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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 1  |  Issue : 2  |  Page : 337-339

Retinal arteriolar calcification and ischemic retinopathy in a patient with chronic kidney disease


1 Department of Vitreo-Retina, Aravind Eye Hospital, Madurai, Tamil Nadu, India
2 Neuro-Ophthalmology, Aravind Eye Hospital, Madurai, Tamil Nadu, India

Date of Submission27-May-2020
Date of Acceptance13-Sep-2020
Date of Web Publication01-Apr-2021

Correspondence Address:
Dr. Chitaranjan Mishra
Department of Vitreo.Retina, Aravind Eye Hospital, Anna Nagar, Madurai - 625 020, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_1696_20

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  Abstract 


A 58-year-female with a history of chronic kidney disease presented with defective vision in both the eyes (BE). Her vision was 20/120 in the right eye (RE) and 20/200 in the left eye (LE). Fundus evaluation revealed retinal arteriolar calcification, neovascularization of the disc (NVD), and drusen at the posterior pole in BE. Optical coherence tomography (OCT) images passing through the retinal arterioles indicated hyper reflective structures with back shadowing suggestive of intravascular calcification. En-face OCT angiography images over the optic discs were suggestive of NVD in BE. Blood investigation revealed elevated levels of phosphate and the product of calcium and phosphate levels. The patient was treated with pan-retinal photocoagulation in BE. However, the visual outcome was poor.

Keywords: Calciphylaxis, end-stage renal disease, optical coherence tomography, secondary hyperparathyroidism, vascular calcification


How to cite this article:
Mishra C, Kannan NB, Chakrabarti A, Kumar M, Ramasamy K. Retinal arteriolar calcification and ischemic retinopathy in a patient with chronic kidney disease. Indian J Ophthalmol Case Rep 2021;1:337-9

How to cite this URL:
Mishra C, Kannan NB, Chakrabarti A, Kumar M, Ramasamy K. Retinal arteriolar calcification and ischemic retinopathy in a patient with chronic kidney disease. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Aug 3];1:337-9. Available from: https://www.ijoreports.in/text.asp?2021/1/2/337/312337



Extraskeletal calcification is a noted complication in patients with end-stage renal disease (ESRD). Blood vessels and soft tissues are frequent sites of calcification in ESRD.[1] Hypocalcemia and hyperphosphatemia are direct results of chronic kidney disease (CKD) and secondary hyperparathyroidism (SHPT) occurs as a metabolic consequence of hypocalcemia. All these factors are proposed to be causing vascular calcification (VC).[2],[3] Retinal vessels are unusual sites of calcification in CKD.[4] In this case report, we describe retinal arteriolar calcification (RAC) in a patient with ESRD.


