ABSTRACT

Background: Myopia or near sightedness is a very common refractive error seen among children and adults. It is an eye disorder where the light focuses in front of the retina  ,causing distant objects to be blurred while the near objects appear normal. High myopia can be visually debilitating and affect one’s day to day activities. Aim: To analyse the efficacy and visual  outcome of using posterior chamber phakic intraocular lens- refractive implantable lens(RIL) for high myopic patients. Methods: A prospective study  was conducted in a tertiary care centre. The study included 50 eyes of 34 patients. The preoperative best corrected visual acuity was compared with their post operative uncorrected visual acuity along with the IOP changes, endothelial cell loss and associated complications. The whole database was recorded and statistically analysed. Results: Out of the 50 eyes that were taken for the study,94% of the eyes had uncorrected visual acuity equal to better than preoperative best corrected visual acuity and  34 %of the eyes had a visual acuity of 6/6. The intraocular pressure was less than 20mmHg postoperatively in all patients. No incidence of cataract was observed in this study. Conclusion: It is found that the visual outcome was favourable and satisfactory after RIL implantation.

Keywords: Myopia, phakic intraocular lens, refractive implantable lens

INTRODUCTION

Myopia is one of the most common refractive error worldwide. Its prevalence can vary with age , gender and ethnicity. However it has been seen that high myopia can be very debilitating visually and affect one’ s daily activities. There are multiple treatment options to correct high myopia like photorefractive keratectomy, laser assisted subepithelial keratectomy, laser in situ keratomileuis(LASIK) and epi- LASIK. However the recent modality of treatment is with phakic intraocular lens.(PIOL)

PIOL has become very popular recently. It was first introduced by Strampelli and later modified by Fyodorov. In the beginning anterior chamber (AC) PIOLs were used, later iris fixated lens were developed2. But recently posterior chamber PIOLs were introduced and it showed great efficacy, safety and most importantly patient satisfaction.  

The PIOL is placed between the iris and the natural crystalline lens in the ciliary sulcus. Compared to LASIK, PIOL implantation  causes lesser higher order aberrations and the contrast sensitivity and visual performance  appears to be superior than LASIK. They can also be used in the patients who are not fit for LASIK and those with mild keratoconus1.

Various PIOL s are available in the market but for our study we have used Refractive implantable lens (RIL) which is introduced by an Indian company, in order to study its efficacy and visual outcome in high myopic patients.

METHODS AND MATERIALS

Patients were selected from the OPD department of Ophthalmology, Chinmaya Mission Hopsital,Bangalore. Verbal consent was taken from the subjects. Prior to the study ,ethical clearance was obtained from the Institutional Ethics Committee.

Sample Size: The study material consisted of 50 eyes of 34 patients.

Inclusion Criteria:

1.Age >18 years

2.Stable refraction for one year

3.Patients having myopia >-6.00D

4.Iridocorneal angle >30°

5.AC depth>2.7 mm

6.Endothelial cell count >2500 cells/mm 2

7.Central corneal thickness > 0.400 mm

Exclusion Criteria:  Patients withcataract, glaucoma, recurrent uveitis, previous ocular surgeries, anterior segment diseases, macular/ retinal pathology, and  connective tissue diseases were contraindicated for the study.

Type of study: A cross sectional descriptive study for a period of 12 months

PROCEDURE:

A detailed history of the selected patients, their best corrected visual acuity(BCVA) using Snellen ‘s  chart, slit lamp examination, retinoscopy, gonioscopy, endothelial cell count with specular microscopy, central cornel thickness with pachymetry, iris diameter-horizontal and vertical with pentacam, white- white distance with a digital calliper, ,A san and B scan ultrasound and anterior segment optical coherence tomography were done.

Under local anaesthesia, a main incision of size 2.8 -3.2mm and two side port incisions are made. The cartridge is loaded with the lens and inserted through the main incision with the bevel down. The injector is placed superficially and the PIOL is injected into the AC which is filled with visco. The leading hole of the lens should be on the right. The lens is then rotated horizontally  with the blunt tip of the manipulator,taking care not to damage the anterior capsule of lens or the iris. The pupil is then contricted with intracameral pilocarpine and a peripheral iridectomy is done superiorly. The visco is then removed and the AC is hydrated.

