metanephrine im 24h-Sammelurin. Nach erfolgter Lokalisationsdiagnostik ist die präoperative pharmakologische Therapie entscheidend für die Vermeidung von Komplikationen und Endorganschäden. Für die präoperative Therapie wird eine initiale Alpha-Blockade mittels des nicht-spezifischen, nicht-kompetitiven, irreversiblen α-Rezeptorantagonisten Phenoxybenzamin dringend empfohlen. Nach einer maximal möglichen Auftitrierung bis zur Normotonie und bis zum Auftreten orthostatischer Beschwerden folgt die Applikation eines nicht-kardioselektiven Beta-Blockers, z. B. Propranolol. Durch konsequente Befolgung dieser Richtlinien sowie durch verbessertes Monitoring und Fortschritte in der Anästhesie konnten die perioperativen Komplikationen deutlich gesenkt werden. Hinsichtlich der Notfalltherapie der hypertensiven Krise beim Phäochromozytom wäre Phentolamin prinzipiell das Mittel der Wahl. Aufgrund schwieriger Verfügbarkeit (importierbar über die Auslands-Apotheke) ist hier Urapidil eine gute Alternative. Beim Gebrauch von Nitroprussid-Natrium sind komplexere pharmakologische und toxikologische Effekte zu beachten.
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References
Williams GH (2001) Hypertensive vascular disease. In: Braunwald E, Fauci AS, Kasper D (eds) Harrison’s principles of Internal Medicine, 15 th ed. McGraw-Hill, New York, p 1414–1429
Williams GH, Hollenberg NK (1990) Pathophysiology of essential hypertension. In: Parmley WW, Chatterjee K (eds) Cardiology, vol. 2. JB Lippincott, Philadelphia, pp 1–18
Landsberg L, Young JB (1994) Harrison’s Principles of Internal Medicine, 13 th ed. Mc Graw-Hill Inc, New York, pp 1976–1979
Williams GH, Moore TJ (1994) Hormonal aspects of hypertension. In: DeGroot LJ, Besser M, Burger HG (eds) Endocrinology, 3rd ed. WB Saunders, Philadelphia, pp 2917–2934
Nawroth PP, Ziegler R (Hrsg) (2001) Klinische Endokrinologie und Stoffwechsel. Springer, Berlin Heidelberg, pp 521–543
Larsen PR, Kronenberg HM, Melmed S, Polonsky KS (eds) (2003) Williams Textbook of Endocrinology, 10th ed. Saunders, Philadelphia, pp 552–562
Bravo EL, Tagle R (2003) Pheochromocytoma: state-of-the-art and future prospects. Endocr Rev 24:539–535
Daly PA, Landsberg L (1992) Pheochromocytoma: diagnosis and management. Bailliere’s Clin Endocrinol Metab 6:143–166
Rosenthal R, Conen D (2000) Phäochromozytom: Diagnose und Therapie. Schweiz Med Wochenschr 130:1298–1304
Levine SN, McDonald JC (1984) The evaluation and management of pheochromocytomas. Adv Surg 17:281–313
Pacak K, Linehan WM, Eisenhofer G, Walther MM et al (2001) Recent advantages in genetics, diagnosis, localization, and treatment of pheochromocytoma. Anesthesiology 66:705–706
Lenders JW, Pacak K, Walther MM et al (2002) Biochemical diagnosis of pheochromocytoma: which test is best? JAMA 287:1427–1434
Hernandez FC, Sanchez M, Alvarez A et al (2000) A five-year report on experience in the detection of pheochromocytoma. Clin Biochim 33:649–655
Sawka AM, Jaeschke R, Singh RJ et al (2003) A comparison of biochemical tests for pheochromocytoma: measurement of fractionated plasma metanephrines compared with the combination of 24-hour urinary metanephrines and catecholamines. J Clin Endocrinol Metab 88:553–558
Bravo EL, Tarazi RC, Fouad FM et al (1981) Clonidine suppression test. A useful aid in the diagnosis of pheochromocytoma. N Engl J Med 305:623–626
Sjoberg RJ, Simcic KJ, Kidd GS (1992) The clonidine suppression test for pheochromocytoma. A review of its utility and pitfalls. Arch Intern Med 152:1193–1197
Bachmann AW, Gordon RD (1991) Clonidine suppression test reliably differentiates phaechromocytoma from essential hypertension. Clin Exp Pharmacol Physiol 18:275–277
Lefkowitz RJ, Caron MG (1988) Adrenergic receptors: models for the study of receptors coupled to guanine nucleotide regulatory proteins. J Biol Chem 263:4993–4996
Ahlquist RP (1991) A study of the adrenotropic receptors. Am J Physiol 260:E243–246
Milligan G, Svoboda P, Brown CM (1994) Why are there so many adrenoceptor subtypes? Biochem Pharmacol 48:277–2990
Caron MG, Lefkowitz RJ (1993) Catecholamine receptors: structure, function, and regulation. Recent Prog Horm Res 48:277–290
Storsberg AD (1995) Structural and functional diversity of β-adrenergic receptors. Ann NY Acad Sci 757:253–260
Yang YT, McElligott MA (1989) Multiple actions of β-adrenergic antagonists on skeletal muscle and adipose tissue. Biochem J 261:1–10
Young WF (2003) Pheochromocytoma. In: Endocrine Society, Meet-The-Professor-Handouts, pp 245–254
Sutton MG, Sheps SG, Lie JT (1981) Prevalence of clinically unsuspected pheochromocytoma: review of a 50-year autopsy series. Mayo Clin Proc 56:354–360
Bravo EL (1994) Evolving concepts in the pathophysiology, diagnosis, and treatment of pheochromocytoma. Endocr Rev 15:356–368
