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DOI: 10.1055/s-2008-1027355
© Georg Thieme Verlag KG Stuttgart · New York
Severe Bilateral Hypertensive Retinopathy and Optic Neuropathy in a Patient with Pheochromocytoma[1]
Schwere beidseitige hypertensive Retinopathie und Optikusneuropathie bei einem Patienten mit PhäochromozytomPublikationsverlauf
received: 14.9.2007
accepted: 18.10.2007
Publikationsdatum:
05. Mai 2008 (online)

Zusammenfassung
Hintergrund: Wir beschreiben einen Fall mit schwerer beidseitiger hypertensiver Retinopathie und Optikusneuropathie wegen Phäochromozytom. Anamnese und Befund: Eine 31-jährige Frau stellte einen schweren beidseitigen Visusverlust verbunden mit Kopfschmerzen und Fotophobie vor. Ophthalmoskopie zeigte beidseitige Papillenschwellungen, weiche Exsudate, Sternfiguren der Makula, Streifenblutungen und Verengung der Arterien. Das Bild war schwerer im rechten Auge. Eine Fluoreszeinangiografie bestätigte die Diagnose einer hypertensive Retinopathie. Der Blutdruck war 220 / 145 mmHg. Eine gründliche systemische Kontrolle enthüllte einen Tumor der rechten Nebenniere. Catecholaminsekretion war normal. Nach der Ablation zeigte sich der Tumor als Phäochromozytom und eine Immunhistochemie zeigte Dopaminsekretion. Therapie und Verlauf: Der postoperative Verlauf war unauffällig. Die antihypertensive Therapie dauerte nur einige Monate. Die visuelle Schärfe wird zurückgegeben und die Netzhautveränderungen verschwanden 8 Monate nach der Operation. Schlussfolgerungen: Schwere hypertensive Retinopathie mit Optikusneuropathie könnte eine Konsequenz der malignen Hypertonie wegen dem Phäochromozytom sein. Sie ist reversibel nach der Ablation des Tumors. Frühdiagnose ist höchst wichtig und ist abhängig von Hormonuntersuchung und Immunhistochemie.
Abstract
Background: We present a case of severe bilateral hypertensive retinopathy and optic neuropathy due to pheochromocytoma. History and Signs: A 31-year-old woman presented with severe bilateral visual acuity loss associated with headaches and photophobia. Ophthalmoscopy showed bilateral optic disc edema, soft exudates, macular star, flame-shaped hemorrhages and arterial narrowing. The situation was more severe in the right eye. Fluorescein angiography confirmed the diagnosis of hypertensive retinopathy. Arterial blood pressure was 220 / 145 mmHg. A thorough systemic control revealed a right adrenal gland mass. Catecholamine secretion was normal. After ablation, the tumor proved to be a pheochromocytoma, and immunohistochemistry showed dopamine secretion. Therapy and Outcome: Postoperative evolution was uncomplicated. Antihypertensive treatment lasted only a few months. Visual acuity was restored and the retinal alterations disappeared 8 months after surgery. Conclusions: Severe hypertensive retinopathy with optic neuropathy may be a consequence of malignant hypertension due to a pheochromocytoma. It is reversible after ablation of the tumor. Early diagnosis is of vital importance and relies on hormonal investigation and immunohistochemistry.
Schlüsselwörter
Phäochromozytom - hypertensive Retinopathie - hypertensive Optikusneuropathie - maligne Hypertonie - Papillenödem - Sternfigur der Makula - Tumor der Nebenniere - Dopamin - Immunhistochemie
Key words
pheochromocytoma - hypertensive retinopathy - hypertensive optic neuropathy - malignant hypertension - optic disc edema - macular star - adrenal gland tumor - dopamine - immunohistochemistry
1 Presented at the 100th Annual Congress of the SOG/SSO, Montreux, 5. - 8.9.2007.
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1 Presented at the 100th Annual Congress of the SOG/SSO, Montreux, 5. - 8.9.2007.
John M. Katsimpris, MD, PhD
Ritsou & Empeirikou Street 73
26335 Patras, Greece
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