During the follow up period the distance between the PIOL and endothelium is measured using the AS –OCT, which ranges between 350 to 600 mm.

Statistical analysis was done using the Statistical Package for Social Sciences (SPSS version 12.0). Chi square test and t- student test was used to compare the variables. Significance was considered if P<0.05

RESULTS

The study was conducted on 50 eyes of 34 patients, the average age being 25.56 years. 52% were male and 48% were female patients.

Figure 1 shows the preoperative best corrected visual acuity(BCVA) of the patients that were taken for our study. Preoperatively 4% eyes had  Best Corrected Visual acuity(BCVA)  of 6/36, 6% eyes had 6/24, 32% eyes had 6/18, 18% eyes had 6/12, 24% eyes had 6/9 and 16% eyes had 6/6

Figure 2 shows the uncorrected visual acuity(UCVA) of the patients after the PIOL implantatation. Post op34% had UCVA of 6/6, 36% had 6/9, 14% had 6/12, 10% had 6/18  and 6% had 6/24. In the 1st week- post op UCVA was less then pre op BCVA in 5% of eyes, whereas 95% of the eyes had equal or better UCVA than preop BCVA. At the end of 3rd month all eyes 100% showed equal or better UCVA then preoperative BCVA.

Figure 3 shows the spherical equivalence preoperatively. Preoperatively spherical equivalence for 16% of eyes was between -6.00D to -10.00 D, 51%  had between -10.25D—15.00D, 33% between -15.25D to – 20.00 D.

Figure 4 shows the residual spherical refraction after the lens implanataion. 3 months postoperatively, 84% had   zero spherical error , 14% had -0.50 D and 2% had between -0.50D to -0.75D

Preoperatively 86% of eyes had an AC depth between 2.5mm to 3.2 mm and 14 % had above 3.2 mm. Post op- 95% of eyes had AC depth between 2.4mm to 2.8 mm and 5 % had above 2.8 mm.AC depth showed a statistically significant reduction , but not below 2.4 mm(P<0.01).

Preoperatively 62% of eyes had IOP between 10-15 mm Hg and 34% between 16 to 20 mmHg . Post operatively in the 1 st week 46 % had  IOP between  10-15 mm Hg , 54% between 16-20mm Hg. However during the  3rd week and 3 rd month  postoperatively  all eyes had IOP <20 mmHg.

Postoperatively 96% of eyes had a reduction in  endothelial cell count  of<100 cells/mm2- which was  insignificant. In the 1st week,3 rd week and 3 rd month post operatively all patients had a vaulting ranging between 300 to 750 µ

Figure 1 shows the preoperative best corrected visual acuity(BCVA) of patients taken for the study.

Figure 2 shows the post operative uncorrected visual acuity(UCVA) of patients after lens implantation.

Figure 3 shows the spherical equivalence of the eyes preoperatively.

Figure 4 shows the residual spherical equivalence after lens implantation

.

DISCUSSION

RIL is an Indian PIOL made of hydrophilic acrylic material. They have 4 peripheral holes that help in the aqueous flow. They have a leading hole on the right and a trailing hole on the left which helps during implantation. The optic diameter ranges between 4.50 mm to 5.80 mm and the overall size ranges between 11.50mm to 13.25mm. It has a refractive index of 1.460.

In our study 50 eyes of 34 patients were taken having a mean age of 25.56 yrs. Perzcamtrodi et al in his study had a mean postoperative spherical equivalent of -1.00D ± 0.50 D, whereas our study showed a mean postoperative SE power of -0.50D ± 1.00D.

 In this study it was seen that the postoperative UCVA of 94% eyes were better than preoperative BCVA and 6% eyes were same as preoperative BCVA. In Uusitalo et al study, 71.5% had equal or better than preoperative BCVA4 and  Ju et al  study showed 96% had equal or better than preoperative BCVA.

 There was no incidence of cataract in our study.But in Lee et al study it was seen that 2% and in Uusitalo et al 2.6%  and in Perzcamtrodi et al  12% of eyes had cataract3.

In this study there was no rise in IOP, whereas in Ju et al study  it showed an increase in 8% of eyes and Le et al observed in 2% of eyes6.

