Corneal Dystrophy

Corneal distrophies are a group of rare and genetic diseases affecting the cornea. It runs in a families and may be present asymptomatic. They affect both the eyes and other parts of the families are not involved. Main cause of corneal dystrophies is foreign material build up in the cornea and that results in clouding of cornea and visual impairment.

The cornea is made up of five distinct layers:
The Epithelium - the outermost, protective layer of the cornea.
The Bowman's membrane - this second layer is extremely tough and difficult to penetrate further protecting the eye
The Stroma - the thickest layer of the cornea, consisting of water, collagen fibers and other connective tissue components that give the cornea its strength, elasticity and clarity.
Descemet's Layer - a thin, strong inner layer that also acts as a protective layer.
The Endothelium - the innermost layer consisting of specialized cells that pump excess water out of the cornea
Types of corneal dystrophies:
There are 20 different types of corneal distrophies but are groped in 3 main categories:

Anterior or superficial corneal dystrophies. These affect the outermost layers of the cornea: the epithelium and Bowman’s membrane.
Stromal corneal dystrophies affect the stroma, which is the middle and thickest layer of the cornea.
Posterior corneal dystrophies affect the innermost parts of the cornea: the endothelium and the Descemet membrane. The most common posterior corneal dystrophy is Fuchs’ dystrophy.
Symptoms of Corneal distrophies:
Symptoms like watery eyes, dry eyes, corneal erosions, Blurry vision, halos around light, light sensitivity and difficulty in seeing at night often overlaps with diseases like glaucoma and cataract. Two differentiating symptoms are pain and a feeling of something in the eye occurs in the corneal distrophies. As the condition worsens vision becomes poor and irregularities in the cornea may lead to fluctuating vision.

Who is at risk for corneal dystropies?
People having family history of the disease are at more risk than others. Corneal dystrophies can appear at any age. Men and women are equally affected by most corneal dystrophies, except for Fuchs’ dystrophy. Fuchs’ affects women more frequently than men.

What are my treatment choices?
Treatment for corneal dystrophies may vary from eye drops to corneal transplant and depends on:

The type of dystrophy
The severity of symptoms
In few cases, people with corneal dystrophy may have repeated corneal erosion. This condition may be treated with antibiotics, lubricating eye drops, ointments, or special soft contact lenses that protect the cornea. If erosion continues, other treatment options may include the use of laser therapy or a technique for scraping the cornea.

In severe cases, a corneal transplant (called keratoplasty) may be necessary. The damaged or unhealthy corneal tissue is removed and clear donor cornea tissue is put in its place. For endothelial dystrophies, such as Fuchs’ dystrophy, a partial cornea transplant (or endothelial keratoplasty) is used.

Keratoconus is a vision disorder that occurs when the normally round cornea (the front part of the eye) becomes thin and irregular (cone) shaped. This abnormal shape prevents the light entering the eye from being focused correctly on the retina and causes distortion of vision. It is characterized by para-central corneal thinning and ectasia so that the cornea takes the shape of a cone. Visual loss occurs primarily from myopia and irregular astigmatism and secondarily from corneal scarring. Keratoconus often begins at puberty and most often is seen in teenagers or young adults.

Keratoconus causes distorted vision that cannot be corrected with eyeglasses. Tiny fibers of protein in your eye called collagen help hold your cornea in place. When these fibers get weak, they can’t hold their shape. Your cornea gets more and more cone-like.

It happens when you don’t have enough protective antioxidants in your cornea. Its cells produce harmful byproducts, the same way a car puts out exhaust. Normally, antioxidants get rid of them and protect the collagen fibers. But if levels are low, the collagen weakens and the cornea bulges.

Symptoms
Signs and symptoms of keratoconus may change as the disease progresses. They include:

Blurred or distorted vision
Increased sensitivity to bright light and glare, which can cause problems with night driving
A need for frequent changes in eyeglass prescriptions
Sudden worsening or clouding of vision
The swelling occurs when the strain of the cornea's protruding cone-like shape causes a tiny crack to develop. The swelling may last for weeks or months as the crack heals and is gradually replaced by scar tissue.
Monocular polyopia (perception of multiple ‘ghost’ images in the eye).
Streaking and flaring distortion around light sources.
Marked anisometropia (difference in vision of two eyes).
Photophobia (increased sensitivity to light).
Eyestrain, in order to read clearly.
Risk factors
Heredity. One in 10 keratoconus sufferers has a close family relative with the disorder.
Frequent eye rubbing, especially aggressive “knuckling” eye rubbing.
Having a history of asthma, allergies, Ehlos Danlers syndrome, Down’s syndrome
Keratoconus is categorised clinically as:
Latent stage: Latent stage was recognisable by placido disc only.
Early stage: Early stages were subdivided into two categories as:

