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Halah Bin Helayel,1 Enmar Mazyad Almazyad,1 Laith Mazyad Almazyad,2 Ruba Qadi,2,3 Alhanoof Almubaiyd,2,4 Sami Al-Shahwan2 1Anterior Segment Division, King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia; 2Fellowship and Residency Training Program, King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia; 3Ophthalmology Department, King Abdulaziz Specialist Hospital, Taif, Saudi Arabia; 4Ophthalmology Department, King Saud Medical City, Riyadh, Saudi ArabiaCorrespondence: Halah Bin Helayel, Anterior Segment Division, King Khaled Eye Specialist Hospital, Uruba Road, Riyadh, 11462, Saudi Arabia, Tel +966114821234, Email hbinhelayel@kkesh.med.saPurpose: To evaluate the adequacy of a Saudi ophthalmology training programs in achieving the competencies outlined by the Saudi Commission for Health Specialties (SCFHS) and the Accreditation Council for Graduate Medical Education (ACGME) and to assess resident satisfaction with their training.Methods: A cross-sectional survey was sent electronically to all trainees (n=50) enrolled in the King Khaled Eye Specialist Hospital (KKESH) ophthalmology training program by email and messaging app (Whatsapp). The survey was sent between January 20, 2021, and January 30, 2021. It evaluated the level of satisfaction with surgical training and skills. The surgical logbooks of all trainees were extracted, and identifiers were removed. Then they were compared with SCFHS and ACGME curriculums for ophthalmology training.Results: Out of 50 invitees, 47 returned the questionnaire. All trainees were confident they could meet surgical requirements by the end of their training. Most trainees were satisfied (n=29, 61.7%) with their performance in core competency requirements. There was no difference between female and male trainees’ comfort levels when performing surgical steps in core competency requirements (P=0.2). Senior trainees seem more satisfied with their performance, especially in core competency requirements (P=0.087). All trainees practice at the wet lab and a virtual reality simulator to improve their skills. Obstacles faced during training were highlighted, including low exposure in the operating theater (n=18, 37.50%), competition on cases (n=5, 10.42%), attending surgeons not willing to teach (n=5, 10.42%), COVID-19 pandemic (n=5, 10.42%), and complex cases (n=4, 8.33%).Conclusion: The outcomes of the current study indicate that trainees at KKESH were adequately satisfied with their surgical training in general. Also, competencies outlined by both the SCFHS and the ACGME were adequately fulfilled.Keywords: ophthalmology training, curriculum, surgical training, medical education

Adel Salah Alahmadi,1,2 Hatlan M Alhatlan,3 Halah Bin Helayel,4 Rajiv Khandekar,4 Ahmed Al Habash,5 Sami Al-Shahwan6,7 1Vitreoretinal Division, King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia; 2Department of Ophthalmology, MOH, Madinah, Saudi Arabia; 3Department of Ophthalmology, King Fahad Hospital, MOH, Hofuf, Saudi Arabia; 4Research Department, King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia; 5Department of Ophthalmology, College of Medicine, Imam Abdulrahman Bin Faisal University, Dammam, Saudi Arabia; 6Glaucoma Division, King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia; 7Residency and Fellowship Office, King Khaled Eye Specialist Hospital, Riyadh, Saudi ArabiaCorrespondence: Ahmed Al HabashDepartment of Ophthalmology, Imam Abdulrahman Bin Faisal University, King Fahd Hospital of the University, King Faisal Road, Dammam 31952, Saudi ArabiaTel +966 138966666 Ext 1323Fax +966 138966770Email ahmedalhabash@gmail.comPurpose: To evaluate the impact of the current pandemic on ophthalmology residency training in Saudi Arabia, focusing on its effects on clinical education, training, and the mental well-being of the trainees.Methods: An online self-administered questionnaire was distributed among residents in the Saudi ophthalmology training programs between July 7 and 14, 2020. In this study, we explored residents’ opinions regarding training disruption and virtual education. The patient health questionnaire (PHQ-9) was used to assess the COVID-19 pandemic’s impact on their mental health. We used descriptive statistics for data analysis.Results: Out of 183 registered ophthalmology residents, 142 participated in this study. Ninety-six participants (35.4%) were rotated at a specialized eye hospital during the COVID-19 pandemic, while 52 (19.2%) had rotations in the ophthalmology department at general hospitals. Those who rotated in both types of hospitals were 123 (45.4%). According to the participants, there was a significant decline in exposure to surgical and office-based procedures compared to emergency eye consultations (Friedman P < 0.001). The COVID-19 pandemic’s effect on mental health was reported by 100 (70.5%) participants. Eighty-five (55.4%) respondents were satisfied with the virtual method of education.Conclusion: COVID-19 pandemic has disrupted residents’ clinical and surgical training in the Saudi ophthalmology training programs. Additionally, we believe that COVID-19 may have a negative impact on trainees’ mental health. Fortunately, the current pandemic provided an innovative education method that will likely be used even after the pandemic.Keywords: ophthalmology training, curriculum, outbreak, mental health, Saudi Arabia, Residency training program, COVID-19, medical education