 In our study it showed that there was  3.5% decrease in the endothelial cell count but in Lee et al study there was an 8% decrease in the endothelial cells. Our study showed that the mean preoperative AC depth was 3.06 mm and postoperative depth was 2.57 mm. There was a significant decrease in the AC depth but it was not <2.4 mm. Ju et al also noted a decrease in AC depth from 3.5 mm to 2.6mm.

Pupillary block was one of the complications noted in Uusitalo et al study whereas pigment dispersion was observed in 5 % of eyes in Ju et al study5. Other complications observed were iritis and retinal detachment which was not seen in our study7.

CONCLUSION

It can be concluded that posterior chamber PIOLs (RIL) are a safe , effective and  reliable option for the treatment of high refractive error in myopic patients. The complications like cataract development, AC depth and IOP changes were not significant. It is important that these patients are regularly monitored on  a long term basis.

CONFLICTS OF INTEREST

There are no conflicts of interest.

BIBLIOGRAPHY

1. JuY,GaoXw,RenB. Posterior chamber phakic intraocular lens implanatation for high myopia. Int J ophthalmology. 2013;vol(6): 831-835

2.Pinenda- Fernandez A,JaramilloJ,Vargas J. Phakic posterior chamber intraocular lens for high myopia. J cataract refract surg .2004;vol(20): 2277-2283

3. Kamiya K, Igarashi A ,Shimizu K. Visual performance after posterior chamber phakic intraocular lens. Am J ophthalmol. 2012;vol( 153): 1178-1860

4.Usitalo RJ, Aine E, Sen NH. Implantable contact lens for high myopia. J cataract refract surg. 2002;vol( 28) 29-35.

5.Hassaballa Ma, Mackay T. Phakic intraocular lens outcomes and complications. Artisan vs. Visian ICL 2011;vol( 25): 1365-1370

6.Lee J, Kim Y. Long term clinical results of posterior chamber phakic intraocular lens implantation to correct myopia. Clin Exp Ophthalmol.2016;vol( 44): 481-487

7. Alfonso JF,Baamonde B. Posterior chamber collagen copolymer phakic intraocular lenses to correct myopia. J cataract Refract Surg. 2011;vol( 37): 873-880

ABSTRACT

Nowadays intraoral local anaesthesia has been widely used to perform various dental procedures. The complications associated with it are very minimal though some of the commonly seen ocular complications include meiosis, nystagmus, diplopia, ptosis, amaurosis. In our case study a 30 year old female came to eye OPD with complaints of reduced vision. She had undergone a dental procedure which was done under intra oral local anaesthesia one week prior, following which her vision started deteriorating. Though various mechanisms have been suggested for the cause of diminution of vision post intra oral local anaesthesia, the exact aetiology still remains unknown. In our study intravascular injection of epinephrine which led to a cilioretinal artery occlusion could be the possible cause. The patient was followed up regularly and four weeks later she had recovered complete vision successfully.

Keywords: intraoral local anaesthesia, arterial occlusion.

INTRODUCTION

In order to perform painless dental procedures intraoral local anaesthesia was very widely used. Rarely the administration of a local anaesthesia intra orally can cause ocular complications. It is noted that complications like ptosis, meiosis, amaurosis , diplopia have occurred following therapeutic injections  given around the nose, lips and eye.

 The first case of blindness following intranasal injection of warm paraffin was noted in the early 90’s and reported by Sir Von Bahr. Many cases of decreased vision have been reported following a maxillary nerve block. However in most of the cases the symptoms developed immediately after the administration of the drug and lasted for a few hours. A case of complete blindness following the injection is extremely rare. Though various theories have been proposed to better understand the cause of ocular complications post intraoral local anaesthesia,the  exact aetiology still remains unclear.

CASE REPORT

A 30 year old female came to eye OPD with complaints of reduced vision the left eye since 1 week. On further investigation it was found that she underwent a dental procedure of the left upper maxillary canine exactly one week ago. She was given intraoral local anaesthesia of lignocaine hydrochloride and epinephrine( 1:100,000 )prior to the procedure.  Immediately after the procedure the patient started developing a headache and a gradual loss of vision. She also complained of seeing flashes of light occasionally. There was no history of nausea or photophobia and phonophobia. No significant medical history or surgical history or any previous ocular surgeries or trauma in the past.