Sheps SG, Jiang NS, Klee GG et al (1990) Recent developments in the diagnosis and treatment of pheochromocytoma. Mayo Clin Proc 65:88–95
Bravo EL (1991) Pheochromocytoma: new concepts and future trends. Kidney Int 40:544–556
Manger WM, Gifford RW J (1993) Pheochromocytoma: current diagnosis and management. Cleve Clin J Med 60:365–378
Plouin PF, Degoulet P, Tugaye A, Ducroc MB, Menard J (1981) Screening for pheochromocytoma: in which hypertensive patients? A semiological study of 2585 patients, including 11 with phaeochromocytoma. Nouv Presse Med 10:869–872
Crout JR, Pisano JJ, Sjoerdsma A (1961) Urinary excretion of catecholamines and their metabolites in pheochromocytoma. Am Heart J 61:375–381
Brouwers F, Lenders JWM, Eisenhofer G, Pacak K (2003) Pheochromocytoma as an endocrine emergency. Rev Endocr Metab Dis 4:121–128
Raisanen J, Shapiro B, Glazer GM, Desai S et al (1984) Plasma catecholamines in pheochromocytoma: effect of urographic contrast media. Am J Roentgenol 143:43–46
Newell KA, Prinz RA, Pickleman J, Braithwaite S et al (1988) Pheochromocytoma multisystem crisis. A surgical emergency. Arch Surg 123:956–959
Werbel SS, Ober KP (1995) Pheochromocytoma: update on diagnosis, localization, and management. Med Clin North Am 79:131–153
Russel WJ, Metcalfe IR, Tonkin AL et al (1998) The preoperative management of phaeochromocytoma. Anaesth Intensive Care 26:196–200
Pullerits J, Ein S, Balfe JW (1988) Anesthesia for phaeochromocytoma. Can J Anaesth 35:525–534
Young WF (2002) Pheochromocytoma. In: Physicians information and education resource (PIER). American College of Physicians
Bravo EL (2002) Pheochromocytoma: an approach to antihypertensive management. Ann NY Acad Sci 970:1–10
Kaplan NM (1998) Clinical hypertension, 7th ed. Williams & Wilkins, Baltimore, pp 345–363
Miura Y, Yoshinaga K (1988) Doxazosin: a newly developed, selective alpha-1-inhibitor in the management of patients with pheochromocytoma. Am Heart J 116:1785–1789
Desmonts JM, Marty J (1984) Anaesthetic management of patients with phaeochromocytoma. Br J Anaesth 56:781–789
Newell KA, Prinz RA, Brooks MH, Glisson SN, Barbato AL, Freeark RJ (1988) Plasma catecholamine changes during excision of pheochromocytoma. Surgery 104:1064–1073
Boutros AR, Bravo EL, Zanettin G, Straffon RA (1990) Perioperative management of 63 patients with pheochromocytoma. Cleve Clin J Med 57:613–617
Ulchaker JC, Goldfarb DA, Bravo EL, Novick AC (1999) Successful outcomes in pheochromocytoma surgery in the modern era. J Urol 161:764–767
Archer SL, Huang JM, Hampl V et al (1994) Nitric oxide and cGMP cause vasorelaxation by activation of a charybdotoxin-sensitive K channel by cGMP-dependent protein kinase. Proc Natl Acad Sci USA 91:7583
Schulz V (1984) Clinical pharmacokinetics of nitroprusside, cyanide, thiosulphate and thiocyanate. Clin Pharmacokinet 9:239
Hall VA, Guest JM (1992) Sodium nitroprussid-induced cyanide intoxication and prevention with sodium thiosulfate prophylaxis. Am J Crit Care 2:19–27
Forth W, Henschler D, Rummel W (1990) Allgemeine und spezielle Pharmakolgie und Toxikologie. BI, Zürich, pp 141–155
Curry SC, Arnold-Capell P (1991) Nitroprusside, nitroglycerin, and angiotensin-converting enzyme inhibitors. In: Blumer JL, Bond GR (eds) Toxic effects of drugs used in the ICU. Critical care clinics. WB Saunders, Philadelphia, pp 555–582
Marino PL (1999) Das ICU-Buch (Hrsg. Taeger K), 2. Aufl. Urban und Fischer, S 254–258
Colson P, Ryckwaert F, Ribstein J et al (1998) Haemodynamic heterogeneity and treatment with the calcium channel blocker nicardipine during phaeochromocytoma surgery. Acta Anaesthesiol Scand 42:1114–1119
Proye C, Thevenin D, Ceca, P, Petillot P et al (1989) Exclusive use of calcium channel blockers in preoperative and intraoperative control of pheochromocytomas: Hemodynamics and free catecholamine assays in ten consecutive patients. Surgery 106:1149–1154
Serfas D, Shoback DM, Lorell BH (1983) Phaechromocytoma and hypertrophic cardiomyopathy: Apparent suppression of symptoms and noradrenaline secretion by calciumchannel blockade. Lancet 2:711–713
Steinsapir J, Carr AA, Prisant LM et al (1997) Metyrosine and pheochromocytoma. Arch Intern Med 157:901
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Serie: Die Notfall- und Intensivtherapie bei endokrinen und metabolischen Erkrankungen Herausgegeben von J. Schölmerich und Ulrike Woenckhaus (Regensburg)
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Schäffler, A. Hypertensive Krisen endokriner Ursache am Beispiel des Phäochromozytoms. Intensivmed + Notfallmed 42, 16–26 (2005). https://doi.org/10.1007/s00390-005-0519-7
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DOI: https://doi.org/10.1007/s00390-005-0519-7