Long-term clinical outcomes of Ahmed valve implantation in patients with refractory glaucoma.

PURPOSE: To evaluate the long-term efficacy of intraocular pressure (IOP) reduction and complications of Ahmed Glaucoma Valve (AGV) implantation in patients with refractory glaucoma. DESIGN: Retrospective study. SUBJECTS: The study involved 302 refractory glaucoma patients who underwent AGV implantation and had a minimum follow-up of 6 months between March 1995 and December 2013. METHODS: An operation was defined as successful when (1) the postoperative IOP remained between 5 and 21 mmHg and was reduced 30% compared to the baseline IOP with or without medication, (2) there was no loss of light perception or vision-threatening severe complications, and (3) no additional filtering or aqueous drainage surgery was required. Clinical records were reviewed. MAIN OUTCOME MEASURES: IOP, anti-glaucoma medications, and complications. RESULTS: The mean follow-up period was 62.25 months (range, 6 to 190 months). The cumulative probability of success was 89% at 6 months, 81% at 1 year, 66% at 3 years, 44% at 10 years, and 26% at 15 years. IOP was reduced from a mean of 32.2 ± 10.5 mmHg to 18.6 ± 9.1 mmHg at 1 month, 15.2 ± 7.0 mmHg at 6 months, and 14.2 ± 3.5 mmHg at 15 years. Surgical failures were significantly increased when preoperative IOP was high, and when severe complications occurred after AGV implantation (P < 0.05). CONCLUSION: AGV implantation was successful for IOP control in patients with refractive glaucoma in the long term. However, the success rate of surgery decreased over time. Preoperative high IOP and severe complications related to the operation were significant risk factors for failure.

Bleb Analysis and Short-Term Results of Biodegradable Collagen Matrix-Augmented Ahmed Glaucoma Valve Implantation: 6-Month Follow-up.

PURPOSE: To evaluate the short-term efficacy of a biodegradable collagen matrix (BCM) as an adjuvant for Ahmed valve implantation surgery to prevent the hypertensive phase. METHODS: This prospective study included 43 refractory glaucoma eyes, all followed for 6 months. Refractory glaucoma was defined as an IOP higher than 20 mm Hg with antiglaucoma eye drops without previous glaucoma surgery. Conventional method was performed in 21 eyes and BCM-augmented Ahmed valve implantation (BAAVI) in 22 eyes. In the BAAVI group, a 10 × 10 × 2-mm BCM was sutured on an Ahmed glaucoma valve FP7 model. Complete success was defined as an IOP of 21 mm Hg or lower (target IOP 1) or 17 mm Hg or lower (target IOP 2) without antiglaucoma medications and qualified success as an IOP of 21 mm Hg or lower with or without medications. Maximal bleb thickness was measured using anterior segment optical coherence tomography images. RESULTS: The preoperative IOPs and numbers of preoperative antiglaucoma medications were similar for both groups. Complete target IOP 1 success rates were 38.1% and 86.4%, complete target IOP 2 success rates were 19.0% and 59.1%, and qualified success rates were 52.4% and 90.9% in the conventional and BAAVI groups, respectively (P < 0.05). The hypertensive phase rate was lower in the BAAVI group (4.5% vs. 47.6%, P = 0.002). Maximal bleb thickness was increased in the BAAVI group on postoperative days 30 and 180 (P < 0.05). CONCLUSIONS: Success rates were higher in the BAAVI group than in the conventional group with the change of bleb morphology. Furthermore, use of BCM significantly decreased the need for antiglaucoma medications for at least 6 months postoperatively.

Surgical results of trabeculectomy and Ahmed valve implantation following a previous failed trabeculectomy in primary congenital glaucoma patients.