  Case Report Top


A 58-year-female presented with defective vision in both the eyes (BE) for the past 10 days. She gave a history of recurrent urinary tract infections for the past 6 years. She had undergone ultrasonography of the abdomen and pelvis and was diagnosed with staghorn calculi in both her kidneys 5 years ago. She had undergone right-sided percutaneous nephrolithotomy with a double J stent twice in the last 5 years. Though the treating nephrologist advised hemodialysis, she never underwent dialysis because she thought that frequent procedures related to dialysis will decrease the quality of her life. There was no history of diabetes, hypertension or cardiac disease. She was on native ayurvedic treatment for the past 4 years; the details of which could not be obtained. Her body mass index was 18 kg/m2. The best-corrected vision in the right eye (RE) was 20/120 and in the left eye (LE) was 20/200. The intraocular pressure (IOP) in BE was 16 mmHg. Anterior segment examination was normal in BE. The fundus examination revealed soft exudates, drusen, and whitish intravascular deposits suggestive of calcification in BE [Figure 1]a and [Figure 1]b. OCT scans passing through retinal arterioles of BE revealed hyperreflective structures with back shadowing suggestive of RAC [Figure 1]c and [Figure 1]d. To know the exact cause of the intravascular deposit of calcium materials, we asked the patient to undergo laboratory evaluation of hemoglobin, serum calcium, serum phosphorus, renal function tests, and urine examination. The patient visited us with laboratory reports after 3 weeks. This time, anterior segment examination revealed the neovascularization of iris and neovascularization of the angles. Her IOP was 17 mmHg in RE and 23 mmHg in LE. The fundus examination revealed the neovascularization of the discs (NVD) in addition to the prior fundus findings [Figure 2]a and [Figure 2]b. OCT scan through the center of the fovea showed disorganization of both the inner and outer retinal layers with damage to the photoreceptor layers, irregular retinal pigment epithelium, and drusen in BE [Figure 2]c and [Figure 2]d. The extent of damage was more severe in the LE [Figure 2]d. Fundus fluorescein angiography was contraindicated due to CKD. En-face OCT angiography (OCTA) images of the superficial layers over the optic discs were suggestive of NVD in BE [Figure 2]e and [Figure 2]f. The blood investigation of the patient revealed hemoglobin level of 7.4 gm/dL (normal range 12-15.5 gm/dL), urea level of 123 mg/dL (normal range 10-40 mg/dL), creatinine level of 12.9 mg/dl (normal range 0.6-1.4 mg/dL), calcium level of 8.2 mg/dL (normal range 8.5-10.1 mg/dL), phosphate level of 7.3 mg/dL (normal range 2.3-4.7 mg/dL), intact parathyroid hormone level of 233.8 pg/mL (normal range 15-68.3 pg/mL), and vitamin D3 level of 26.96 ng/dL (normal range is more than 30 ng/dL). The product of calcium and phosphate (Ca x P) was 59.86 mg2/dL2 (normal level is less than 55 mg2/dL2). Her urine examination was positive for amorphous crystals of phosphorus. The estimated glomerular filtration rate was 26 mL/min/1.73m2 (normal range is 90-120 mL/min/1.73 m2), which was suggestive of stage 4 CKD. She was diagnosed to be a case of RAC with ischemic retinopathy. She was advised and underwent pan-retinal photocoagulation (PRP) in four sittings in BE. She was prescribed topical antiglaucoma medications (eye drops timolol 0.5% BD) starting from the first sitting of PRP in BE. The patient visited us 6 weeks after PRP with a further decrease in vision to counting finger close to face in BE. There was the persistence of NVD and drusen in BE [Figure 3]a and [Figure 3]b. OCT scans taken through the center of the fovea revealed disorganization of both the inner and outer retinal layers with damage to the photoreceptor layers (LE more than RE), irregular retinal pigment epithelium, and drusen in BE [Figure 3]c and [Figure 3]d. En-face OCTA of the superficial layers revealed persistence of NVD in BE [Figure 3]e and [Figure 3]f.
Figure 1: Wide-angle fundus image (Optos Daytona-Plus (Optos PLC, Dunfermline, Scotland, UK)) of the (a) right and (b) left eyes, showing whitish intravascular deposits suggestive of retinal arteriolar calcification (RAC). There are drusen and soft exudates at the macula in both the eyes. Also, note the superficial retinal hemorrhage over the right optic disc margin. (c) OCT B scan of the right eye and (d) OCT B scan of the left eye. The scan is taken passing through the blood vessels (white triangles). White arrows indicate hyperreflective structures with back shadowing corresponding to the retinal vessels suggestive of RAC

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Figure 2: Wide-angle fundus image (Zeiss Clarus 500) of the (a) right and (b) left eyes, showing retinal arteriolar calcification (RAC). There are drusen and soft exudates at the macula in both the eyes (BE). Also, note the neovascularization of the optic discs (NVD) in BE. OCT image of the (c) right and (d) left eyes, showing disorganization of both the inner and outer retinal layers with damage to the photoreceptor layers, irregular retinal pigment epithelium and drusen in BE. (e and f) En-face OCTA image of the superficial layers over the optic disc of BE suggestive of NVD

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Figure 3: Fundus image (Zeiss Clarus 500) of the (a) right and (b) left eyes, showing retinal arteriolar calcification (RAC), NVD, and drusens in both the eyes (BE). Note the PRP laser spots in BE. OCT image of the (c) right and (d) left eyes, showing disorganization of both the inner and outer retinal layers with damage to the photoreceptor layers (left eye more than the right eye), and drusens in both the eyes. (e) and (f) En-face OCTA image of the superficial layers over the optic discs of BE suggestive of the persistence of NVD

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  Discussion Top


RAC is an infrequent complication of CKD. RAC is associated with gross retinal ischemia and its complications. The complications of gross retinal ischemia in CKD include neovascularization, hemorrhages, drusen-like deposits, and soft exudates.[5],[6],[7],[8] Noninvasive investigational modalities like OCT, OCTA, and wide angle fundus photo help in the documentation of these ocular findings. PRP is the ocular treatment done to decrease the ischemic damage to the retina.

A case of RAC reported by Patel et al. in 2002 was having retinal findings similar to our case.[4] However, the age of the patient was younger (33 years vs 58 years), the duration of the disease was longer (10 years vs 5 years), and the patient was on treatment with hemodialysis in the previously reported case. Serum calcium and phosphorus levels were elevated in both the previous reported case and in our patient.

Metastatic calcification occurs due to abnormal metabolism of calcium and phosphate. Extensive calcification of vascular wall and soft tissues has been mentioned to be a frequent feature of patients with ESRD and has been attributed to hyperphosphatemia and SHPT.[2],[4] Kidneys play an active role in the normal physiology of vitamin D, serum calcium, and phosphorus. In kidneys, 25(OH) D3 gets converted to active vitamin D3 [1,25-(OH) 2D3] by renal 1α hydroxylase. Active vitamin D3 increases calcium absorption from the gut, increases serum calcium, and thus downregulates the production of parathyroid hormone (PTH). In CKD, renal 1α hydroxylase production is decreased; thus, the production of active vitamin D3 is reduced. As a result, serum calcium is decreased and the PTH level increases. Elevated levels of phosphate and the product of serum calcium and phosphate (Ca x P) are important causes of vascular and soft tissue calcification and cardiovascular mortality in ESRD patients.[3] Our patient had elevated levels of both these parameters.