On examination the patient’s vision  was 20/25(OS) and 20/20 (OD). The intraocular pressure was measured with applanation tonometer and was 12 mmHg in both eyes. Colour vision was slightly abnormal in the left eye. A relative afferent pupillary defect was noted in the left eye. The extra ocular movements were full in all directions with no evidence of internuclear ophthalmolegia. There were no signs of inflammation, no cells in the anterior chamber or vitreous.

 The visual field test was done and it showed a relative paracentral scotoma in the left eye and  normal field  in the right eye. A dilated fundus examination was done and it showed swelling of the optic disc along with cotton wool spots, hard exudates near the papillomacular bundle. The right eye fundus was normal. Optical coherence tomography was done (OCT) and it showed hypo perfusion in the superficial and deep retinal capillary plexus in the affected areas.

Figure  a: Fundus picture of the left eye showing a swollen optic disc with blurred disc margins along with cotton wool spots near the macular region. 

Figure b: optical coherence angiogram showing capillary dropout along the superficial and deep retinal plexus in the macular region.

A complete blood examination was then done and it normal. The C reactive protein, glucose, liver function test, renal function test, cholesterol, ESR was well within the normal limits. No past history of any cardiovascular diseases.

 A probable diagnosis of cilioretinal artery occlusion was made. The patient was started on 100 Mg of aspirin as a prophylactic measure and was asked to come for a follow up after 4 weeks. The patient showed a significant improvement and the vision improved to 20/20. Fundus examination showed a normal optic disc with very few hard exudates. OCT was also done and was normal except for a thinning of the outer retinal layer near the papillomacular bundle.

DISCUSSION

The cilioretinal artery arises from the short posterior ciliary artery. It is reported to be present in 50% of the eyes and can vary in size, number, distribution and point of origin. Isolated cilioretinal artery occlusion can occur but has a very good prognosis with 90% of the eyes returning to a 6/12 vision and over 60% returning to 6/6. In cases of retinal vascular occlusion, the presence of a cilioretinal artery can play a vital role as they supply the papillomacular bundle and macular region of the retina.

 Epinephrine is very commonly used in dental procedures. Epinephrine causes vasoconstriction and provides a bloodless field for the procedure and also prolongs the duration of the anaesthetic effect. Epinephrine when it reaches the orbit can cause vasoconstriction of the ophthalmic and ciliary arteries. But the cilioretinal arteries are more vulnerable due to their small diameters.

The presence of hard exudates led us to a differential diagnosis of malignant hypertensive retinopathy. The clinical presentation of hypertensive retinopathy is usually bilateral and unilateral cases are very rare. However vessel changes such as arteriovenous nipping, silver wiring typical of hypertensive retinopathy were absent. Clinically the blood pressure was well within the normal limits.

Presence of emboli was another differential. In such cases permanent visual loss can occur after an arterial occlusion due to emboli. In our case the patient started developing the symptoms around 5 to 7 minutes after the injection but she started improving gradually with 20/20 at the end of 4 weeks. A complete cardiovascular evaluation was also done and nothing significant was detected.

There was no history of previous attacks of migraine in the past or a similar family history. During migraine the vasospasm can also lead to a permanent retinal artery occlusion.

A number of theories were put forward to understand the mechanism by which the anaesthetic agent reaches the orbit. Some of them being diffusion, inadvertent needle penetration of the orbit, venous injection and retrograde arterial injection. One of the studies showed that retrograde flow of the epinephrine through the maxillary artery was one of the causes for reduced vision.  

In our study it is considered that the inadvertent administration of epinephrine led to vasospasm and insufficient blood flow to the cilioretinal artery causing a transient loss of vision. The onset of action of epinephrine is between 1 to 3 minutes  and our patient started developing the symptoms within 5 to 7 minutes. 

CONCLUSION.

Vasoconstriction of the cilioretinal artery following the injection of epinephrine is a very dreadful complication. In order to avoid such complication, it is important to aspirate before injection, or inject small quantities of the drug along with moving the needle while injecting to avoid accumulation of large quantities of epinephrine at one particular site. Hence it is important hat clinicians should be aware of this complication and take the necessary precautions.