PURPOSE: To compare the surgical results of trabeculectomy and Ahmed glaucoma valve implantation after a previous failed trabeculectomy. METHODS: A retrospective comparative case series review was performed on 31 eye surgeries in 20 patients with primary congenital glaucoma who underwent trabeculectomy or Ahmed glaucoma valve implantation after a previous failed trabeculectomy with mitomycin C. RESULTS: The preoperative mean intraocular pressure was 25.5 mmHg in the trabeculectomy group and 26.9 mmHg in the Ahmed glaucoma valve implantation group (p = 0.73). The 48-month postoperative mean intraocular pressure was 19.6 mmHg in the trabeculectomy group and 20.2 mmHg in the Ahmed glaucoma valve implantation group (p = 0.95). The 12-month trabeculectomy success rate was 69%, compared with 64% for Ahmed glaucoma valve implantation, and the 48-month success rates were 42% and 36% for trabeculectomy and valve implantation, respectively. The success rates following the entire follow-up period were not significantly different between the two groups (p > 0.05 by log rank test). Postoperative complications occurred in 25% of the trabeculectomy-operated eyes and 9% of the Ahmed-implanted eyes (p = 0.38). CONCLUSIONS: There was no significant difference in surgical outcome between the trabeculectomy and Ahmed glaucoma valve implantation groups, neither of which had favorable results. However, the trabeculectomy group demonstrated a higher prevalence of adverse complications such as post-operative endophthalmitis.

Scleral fixation of standard capsular tension ring and in-the-bag intraocular lens implantation in patients with severe lens subluxation.

The authors introduce a new technique for scleral fixation using a standard capsular tension ring for in-the-bag use in patients with a lens dislocated by more than 180°. Three patients with zonulysis and phacodonesis underwent surgery with this technique, and all of the patients showed stable maintenance in visual acuity after an average of 22.7 months (range: 14 to 30 months) of follow-up. None of them showed any serious postoperative complications. This technique appears to be safe and effective in terms of lens stability and visual rehabilitation.

Surgical results of Ahmed valve implantation with intraoperative bevacizumab injection in patients with neovascular glaucoma.

PURPOSE: To investigate the surgical results of Ahmed valve implantation with intraoperative bevacizumab injection in patients with neovascular glaucoma (NVG). METHODS: A retrospective comparative case series review was conducted on 52 eyes with NVG who underwent Ahmed glaucoma valve implantation with or without intraoperative bevacizumab intravitreal injection. In the intraoperative intravitreous bevacizumab injection group (IVB group, 20 eyes), 1.25 mg of bevacizumab was injected into the vitreous cavity during Ahmed valve implantation. In the control group (32 eyes), only Ahmed valve implantation was performed. Surgical failure was defined when (1) the postoperative intraocular pressure was over 21 mm Hg at consecutive clinic visits, (2) the visual acuity became light perception negative, (3) additional antiglaucomatic surgery was required, or (4) devastating operative or postoperative complications were noted. RESULTS: Although the success rate in the IVB group (70.0%) was higher than that in the control group (62.5%) 1 year after operation, the differences were not statistically significant (P=0.828 by log-rank test). Mean intraocular pressures in the IVB group were significantly lower than those of the control group at 12 and 15 months (P<0.05 by the Mann-Whitney U test). Postoperative complications were similar between the 2 groups. Preoperative history of trabeculectomy was a significant risk factor for surgical failure of Ahmed valve implantation in NVG (relative risk=4.618; P=0.018 by Cox regression model). CONCLUSIONS: Intraoperative IVB injection does not seem to be helpful for better surgical outcomes of Ahmed valve implantation in NVG. A history of trabeculectomy is a risk factor for failure after Ahmed valve implantation in patients with NVG.

Intraocular pressure reduction in normal-tension glaucoma patients in South Korea.

To evaluate the potential benefit of intraocular pressure (IOP) reduction in normal-tension glaucoma (NTG) patients in South Korea. A retrospective, multi-center analysis of Korean NTG patients with 5-years follow-up, typical glaucomatous optic disc and/or visual field changes and no recorded IOP >21 mmHg. Progression was identified by Advanced Glaucoma Intervention Study visual field scoring. There were 90 (42%) progressed patients and 127 (58%) stable patients included in the study. Mean IOP measured higher in the progressed (14.3 ± 2.2 mmHg) than stable patients (14.0 ± 1.9 mmHg), but was not statistically different between the groups (P = 0.29). The mean IOP that best discriminated stable patients was ≤15 mmHg, but no statistical difference existed in the numbers of progressed versus stable patients at ≤15 mmHg compared to >15 mmHg (P = 0.07). Multivariate regression analysis showed that the baseline number of glaucoma medicines and visual field as well as mean, peak and fluctuation of IOP were significant risk factors for glaucomatous progression (P < 0.01). This study suggests that in Korean NTG patients, despite relatively similar IOPs between progressed and stable patients, and based on multivariate regression analysis, IOP may be a risk factor for glaucomatous progression.

Split-thickness hinged scleral flap in the management of exposed tubing of a glaucoma drainage device.