Keratoconus fruste, which entailed 1- to 4-degree deviation of horizontal axis of the placido disc.
Early or mild keratoconus, which entailed 5- to 8-degree deviation of horizontal axis.
Causes
A family history of keratoconus has been established in some cases. Most researchers believe that multiple, complex factors are required for the development of keratoconus including both genetic and environmental factors.

With the advent of videokeratography to assess family members, however, pedigrees have been analysed. These studies show corneal changes consistent with keratoconus in some family members, which suggest an autosomal dominant pattern of inheritance.

Keratoconus may be associated with wide variety of systemic and ocular conditions.

Systemic associations:
Atopy (a genetic predisposition to develop an allergic reaction): Eye rubbing seen in systemic atopy may play a role in the development of keratoconus.
Down syndrome (Trisomy 21): In Down syndrome (Trisomy 21), frequency of acute hydrops is higher, perhaps because of eye rubbing and/or these patients are treated infrequently with keratoplasty and their disease is allowed to progress further.
Ehlers-Danlos syndrome.
Marfan syndrome.
Ocular associations:
Retinitis pigmentosa.
Retinopathy of prematurity.
Fuchs’ corneal endothelial dystrophy.
Posterior polymorphous dystrophy.
Contributory factors such as:
Enzyme abnormalities in corneal epithelium: Enzyme abnormalities such as increased expression of lysosomal enzymes (catalase and cathepsin) and decreased levels of inhibitors of proteolytic enzymes (tissue inhibitor matrix metalloproteinases), may play a role in corneal stromal degradation.
Differentially expressed corneal epithelium: Differentially expressed corneal epithelium between keratoconus and myopes (as controls) in both genetic expression and protein expression.
Molecular defect: Molecular defect producing unusual absence of water channel protein aquaporin 5 in keratoconus as compared to normal corneal epithelium.
Gelatinolytic activity: Gelatinolytic activity in stroma has been described, which may be due to decreased function of enzyme inhibitors.
Abnormalities in corneal collagen and its cross-linking: Abnormalities in corneal collagen and its cross-linking may be the cause of keratoconus.
Hard contact lens wear.
Pathophysiology:
First is thinning of the corneal stroma then fragmentation of the Bowman layer and the deposition of iron in the basal epithelial cells, forming the Fleischer ring. Folds and breaks in the Descemet’s membrane result in acute hydrops and striae, which produces variable amount of diffuse scarring.

How diagnosis is made?
Certain tests like refraction, keratometry, corneal topography/Computerised videokeratography, ultrasound pachymetry and slit lamp microscopy help in reaching final conclusion.

Computerized videokeratography, which takes pictures of your cornea so a map can be made of the surface while also measuring the thickness of your cornea

Severity of keratoconus depends on shape of cone:
Nipple cones
Oval cones
Globus cones
Treatment
If your keratoconus is progressing, corneal collagen cross-linking might be indicated to slow or stop the progression. Contact lenses can be used to correct astigmatism and mild near-sightedness. Improving your vision depends on the severity of keratoconus. Mild to moderate keratoconus can be treated with eyeglasses or contact lenses.

Lenses
Hard contact lenses. Hard lenses may feel uncomfortable at first, but many people adjust to wearing them and they can provide excellent vision. This type of lens can be made to fit your corneas.
Piggyback lenses. If rigid lenses are uncomfortable, your doctor may recommend "piggybacking" a hard contact lens on top of a soft one.
Eyeglasses or soft contact lenses. Glasses or soft contact lenses can correct blurry or distorted vision in early keratoconus. But people frequently need to change their prescription for eyeglasses or contacts as the shape of their corneas change.
Hybrid lenses. These contact lenses have a rigid center with a softer ring around the outside for increased comfort. People who can't tolerate hard contact lenses may prefer hybrid lenses.
Scleral lenses. These lenses are useful for very irregular shape changes in your cornea in advanced keratoconus. Instead of resting on the cornea like traditional contact lenses do, scleral lenses sit on the white part of the eye (sclera) and vault over the cornea without touching it.
Surgical Interventions
Some form of surgery may become necessary if the cornea progresses in its shape-changing until it is so steep that contacts cannot be tolerated at all.