Ramzi Al Judaibi,1 Khaled K Abu-Amero,2,3 Jose Morales,1 Sami Al Shahwan,1 Deepak P Edward1,4 1King Khaled Eye Specialist Hospital, 2Department of Ophthalmology, College of Medicine, King Saud University, Riyadh, Kingdom of Saudi Arabia; 3Department of Ophthalmology, College of Medicine, University of Florida, Jacksonville, FL, USA; 4Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore MD, USA Purpose: Here, we present two patients with congenital anterior staphyloma, with mutations in the CYP1B1 gene. Methods: We reviewed the medical records, including the genetic analysis. Results: Two unrelated patients presented with congenital anterior staphylomas. Both patients showed mutations in the CYP1B1 gene. The first patient, the product of a consanguineous marriage, showed a homozygous misssense mutation g.3987G>A (p.G61E). The second patient had compound heterozygous misssense mutations [g.4160 G>T (p.A119S) and g.8131 C>G (p.L432V)]. Conclusion: CYP1B1 gene mutation may be associated with congenital anterior staphylomas. Keywords: mutation analysis, congenital glaucoma, consanguinity, congenital aphakia

Purpose: To present the outcomes of Ahmed glaucoma valve (AGV) implantation in uveitic glaucoma (UG) in a tertiary eye center in Riyadh, Saudi Arabia., Methods: A retrospective review of all UG patients who underwent AGV implantation at a single, tertiary care eye center from January 2008 to December 2018. The main outcome measures were change in mean intraocular pressure (IOP), number of antiglaucoma medications, overall success rate in different follow-up periods, complete and qualified success from last follow up, and complications. The success of the treatment was defined as patients achieving intraocular pressure (IOP) levels between 6- and 21-mm Hg with or without additional antiglaucoma medications and/or a minimum of 20% reduction from baseline IOP., Results: The study included 74 patients (86 eyes), with a mean follow-up of 4.9 ± 2.9 years. The mean IOP was 32.2 ± 8.5 mmHg at baseline and 16.3 ± 6.6 mmHg at the last follow-up ( p  < 0.001). The mean number of antiglaucoma medications decreased from 3.5 ± 0.7 preoperatively to 1.3 ± 1.4 at the last follow-up ( p  < 0.001). Overall survival rates at 1, 3, 5, and 10 years were 96.5%, 92.7%, 91%, and 56.9%, respectively. Complete and qualified success rates were 37.9% and 48.3% at the end of follow up, respectively. The rate of postoperative complications was 56%, of which 18 eyes (20%) required surgical revision. Bivariate Cox proportional hazards regression analysis did not reveal any factors significantly associated with failure., Conclusions: AGV is safe and effective in providing intermediate-term IOP control in UG patients. Complications reported in our cohort were mostly transient and conservatively treated.

Purpose: Glaucoma, a disease characterized by optic nerve damage and associated visual field defects, is the main cause of irreversible loss of vision, both globally and locally. Appropriate management of glaucoma involves early diagnosis in addition to early and continuous control of the disease to protect the optic nerve from further damage. This study aimed to assess the knowledge, attitude, and practice (KAP) towards glaucoma among patients making their first visit to the glaucoma clinics, at King Khaled Eye Specialist Hospital in Riyadh, and their first-degree relatives., Methods: This cross-sectional study used self-administered questionnaires. Informed consent was obtained prior to questionnaire distribution. The questionnaire included four sections on sociodemographic data, and awareness, and KAP towards glaucoma., Results: Overall, 120 participants were included in our study: 70 (58.3%) were glaucoma patients and 50 (41.7%) were first-degree relatives. Forty-four (62.9%) participants in the patient group and 39 (78%) in the relative group were men. Thirty-four (48.6%) patients were aged 41-65 years, whereas 41 (82%) relatives were aged 18-40 years. Thirty-six patients (52.2%) and 35 relatives (70%) had heard of the term 'glaucoma'. The correct definition of glaucoma was selected by only 12 participants (17.1%) in the patient group and 4 (8.2%) in the relative group. Living outside the central region of Saudi Arabia and having a negative systemic medical history were associated with poorer knowledge of glaucoma definition (p < 0.05). Although 84.8% of patients believed that compliance with anti-glaucoma medications helps in disease control, only 22.7% reported good compliance. The correct method of instilling eye drops was stated by 43.9% of the patients, and good hospital visit habits were reported by 53.03%. Eighty percent of glaucoma patients strongly recommended that their first-degree relatives be screened for glaucoma., Conclusion: Knowledge about glaucoma is still limited, and awareness programs need to be further activated to spread correct knowledge about the nature of glaucoma, the importance of compliance with treatment, the correct method of instilling eye drops, and the significance of screening at-risk individuals. Patient families should also be involved in educational programs., Competing Interests: Declaration of conflicting interestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Purpose: To compare the outcomes of the Ahmed glaucoma valve (AGV) (New World Medical, Inc) with the Baerveldt glaucoma implant (BGI) (Johnson & Johnson Vision) in primary congenital glaucoma (PCG)., Methods: This was a retrospective review of children with PCG who underwent AGV or BGI implantation with a minimum follow-up of 6 months. Main outcome measures were intraocular pressure (IOP), the number of glaucoma medications, success rate, complications, and surgical revisions., Results: The study included 153 eyes of 86 patients (120 eyes in the AGV group and 33 eyes in the BGI group), and the mean follow-up period was 58.7 ± 6.9 months in the AGV group and 58.5 ± 5.0 months in the BGI group. At baseline, IOP was lower in the AGV group (33 ± 6.3 vs 36.4 ± 6.1 months; P = .004) and the number of glaucoma medications was comparable between groups (3.4 ± 0.9 vs 3.6 ± 0.5 medication; P = .183). At 5 years, the mean IOP (18.4 ± 5.0 vs 16.3 ± 2.5 mm Hg; P = .004) and number of glaucoma medications (2.1 ± 1.3 vs 1.0 ± 1.0 medication; P = .001) were significantly less in the BGI group. Furthermore, the surgical success was 53.4% in the AGV group and 78.8% in the BGI group ( P = .013)., Conclusions: Both the AGV and BGI were successful in providing adequate IOP control in patients with PCG. Long-term follow-up showed that the BGI was associated with a lower IOP, fewer glaucoma medications, and a higher success rate. [ J Pediatr Ophthalmol Strabismus . 2023;60(6):448-454.] .