The lesions of microvascular retinopathy in patients of CKD may be the effect of associated systemic hypertension, diabetic retinopathy, or nontraditional pathways for vascular leakage-like inflammation, calcification, and endothelial damage.[5] In the absence of hypertension and diabetes probably the nontraditional pathways for vascular leakage were responsible for widespread ischemia in our patient.

Calciphylaxis is a life-threatening syndrome that results due to VC in the subcutaneous tissue in patients with ESRD.[9] The resulting ischemia and infarction cause intense pain and gangrene in the patients.[9] RAC has been mentioned to precede calciphylaxis in CKD patients. Our patient did not have any skin or extremity lesions. However, close follow-up is needed since the life expectancy is less than 1 year in patients of calciphylaxis.[9]

Some similar-looking fundus appearances must be differentiated form RAC with the help of proper clinical and laboratory evaluation. These differentials include cream-colored retinal vessels in lipaemia retinalis, metabolic abnormalities like oxalosis, and dystrophic calcification of retinal vessels in long-standing proliferative diabetic retinopathy and retinal vein occlusions.

Treatment of the patients of ESRD includes hemodialysis and renal transplant.[10] However, palliative and supportive care have a role in ethical decision making in patients not willing for either dialysis or renal transplant.[10] The management of hyperphosphatemia and hypercalcemia include dietary phosphate restriction, calcium-based and non-calcium-based phosphate binders, activated vitamin D3 supplement and calcimimetic agents, e.g., cinacalcet.[11] PRP in multiple sessions is the prescribed ocular management for retinal ischemia.


  Conclusion Top


The patients of CKD with RAC-related ocular ischemia should be treated with hemodialysis and PRP appropriately to prevent further damage to the retina. In spite of the available treatment modalities, the mortality of these patients is high and visual prognosis is poor.

Declaration of patient consent

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

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Moe SM, Chen NX. Mechanisms of vascular calcification in chronic kidney disease. J Am Soc Nephrol 2008;19:213-6.  Back to cited text no. 1
    
2.
Cozzolino M, Gallieni M, Brancaccio D. The mechanisms of hyperphosphatemia-induced vascular calcification. Int J Artif Organs 2008;31:1002-3.  Back to cited text no. 2
    
3.
Block GA. Prevalence and clinical consequences of elevated Ca x P product in hemodialysis patients. Clin Nephrol 2000;54:318-24.  Back to cited text no. 3
    
4.
Patel DV, Snead MP, Satchi K. Retinal arteriolar calcification in a patient with chronic renal failure. Br J Ophthalmol 2002;86:1063.  Back to cited text no. 4
    
5.
Deva R, Alias MA, Colville D, Tow FK, Ooi QL, Chew S, et al. Vision-threatening retinal abnormalities in chronic kidney disease stages 3 to 5. Clin J Am Soc Nephrol 2011;6:1866-71.  Back to cited text no. 5
    
6.
Evans RD, Rosner M. Fellows' forum: Ocular abnormalities associated with advanced kidney disease and hemodialysis. Semin Dial 2005;18:252-7.  Back to cited text no. 6
    
7.
Ahsan MK, Alam MR, Khanam A, Ahmed AH, Faroque MO, Hadiuzzaman KB, et al. Ocular fundus abnormalities in pre-dialytic chronic kidney disease patients. J Biosci Med 2019;7:20.  Back to cited text no. 7
    
8.
Liew G, Mitchell P, Wong TY, Iyengar SK, Wang JJ. CKD increases the risk of age-related macular degeneration. J Am Soc Nephrol 2008;19:806-11.  Back to cited text no. 8
    
9.
Nigwekar SU, Wolf M, Sterns RH, Hix JK. Calciphylaxis from nonuremic causes: A systematic review. Clin J Am Soc Nephrol 2008;3:1139-43.  Back to cited text no. 9
    
10.
Rak A, Raina R, Suh TT, Krishnappa V, Darusz J, Sidoti CW, et al. Palliative care for patients with end-stage renal disease: Approach to treatment that aims to improve quality of life and relieve suffering for patients (and families) with chronic illnesses. Clin Kidney J 2017;10:68-73.  Back to cited text no. 10
    
11.
Dong BJ. Cinacalcet: An oral calcimimetic agent for the management of hyperparathyroidism. Clin Ther 2005;27:1725-51.  Back to cited text no. 11
    


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  [Figure 1], [Figure 2], [Figure 3]



 

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