All about the eye in kidney diseases or renal diseases: ocular changes in kidney failure, transplant, and dialysis, and other eye problems or ocular manifestation of kidney diseases.
Introduction to Kidney Diseases
The kidneys are the filter organs of the body. There are two bean-shaped kidneys on either side of the spine, below the ribs, and behind the belly. The kidney is about 4 or 5 inches long, roughly the size of a large fist.
The main function of the kidney is to filter wastes and excess fluids from blood, which are then excreted in the urine.
Chronic kidney disease, also called chronic kidney failure, describes the gradual loss of kidney function.
When chronic kidney disease reaches an advanced stage, dangerous levels of fluid, electrolytes, and wastes can build up in the body.
Most common kidney diseases include Alport Syndrome, Lowes Syndrome, Familial Juvenile Neprophthisis, Aniridia syndrome, Kidney Failure, among many others.
Kidney Eye Connection: Vision Problem & Eye in Kidney Diseases
When the normal functioning of the kidney is compromised, it affects not only the kidney but also the other several vital organs of the body, including the eyes. Let’s have a look at some of the common renal or kidney diseases and the associated ocular signs and symptoms.
Alport syndrome is a genetic condition characterized by kidney disease, hearing loss, and eye abnormalities. It is found that males are affected more frequently and more severely than females.
The most frequent onset of Alport syndrome is discovered in the people of age group 5-20 years but has been noted as early as 5 months.
It is estimated that the Alport occurs in 1 baby per 50,000 newborns.
The signs and symptoms typically begin in childhood, and the first sign of the condition is usually the presence of blood in the urine (haematuria). In addition, there can be proteinuria.
Over time, an affected person may experience swelling (edema), bone weakening, and joint pain
Alport syndrome also causes sensorineural hearing loss, which is caused by abnormalities of the inner ear’s nerves not working properly.
Alport syndrome is also characterized by specific eye changes.
Alport Syndrome Eye Symptoms
Ocular abnormalities are estimated to occur in about 10 percent of the patients with Alport syndrome
Following eye structures may be affected by the syndrome.
- Conjunctiva-calcium crystals with granulomatous response
- Cornea-juvenile corneal arcus, corneal endothelial pigmentation, bilateral non-simultaneous corneal ring, abscess
- Anterior chamber (AC) angle-Pigment dispersion, angle-closure glaucoma
- Lens-anterior lenticonus, anterior polar cataract, posterior cortical opacities
- The retina-Abnormal coloration of the retina, dot and fleck retinopathy, maybe the presence of numerous discrete white intraretinal dots, resembling fundus albipunctatus, retinitis pigmentosa, ERG abnormal (Reduced b-wave), and EOG normal. As a result of severe kidney failure that may occur, a patient may develop marked hypertension and hypertensive retinopathy.
- Optic nerve head-optic disc edema, optic nerve head drusen
Alport syndrome is suspected based on family history or clinical signs of the conditions.
A kidney biopsy is done to look for signs of condition such as abnormalities of cells in glomeruli. It also allows testing especially for type IV collagen protein, as this protein would be absent in a patient with Alport syndrome.
The test is performed to measure the amount of blood and protein in the urine.
An ophthalmic exam may be used to look for signs, characteristics of a condition such as anterior lenticonus, and among others.
Although there is no one specific treatment for Alport syndrome, the goals of treatment include monitoring and controlling the progression of the disease and treating the symptoms.
The strict control of blood pressure is very important in order to keep the kidneys as healthy as possible. Research suggests that ACE inhibitors, angiotensins, and statins can help reduce proteinuria and the progression of kidney disease.
However, treatment of chronic kidney failure often becomes necessary. This can include dietary modifications and fluid restriction.
Treatments for other aspects of the condition are addressed as needed. For instance, surgical repairs of the eye manifestations may be recommended.
Loss of hearing is likely to be permanent, but affected individuals can be assisted with hearing aids.
Lowe syndrome also called oculocerebrorenal syndrome (OCRS) and oculocerebrorenal syndrome of Lowe (OCRL), is an X-linked recessive metabolic disorder described by Lowe and coworkers in 1952.