Purpose: To identify the risk factors for glaucoma drainage device (GDD) exposure in children., Design: Retrospective case-control study., Methods: The study population comprised children (one eye each) who presented with a history of GDD exposure to a tertiary care eye hospital over the period January 2014 to January 2020. Consecutive children (age <18 years) were included. A control group from the same time period (children without GDD exposure) were included in the ratio of 5 controls to every 1 case of exposure. The main outcome measures included risk factors for exposure (univariate and multivariate analysis)., Results: A total of 21 eyes (of 21 children with implant exposure) and 115 eyes (of 115 children without exposure) were included in this study. During the same study period, a total of 494 eyes had undergone GDD implantation, giving an estimated incidence of exposure of 4.25%). In the bivariate analysis, GDD exposure was associated with multiple previous ocular surgeries (P = .001), longer follow-up duration (P < .001), combined procedure at the time of primary implantation (P = .002), and a younger age at the time of primary implantation (P = .006). The former 3 risk factors continued to prove a statistically significant association on multivariable regression analysis. Postoperative use of eye lubricants was more common among children in the control group (P = .007)., Conclusion: In pediatric glaucoma patients, younger age and combined procedure at the time of primary GDD implantation, in addition to multiple previous ocular surgeries, were associated with greater risk of implant exposure. Postoperative use of eye lubricants may be protective., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Purpose: Studies have shown that there are significant differences in the concepts of medical professionalism among residents. Very few studies have specifically evaluated the perception of medical professionalism in a specific specialty. The aim of the study is to evaluate the perception of medical professionalism among ophthalmology residents and to assess the differences in the concept of professionalism between junior and senior residents., Methods: This cross-sectional study used a questionnaire to survey ophthalmology residents enrolled in different regions of Saudi Arabia in 2020. The ophthalmology residents included both junior and senior residents. In this study, "professionalism" concurred with the definition in the American Board of Internal Medicine's "Project Professionalism" and the Accreditation Council for Graduate Medical Education., Results: A total of 167 participants were recruited in the study. Out of the 127 participants who completed the survey, 60 (47.24%) were junior residents and 67 (52.76%) were senior residents. The scores for the attributes varied markedly. The lowest score obtained was 6.54 ± 3.349 for the attribute "In an emergency putting the welfare of others over my own safety" in the "Altrusim" domain and the highest score obtained was 9.62 ± 0.854 for the attribute "respect for co-workers" in the "Respect for others" domain. Out of the total residents who completed the survey ( n = 127), 84 (66.14%) agreed that professionalism is teachable through a residency curriculum and 85 (66.93%) thought that it is testable., Conclusion: The characteristics of different attributes of professionalism varied significantly among the residents. The findings of the present study highlights that the conceptualization and perception of professionalism is changing and there is a need to focus on the resident curriculum to instill the values of professionalism. Targeted training is needed on the concepts of professionalism to develop behaviors consistent with medical professionalism and to evaluate the levels of professionalism among residents., Competing Interests: There are no conflicts of interest., (Copyright: © 2022 Middle East African Journal of Ophthalmology.)