BIBLIOGRAPHY

  1. Steenen SA, Dubois L .Ophthalmologic complications after intra oral local anaesthesia : a case report and review of literature . Oral Surg. 2012;vol( 113): 1-5
  2. Von Bahr. Multiple embolisms in the fundus of an eye after injection in the scalp. Acta Ophthalmol. 1963;vol( 41): 85-91
  3. Boynes SG, Echeverria Z. Ocular complications  associated with local anaesthesia administration in dentistry. Dent Clin North. 2010;vol(54): 677-686
  4. Von Arx, Lozanoff S. Ophthalmic complications and local anaesthesia. Swiss Dent J. 2014;vol( 124): 1189-1203
  5. Hayreh S S. The cilio retinal arteries. Br J Ophthalmol. 1963;vol( 47): 71-89
  6. Said Yekta- Michael S, Stein MJ. Evaluation of the anaesthetic effect of epinephrine. Head Face Med. 2015;vol(11): 2
  7. Sott Db, Jebson PJ. Factors affecting plasma levels of lignocaine and prilocaine .Br J Anaesth. 1972;vol( 44 ):1040-1049

Abstract

Transient loss of vision after using a smart phone is a temporary phenomenon where in a person has loss of vision in one eye for a short duration after prolonged exposure to the smart phone in the dark. This phenomenon is attributed to the bleaching of the retinal pigments. It is important to be aware of this condition and careful history needs to be taken from the patient before the patient is started on lifelong treatment.

KEYWORDS.  Smart phone, loss of vision

CASE 1:

 A 30 year old male patient had come to our OPD with complaints of transient loss of vision in his right eye. When asked in detail he revealed that he was lying on his left side and was looking at his smart phone at night for more than one hour. When the lights went off he was unable to see anything through his right eye. But however after a period of 20 minutes it stated improving and he was slowly able to see with his right eye.

CASE 2

A 40 year old lady said that she had loss of vision in her right eye at night which lasted for close to 30 minutes. During this period she could  see various shapes floating around. Prior to that, she was using her phone for close to 45 minutes. Since she was clueless and worried, she immediately closed her eyes and went to sleep and was happy to regain her vision the next day.

Both these individuals when consulted a physician diagnosed it as a possible cause of transient ischemic attack and was advised to start on aspirin and statins.

Another lady aged 60 years had a similar episode of transient unilateral loss of vision twice and she failed to report the usage of phone prior to the episode as she considered it as irrelevant. Later she was diagnosed as a case of multiple sclerosis and was started on medications.

All these patients had visual symptoms during the night after looking at their smart phones for a very long duration. Transient smart phone blindness was first discovered in the year 2016. There was temporary loss of vision which occurred at night after prolonged usage of smart phones which was due to the differential bleaching of the photopigment . However the symptoms resolved after a couple of minutes and this was due to the scotopic recovery after the bleach.

A study done showed that viewing the smart phone monocularly  at an arm’s length for several hours did lead to blurring of vision which recovered  after several minutes. Hence it shows that it is very important to assess the history if they have had exposure to smart phones in the dark for a long period. If not assessed ,it can be misdiagnosed as a case of a TIA or MS. This can help the patients from being started on unwanted medications and just a simple word of reassurance would be enough.

 Since smart phones have become an essential commodity in one’s life this unique phenomenon should always be kept in mind. Some of the other complications due to the use of smart phones is traffic accidents caused by the typical smart phone zombies and nomophobia or a sort of anxiety disorder of being out of cellular phone contact. It seems to have a very large population as shown by certain studies.

Other dangerous consequences due to improper use of smart phones include traffic accidents caused by “smartphone zombies” and selfie‑related injuries and deaths. Half of selfie‑related deaths in 2015 happened in India. Some countries have traffic lights on the road at pedestrian crossings and have dedicated pavements for smartphone zombies .

Bibliography

References 1. Alim‑Marvasti A, Bi W, Mahroo OA, Barbur JL, Plant GT. Transient smartphone “Blindness”. N Engl J Med 2016;374:2502‑4. 2. Irshad F, Adhiyaman V. Transient smartphone blindness. Can J Ophthalmol 2017;52:e107‑8. 3. Sathiamoorthi S, Wingerchuk DM. Transient smartphone blindness: Relevance to misdiagnosis in neurologic practice. Neurology 2017;88:809‑10.