INTACS are described as arc-like and plastic. These pieces are inserted into the center of the cornea to flatten it, thereby making the eye more contact lens-tolerant.
Collagen crosslinking (CXL) with UVA is a complex surgery that involves removing the topmost layer of your cornea, adding vitamin drops and then exposing the eye to a special UV lamp that helps the cornea fibers multiply, strengthening the cornea.
Corneal transplant surgery is the last resort for most doctors. In this procedure cornea would be removed and replaced with a healthy, normal-shaped cornea. This surgery has a long recovery time, a year or more in some cases, for clear vision.
Penetrating keratoplasty. If you have corneal scarring or extreme thinning, you'll likely need a cornea transplant (keratoplasty). Penetrating keratoplasty is a full-cornea transplant. In this procedure, doctors remove a full-thickness portion of your central cornea and replace it with donor tissue
Deep anterior lamellar keratoplasty (DALK). The DALK procedure preserves the inside lining of the cornea (endothelium). This helps avoid the rejection of this critical inside lining that can occur with a full-thickness transplant.
Important Reminder: This information is only intended to provide guidance, not a definitive medical advice. Please consult eye doctor about your specific condition. Only a trained, experienced board certified eye doctor can determine an accurate diagnosis and proper treatment.

To schedule an appointment with our experts for Keratoconus Treatment in Ghatkopar, please call us at +91 8451045935, +91-8451045934 or visit our clinic at Address.

For more information = https://www.mumbaieyecare.com/

PCRRT Groups Guidelines On AKI In Pediatric Hematopoietic Cell Transplantation Now Published In Journal Of Pediatric Nephrology
Hematopoietic cell transplantation is a therapy to treat neoplastic & metabolic disorders, hematological diseases, & fatal immunological deficiencies, but a common complication is AKI. Read this evidence-based Guideline compiled by the PCRRT working group.

About Dr.

Dr. Sidharth Kumar Sethi
Kidney & Urology Institute
He was trained as a Fellow (International Pediatric Nephrology Association Fellowship) and Senior Resident in Pediatric Nephrology at All India Institute of Medical Sciences and Division of Pediatric Nephrology and Transplant Immunology, Cedars Sinai Medical Centre, Los Angeles, California. He has been actively involved in the care of children with all kinds of complex renal disorders, including nephrotic syndrome, tubular disorders, urinary tract infections, hypertension, chronic kidney disease, and renal transplantation. He has been a part of 8-member writing committee for the guidelines of Steroid Sensitive Nephrotic Syndrome and Expert committee involved in the formulation of guidelines of Pediatric Renal Disorders including Steroid Resistant Nephrotic Syndrome and urinary tract infections. He has more than 30 indexed publications in Pediatric Nephrology and chapters in reputed textbooks including Essential Pediatrics (Editors O.P. Ghai) and “Pediatric Nephrology” (Editors A Bagga, RN Srivastava). He is a part of Editorial Board of “World Journal of Nephrology” and “eAJKD- Web version of American Journal of Kidney Diseases”. He is a reviewer for Pediatric Nephrology related content for various Pediatric and Nephrology journals.

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Cataract Surgery In Ghatkopar
Cataract surgery is replacing the faulty lens with an artificial lens. It’s a common and safe procedure done by ophthalmologist when you are having cloudy vision. Decision to undergo cataract surgery depends on the level of difficulty; a patient is facing on daily routine activities.

What is Cataract?
Cataract is progressive in nature and develops with time you grow older. Cataract affects most of people and becomes prominent with age. It can be described as blurry, cloudy or opaque vision. Natural eye lens focuses light on the retina to form clear and undistorted image. Clouding of this lens results in blurred and distorted image. It commonly affects both eyes but in few cases one eye cataract might advances rapidly. If not treated on time, may result is complete loss of sight.

Causes of Cataract
Cataract can be seen in children as a hereditary defect, while eye trauma, smoking, diabetes, excessive use of alcohol, prolonged exposure of sunlight or ultraviolet rays, extended use of corticosteroids are few causes of cataract occurring in elderly people.

