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NDT Advance Access originally published online on April 1, 2007
Nephrology Dialysis Transplantation 2007 22(6):1769-1771; doi:10.1093/ndt/gfm034
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© The Author [2007]. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

Retinal arterial and venous occlusions in patients with ADPKD

Qi Qian1,2, Brian R. Younge{dagger} and Vicente E. Torres1

1Division of Nephrology and Hypertension and 2Department of Physiology and Biomedical Engineering, {dagger}Department of Ophthalmology, Mayo Clinic College of Medicine and Mayo Graduate School, Rochester, MN, USA

Correspondence and offprint requests to: Qi Qian, Assistant Professor of Medicine and Physiology, Division of Nephrology and Hypertension, Mayo Clinic College of Medicine, 200 First Street SW, Rochester, MN 55905, USA. Email: qian.qi{at}mayo.edu

Keywords: ADPKD; central retinal vascular occlusion; hypertension; vasculopathy



   Introduction
Top
 Introduction
Case report
 Discussion
 Acknowledgements
 References
 
Autosomal dominant polycystic kidney disease (ADPKD) is a systemic disease with renal and extra renal, cystic and non-cystic manifestations. The cystic disease involves kidney, liver, pancreas, seminal vesicles and meninges. Non-cystic manifestations include aneurysms and dolichoectasias (intracranial, thoracic aorta, coronary and other arteries), valvular heart disease, hernias and possibly intestinal diverticula [1]. Hypertension, arterial vasospasm, and extensive remodelling of small renal arteries and arterioles at early stages of cystic disease are also evident [2–5]. Cardiovascular complications are the leading cause of death in ADPKD. Here, we report eight cases of retinal arterial and/or venous occlusions in patients with ADPKD and propose that these conditions may be part of the generalized vasculopathy associated with ADPKD.



   Case report
Top
 Introduction
 Case report
Discussion
 Acknowledgements
 References
 
A 43-year-old male presented to the emergency room with a sudden loss of vision in his right eye. The vision loss occurred without any associated symptoms or notable preceding event. His medical history was significant for diagnoses of ADPKD at age 19 and hypertension at 26. The hypertension was well controlled with a combination regimen of atenolol and hydrochlorothiazide. His glomerular filtration rate was estimated at 27 ml/min/1.73 m2. He had no prior vision problem. On physical examination, blood pressure was 138/88 mmHg and polycystic kidneys palpable. Visual acuity was sharply reduced in the right eye (OD: 20/400) from previous 20/20; the left eye was unaffected (OS: 20/20). Ophthalmoscopic examination of the right eye revealed multiple areas of retinal haemorrhages with venous dilatations adjacent to the disc, cotton wool patches and focal areas of retinal opacifications. These findings were consistent with combined central retinal vein occlusion (CRVO) and branch retinal artery occlusion (BRAO). Throughout the ensuing year, he was treated with multiple sessions of pan-retinal photocoagulations, but the vision in his right eye remained negligible.

In the last 20 years, we have seen a number of ADPKD patients with a similar clinical presentation. These cases are summarized in Table 1. Most of them developed retinal vascular occlusions at a much younger age than typically found in the general population. Apart from a diagnosis of ADPKD, the only predisposing condition was hypertension, which was controlled in all cases. None of the patients had diabetes mellitus. Seven of the eight were non-smokers (the only smoker, patient #7, reported smoking 6–8 cigarettes/day at the time of presentation). We propose that retinal vascular occlusion may be a manifestation of the vasculopathy associated with ADPKD.