Purpose: To examine barriers and adherence to topical glaucoma medication in a pediatric glaucoma population in Saudi Arabia., Methods: This cross-sectional study was undertaken at the King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia during 2016-2017. Parents (father or mother) of children aged 6 months to 15 years with a history of glaucoma were interviewed. Rasch analysis was performed to assess the psychometric properties of the developed 12-item barriers-to-adherence tool (BAT-12) and to identify barriers deemed highly important. The 12-items were adapted from previous studies examining different barriers to adherence in different populations. Medication adherence was assessed by asking parents a series of closed-ended questions about the frequency of missed medications. Determinants of missed medications on at least 1 out of the last 3 days were examined using Poisson regression., Results: One hundred and six parents were interviewed. The 12-item scale had acceptable psychometric properties. Barriers deemed important were forgetfulness (logit -0.59), complex dosing regimen (logit 0.09), and being too busy with other work/activities (logit 0.14). When asked how often, on average, their child missed the prescribed medication, 26 (24.5%) reported daily, 17(16.0%) reported few times per week, and 32 (30.2%) reported once per week. A third of parents (n = 37, 34.9%) reported having missed giving all drops on at least one day in the last 3 days. Poisson regression with robust variance revealed that increase in age of the child (prevalence ratio, 1.08 [95% CI, 1.03-1.14]; p = 0.003) and increase in parents' barriers score (prevalence ratio, 2.13 [95% CI, 1.49, 3.03]; p < 0.001) were significantly associated with having missed medications on at least one out of the last three days while adjusting for the effect of father's current employment status and area of residence., Conclusions: The tool we used to examine barriers to glaucoma medication had acceptable psychometric properties and could be used and strengthened in future studies. Our study highlights poor adherence to glaucoma medications among children with glaucoma.

Objectives: To determine the incidence of ocular hypertension (OHT) and glaucoma in patients with acanthamoeba keratitis (AK) and to outline the risk factors for the development of glaucoma., Methods: A retrospective review of patients diagnosed with AK at our institute during the period from 2000 to 2018. The main outcome measures were the incidence of OHT and glaucoma, and risk factors for the development of glaucoma., Results: Fifty-two eyes diagnosed with AK were included. The incidence of OHT and glaucoma was 51.9% and 32.7%, respectively. The mean duration from disease onset to the first attack of elevated intraocular pressure was 8.4±16.6 months. The use of corticosteroids in the treatment regimen was significantly associated with the development of glaucoma (odds ratio, 3.93; 95% confidence interval, 0.96-16.15; P=0.049). At the last follow-up visit, both patients with glaucoma and nonglaucoma patients had improved visual acuity without a difference in the mean amount of logarithm of the minimum angle of resolution acuity improvement among them (0.56±0.91 vs. 0.67±0.87, P=0.686)., Conclusion: Our findings confirm that OHT and glaucoma are frequent complications in AK. Patients treated with a regimen containing corticosteroids are at a higher risk; thus, they should receive close intraocular pressure monitoring., Competing Interests: The authors have no funding or conflicts of interest to disclose., (Copyright © 2021 Contact Lens Association of Ophthalmologists.)

Background: Crouzon syndrome is the most common form of craniosynostosis, and mutations in the fibroblast growth factor receptor 2 and 3 (FGFR2 and FGFR3) genes are implicated in its pathogenesis., Observation: A 10-year-old female patient with Crouzon syndrome and congenital glaucoma treated with trabeculectomy and ocular hypotensive medications was examined. The patient had proptosis, exposure keratopathy, megalocornea, thin central corneal thickness, a shallow anterior chamber, mild anterior subcapsular cataract, and a cup-to-disc ratio of 0.9. Ultrasound biomicroscopy revealed a shallow anterior chamber, posterior synechiae, and closed angle in the right eye, as well as a narrow angle in the left eye, despite an axial length of 28.9 mm in the right eye and 30.0 mm in the left eye, measured by A-scan ultrasound biometry. The crystalline lens thickness measured by ultrasound biomicroscopy was 4.18 mm in the right eye and 4.12 mm in the left eye., Conclusions and Importance: Despite long axial lengths, shallow anterior chambers with occluded angles are possible in Crouzon syndrome and are most likely caused by FGFR2-related anterior segment dysgenesis. To the best of our knowledge, this is the first report that describes closed angles and anterior segment dysgenesis as a secondary cause of congenital glaucoma in Crouzon syndrome.

Impervious encapsulation around Ahmed glaucoma valve (AGV) results in surgical failure raising intraocular pressure (IOP). Dysregulation of extracellular matrix (ECM) molecules and cellular factors might contribute to increased hydraulic resistance to aqueous drainage. Therefore, we examined these molecules in failed AGV capsular tissue. Immunostaining for ECM molecules (collagen I, collagen III, decorin, lumican, chondroitin sulfate, aggrecan and keratan sulfate) and cellular factors (αSMA and TGFβ) was performed on excised capsules from failed AGVs and control tenon's tissue. Staining intensity of ECM molecules was assessed using Image J. Cellular factors were assessed based on positive cell counts. Histopathologically two distinct layers were visible in capsules. The inner layer (proximal to the AGV) showed significant decrease in most ECM molecules compared to outer layer. Furthermore, collagen III (p = 0.004), decorin (p = 0.02), lumican (p = 0.01) and chondroitin sulfate (p = 0.02) was significantly less in inner layer compared to tenon's tissue. Outer layer labelling however was similar to control tenon's for most ECM molecules. Significantly increased cellular expression of αSMA (p = 0.02) and TGFβ (p = 0.008) was detected within capsular tissue compared to controls. Our results suggest profibrotic activity indicated by increased αSMA and TGFβ expression and decreased expression of proteoglycan (decorin and lumican) and glycosaminoglycans (chondroitin sulfate). Additionally, we observed decreased collagen III which might reflect increased myofibroblast contractility when coupled with increased TGFβ and αSMA expression. Together these events lead to tissue dysfunction potentially resulting in hydraulic resistance that may affect aqueous flow through the capsular wall.