Eye Examination
Cataracts can be diagnosed with simple eye exam. The eye exam contains a vision test and an examination of your eyes using a slit lamp microscope. The pupils are dilated with special eye drops to provide a better view of the back of the eye, where the retina and optic nerve are located.

Symptoms of cataract
Ask yourself few questions

Do you feel difficulty in seeing at night?

Is reading or watching television becoming difficult for you?

Driving at night or seeing in bright light has become difficult?

Do you need assistance for finding ways while walking, cooking, shopping or daily activities of your visi

Are you opting for alternate ways to substitute your vision problems?

Glare or sensitivity to light is another symptom of cataract. Diplopia or double vision is also seen in cataract patients. It also affects color vision, leading to yellowish or brownish tint to your eyesight. Patients often need frequent changes in their eyeglasses.

Cataract Treatment in Ghatkopar
Cataract develops as a result of thickened eye lens and treatment is to replace faulty lens with a new lens. Cataract removal is a routine treatment procedure at opthalomogist clinic and carried out across the globe with rarely any accompanying complications.

To prepare you for surgery, your doctor calls you for eye checkup (a week before), to choose which eye lens is best for you.

On the day of surgery, it’s recommended not to eat for 12 hours before surgery.

Bladeless Cataract Surgery
Cataract surgery at Mumbai Eye Care, Ghatkopar, is performed using the latest robotic technology which is completely bladeless and with ultrafine precision using the FEMTOSECOND LENSX LASER, ALCON, USA. This is an US FDA approved laser cataract surgery performed by our eye doctors and eye surgeon at Ghatkopar, Mumbai. This ensures complete blade free surgery, with high level of precision and excellent visual results. This technology makes the surgery very safe and reduces the chances of any complications to negligible.

How the Femtosecond Laser Works
Femtosecond laser performs most of the steps of cataract surgery that are otherwise performed using hand, thus decreasing the chances of human error and improves the precision.

Femtosecond LASER makes the cuts or incision on the cornea that were traditionally performed using blade. These incision are perfectly self sealing and thus do not need any sutures and heal rapidly.

The capsulorrhexis can be altered in terms of size and one can get a perfectly centered capsulorrhexis thus ensuring perfect IOL or lens positioning and better outcomes in cases of premium IOLs such as toric IOL and Multifocal IOL.

Benefits of Femtosecond/ Robotic/ Bladeless/ Bladefree cataract surgery at Mumbai Eye Care, Ghatkopar.

Most Advanced technology available today
A Complete Blade Free Procedure
Laser precise predictable outcomes
Enhanced Safety
Customized Procedure

Phacoemulsification: It is a simple procedure of less than 1 hour duration. Local anesthesia or sedation makes it painless for the patient. A tiny incision is made in front of eye with laser and inserts a pen-shaped probe to break the cloudy lens into pieces. This process is phacoemulsification. Suction then removes the pieces and surgeon places an artificial intraocular lens (IOL). Lens can be made of plastic, silicone or acrylic. There are varieties of lens available for implantation as per the patient requirements like Monofocal, Toric and Multifocal intraocular lenses.

Itracapsular cataract surgery: Its rare nowadays, this technique needs larger incision and along with cataract surrounding capsule is also removed. Intraocular lens is placed in front of iris in this procedure.

Recovery
Usually it is a complication free procedure, but it is recommended to be in contact with your surgeon after the surgery. Initially, an eye drop is given to reduce inflammation and prevent infection. You will need frequent visits to the doctor to monitor any complications. Cataract removal procedure doesn’t need night stay, as it’s a small daycare surgery. Most people observe an improvement in their visual function within few days of the surgery, enabling them to restart most of their daily activities including work.

Majority of people regain full activity after a few days of getting the procedure. Your doctor might fit you with a pair of glasses (if needed) once your vision is stabilized. This also depends upon the type of intraocular lens implanted in your eye.

Dr. Amod Manocha - Pain Specialist In Delhi and Gurgaon

Dr. Amod Manocha is the Head of Pain Management Services at Max Super Speciality Hospital, Saket. He is trained as a Pain Management Specialist and an Anaesthetist in the UK. He has over 13 years of work experience in the UK including working as a Chronic Pain Consultant in many UK hospitals. Dr. Manocha believes in multidisciplinary approach and providing evidence-based treatments at par with international standards. He is committed to providing quality care and believes in building long-term relationship with patients based on honest communication and keeping their interests foremost.