Skip to main content
Log in

Hypertensive Krisen endokriner Ursache am Beispiel des Phäochromozytoms

Management of hypertensive emergency in pheochromocytoma

  • ÜBERSICHT
  • Published:
Intensivmedizin + Notfallmedizin

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.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References

  1. 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

  2. Williams GH, Hollenberg NK (1990) Pathophysiology of essential hypertension. In: Parmley WW, Chatterjee K (eds) Cardiology, vol. 2. JB Lippincott, Philadelphia, pp 1–18

  3. Landsberg L, Young JB (1994) Harrison’s Principles of Internal Medicine, 13 th ed. Mc Graw-Hill Inc, New York, pp 1976–1979

  4. 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

  5. Nawroth PP, Ziegler R (Hrsg) (2001) Klinische Endokrinologie und Stoffwechsel. Springer, Berlin Heidelberg, pp 521–543

  6. Larsen PR, Kronenberg HM, Melmed S, Polonsky KS (eds) (2003) Williams Textbook of Endocrinology, 10th ed. Saunders, Philadelphia, pp 552–562

  7. Bravo EL, Tagle R (2003) Pheochromocytoma: state-of-the-art and future prospects. Endocr Rev 24:539–535

    Google Scholar 

  8. Daly PA, Landsberg L (1992) Pheochromocytoma: diagnosis and management. Bailliere’s Clin Endocrinol Metab 6:143–166

    Google Scholar 

  9. Rosenthal R, Conen D (2000) Phäochromozytom: Diagnose und Therapie. Schweiz Med Wochenschr 130:1298–1304

    Google Scholar 

  10. Levine SN, McDonald JC (1984) The evaluation and management of pheochromocytomas. Adv Surg 17:281–313

    Google Scholar 

  11. 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

    Google Scholar 

  12. Lenders JW, Pacak K, Walther MM et al (2002) Biochemical diagnosis of pheochromocytoma: which test is best? JAMA 287:1427–1434

    Google Scholar 

  13. 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

    Google Scholar 

  14. 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

    Google Scholar 

  15. 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

    Google Scholar 

  16. 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

    Google Scholar 

  17. Bachmann AW, Gordon RD (1991) Clonidine suppression test reliably differentiates phaechromocytoma from essential hypertension. Clin Exp Pharmacol Physiol 18:275–277

    Google Scholar 

  18. 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

    Google Scholar 

  19. Ahlquist RP (1991) A study of the adrenotropic receptors. Am J Physiol 260:E243–246

    Google Scholar 

  20. Milligan G, Svoboda P, Brown CM (1994) Why are there so many adrenoceptor subtypes? Biochem Pharmacol 48:277–2990

    Google Scholar 

  21. Caron MG, Lefkowitz RJ (1993) Catecholamine receptors: structure, function, and regulation. Recent Prog Horm Res 48:277–290

    Google Scholar 

  22. Storsberg AD (1995) Structural and functional diversity of β-adrenergic receptors. Ann NY Acad Sci 757:253–260

    Google Scholar 

  23. Yang YT, McElligott MA (1989) Multiple actions of β-adrenergic antagonists on skeletal muscle and adipose tissue. Biochem J 261:1–10

    Google Scholar 

  24. Young WF (2003) Pheochromocytoma. In: Endocrine Society, Meet-The-Professor-Handouts, pp 245–254

  25. 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

    Google Scholar 

  26. Bravo EL (1994) Evolving concepts in the pathophysiology, diagnosis, and treatment of pheochromocytoma. Endocr Rev 15:356–368

    Google Scholar 

  27. Sheps SG, Jiang NS, Klee GG et al (1990) Recent developments in the diagnosis and treatment of pheochromocytoma. Mayo Clin Proc 65:88–95