A split-thickness hinged scleral flap technique was devised to aid in tube exposure after glaucoma drainage device implantation. This technique resulted in the successful recovery of the tube without reexposure or serious complications in all 3 observed cases. Split-thickness hinged scleral flaps may be an effective surgical technique for the management of repeated tube exposure after glaucoma drainage device implantation.

Modified Siepser sliding knot technique for scleral fixation of subluxated posterior chamber intraocular lens.

UNLABELLED: We describe a new technique of scleral fixation for the management of dislocated intraocular lenses (IOL). A suture is tied inside the eye around the dislocated haptic to prevent slippage of the IOL after surgery. The technique, which is safe and less invasive than previous methods, was used in 3 patients and the results are presented. FINANCIAL DISCLOSURE: No author has a financial or proprietary interest in any material or method mentioned.

A case of decreased visual field after uneventful cataract surgery: nonarteritic anterior ischemic optic neuropathy.

The purpose of this article is to report a case of nonarteritic anterior ischemic optic neuropathy (NAION) after uneventful cataract surgery. A 53-year-old Filipina underwent cataract surgery. She had a small optic disc with cup-to-disc ratio of 0.2 in the left eye and 0.3 in the right eye. On the first postoperative day, the uncorrected visual acuity (UCVA) was 20/20, with an intraocular pressure (IOP) of 20 mmHg in the left eye. At one week after operation, the UCVA was 20/20 and the IOP was 15 mmHg. Three weeks later, she underwent cataract surgery in the right eye. On the first postoperative day, her UCVA was 20/20 in both eyes, but she complained of a visual field decrease in the left eye. A relative afferent pupillary defect (RAPD) was noted and the optic disc was pallid and swollen diffusely. A red-free photo showed defect surrounding the optic disc. A visual field test showed tunnel vision sparing the central vision. In this report, the authors hypothesize an association between cataract extraction and delayed NAION. Since the risk of NAION in the fellow eye is 30-50%, visual acuity, visual field, fundus exam and RAPD should be routinely checked.

Level of vascular endothelial growth factor in aqueous humor and surgical results of ahmed glaucoma valve implantation in patients with neovascular glaucoma.

PURPOSE: To determine the associations between the levels of growth factors in aqueous humor and the surgical outcomes of Ahmed glaucoma valve implantation in patients with neovascular glaucoma (NVG). METHODS: From 19 NVG eyes of 19 patients, a sample of aqueous humor was taken just before Ahmed glaucoma valve implantation. Levels of vascular endothelial growth factor (VEGF), transforming growth factor (TGF)-beta1, and TGF-beta2 in aqueous humor aspirates were measured using a sandwich enzyme-linked immunosorbent assay method. When the postoperative intraocular pressures were >or=21 mm Hg with or without antiglaucoma medications at 2 successive visits or when any other surgical interventions were needed to lower intraocular pressure, that surgery was considered a failure. After patients were classified into success versus failure groups, the levels of the growth factors between the 2 groups was compared. The cumulative probability of success according to Kaplan-Meier analysis was also determined. RESULTS: Mean postoperative follow-up period was 40.9+/-19.6 months and cumulative probability of success was 43% at 57 months after surgery. Mean VEGF level in the failure group was higher than that of the success group (P=0.014). However, there was no statistical difference in the levels of TGF-beta1, TGF-beta2, and protein between 2 study groups (all P>0.05). CONCLUSIONS: The level of VEGF in aqueous humor was significantly higher in the failure group after the Ahmed glaucoma implantation compared with the success group. Our results imply that VEGF may play a role in determining surgical success after Ahmed valve implantation in patients with NVG.

Potential benefit of intraocular pressure reduction in normal-tension glaucoma in South Korea.

