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Year : 2022  |  Volume : 60  |  Issue : 1  |  Page : 86-88

Pheochromocytoma-induced hypertensive retinopathy

1 Department of Ophthalmology, Sri Sankaradeva Nethralaya, Guwahati, Assam, India
2 Department of Ocular Pathology, Uveitis and Neuro-Ophthalmology Services, Sri Sankaradeva Nethralaya, Guwahati, Assam, India
3 Department of Oculoplasty and Neuro-Ophthalmology Services, Sri Sankaradeva Nethralaya, Guwahati, Assam, India

Date of Submission18-Jul-2021
Date of Acceptance27-Dec-2021
Date of Web Publication22-Mar-2022

Correspondence Address:
Dr. Suklengmung Buragohain
Department of Ophthalmology, Sri Sankaradeva Nethralaya, Beltola, Guwahati - 781 028, Assam
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/tjosr.tjosr_108_21

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Pheochromocytoma is an important cause of secondary hypertension that can lead to hypertensive retinopathy. In such cases, changes are often reversible and the condition remains stable once the required treatment is done at an early stage. In this case report, we present a case of pheochromocytoma-induced hypertensive retinopathy which regressed after adrenalectomy.

Keywords: Hypertensive retinopathy, pheochromocytoma, secondary hypertension

How to cite this article:
Buragohain S, Das D, Bhattacharjee H, Kuri GC, Ghosh R. Pheochromocytoma-induced hypertensive retinopathy. TNOA J Ophthalmic Sci Res 2022;60:86-8

How to cite this URL:
Buragohain S, Das D, Bhattacharjee H, Kuri GC, Ghosh R. Pheochromocytoma-induced hypertensive retinopathy. TNOA J Ophthalmic Sci Res [serial online] 2022 [cited 2022 Jul 2];60:86-8. Available from: https://www.tnoajosr.com/text.asp?2022/60/1/86/340336

  Introduction Top

Hypertensive retinopathy is a condition characterized by retinal vascular changes in the retina in the event of increased blood pressure (BP) (hypertension). Although most frequently the changes are seen in the retina, other structures such as the choroid and optic nerve can be involved as well.[1] Systemic hypertension has been subclassified into several groups although to keep it simple it can be divided mainly into primary and secondary hypertension.[2] Even though secondary hypertension is less common than primary, they are most often correctable.[3] Pheochromocytoma is one such important cause of secondary hypertension among many (renal artery stenosis, renal parenchymal disease, estrogen use, hyperaldosteronism, Cushing's syndrome, etc.).[4] We present a case of pheochromocytoma-induced hypertensive retinopathy in which timely intervention led to the regression of the effects on the retina due to uncontrolled BP.

  Case Report Top

A 33-year-old female came with complaints of left eye diminution of vision for the last 2 years which was gradual in onset and worsening. Past ocular history was not significant. Past medical history was positive for diabetes mellitus and for pheochromocytoma-induced hypertension for which she underwent left adrenalectomy 2 months back. The patient had been experiencing symptoms of severe headache and sweating for 2 years for which she underwent a thorough systemic evaluation 3 months back. On systemic evaluation, her BP was 250/150 mmHg with a pulse rate of 150 beats/min. Ultrasonography and computed tomography scan of the abdomen revealed a heterogeneously enhancing mass lesion arising from the left adrenal gland [Figure 1]. Urine metanephrine levels were high (726 μg/24 h). A diagnosis of pheochromocytoma was made and left adrenalectomy was done.
Figure 1: Computed tomography scan image of the abdomen showing a heterogeneously enhancing mass lesion arising from the left adrenal gland

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On examination, the unaided visual acuity (VA) was 6/9 in the right eye and 6/6 in the left eye. After correction, the VA improved to 6/6P in the right eye. The intraocular pressure was 19 and 16 mmHg, respectively, in the right and left eyes. On slit-lamp biomicroscopy, the anterior segment had normal findings in both eyes. Fundoscopy of the right eye [Figure 2]a showed an absent foveal reflex with maculopathy along with generalized arteriolar attenuation, silver wiring of the vessels, nicking of the vessels, and fibrous tissue over the disc along with mild disc pallor. The left eye [Figure 2]b funduscopy had similar findings in addition to regressed macular edema. High-resolution optical coherence tomography of both the eyes showed intraretinal edema along with retinal pigment epithelium alterations suggestive of regressed maculopathy [Figure 3]. The case was diagnosed as both eyes regressed hypertensive retinopathy secondary to pheochromocytoma-induced hypertension. The patient was continued on the antihypertensive medications, and on follow-up after 3 months, her vision remained stable.
Figure 2: Fundus photograph showing maculopathy along with generalized arteriolar attenuation, silver wiring of the vessels, nicking of the vessels, and fibrous tissue over the disc along with mild disc pallor in the right eye (a), and in addition to the above, regressed macular edema in the left eye (b)

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Figure 3: High-resolution optical coherence tomography scan of both the eyes (right [a] and left [b]) showing intraretinal edema along with retinal pigment epithelium alterations