MRIDULA V AMARNATH

Chinmaya Mission Hospital,Bangalore,Karnataka

*Corresponding author email: [email protected]

ABSTRACT

A 10 year old female child came to the OPD with complaints of decreased vision. She is a known case of lupus nephritis. Though her vision could be improved after refraction in both eyes, more shocking revelation was the deposition of   a dense yellow brown deposit of Kayser Fleischer(KF) ring and a sunflower cataract. She was further carried out various diagnostic tests for Wilson’s disease and was immediately treated for the same. Unfortunately the child died within two months due to hepatic encephalopathy and sepsis. Hence it is important to understand the significance of early detection of KF ring which is a definite and a long standing indicator of neurological Wilson disease.

Keywords: Kayser Fleischer ring, Wilson disease


INTRODUCTION

Wilson’s disease also known as hepatic degeneration and progressive lenticular degeneration is a medical condition having a wide clinical spectrum. It tends to affect 1 in 30,000 people worldwide. It is a very rare genetic disorder where the liver fails to remove the excess copper, which eventually tends to build up in organs like the brain, liver and eye and cause various abnormalities. Kayser Fleischer ring is one of the earliest manifestations of Wilson s disease. Early and prompt clinical diagnose of the KF ring is extremely crucial and life saving for the patient.

CASE REPORT

A 10 year old female child came to the eye OPD with complaints of decreased vision since 15 days. She was diagnosed as a case of lupus nephritis when she developed symptoms of hematuria, facial puffiness and pedal edema on and off. After blood investigations, urine routine examination and ultrasound of the kidney, ureter and bladder it was confirmed to be a case lupus nephritis with positive ANA.

Her uncorrected vision in her right and left eye were 6/36 and 6/60 respectively and she improved after refraction to 6/9 and 6 / 12 respectively in the right and left eye. On examination of the anterior segment, the cornea showed a very dense yellow brown KF ring in all the quadrants. After dilating the pupil ,a central disc of golden brown deposits with spokes appearance over the anterior lens capsule suggested that of a sunflower cataract which could be the  reason for her decrease in vision in both eyes. Fundus examination was done and it appeared normal in both the eyes.

Based on the above findings the child was assessed further for Wilson’s disease and it showed a low ceruloplasmin level and a high urinary copper level. She was immediately treated with steroids, mycophenolate mofetil , penicillamine and calcium

 Though initially in the first month the child responded well to the treatment ,but gradually during the follow up there were alterations in the blood parameters. The child started developing hypertension, behavioral changes , dystonia, dysphagia. She soon developed focal seizures which suggested significant hepatic encephalopathy  and it eventually led to the death of the child.

DISCUSSION

Wilson’s disease is a rare inherited autosomal recessive disorder. It occurs due to mutation in the ATP7B gene that mainly encodes for copper transporting P type adenosine triphosphate expressed in the liver and kidney2. Copper which gets absorbed from food through albumin in the small intestine reaches the liver cells with the help of membrane transporters. The gene ATP7B helps in the binding of copper to ceruloplasmin and the excess of copper gets eliminated into the bile. Impairment in the biliary excretion leads to an improper accumulation of copper into the extra hepatic sites like the brain, kidney, cornea and heart which lead to further manifestations. Most people are diagnosed between the age group of 5 to 35 years.

Clinical features of Wilson‘s disease don’t appear until the copper builds up in the brain liver and other organs. The features vary depending on the body part it is affected with.  Neurological manifestations like dystonia, tremors, dysarthria are common4. They may also have fatigue, lack of appetite, abdominal pain, accumulation of fluid in the legs and abdomen. Uncontrolled movements and stiffness of the muscles , yellowish discoloration of the skin and the conjunctiva of the eye are also seen . Deposition of yellowish brown pigment around the cornea called the Kayser Fleischer ring along with sunflower cataract is a very common finding. Hemolytic anemia, thrombocytopenia, renal tubular dysfunction, hypercalciuria, hyperphosphaturia, hypokalemia, cardiovascular dysfunction are also seen.