PURPOSE: The aim of this study was to evaluate the potential benefit of intraocular pressure (IOP) reduction in normal tension glaucoma (NTG) Asian adult patients in South Korea. METHODS: This was a retrospective, multicenter analysis of 166 NTG Asian adult patients in South Korea. The patient population consisted of Korean patients with NTG with at least 5 years of records available for evaluation. Patients all had typical glaucomatous optic-disc and/or visual-field changes but had never had a recorded IOP >21 mmHg. RESULTS: Overall, 48 (29%) patients were progressed and 116 (71%) were stable over the follow-up period. Of patients with IOPs or=15 mmHg (27/73) 37% progressed (P = 0.041). The mean IOP for the stable group was 14.0 +/- 1.8 mmHg, whereas with the progressed group the average mean IOP was 14.4 +/- 1.6 mmHg (P = 0.20). The mean peak IOP was 17.4 +/- 2.2 mmHg in the stable group and 17.8 +/- 2.0 mmHg in the progressed group (P = 0.26). Multivariate linear regression analysis did not any identify independent risk factors for progression, including age, gender, or mean and peak IOP. CONCLUSIONS: This study provides initial evidence that Korean patients with normal-tension glaucoma, treated to lower IOPs, may have a decreased incidence of progression over 5 years than those patients with higher IOPs. More research is required to confirm this finding.

Anterior chamber measurements by pentacam and AS-OCT in eyes with normal open angles.

PURPOSE: To assess the reproducibility and agreement of anterior chamber measurements between the Pentacam (PTC) and the Anterior segment optical coherence tomography (AOCT) in normal healthy eyes with open angle. METHODS: Prospective cross-sectional comparative case series. A total of 162 eyes of 81 healthy volunteers with normal open angle were included in this study. Anterior chamber angle (ACA) and anterior chamber depth (ACD) were measured with PTC and AOCT. Intra-observer variability and inter-methods agreement of both instruments for ACA and ACD were evaluated. RESULTS: Values of temporal and nasal ACA measured by two instruments were similar, and the results of ACD were also not significantly different between modalities (p > 0.01). ACA and ACD measurements by PTC and AOCT showed good intra-observer and inter-method agreements (all > 0.9). CONCLUSIONS: PTC and AOCT are presumed to be very useful for the anterior chamber angle examination. They may provide good images and quantitative data about the angle structures including ACA and ACD.

Latent asymmetric intraocular pressure as a predictor of visual field defects.

OBJECTIVE: To investigate the association of latent asymmetric intraocular pressure (IOP) (IOP asymmetry between fellow eyes when patients were in the supine position but not when sitting) with visual field (VF) defects in patients with open-angle glaucoma. METHODS: Fifty-three patients with open-angle glaucoma, who were receiving the same topical medication in both eyes, were enrolled and were housed in a sleep laboratory for 24 hours. Intraocular pressures were measured when the patients with open-angle glaucoma were in the supine position or were sitting. A group of patients with latent asymmetric IOP was identified. Intraocular pressure asymmetry, monocular diurnal IOP fluctuation, and VF indexes were compared between the groups with and without latent asymmetric IOP. RESULTS: Among the study population, 16 patients had latent asymmetric IOP. Compared with fellow eyes, their hypertensive eyes demonstrated greater IOP fluctuations in the sitting and supine positions and had more aggressive VF defects. In addition, the eyes in patients having latent symmetric IOP showed significantly greater diurnal IOP fluctuations in the sitting and supine positions and more severe VF defects compared with the eyes in patients having symmetric sitting and supine position IOPs. CONCLUSIONS: Patients with latent asymmetric IOP are at increased risk of VF deterioration. Latent asymmetric IOP may be a predictor of glaucomatous VF defects. Further investigation in a larger, more diverse group of patients is needed to assess the diagnostic implications of latent asymmetric IOP relative to glaucoma therapy.

Scleral fixation of a silicone plate haptic intraocular lens in a patient showing repeated pupillary intraocular lens capture.

A 39-year-old man with Marfan syndrome underwent scleral fixation using a three-piece foldable intraocular lens and experienced repeated pupillary intraocular lens captures secondary to a pliable iris. As a result, the patient needed an additional pupilloplasty. In the opposite eye, a silicone plate haptic intraocular lens was used for scleral fixation because it was thought that the plate design could prevent pupillary intraocular lens capture, even in cases involving a pliable iris. At the 27-month follow-up examination, the patient showed good centration of the intraocular lens in the eye that used the silicone plate haptic intraocular lens without pupillary intraocular lens capture.

Relationship between the retinal thickness analyzer and the GDx VCC scanning laser polarimeter, Stratus OCT optical coherence tomograph, and Heidelberg retina tomograph II confocal scanning laser ophthalmoscopy.

