|Year : 2016 | Volume
| Issue : 2 | Page : 92-95
Bilateral exudative retinal detachment in a case of paraganglioma
Vishal Vohra, Harshika Chawla, Praveen Malik
Department of Ophthalmology, Dr. Ram Manohar Lohia Hospital and Post Graduate Institute of Medical Education and Research, New Delhi, India
|Date of Submission||30-Oct-2014|
|Date of Acceptance||14-Apr-2015|
|Date of Web Publication||9-Jun-2016|
Doctor Hostel, Room 317, Department of Ophthalmology, Dr. Ram Manohar Lohia Hospital and Post Graduate Institute of Medical Education and Research, New Delhi - 110 001
Source of Support: None, Conflict of Interest: None
Bilateral exudative retinal detachment due to extra-adrenal pheochromocytomas in a pediatric age group has been rarely reported. We report a case of a 12-year-old female child with exudative retinal detachment and hypertensive retinopathy changes secondary to hypertensive emergency due to extra-adrenal pheochromocytoma. The patient presented with sudden painless diminution of vision in both eyes since 15 days. Fundus examination showed exudative retinal detachment with Grade 4 hypertensive retinochoroidopathy changes. Supine blood pressure of 190/140 mm of Hg was recorded. Contrast-enhanced computed tomography (CECT) of the abdomen revealed a heterogeneously enhancing right para-aortic mass likely representing extra-adrenal pheochromocytoma. Hypertensive ocular damage represents target organ damage in humans with systemic arterial hypertension. Fundus examination is a rapid and low cost modality for detecting previously existing and new onset vascular damage. Prompt recognition and timely referral of such cases have prodigious implications for both ocular and general health of a person.
Keywords: Hypertensive choroidopathy, paraganglioma, serous retinal detachment
|How to cite this article:|
Vohra V, Chawla H, Malik P. Bilateral exudative retinal detachment in a case of paraganglioma. J Clin Ophthalmol Res 2016;4:92-5
|How to cite this URL:|
Vohra V, Chawla H, Malik P. Bilateral exudative retinal detachment in a case of paraganglioma. J Clin Ophthalmol Res [serial online] 2016 [cited 2020 Sep 20];4:92-5. Available from: http://www.jcor.in/text.asp?2016/4/2/92/183720
Hypertension leads to persistent pathologic elevation of blood pressure and exaggerated total peripheral resistance that is related to vascular lesions within the brain, heart, kidneys, and eyes. Rise of blood pressure causes focal and generalized retinal arteriolar constriction, presumably mediated by autoregulation, that if prolonged results in breakdown of the inner blood-retinal barrier, with extravasation of plasma fluid and red blood cells. Consequently, retinal hemorrhages, cotton wool spots, intraretinal lipid, and, sometimes, a macular star configuration of hard exudates are often seen.  Although the retinal vascular changes and optic neuropathy secondary to hypertension are renowned, hypertensive choroidopathy is seldom reported.  We report a case of hypertensive choroidopathy with bilateral serous retinal detachments.
| Case Report|| |
A 12-year-old girl child presented to ophthalmology outpatient department with complaint of sudden painless diminution of vision in both eyes since 15 days. The patient gave an account of four to five episodes of throbbing headache associated with sweating and nausea, which used to get resolved spontaneously. There was history of death of her father at the age of 28 years of similar preceding symptoms. No records of treatment of father were available.
Ophthalmologic examination revealed visual acuity of counting fingers at 2 meters in right eye (RE) and at 1 meter in left eye (LE) with no improvement of vision on pin hole and refraction. Pupils were sluggishly reacting. Intraocular pressures were 10 and 12 mm of Hg in RE and LE, respectively. Fundus examination of both eyes showed presence of extensive disc edema (LE >RE) extending to peripapillary region with diffuse arteriolar attenuation and a few flame-shaped hemorrhages surrounding the disc [Figure 1]. Macular fan was seen on nasal side of the macula in both the eyes. Elschnig spots were also seen temporal to macular area [Figure 2]. Inferior retinal detachment with a smooth surface, spanning an area from 3 to 9'O clock just below the lower extent of macula in prone position was seen [Figure 3]. Detachment extended to macula in supine position due to shifting of fluid. B mode ultrasound with A scan overlay showed inferior retinal detachment with subretinal fluid shifting to the most dependent position on movement of head position [Figure 4], confirming the diagnosis of exudative retinal detachment in both eyes.
|Figure 1: Colored fundus photographs showing extensive disc edema with diffuse arteriolar attenuation and a few flame shaped hemorrhages surrounding the disc and macular fan on nasal side of the macula in both the eyes|
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|Figure 3: Colored fundus photographs of both eyes showing inferior retinal detachment|
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|Figure 4: B scan with A scan overlay showing inferior retinal detachment with shifting subretinal fluid|
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Supine blood pressure of 190/140 mm of Hg was recorded. Patient was then immediately referred to pediatric department for urgent management and workup of the cause of malignant hypertension. She was started on sodium nitroprusside and amlodipine for management of hypertensive emergency and was asked to rest in propped up position to minimize the extension of retinal detachment into macular region.