It is a multisystem disorder that primarily affects the eyes, nervous system, and kidneys.
The Lowe syndrome is found to affect males more than females.
It is characterized by congenital cataracts, mitotic pupil, infantile glaucoma, neonatal or infantile hypotonia, intellectual impairment, and renal tubular dysfunction (Fanconi syndrome), Behavioral problems and seizures have also been reported.
Renal Fanconi syndrome frequently develops in individuals with Lowes syndrome
In this syndrome, the kidneys are unable to reabsorb important nutrients into the bloodstream. Instead, the nutrients are excreted in the urine.
These kidney problems lead to increased urination, dehydration, and abnormally acidic blood (metabolic acidosis). A loss of salts and nutrients may also impair growth and result in soft, bowed bones (hypophosphatemic rickets), especially in the legs.
Progressive kidney problems in older children and adults with Lowe syndrome can lead to life-threatening renal failure and end-stage renal disease (ESRD).
Lowe syndrome is an uncommon condition. It has an estimated prevalence of 1 in 500,000 people.
Eye Symptoms of Lowe Syndrome
- Cornea-corneal keloids extending through the full corneal thickness
- Anterior chamber angle-anomalous to typical congenital glaucoma
- Ciliary processes are rudimentary and anteriorly displaced
- Iris blood vessels have abnormal fenestration
- Segmental hypoplasia of the pupillary dilator muscle with adhesion to the anterior lens surface.
- Lens-a a small discoid cataractous lens with peculiar capsular and epithelial changes present at birth in nearly 100% of affected males.
- Ring-shaped (sommering’s) lens
- Posterior lenticonus adherent to condensed anterior vitreous
- Strabismus and amblyopia also occur either from poor vision or from poor fusion control secondary to the bilateral aphakes.
Familial Juvenile Nephronophthisis
Familial juvenile nephronophthisis is a chronic autosomal recessive kidney disease responsible for 15% of end-stage renal failure in children. In 1951 Fanconi et al. described a familial juvenile nephronophthisis (FJN) in two families.
The initial symptom occurred between ages 2 and 3 and consisted of polydipsia, polyuria, nycturia, and signs of renal insufficiency became apparent only in the late stages of disorders.
As the condition progresses, anemia and progressive renal failure ensue, with low creatinine clearance rate, azotemia, and inability to concentrate urine
Affected kidneys are small with multiple intraretinal cysts in both cortex and medulla, diffuse chronic interstitial fibrosis with variable areas of tubular atrophy and dilatation.
Other names of Familial Juvenile Nephronophthisis
- Medullary cystic disease
- Cystic disease of the renal medulla
- Progressive hereditary nephropathy
- The familial disease of the renal medulla
- Chronic idiopathic tubulointerstitial nephropathy
- Familial uremic medullary cystic disease
Eye Symptoms of Familial Juvenile Nephronophthisis
Affected children may have poor vision, nystagmus, and fundus changes indicative of generalized abnormality of the retina, a clinical picture resembling Leber’s congenital amaurosis
In some typical retinitis pigmentosa may be present
Leber’s congenital amaurosis may be considered as a type of retinitis pigmentosa under a broader framework of generalized heredoretinal degenerations.
But it is usually classified separately because of its congenital nature and its relative lack of progression and the presence of nystagmus.
Term renal-retinal dysplasia is used to describe this syndrome (i.e., FJN along with a number of skeletal, hepatic, and neuromuscular disorders).
Atypical forms of retinitis pigmentosa in association with FJN include retinitis punctata albescence, retinitis pigmentosa, and central retinitis pigmentosa.
Sectoral retinitis pigmentosa and congenital stationary night blindness may be present. Likewise, mild ERG and EOG abnormalities may be present.
Kidney Failure, Dialysis, and Kidney Transplantation
Renal or kidney failure may be acute or chronic
Acute renal failure
Acute kidney failure is of rapid onset and is often reversible. It is characterized by acute loss of renal function manifested by oliguria or anuria.
This sudden change may be due to vascular disease-malignant hypertension or vasculitis such as periarteritis nodosa, rapidly progressive glomerulonephritis, acute interstitial nephritis, acute tubular necrosis, acute urinary obstruction
These alterations lead to a decrease in glomerular filtration rate is primarily the vascular cause of acute renal failure that produce concurrent ocular manifestation.