Purpose: Encapsulation of the Ahmed glaucoma valve (AGV) plate is a common cause for postoperative elevation of intraocular pressure, especially in children. Many reports have described the outcomes of AGV revision in adults. However, the outcomes of AGV revision in children are poorly documented. The aim of this study was to determine the outcomes of AGV revision in children., Design: Retrospective cross-sectional study., Methods: A retrospective chart review of patients less than 15 years of age who underwent AGV revision with a minimum postoperative follow-up of 6 months was conducted. Outcome measures included reduction in intraocular pressure from baseline, survival analysis, and reduction in the number of antiglaucoma medications. Postoperative complications were also noted. Complete success was defined as an IOP of 21 mm Hg or less without medications, while qualified success was defined as having an IOP of 21 mm Hg or less with medications., Results: A total of 44 eyes met the inclusion criteria. Primary congenital glaucoma was present in 39 eyes (88.6%), aphakic glaucoma in 4 eyes (9.1%), and Peters anomaly-associated glaucoma in 1 eye (2.3%). The mean number of previous surgeries was 1.4, and the mean age was 6.7 years (range, 1.9-13 years) with a median follow-up of 12 months (range, 6-24 months). The IOP was reduced from a preoperative mean of 30.4 (± 10.3) to 24.9 (± 10.6) mm Hg at 6 months postoperatively. Kaplan-Meier analysis showed that the complete success rate at 1 month was 100% followed by a rapid decline at 6 months to 38.6%, 27.7% at 1 year, and 5.5% at 2 years. Qualified success rate was 100% at 1 month followed by a 6-month and 1-year survival rate of approximately 50% and a 2-year survival rate of approximately 16%. The median survival time was 14 months. No specific risk factors for failure were identified. Visual acuity remained unchanged following revision. The most common complication was recurrence of encapsulation with elevated IOP (15.9%). Other complications included hyphema (n = 3; 6.8%), endophthalmitis (n = 1; 2.3%), wound leak (n = 1; 2.3%), and choroidal detachment (n = 2; 4.5%)., Conclusion: Although the short-term success rate of AGV revision in children is high, with longer follow-up the success rate decreases significantly., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Purpose: To evaluate the systemic pharmacokinetics (PKs) of travoprost 0.004% preserved with Polyquad ® (TRAVATAN ® ) in pediatric patients with glaucoma or ocular hypertension., Methods: This was a phase 1, open-label, multicenter clinical study of patients aged ≥2 months to <18 years. Patients received daily administration of travoprost 0.004% preserved with Polyquad in both eyes for 7 days. Plasma samples were collected 30 min before the final dose and at 10, 20, 40, and 80 min postdose. The main outcome measure was maximum concentration of travoprost free acid in plasma (C max )., Results: Included in the PK analysis were 24 patients (average age 9.6 ± 4.9 years). At least 1 sample with quantifiable levels of travoprost free acid was collected for 11 patients. The mean C max was 0.0471 ± 0.0105 ng/mL for patients aged 2 months to <3 years; 0.0258 ± 0.0128 ng/mL for ages 3 to <12 years; and 0.0109 ± 0.0005 ng/mL for ages 12 to <18 years. Travoprost was undetectable in samples collected predose from pediatric patients. Treatment-related adverse events (AEs) included hyperemia, eye pain, and eye pruritus (n = 1 each). There were no discontinuations or drug-related serious AEs., Conclusions: Travoprost free acid concentration in plasma was low in pediatric patients, detectable in only 11 of 24 patients. There was no accumulation of travoprost over the course of treatment. No clear relationship was observed between age/body surface area and C max . No increased risk was identified for the use of travoprost 0.004% preserved with Polyquad in patients <18 years of age.