Routine laboratory workups were normal. Contrast-enhanced computed tomography (CECT) of the abdomen revealed a heterogeneously enhancing right para-aortic mass causing right ureteric compression with right sided moderate hydronephrosis, which in a clinical setting of hypertensive crisis likely represented extra-adrenal pheochromocytoma [Figure 5]. Twenty-four hour urine metanephrines were 460 μg/24 h and normetanephrines were 620 μg/24 h, confirming the diagnosis.
|Figure 5: CECT abdomen showing heterogeneously enhancing right para-aortic mass causing right ureteric compression with right-sided moderate hydronephrosis. CECT = Contrast-enhanced computed tomography|
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Patient was followed-up in pediatric surgery department. Patient underwent surgical resection of tumor after control of hypertension. Patient was then lost to follow-up.
| Discussion|| |
Our patient showed Grade 4 hypertensive retinochoroidopathy with exudative retinal detachment. Although a classic ocular sequelae of severe hypertension, these findings are relatively uncommon to the extent manifested in our patient. Among the varied etiologies of exudative retinal detachment, malignant hypertension comprises a minor cluster of cases.  Hypertension causes hypertensive retinopathy, optic neuropathy, and choroidopathy.  Among the three, hypertensive choroidopathy is less known. Hypertensive choroidopathy usually occurs later than retinal arteriolar narrowing and arteriovenous changes. 
When the choroidal vessels are severely plagued by elevated blood pressure, as in acute hypertension, fibrinoid necrosis of choroidal arterioles occurs, with a resultant breakdown of the outer blood-retinal barrier. Hypertensive choroidopathy has been reported in toxemia of pregnancy, renal disease, pheochromocytoma, and malignant hypertension.  Choroid receives sympathetic innervation and is sensitive to circulating vasoconstrictive factors like angiotensin II, adrenaline, and antidiuretic hormone (ADH). These factors and neural stimulation initiate constriction of the choroid and choriocapillaris.  Hypertensive choroidopathy occurs in three phases, viz., acute ischemic, chronic occlusive, and chronic reparative phase. Throughout the acute ischemic phase, choroidal arterioles constrict, causing necrosis of the retinal pigment epithelium (RPE) and choriocapillaris leading to accumulation of exudates within the subretinal space and focal serous retinal detachment. The chronic occlusive phase is characterized by narrowing or occlusion of the choroidal capillaries and leak of the RPE overlying these regions. Degenerative RPE lesions develop over the macula and periphery, which intensify over time. Finally, in the chronic reparative phase, the occluded vessels recanalize, RPE heals, and the retina reattaches. The more ordinarily described features of hypertensive choroidopathy are Elschnig spots which are focal areas of chronic RPE degeneration of chronic hypertension.  Siegrist's streaks, linear retinal pigment epithelial changes, which are the sequelae of acute hypertensive choroidopathy, indicate a poor prognosis.  Serous retinal detachment, a prominent feature within the animal studies, is less commonly seen in clinical settings. 
Malignant hypertension can be labeled as a medical emergency. Its rare causes are pheochromocytomas and paragangliomas. The extra-adrenal pheochromocytomas are referred to as paragangliomas. A majority of the extra-adrenal tumors occur intra-abdominally (85% occur below the diaphragm), from the organ of Zuckerkandl or along the sympathetic chain.  Almost all sympathetic extra-adrenal paragangliomas and pheochromocytomas produce, store, metabolize, and secrete catecholamines or their metabolites.
Most pheochromocytomas represent sporadic tumors. However, 15-30% cases have disease as part of a familial disorder. Sporadic tumors are usually unicentric and unilateral, while familial ones are often multicentric and bilateral. Familial disorders associated with adrenal pheochromocytoma are Von Hippel-Lindau (VHL) syndrome, multiple endocrine neoplasia type 2 (MEN2), and neurofibromatosis type 1 (NF1); all of which have autosomal dominant inheritance. Hereditary pheochromocytoma typically present at a younger age than sporadic forms. 
Pheochromocytoma presents with high blood pressure due to catecholamine excess in 95% of cases.  Clinical picture of hypertension vary and may show either a persistent or paroxysmal pattern.  In some patients, paroxysms can occur along with sustained hypertension. On the contrary, a small, however a vital proportion of patients with pheochromocytoma are normotensive. Additional symptoms seen in pheochromocytoma patients include headache, palpitation, anxiety, and sweating.  It can result in angina, myocarditis, arrhythmias, heart attack, cardiomyopathies, postural hypotension, organ ischemia, acute renal failure, pulmonary edema, encephalopathy, shock, and a flamboyant range of ocular complications. 
While the ultimate management of pheochromocytoma is surgical, medical treatment still remains significant in perioperative management of blood pressure, as well as in cases of inoperable metastatic disease. It is a widely acknowledged fact that tumor handling during surgery can unharness tremendous amounts of catecholamines into circulation, which could overpower the pharmacological antagonism. So as to control blood pressure before the surgery, even in preoperative normotensive patients, it is advocated that patients undergo preoperative medical treatment.  Phenoxybenzamine is a noncompetitive alpha antagonist that reduces blood pressure fluctuation and blocks vasoconstriction, preventing an intraoperative hypertensive surge. 
| Conclusion|| |
Hypertensive ocular damage represents target organ damage in humans with systemic arterial hypertension. Fundus examination is a rapid and low cost modality for detecting previously existing and new onset vascular damage. For prompt recognition and timely referral of such cases have prodigious implications for both ocular and general health of a person.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]