Chronic renal failure
Chronic kidney failure occurs when there is a permanent reduction in GFR and it is irreversible
Reduction may be slowly, progressive, leading to chronic renal insufficiency and over a period of years, end-stage renal disease. Hypertension may complicate the picture.
Uremia is an indication of far advanced destruction of renal tissue characterized by signs and symptoms that affect multiple organ systems.
Kidney Failure Eye Symptoms
Hypertension may result from many renal diseases, or it may be the primary cause of renal failure. Malignant or accelerated hypertension can result in both severe HTN retinopathy and acute renal failure and may be superimposed on chronic vascular disease in both kidney and eye.
Retinopathy is always present in advanced diabetic retinopathy and tends to be more severe as kidney failure develops.
Other Ocular Manifestations of Kidney Failure
- Proliferative diabetic retinopathy
- Non-rhegmatogenous retinal detachment
- Visual acuity reduced
- Optic nerve and disc edema
- Acute inflammation
- Band keratopathy
Eye Findings in Hemodialysis and Kidney Transplantation
With increased control of hypertension and advances in photocoagulation for diabetic retinopathy, progression of visual and retinal changes has sharply been curtailed.
Currently, less than 5 percent of patients on dialysis develop blindness. Acute changes can occur during or immediately following dialysis and they are:
- Central retinal vein occlusion
- Cortical blindness
- Anterior ischaemic retinopathy
Long term Eye Changes in hemodialysis and kidney transplantation
- Posterior subcapsular cataract
- Calcification of cornea and conjunctiva
- Pigmentary changes in posterior segments
Following successful kidney transplantation, visual acuity often remains stable but retinopathy can progress and proliferative retinopathies do develop in these patients.
Immunosuppressive therapy following renal transplantation introduces possibilities of following ocular changes.
- Ocular and periocular neoplasms
- Cytomegalovirus retinopathy
- Bacterial and fungal endophthalmitis (staphylococcus, pseudomonas, candida, Nocardia species)
- Herpes simplex keratitis
- Pneumocystis infection
Immunosuppressed renal transplant recipients are also prone to develop particular types of cancers:
- Squamous cell carcinoma of eyelids
- Ocular lymphoma
- Activation of a uveal malignant melanoma
Wilms’ Tumor (Aniridia Syndrome)
Wilms’ tumor is also known as Miller’s syndrome. It is an embryonal malignancy of mixed origin that arises in the kidney.
It accounts for 20% of malignant tumors in children. The incidence of the syndrome is between 1 in 10,000 in the general population and 1 in 50,000 live births.
Approximately 40% are hereditary and transmission is autosomal dominant.
Presenting manifestations in childhood are:
- Abdominal swelling or mass (50 to 60%)
- Pain (20 to 30%)
- Hematuria (5 to 10%)
- Hypertension may occur from increased renin levels caused by renal ischemia
Ocular Manifestation of Wilms’ Tumor
- Bilateral aniridia, affecting not only iris but also the cornea, anterior chamber (AC) angle, lens, retina, and optic nerve
- Iris hypoplasia
The Visual impairment in Wilms’ tumor correlates to
- Absence of macular reflex
- Optic nerve hypoplasia
- Corneal opacification
Renal or kidney disease that affect the eye may cause significant ocular changes including retinal changes and visual disturbances
In some cases, eye changes are seen before the systemic changes and may help in the diagnosis of kidney diseases.
Different renal diseases are characterized by specific abnormalities and degrees of severity. So, eye care professionals (optometrists and ophthalmologists) may help determine the patient’s prognosis because the presence of retinal changes and other eye changes correlates with the early onset of kidney failure.
- The Eye in Systemic Disease, 2nd Edition – December 13, 1990, Authors: Jack J. Kanski, Dafydd J. Thomas, eBook ISBN: 9781483164366
- Kanski’s Clinical Ophthalmology: A Systematic Approach by Jack J. Kanski MD MS FRCS FRCOphth and Brad Bowling FRCSEd(Ophth) FRCOphth FRANZCO
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