Leigh Syndrome : Two cases report

Leigh syndrome is a subacute necrotizing encephalomyelopathy of infancy or early childhood. The clinical presentation can be highly variable. The classical presentations are central hypotonia, developmental regression or arrest, ataxia, ophthalmoplegia, and abnormal respiratory pattern. Diagnosis is usually confirmed by radiologic evidence of focal, bilateral and usually symmetric lesions of the both gray and white matter in the brain and the spinal cord. We experienced 2 cases of Leigh syndrome in a brother and sister.The first case, 4 year-old boy, was misdiagnosed as a cerebral palsy initially, but after acute infection, he revealed developmental regression and abnormal movement. His disease was confirmed by typical magnetic resonance imaging findings. The second case, 1 year-old girl with nystagmus, showed bilateral symmetric high signal intensity in globus pallidus on T2WI. We reported these cases with a brief review of the related literature.

Transscleral Fixation of Posterior Chamber Intraocular Lens in Aphakia

We evaluated visual acuity, refractive error, corneal astigmatism, and complications after transscleral fixation of posterior chamber lenses in 46 aphakic eyes of 45 patients from January 1992 to April 1999. According to the causes of aphakia, there were 20 eyes for senile cataract surgery, 15 eyes for traumatic cataract surgery, 11 eyes for retinal detachment surgery including lensectomy. We used PMMA lenses in 35 eyes but foldable lenses in the remaining eyes. Anterior vitrectomy was done in 14 eyes. In 8 eyes which had some capsular support, one haptic was inserted at ciliary sulcus and the other haptic was sutured. Postoperative uncorrected visual acuity(UCVA)and best-corrected visual acuity(BCVA)were best in senile cataract surgery group according to the causes of aphakia, better in PMMA lens group according to the lens type, and better in the combined anterior vitrectomy group. But, there was no significant difference in the proportion of the cases that BCVA after 1 month of operation was not less than preoperative BCVA, according to the causes of aphakia, lens types, or combined anterior vitrectomy. The postoperative refractive error showed mild myopic shift, and the corneal astigmatic change was within 1. 5 diopter and stable up to 2 month after operation and did not differ significantly according to lens types. The incidence of complications was not high. In conclusion, transscleral fixation of posterior chamber lens in aphakia is effective.

Early Effect of Autologous Limbal Transplantation Immediately following Total Limbal Injury in Rabbits

Limbal epithelium plays a great role in reconstruction of damaged corneal epithelium and early limbal transplantation showed better results in limbal injury.However, there is no consensus about the appropriate time for limbal transplantation.We, therefore, investigated the results of autologous limbal transplantation (ALT)immediately following limbal epithelial injury in rabbits.We classified the rabbits in three groups whether the application of ALT and therapeutic contact lens were done or not.Injury of limbal and corneal epithelium was made by application of n-heptanol and tarsorrhaphy was done in all groups.ALT from the healthy fellow eye was done in Group 1 and Group 2 but not in Group 3, control group.Therapeutic soft contact lens was applied to Group 2 after ALT.We evaluated epithelial defect, haze, and neovascularization of corneas at 3 days, 1 week, and 2 weeks after operation.We also examined tissue specimen of corneas after two weeks of operation.Epithelial defect was almost healed within 2 weeks after operation in Group 1 and Group 2, but Group 3 showed persistent epithelial defect. Corneal neovascularization and haze were the most severe in Group 3, but was not so severe in Group 1 and Group 2, and there was no significant difference between the two groups.On histopathologic examination, Group 1 and Group 2 showed almost normal corneal epithelium though a few inflammatory cells and goblet cells were observed in some cases but control group showed severe inflammaton and many new vessels and goblet cells.In conclusion, ALT immediately following severe limbal injury is effective in reconstructing corneal epithelium.