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  Discussion Top

Pheochromocytomas are rare tumors arising in chromaffin cells of the adrenal medulla or paraganglia.[5] They can give rise to malignant hypertension in which there is an acute rise in BP (defined as systolic BP >200 mmHg and diastolic >140 mmHg). It is a life-threatening condition and is histologically characterized by fibrinoid necrosis of arterioles.[6]

Both acute and chronically raised states of Blood pressure can lead to characteristic changes in the intraocular tissues such as the retina, choroid, and optic nerve. Signs of a chronic hypertensive state include generalized arteriolar narrowing and arteriovenous nicking. In contrast, signs such as focal arteriolar narrowing, cotton-wool spots, and retinal hemorrhages are more commonly seen in acute states of elevated BP.[7],[8] In our case, the clinical picture was analogous to that of regressed hypertensive retinopathy after an event of malignant hypertension. Silver wiring of the vessels, nicking of the vessels, and fibrous tissue over the disc along with mild disc pallor were all traces of a past episode of malignant hypertension. Clinical features are mainly a result of constriction of vessels by the circulating catecholamines, the obstruction of arterioles, and the disruption of the blood–retina barrier.[9]

In our patient, although stable, there was a minimal reduction in the VA. Visual morbidity in such cases arises due to persistent papilledema or retinal pigment changes secondary to serous retinal detachment.[6] Pheochromocytoma-induced hypertension may present with many physical symptoms and signs, although sometimes the ocular features are the only clues that are present. A thorough fundus examination can give a great amount of information regarding the state of the cardiovascular system, and the signs of hypertensive retinopathy should not be missed.

The changes that take place in the ocular tissues due to secondary hypertension are reversible if treatment is done at an early stage.[3] This makes it paramount that the disease is diagnosed as early as possible. Clinical suspicion of secondary hypertension should always be kept in mind with regard to patients that present with hypertensive retinopathy changes at an age group that is atypical for the condition. Apart from the clinical features, other investigative modalities are available which can be utilized for the diagnosis of pheochromocytoma. Biochemical testing is an important tool available to diagnose pheochromocytoma or to rule it out and should be utilized.[10] In our case, urine metanephrines were high and that aided in the diagnosis of pheochromocytoma.

Once the diagnosis is established, the essential treatment can be done. In our case, after adrenalectomy, the patient had a reversal of the hypertensive retinopathy changes and maintained a stable vision till the last follow-up.

In conclusion, pheochromocytoma-induced hypertensive retinopathy, although rare, is an important cause of visual and systemic morbidity. Timely diagnosis is important, and the utilization of hormonal investigations along with immunohistochemistry plays a big role in that aspect. The changes are reversible with resection of the tumor, and visual prognosis is good in the early stages.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that her name and initials will not be published and due efforts will be made to conceal her identity, but anonymity cannot be guaranteed.


We would like to thank Sri Kanchi Sankara Health and Educational Foundation, Guwahati, India.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Update on the Ocular Manifestations of Systemic Arterial Hypertension – PubMed. Available from: https://pubmed.ncbi.nlm.nih.gov/15118507/. [Last accessed on 2021 Jul 18].  Back to cited text no. 1
Abul-Ezz S, Bunke C, Singh H. Arterial hypertension. In: Cecil's Essentials of Medicine. Philadelphia: W.B. Saunders; 1997. p. 227-31.  Back to cited text no. 2
Laragh J. Laragh's lessons in pathophysiology and clinical pearls for treating hypertension. Am J Hypertens 2001;14:491-503.  Back to cited text no. 3
Braunwald E. Hypertension. In: Harrison's Principles of Internal Medicine: Companion Handbook. New York: McGraw-Hill; 1995. p. 340-6.  Back to cited text no. 4
Bravo EL, Tagle R. Pheochromocytoma: State-of-the-art and future prospects. Endocr Rev 2003;24:539-53.  Back to cited text no. 5
Spencer CG, Lip GY, Beevers DG. Recurrent malignant hypertension: A report of two cases and review of the literature. J Intern Med 1999;246:513-6.  Back to cited text no. 6
Wong TY, Mitchell P. Hypertensive retinopathy. N Engl J Med 2004;351:2310-7.  Back to cited text no. 7
Sharrett AR, Hubbard LD, Cooper LS, Sorlie PD, Brothers RJ, Nieto FJ, et al. Retinal arteriolar diameters and elevated blood pressure: The Atherosclerosis Risk in Communities Study. Am J Epidemiol 1999;150:263-70.  Back to cited text no. 8
Hayreh S. Hypertensive fundus changes. In: Retinavitreous-Macula. Philadelphia: Saunders; 1999. p. 345-71.  Back to cited text no. 9
Eisenhofer G, Goldstein DS, Sullivan P, Csako G, Brouwers FM, Lai EW, et al. Biochemical and clinical manifestations of dopamine-producing paragangliomas: Utility of plasma methoxytyramine. J Clin Endocrinol Metab 2005;90:2068-75.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3]


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