Figure 1:KF ring

http://www.tnoajosr.com/articles/2017/55/4/images/TNOAJOphthalmicSciRes_2017_55_4_307_231127_f1.jpg

Figure 2 : Sunflower Cataract

The diagnostic tests used for the diagnosis of Wilson disease include

  • low serum ceruloplasmin levels(<0.20 g/l, normal is 0.20 -0.40 g/l)
  • 24 hour urinary copper excretion (>100µ g/day or1.0mol/day)
  • 24 hour urinary copper excretion after D penicillamnie(>25mol/day)
  • Hepatic copper level on liver biopsy(>250µg/g dry weight, norml is <50 µg/g dry weight).


It is important for the early recognition of Wilson disease either by clinical ,biochemical or genetic examinations in order to prevent the progression of disease .

Kayser Fleischer ring is a characteristic feature in Wilson disease. It is seen that KF rings are seen in 100 percent of the neurological involvement, 85% in hepatic involvements and about 54% in pre -symptomatic patients. KF ring was first described by a German ophthalmologist by name Bernhard Kayser in the year 1902 and Bruno Fleischer in the year 19031. The ring occurs due to deposition of copper in the descemets membrane of the cornea and limbus. The free copper which is loosely bound to the albumin enters the aqueous humor and then the descemets membrane3.  It starts from the Schwalbe’s line and extends 5 mm on the corneal surface. It initially starts at the superior pole in the form of an arc from the 10 to 2 o clock position followed a similar inferior pole before it finally encircles the cornea.

 A study was done by Inne Et al reported a case of unilateral KF ring in a patient with WD. The patient had a reduced intraocular pressure with decreased aqueous production in the scarred eye. And hence the KF  ring was not manifested. Thus it was postulated that copper deposition is though the aqueous rather than the limbal circulation which was well maintained in the scarred eye. Both passive diffusion and a cellular activity may be responsible for the copper granule deposition.

The KF ring tends to evolve either partially or completely once the patient is started on penicillamine treatment or following a liver transplant. Other disorders where KF ring can be seen are cholestasis, primary biliary cirrhosis5 .Anterior segment optical coherence tomography(AS-OCT) can be used to visualize the KF ring. It appears as an intense hyperreflectivity of the decement membrane in the peripheral cornea. This is a very useful indicator, as if the KF cannot be captured using a slit lamp then a  AS-OCT comes in handy .

Compared to the KF ring, sunflower cataracts are comparatively more rare. They are seen in nearly only 17 % of the patients with Wilson disease. It was first described by Siemerlingg and Oloff in the year 1922. It is mainly seen due to the deposition of copper on the anterior capsule. Posterior capsule tends to be more uniform. Cataract consists of a central disc with folds radiating at the periphery. As reported by Duke Elder the radiating folds are due to the impression of the posterior surface of the iris on the anterior capsule of the lens.

CONCLUSION

This case was reported to understand the importance of KF rings and its significance. A young girl who was a case of lupus nephritis had undergone an ophthalmological examination as she complained of reduced vision during which the KF ring and sunflower cataract was diagnosed. It was a coincidental finding. Prompt referral by the pediatrician and early identification by the ophthalmologist is very crucial and life saving in a case of Wilson s disease. Though KF rings can occur in other conditions but it is most commonly seen in Wilson s disease and the necessary investigations and treatment should be started without any delay. In case of subclinical occurrence of KF ring, an AS -OCT should be done to confirm the above.

BIBLIOGRAPHY

  1. Ramya Silpa,Chidvila V. Wilson disase.Int J Pharma. 2013 ;(2): 18-23
  2. Pfeiffer Rf. Wilson disease.Neurophthal .2007 ;(27): 123-132
  3. Meenkshi Sundaram,Sinha S .Wilson disease- description of 300 patients evaluated over 3 decades. 2007;(86): 112-121
  4. Masud H, Bashir T. Kayser Fleischer ring in Wilson disease. Pak J ophthalmol. 2014;( 30): 112-116
  5. Lin KJ,TSai RT .Ocular manifestations as the initial presentation of Wilson Disease. Tiawan J ophthalmol. 2013 ;(3 )173-175