A Case of Microbial Keratitis as a Complication of Orthokeratology Contact Lens Wear

Orthokeratology is a nonsurgical measure for correcting ametropia, especially, myopia by changing corneal curvature and refractive powers with the use of tight-fitting, progressively flatter, and rigid contact lenses. Contact lens-related microbial keratitis is already well-known, but keratitis which was developed in orthokeratology lens is not reported yet. Only one case of filtering bleb infection as a complication of orthokeratology lens wear is reported abroad in a patient who had been performed a right trabeculectomy in combination with phacoemulsification and insertion of an intraocular lens implant before. We treated successfully the microbial keratitis which developed in a 11-year-old boy with fortified antibiotics who had worn orthokeratology lens for one month.

A Case of Unilateral Pellucid Marginal Corneal Degeneration Diagnosed by Computerized Topography

Pellucid marginal degeneration is a noninflammatory peripheral corneal thining disorder, which progresses slowly, affects inferior cornea bilaterally, occurs usually between twenties and fifties. Protrusion of the cornea with normal corneral thickness occurs above a band of thinning which is located 1 to 2mm from the limbus and measures 1 to 2mm in width and the patient complaints of progressive visual disturbance due to high irregular astigmatism. Topographically, the lowest corneal power is located along a narrow corridor of the central and peracental cornea and corneal power is highest along the inferior peripheral cornea and in the mid-peripheral cornea along the inferior oblique corneal meridian in a band-like fashion and is decreased slowly superior to that meridian. We experienced a case of pellucid marginal degeneration who had a history of progressive visual loss due to high irregular astigmatism. A 67 year-old woman had the characteristic topographic pattern and clinical feature of pellucid marginal degeneration only in her righ eye and we report it as the first case of unilateral pellucid marginal degeneration in Korea.

A Case of Unilateral Pellucid Marginal Corneal Degeneration Diagnosed by Computerized Topography

Pellucid marginal degeneration is a noninflammatory peripheral corneal thining disorder, which progresses slowly, affects inferior cornea bilaterally, occurs usually between twenties and fifties. Protrusion of the cornea with normal corneral thickness occurs above a band of thinning which is located 1 to 2mm from the limbus and measures 1 to 2mm in width and the patient complaints of progressive visual disturbance due to high irregular astigmatism. Topographically, the lowest corneal power is located along a narrow corridor of the central and peracental cornea and corneal power is highest along the inferior peripheral cornea and in the mid-peripheral cornea along the inferior oblique corneal meridian in a band-like fashion and is decreased slowly superior to that meridian. We experienced a case of pellucid marginal degeneration who had a history of progressive visual loss due to high irregular astigmatism. A 67 year-old woman had the characteristic topographic pattern and clinical feature of pellucid marginal degeneration only in her righ eye and we report it as the first case of unilateral pellucid marginal degeneration in Korea.

Clinical Aspects of Infectious Endogenous Endophthalmitis

Infectious endogenous endophthalmitis is a relatively rare, but seriously devasting disease. To evaluate clinical aspects of this disease, we performed a retreospective study on 7 eyes of 7 patients who had been treated with this diagnosis from July 1994 to January 1998 at Asan Medical Center. The preceding systemic diseases consisted of liver abscess, non-small cell lung cancer, spontaneous bacterial peritonitis, acute cholangitis, cystitis, systemic candidiasis. Microorganisms were recovered in blood culture in 4 of them(3 Klebsiella pneumoniae and one Candida albicans). One among these patients also revealed culture positive for Klebsiella pneumoniae in the vitreous as well as in his blood. All patients received intravitreal vancomycin and amikacin injection following vitreous and aqueous tapping. Four patients underwent pars plana vitrectomy as the second procedure and 2 of them expired. At the final follow up, retina remained attached succesjully in six of 7 eyes, yet the visual outcome was so poor that only two recovered over 5/200 or better. Infectious endogenous endophthalmitis has guarded prognosis since causative microorganisms are often extremely virulent, detection of this disease is likely to be delayed and appropriate management can not be started immediately. High index of suspicion for the diagnosis and the intensive treatment are strongly suggested to achieve the best result.