PURPOSE: To assess the relationship between the retinal thickness analyzer (RTA) parameters, and those of the GDx VCC scanning laser polarimeter (GDx VCC), Stratus OCT optical coherence tomography (Stratus OCT), and Heidelberg retinal tomograph II confocal scanning laser ophthalmoscopy (HRT II). METHODS: Twenty-nine primary open-angle glaucoma patients were retrospectively included in this study. Measurements were obtained using the RTA, GDx VCC, Stratus OCT, and HRT II. We calculated the correlation coefficients between the parameters of RTA and those of the other studies. RESULTS: Among the optic disc parameters of RTA, the cup volume was best correlated with Stratus OCT (R=0.780, p<0.001) and HRT II (R=0.896, p<0.001). Among the posterior pole retinal thickness parameters, the posterior pole abnormally thin area (PPAT) of the RTA and the inferior average of the GDx VCC were best correlated (R=-0.596, p=0.001). The PPAT of the RTA and the inferior maximum of the Stratus OCT were best correlated (R=-0.489, p=0.006). The perifoveal minimum thickness (PFMT) of the RTA and the cup shape measurement of the HRT II were best correlated (R=-0.565, p=0.004). CONCLUSIONS: Many RTA optic disc parameters were significantly correlated with those of the Stratus OCT and HRT II. The RTA posterior pole retinal thickness parameters were significantly correlated with those of the GDx VCC, Stratus OCT and HRT II. The RTA optic disc and posterior pole retinal thickness parameters may be valuable in the diagnosis of glaucoma.

The effect of swimming goggles on intraocular pressure and blood flow within the optic nerve head.

PURPOSE: Goggles are frequently worn in the sport of swimming and are designed to form a seal around the periorbital tissue orbit. The resultant pressure on the eye may have the potential to affect intraocular pressure and blood flow of the optic nerve head. This study evaluates the influence of wearing swimming goggles on intraocular pressure (IOP) and blood flow of the ocular nerve head (ONH) in normal subjects. MATERIALS AND METHODS: Thirty healthy participants took part in this study. The IOP of each participant was measured using a Goldmann tonometer. Measurements were taken immediately before putting on swimming goggles, at 5, 10, 30, and 60 minutes after putting on swimming goggles, and then immediately after taking off the goggles. Blood flow of the ONH was measured using the Heidelberg retinal flowmeter. RESULTS: The average IOP before, during and after wearing the swimming goggles were 11.88 +/- 2.82 mmHg, 14.20 +/- 2.81 mmHg and 11.78 +/- 2.89 mmHg, respectively. The IOP increased immediately after putting on the goggles (p < 0.05) and then returned to normal values immediately after removal (p > 0.05). Blood flow of the ONH was 336.60 +/- 89.07 Arbitrary Units (AU) before and 319.18 +/- 96.02 AU after the goggles were worn (p < 0.05). CONCLUSION: A small but significant IOP elevation was observed immediately after the swimming goggles were put on. This elevated IOP was maintained while the goggles were kept on, and then returned to normal levels as soon as they were taken off. Blood flow of the ONH did not change significantly throughout the experiment. These facts should be considered for safety concerns, especially in advanced glaucoma patients.

Prognosis for patients in a Korean population with ocular adnexal lymphoproliferative lesions.

PURPOSE: To analyze the clinical features, treatment outcomes, and prognostic factors associated with lymphoproliferative lesions of the ocular adnexa in a Korean population. METHODS: Data from 69 patients treated for adnexal lymphoproliferative disease in the 12-year period from 1991 to 2002 were retrospectively evaluated. RESULTS: The 69 patients had a median age of 46 years (range, 15-73 years); 60 of these patients were diagnosed with extranodal marginal zone B cell lymphomas (MALT lymphomas) and had 10 year cause specific survival and relapse free survival rates of 95.6% and 82.6%, respectively. Of 6 patients (8.7%) with concurrent systemic lymphoma, including 4 diagnosed with stage IV disease, 3 died from lymphoma. Only one patient with a primary ocular adnexal MALT lymphoma developed systemic lymphoma, which was treated with surgical resection. Local recurrence either at the primary site or in the fellow eye occurred in 11.6% of patients at a median follow-up time of 102 months (range, 79-132 months), and was controlled using repeat irradiation in all cases. Statistical analysis showed the presence of concurrent systemic lymphoma, bilateral disease, and an advanced stage at diagnosis, were linked to lymphoma-related death (Log-rank test, p < 0.05) and systemic progression (Fisher's exact test, p < 0.05), and that the tumor location was not a prognostic factor for lymphoma-related death or relapse at any site. CONCLUSIONS: Ocular adnexal lymphoproliferative disease in Koreans occurred at a relatively young age, and was mostly orbitally located and of the MALT subtype, which is highly localized and rarely associated with extraorbital relapse. Primary or secondary status, stage at presentation, and bilaterality were found to be prognostic factors.