Purpose: The purpose of this study was to compare outcomes of transscleral diode cyclophotocoagulation using short duration (SD) versus longer duration (LD) treatment., Patients and Methods: Data were collected retrospectively on patients with glaucoma who underwent cyclophotocoagulation with LD (4000 ms) treatment with variable power ≤1000 mW versus SD using 1500 ms with variable power ≤2000 mW. The outcome measures included: intraocular pressure (IOP) at 12 months, decrease in number of antiglaucoma medications, complications and visual acuity (VA) at the final follow-up visit., Results: There were 71 patients and 70 patients in the SD and LD groups, respectively. The mean IOP±SD pretreatment baseline was 33±10 versus 36±10 mm Hg for SD and LD groups, respectively (P>0.05). At 1 year postoperatively, the IOP decreased significantly in both groups (16.7 vs. 17.4 mm Hg for SD and LD, respectively; P<0.0001). This decrease in IOP was comparable between groups (P=0.5). The average number of IOP lowering medications were 2.5 and 2.9 at baseline in the SD and LD groups, respectively, and decreased to 1.3 in both groups (P=0.83). The mean LogMAR VA decreased significantly from 1.87 at baseline to 1.96 at 1 year in the SD group (P=0.276), and 2.03 to 2.3 (P≤0.001) in the LD group, this change was comparable (0.19; P=0.075) between the 2 groups. Complications were also comparable., Conclusions: Both LD and SD burns during cyclophotocoagulation effectively lowered IOP at 1 year. Neither treatment resulted in a greater reduction of IOP. LD treatment appeared to result in a decrease in VA and greater postoperative inflammation.

Primary angle closure glaucoma (PACG) is a major cause of blindness worldwide. We conducted a genome-wide association study (GWAS) followed by replication in a combined total of 10,503 PACG cases and 29,567 controls drawn from 24 countries across Asia, Australia, Europe, North America, and South America. We observed significant evidence of disease association at five new genetic loci upon meta-analysis of all patient collections. These loci are at EPDR1 rs3816415 (odds ratio (OR) = 1.24, P = 5.94 × 10(-15)), CHAT rs1258267 (OR = 1.22, P = 2.85 × 10(-16)), GLIS3 rs736893 (OR = 1.18, P = 1.43 × 10(-14)), FERMT2 rs7494379 (OR = 1.14, P = 3.43 × 10(-11)), and DPM2-FAM102A rs3739821 (OR = 1.15, P = 8.32 × 10(-12)). We also confirmed significant association at three previously described loci (P < 5 × 10(-8) for each sentinel SNP at PLEKHA7, COL11A1, and PCMTD1-ST18), providing new insights into the biology of PACG.

Purpose: To investigate the lymphatic vascular microvessel density (LVD) and the blood vascular microvessel density (MVD) and their distribution in excised leaking blebs after mitomycin C trabeculectomy and normal conjunctiva specimens., Materials and Methods: LVD and MVD in normal human conjunctiva (n=8) and excised blebs in the hypocellular stroma and peribleb tissue (conjunctiva adjacent to hypocellular bleb tissue) (n=8) were evaluated by immunohistochemistry using antibodies raised against Lymphatic Vessel Endothelial Receptor 1 (D2-40, lymphatic endothelium) and CD34 (vascular endothelium). LVD and MVD counts were performed by light microscopy in 5 fields at ×20 magnification by 3 observers. Differences were determined using Mann-Whitney U test (P<0.05 was considered significant)., Results: The leaking blebs showed typical epithelial-stromal domes with areas of acellular stroma covered by attenuated epithelium and surrounded by normal conjunctival epithelium and a dense scar-like matrix replacing the substantia propria. The LVD and MVD were significantly reduced to nil in the hypocellular conjunctival stroma of the excised blebs compared with normal conjunctiva (21.42 vs. 1.16, P<0.002 and 24.28 vs. 1, P<0.008, respectively). The LVD and MVD was also reduced (2- to 2.5-fold) in the peribleb stroma when compared with normal conjunctiva specimens., Conclusions: In this study we show reduced LCD and MVD in the hypocellular and peribleb stroma. These results may suggest a role of these vessels in an altered immune response in leaking blebs leading to an increased risk for blebitis.

Purpose: To evaluate changes in intraocular pressure (IOP), vision, and medications at least 1 year after phacoemulsification combined with endocyclophotocoagulation for advanced glaucoma and cataract., Methods: A retrospective chart review was conducted on patients with advanced glaucoma who underwent phacoemulsification combined with endocyclophotocoagulation at King Khaled Eye Specialist Hospital between 2005 and 2012. Data were collected on patient demographics, type of glaucoma, IOP over time, best-corrected visual acuity, number of glaucoma medications, comorbidities, and previous surgeries. Absolute success was defined as IOP≤15 mm Hg without medication and qualified success was IOP≤15 mm Hg with medications. Statistical significance was indicated by P<0.05., Results: The study sample comprised 104 eyes (104 patients). Mean follow-up was 17.3±1.8 months. Mean IOP decreased from 17±1.4 mm Hg preoperatively to 14.7±1.3 mm Hg at the last postoperative visit. Absolute success was achieved in 11.9% (95% confidence interval, 5.6-18.2) of the eyes. Qualified success was achieved in 72.3% (95% confidence interval, 63.5-81.0) of the eyes. Best-corrected visual acuity improved by ≥2 lines in 76 (73%) eyes. Eyes with primary open-angle glaucoma had the higher absolute and qualified success rates compared with primary angle-closure and pseudoexfoliation glaucoma (P>0.05). Only 48 (46%) patients required >3 medications for IOP control compared with 78 (75%) patients before surgery., Conclusions: At ≥1 year postoperatively, the absolute success rate of treating advanced glaucoma by endocyclophotocoagulation and phacoemulsification was low. However, medication burden was reduced. Owing to the significant variation in the success rate based on the type of glaucoma, patients with advanced glaucoma should be carefully selected and counseled.