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  		Table 1. Eight cases of retinal vascular occlusions in patients with ADPKD

 


   Discussion
Top
 Introduction
 Case report
 Discussion
Acknowledgements
 References
 
Despite their critical role in maintaining the central vision, the retinal artery and vein are anatomically arranged in a way that makes the central retina vulnerable to factors that alter the blood flow. The central retinal artery and its branches course together with a single retinal vein and its branches in a tight fibrous sheath (optical nerve sheath); thereby, a thickening of the central retinal artery may compress the adjacent vein (especially at points of arteriovenous crossings), leading to venous occlusion. As a sole provider of blood to the inner two-thirds of the retina through all layers of capillary networks, the central retinal artery travels centrifugally from the centre of the optic disc to the periphery of the retina with a progressive slowing in linear blood flow. This flow pattern results in a high O2 extraction with an arterial-venous PO2 gradient of ~40% [6]. The central retina is, therefore, highly susceptible to a reduction and/or stagnation in blood flow [7].

The prevalence of retinal artery or vein occlusion in the general population increases with age. Central retinal artery occlusion (CRAO) occurs at 0.01% in persons with an average age of 60–65 years [8]. CRVO occurs at 0.7% in persons between 49 and 60 years of age. The prevalence progressively increases to 4.6% in persons older than 80 years [9]. Because CRAO and CRVO are rare in persons under 50 years, an investigation to rule out predisposing conditions is advocated for patients presenting at ages under 50. The risk factors for developing CRAO and CRVO include hypertension, diabetes, hyperlipidaemia, cigarette smoking, hyperviscosity or hypercoagulable states and hyperhomocysteinaemia [10]. Although its role in retinal vascular occlusive diseases has not been ascertained, renal dysfunction per se is not considered an independent risk factor.

Patients with central retinal vascular occlusions typically present with a sudden, painless impairment of central vision. CRAO is generally associated with a more severe degree of vision loss than that in CRVO. Ischaemic (non-perfused) occlusions are associated with worse vision loss than the non-ischaemic (partially perfused with venous stasis) ones [11]. When the branches of the retinal artery or vein become occluded (BRAO) or branch retinal venous occlusion (BRVO), the vision loss is usually limited to the regions perfused by the branches. Ophthalmoscopic evaluation (Figure 1) typically shows varying degrees of retinal ischaemia and opacity for CRAO; retinal haemorrhages with venous dilatation and cotton wool spots for CRVO; and retinal/macular oedema for both conditions. Long-term complications for both CRAO and CRVO include retinal neovascularization, macular detachment with vitreous haemorrhage and neovascular glaucoma. The final visual acuity depends on the visual acuity at presentation, the duration of visual impairment and the severity of the subsequent complications. In general, the final vision after CRAO is worse than that in CRVO [12,13].


Figure 1
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  		Fig. 1. (A) CRAO showing retinal opacification, a cherry-red spot in fovea, and the boxcar gaps in the veins. (B) CRVO, the picture taken from patient #6 at presentation showing multiple blotchy retinal haemorrhages with dilated veins.

 
Aggressive control of the risk factors is critical to the prevention of retinal vascular diseases and their complications. After the onset of the disease, treatment has been focused primarily on correcting identifiable inciting factors. In recent years, new treatment modalities have been emerging with promising results. For CRVO, methods have been developed to promptly re-establish the blood egress, including optic nerve sheath decompression, lamina puncture and chorioretinal anastomosis [14]. For the patients with neovascularization following CRAO/CRVO, pan-retinal laser photocoagulation can reduce the long-term complications and improve final visual acuity.

We report the occurrence of central retinal vascular occlusions in eight patients with ADPKD at a median age of 51.2 years, contrasted with an approximately 63–75 years in the general population [8,9]. Thus, as in the case of aneurysmal subarachnoid haemorrhage [15], ADPKD patients are at risk for developing central retinal vascular occlusions at a younger age. We suggest that retinal vascular occlusion is another manifestation of the systemic vasculopathy in ADPKD. Enhanced vasocontraction and arterial remodelling [2,4,5], as shown in Pkd2+/– (mouse orthologous model of ADPKD) conduit and resistance arteries [16,17], may increase the risk of these patients for this complication. Increased awareness of this manifestation could lead to an earlier diagnosis and treatment before irreversible vision loss develops.