    Google Scholar 

  28. Bravo EL (1991) Pheochromocytoma: new concepts and future trends. Kidney Int 40:544–556

    Google Scholar 

  29. Manger WM, Gifford RW J (1993) Pheochromocytoma: current diagnosis and management. Cleve Clin J Med 60:365–378

    Google Scholar 

  30. 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

    Google Scholar 

  31. Crout JR, Pisano JJ, Sjoerdsma A (1961) Urinary excretion of catecholamines and their metabolites in pheochromocytoma. Am Heart J 61:375–381

    Google Scholar 

  32. Brouwers F, Lenders JWM, Eisenhofer G, Pacak K (2003) Pheochromocytoma as an endocrine emergency. Rev Endocr Metab Dis 4:121–128

    Google Scholar 

  33. 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

    Google Scholar 

  34. Newell KA, Prinz RA, Pickleman J, Braithwaite S et al (1988) Pheochromocytoma multisystem crisis. A surgical emergency. Arch Surg 123:956–959

    Google Scholar 

  35. Werbel SS, Ober KP (1995) Pheochromocytoma: update on diagnosis, localization, and management. Med Clin North Am 79:131–153

    Google Scholar 

  36. Russel WJ, Metcalfe IR, Tonkin AL et al (1998) The preoperative management of phaeochromocytoma. Anaesth Intensive Care 26:196–200

    Google Scholar 

  37. Pullerits J, Ein S, Balfe JW (1988) Anesthesia for phaeochromocytoma. Can J Anaesth 35:525–534

    Google Scholar 

  38. Young WF (2002) Pheochromocytoma. In: Physicians information and education resource (PIER). American College of Physicians

  39. Bravo EL (2002) Pheochromocytoma: an approach to antihypertensive management. Ann NY Acad Sci 970:1–10

    Google Scholar 

  40. Kaplan NM (1998) Clinical hypertension, 7th ed. Williams & Wilkins, Baltimore, pp 345–363

  41. 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

    Google Scholar 

  42. Desmonts JM, Marty J (1984) Anaesthetic management of patients with phaeochromocytoma. Br J Anaesth 56:781–789

    Google Scholar 

  43. Newell KA, Prinz RA, Brooks MH, Glisson SN, Barbato AL, Freeark RJ (1988) Plasma catecholamine changes during excision of pheochromocytoma. Surgery 104:1064–1073

    Google Scholar 

  44. Boutros AR, Bravo EL, Zanettin G, Straffon RA (1990) Perioperative management of 63 patients with pheochromocytoma. Cleve Clin J Med 57:613–617

    Google Scholar 

  45. Ulchaker JC, Goldfarb DA, Bravo EL, Novick AC (1999) Successful outcomes in pheochromocytoma surgery in the modern era. J Urol 161:764–767

    Google Scholar 

  46. 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

    Google Scholar 

  47. Schulz V (1984) Clinical pharmacokinetics of nitroprusside, cyanide, thiosulphate and thiocyanate. Clin Pharmacokinet 9:239

    Google Scholar 

  48. Hall VA, Guest JM (1992) Sodium nitroprussid-induced cyanide intoxication and prevention with sodium thiosulfate prophylaxis. Am J Crit Care 2:19–27

    Google Scholar 

  49. Forth W, Henschler D, Rummel W (1990) Allgemeine und spezielle Pharmakolgie und Toxikologie. BI, Zürich, pp 141–155

  50. 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

  51. Marino PL (1999) Das ICU-Buch (Hrsg. Taeger K), 2. Aufl. Urban und Fischer, S 254–258

  52. 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

    Google Scholar 

  53. 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

    Google Scholar 

  54. 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

    Google Scholar 

  55. Steinsapir J, Carr AA, Prisant LM et al (1997) Metyrosine and pheochromocytoma. Arch Intern Med 157:901

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to A. Schäffler.

Additional information

Serie: Die Notfall- und Intensivtherapie bei endokrinen und metabolischen Erkrankungen Herausgegeben von J. Schölmerich und Ulrike Woenckhaus (Regensburg)

Rights and permissions

Reprints and permissions

About this article

Cite this article

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

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00390-005-0519-7

Key words

Schlüsselwörter

Navigation