Purpose: Ahmed glaucoma valve (AGV) implant is an aqueous shunt device used to control intraocular pressure in glaucoma. Implant failure results from impervious encapsulation of the shunt plate causing increased hydraulic resistance and raised intraocular pressure. We hypothesized that deregulation of fibrosis pathway contributes to capsular resistance. We tested this by studying fibrosis related gene expression in failed AGV implants., Methods: Differential gene expression was examined in failed AGV capsules and compared to normal control tenon. Following total RNA extraction, 84 key genes in fibrosis pathway were examined by real-time PCR using RT2 Profiler PCR Array. Relative gene expression was calculated using ΔΔCt method. Gene specific TaqMan assays were used to validate select genes with ≥2 fold differential expression in the array expression profile., Results: We observed differential expression in several genes in the fibrosis pathway. Almost half (39/84) of examined genes showed ≥2 fold differential expression in majority of capsules examined on the array. TaqMan assays for select genes including CCN2 (CTGF), THBS1, SERPINE1, THBS2, COL3A1, MMP3, and IL1A in an increased validation sample set showed significant changes in expression (p value from <0.001 to 0.022) at a high frequency in concurrence with our array results., Conclusions: Pathway-focused analyses identified candidate genes with altered expression providing molecular evidence for deregulation of the fibrosis pathway in AGV failure.

Exfoliation syndrome (XFS) is the most common recognizable cause of open-angle glaucoma worldwide. To better understand the etiology of XFS, we conducted a genome-wide association study (GWAS) of 1,484 cases and 1,188 controls from Japan and followed up the most significant findings in a further 6,901 cases and 20,727 controls from 17 countries across 6 continents. We discovered a genome-wide significant association between a new locus (CACNA1A rs4926244) and increased susceptibility to XFS (odds ratio (OR) = 1.16, P = 3.36 × 10(-11)). Although we also confirmed overwhelming association at the LOXL1 locus, the key SNP marker (LOXL1 rs4886776) demonstrated allelic reversal depending on the ancestry group (Japanese: OR(A allele) = 9.87, P = 2.13 × 10(-217); non-Japanese: OR(A allele) = 0.49, P = 2.35 × 10(-31)). Our findings represent the first genetic locus outside of LOXL1 surpassing genome-wide significance for XFS and provide insight into the biology and pathogenesis of the disease.

Purpose: Primary congenital glaucoma is a common disorder in the Middle East mainly caused by mutations in the the CYP1Bl gene. We report a family with three siblings that presented with recalcitrant childhood glaucoma, aniridia in two siblings with a novel CYP1B1 gene mutation., Materials and Methods: Review of pedigree, clinical history and clinical course of the family. Genetic testing in the affected family members., Results: Three sisters presented with clinical findings of severe congenital glaucoma and a positive family history. Clinical examination of two of sisters revealed corneal scarring, bilateral aniridia with severe glaucoma that required multiple surgical procedures to control intraocular pressure. The third sibling presented with garden-variety primary congenital glaucoma. Genetic analysis revealed a novel CYP1B1 gene mutation (g.8291 C > T; p.S485F)., Conclusion: CYP1B1 mutation related congenital glaucoma can present with an extreme form of anterior segment dysgenesis that includes recalcitrant glaucoma, corneal opacification and aniridia.

Purpose: To compare the scanning electron microscopy (SEM) features of the anterior capsule edge created by continuous curvilinear capsulorhexis (CCC) and femtosecond laser-assisted capsulotomy using angular second moment and contrast., Setting: King Khaled Eye Specialist Hospital, Riyadh, Kingdom of Saudi Arabia., Design: Prospective comparative series., Methods: Anterior capsule specimens following CCC (n = 10) or femtosecond laser-assisted capsulotomy using 2 platforms (Lensx, n = 9, and Victus, n = 10) were studied by SEM. Irregularity of the capsule edge was quantified using 2 parameters (angular second moment and contrast) using ImageJ software. The clinical features and laser parameters were correlated with angular second moment and contrast., Results: By SEM, the femtosecond laser-assisted capsulotomy edge surfaces created by both lasers showed marked irregularity compared with the smooth edge of the CCC. The angular second moment and contrast measures for both lasers differed significantly from those obtained for CCC (P<.001). There was no between-laser difference in angular second moment and contrast measures. The angular second moment showed only a weak negative correlation with increasing laser power, whereas contrast showed a weak positive correlation with increasing power., Conclusions: Both laser platforms created an irregular capsulotomy edge. The angular second moment and contrast quantified capsule edge irregularities for further comparisons. These measures could be used to quantify efforts to reduce capsule-edge irregularity from femtosecond laser-lens capsule interaction., (Copyright © 2014 ASCRS and ESCRS. Published by Elsevier Inc. All rights reserved.)