   Acknowledgements
Top
 Introduction
 Case report
 Discussion
 Acknowledgements
References
 
NIH DK63064 and Mayo Clinic FUTR (QQ).

Conflict of interest statement. None declared.



   References
Top
 Introduction
 Case report
 Discussion
 Acknowledgements
 References
 

  1. Torres V, Holley K, Offord K. Epidemiology. In: Problems in Diagnosis and Management of Polycystic Kidney Disease—Grantham J, Gardner K, eds. (1985) Kansas City: PKR Foundation. 49–69.
  2. King BF, Torres VE, Brummer ME, et al. Magnetic resonance measurements of renal blood flow as a marker of disease severity in autosomal-dominant polycystic kidney disease. Kidney Int (2003) 64:2214–2221.[CrossRef][Web of Science][Medline]
  3. Chapman AB, Rubinstein D, Hughes R, et al. Intracranial aneurysms in autosomal dominant polycystic kidney disease. New Engl J Med (1992) 327:916–920.[Abstract]
  4. Gabow PA, Ikle DW, Holmes JH. Polycystic kidney disease: prospective analysis of nonazotemic patients and family members. Ann Intern Med (1984) 101:238–247.[Abstract/Free Full Text]
  5. Schacht F. Hypertension and vascular studies in congenital polycystic kidney [master's thesis]. In: Thesis for Master of Science in Urology (1930) St Paul, MN: University of Minnesota.
  6. Henkind P, Hansen RI, Szalay J. Ocular circulation. In: Physiology of the Human Eye and Visual System—Records RE, ed. (1979) New York: Harper & Row. 98–155.
  7. Hill DW. The regional distribution of retinal circulation. Ann R Coll Surg Engl (1977) 59:470–475.[Web of Science][Medline]
  8. Lorentzen SE. Occlusion of the central retinal artery. A follow-up. Acta Ophthalmol (Copenh) (1969) 47:690–703.[Medline]
  9. Mitchell P, Smith W, Chang A. Prevalence and associations of retinal vein occlusion in Australia. The Blue Mountains Eye Study. Arch Ophthalmol (1996) 114:1243–1247.[Abstract/Free Full Text]
  10. Colucciello M. Retinal vascular disease in hypertension. Risk factor modification optimizes vision outcomes. Postgrad Med (2005) 117:33–38, 41–42.[Medline]
  11. Hayreh SS, Rojas P, Podhajsky P, et al. Ocular neovascularization with retinal vascular occlusion-III. Incidence of ocular neovascularization with retinal vein occlusion. Ophthalmology (1983) 90:488–506.[Web of Science][Medline]
  12. Augsburger JJ, Magargal LE. Visual prognosis following treatment of acute central retinal artery obstruction. Br J Ophthalmol (1980) 64:913–917.[Abstract/Free Full Text]
  13. Group TCVOS. Natural history and clinical management of central retinal vein occlusion. The Central Vein Occlusion Study Group. Arch Ophthalmol (1997) 115:486–491.[Abstract/Free Full Text]
  14. Shahid H, Hossain P, Amoaku WM. The management of retinal vein occlusion: is interventional ophthalmology the way forward? Br J Ophthalmol (2006) 90:627–639.[Abstract/Free Full Text]
  15. Schievink WI, Torres VE, Piepgras DG, et al. Saccular intracranial aneurysms in autosomal dominant polycystic kidney disease. J Am Soc Nephrol (1992) 3:88–95.[Abstract]
  16. Qian Q, Hunter LW, Li M, et al. Pkd2 haploinsufficiency alters intracellular calcium regulation in vascular smooth muscle cells. Hum Mol Genet (2003) 12:1875–1880.[Abstract/Free Full Text]
  17. Qian Q, Hunter LW, Du H, et al. Pkd2+/- Vascular smooth muscles develop exaggerated vasocontraction in response to phenylephrine stimulation. J Am Soc Nephrol (2007) 18:485–493.[Abstract/Free Full Text]
Received for publication: 30.12.06
Accepted in revised form: 10. 1.07


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