Measurement of contrast sensitivity and glare under mesopic and photopic conditions following wavefront-guided and conventional LASIK surgery.

PURPOSE: To compare contrast and glare vision in a prospective study of eyes treated using conventional and wavefront-guided LASIK surgery. The reproducibility of a glaremeter device used to quantitatively measure glare and halo was also determined. METHODS: Ninety-two eyes of 46 patients underwent conventional LASIK surgery and 104 eyes of 52 patients underwent wavefront-guided LASIK surgery. Visual acuity, glare disability measured using a glaremeter, and contrast sensitivity assessed using a Pelli-Robson chart were measured monthly for 6 months postoperatively. Glaremeter testing was performed under both mesopic (5.4 +/- 0.4 cd/m2) and photopic (78.3 +/- 4.4 cd/m2) conditions. To evaluate the reproducibility of the glaremeter, 36 eyes of 18 nonoperated myopic patients were tested. RESULTS: The coefficient of variation and the reliability coefficient for the glare test were 13.6% and 95.2%, respectively. The glaremeter showed that glare disability under mesopic conditions differed between conventional and wavefront-guided LASIK eyes over 6-month follow-up (907.5 +/- 491.5 vs 986.1 +/- 448.0 pixels preoperatively and 1717.1 +/- 521.2 vs 1407.8 +/- 411.3 pixels at 6 months, P<.0001). At 6 months, contrast sensitivity log values were 1.62 +/- 0.31 and 1.78 +/- 0.34 for conventional and wavefront-guided LASIK eyes, respectively (P=.010). The visual complaint score was lower in the wavefront-guided LASIK group (P=.0116). CONCLUSIONS: Compared to conventional ablation, wavefront-guided ablation provided superior outcomes in terms of postoperative glare under mesopic conditions, subjective complaints, and contrast sensitivity. In addition, it appears the glaremeter can be used for clinical quantitative evaluation of glare and halo.

Anterior transposition of inferior oblique muscle for treatment of unilateral superior oblique muscle palsy with inferior oblique muscle overaction.

Although many weakening procedures for the inferior oblique muscle have been advocated, there is some controversy as to the most beneficial procedure for weakening overacting inferior oblique muscles. This study was undertaken to determine if unilateral anterior transposition of the inferior oblique muscle alone could be a safe and effective procedure for treating unilateral superior oblique palsy from the perspective of hypertropia, inferior oblique overaction, and abnormal head posture. The records of 33 patients, who underwent anterior transposition of the inferior oblique muscle for unilateral superior oblique palsy at our institution between Jan 1995 and Dec 2002, were retrospectively reviewed. The average preoperative inferior oblique overaction was 2.3 +/- 0.64, and the hypertropia in the primary position was 12.3 +/- 7.69 prism diopter (PD). Twenty-six patients showed head tilt to the opposite direction preoperatively. After the anterior transposition of the inferior oblique, inferior oblique overaction was diminished in 32 patients (97%). Twenty-six out of 33 patients (79%) had no hypertropia in the primary position at last postoperative assessment. Of the 26 patients with head tilt before surgery, 21 patients (81%) achieved full correction after surgery. Satisfactory results were obtained in most of the patients in our study with the exception of three patients who required additional surgery. No patient demonstrated postoperative hypotropia in the primary position. None of the patients noticed elevation deficiency or lower lid elevation. The anterior transposition of the inferior oblique was found to be safe and effective for treating superior oblique palsy with secondary overaction of the inferior oblique muscle.

Kearns-Sayre syndrome -3 case reports and review of clinical feature.

Kearns-Sayre syndrome, first described by Kearns and Sayre in 1958, is a rare disorder consisting of ptosis, limited movement of both eyes and atypical retinal pigmentary change (salt-pepper like appearance). Most cases have shown an increase in the concentration of mitochondria and ragged-red fiber under Gomori-trichrome staining on muscle biopsy. Occasionally, it is combined with other neurologic and endocrinologic symptoms such as ataxia, dementia, diabetes, and hyperaldosteronism. We recently experienced three cases of male teenaged patients who expressed the clinical features of Kearns-Sayre syndrome.