Background: Sympathetic ophthalmia is a rare form of bilateral uveitis that develops following an ocular penetrating injury to one eye. Several cases of sympathetic ophthalmia have been reported after non-penetrating cyclodestructive procedures including Nd:YAG and diode laser cyclophotocoagulation (CPC) but information on inciting factors and outcome is limited. In this case series we investigated the inciting factors, clinical features, course and outcome of six patients who developed sympathetic ophthalmia after diode CPC., Methods: Six patients who developed sympathetic ophthalmia after diode CPC were identified from the King Khaled Eye Specialist Hospital uveitis database between 2008 and 2013. The medical records of these patients were reviewed for demographic information, ocular history, diode laser parameters, clinical presentation, treatment and outcome., Results: Six patients were diagnosed with sympathetic ophthalmia following treatment with diode laser CPC, an incidence of 0.001% during the study period. Two patients had a history of accidental trauma and all patients had undergone at least one ocular surgery that included a variety of procedures. At presentation, two patients had predominantly posterior segment findings in the sympathising eye, one patient had only anterior segment findings and the rest had panuveitis. All patients were treated with topical and systemic corticosteroids and/or immunosupressive therapy that resulted in complete resolution of inflammation and return to baseline vision in the sympathising eye within 2-48 months of treatment., Conclusions: Sympathetic ophthalmia after diode CPC is rare and is seen in patients with a history of intraocular surgery with or without previous trauma. Early recognition of sympathetic ophthalmia and aggressive management with immunosuppressive therapy results in good outcomes for these patients., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.)

In this brief communication, we present the steps used to establish a web-based congenital glaucoma registry at our institution. The contents of a case report form (CRF) were developed by a group of glaucoma subspecialists. Information Technology (IT) specialists used Lime Survey softwareTM to create an electronic CRF. A MY Structured Query Language (MySQL) server was used as a database with a virtual machine operating system. Two ophthalmologists and 2 IT specialists worked for 7 hours, and a biostatistician and a data registrar worked for 24 hours each to establish the electronic CRF. Using the CRF which was transferred to the Lime survey tool, and the MYSQL server application, data could be directly stored in spreadsheet programs that included Microsoft Excel, SPSS, and R-Language and queried in real-time. In a pilot test, clinical data from 80 patients with congenital glaucoma were entered into the registry and successful descriptive analysis and data entry validation was performed. A web-based disease registry was established in a short period of time in a cost-efficient manner using available resources and a team-based approach.

Acute necrotizing encephalopathy is a rare, clinically distinct entity of acute encephalopathy triggered by acute febrile diseases, mostly viral infections. It is postulated to arise from uncontrolled cytokine release during a febrile illness, and is most often seen in East Asia. We describe a rare Saudi patient of acute necrotizing encephalopathy attributable to enterovirus in a 4 years and 6 months old girl. A work-up revealed elevations in serum and cerebrospinal fluid interleukin-6 and tumor necrosis factor-α. The outcome on intravenous pulse methylprednisolone was good. This case is the first, to the best of our knowledge, of acute necrotizing encephalopathy reported from Saudi Arabia with a good outcome despite severe magnetic resonance imaging findings and delay in the steroid treatment., (Copyright © 2012 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.)

Purpose: To compare the demographic and clinical distribution of primary and secondary congenital glaucoma from a registry at King Khaled Eye Specialist Hospital., Design: Registry-based cohort study., Methods: Review of registry data that included new patients with congenital glaucoma seen between 2001 and 2003 (29 months); analysis of the demographic data and clinical features of primary and secondary congenital glaucoma at presentation., Results: A total of 325 eyes of 180 patients were included. Most patients had primary congenital glaucoma (80%). The mean (± SD) age at presentation for primary congenital glaucoma was 3.8 ± 10.7 months and for secondary congenital glaucoma was 4.3 ± 7.9 months. Most primary congenital glaucoma and secondary congenital glaucoma patients had bilateral disease (82.6%; 74.3%). Primary congenital glaucoma was equally distributed by sex but secondary congenital glaucoma was 1.5-fold more common in male patients. A positive family history was elicited in 30%, and almost 60% had a history of consanguinity in both groups. The mean intraocular pressure and corneal diameter were comparable in both groups but the axial length was significantly longer in primary congenital glaucoma and cup-to-disc (C/D) ratio greater in secondary congenital glaucoma. In the primary congenital glaucoma group, corneal haze showed a significant relationship with most clinical parameters. In primary congenital glaucoma, a positive correlation was noted between age at presentation and increasing corneal diameter and axial length but a negative relationship was noted with C/D ratio and corneal haze, whereas for secondary congenital glaucoma only axial length was positively correlated., Conclusion: The congenital glaucoma registry provides unique baseline data on primary congenital glaucoma and secondary congenital glaucoma in Saudi Arabia that will enable us to better understand the disease in the Kingdom and region., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Currently, there are numerous choices for the treatment of pediatric glaucoma depending on the type of glaucoma, the age of the patient, and other particularities of the condition discussed in this review. Traditionally, goniotomy and trabeculotomy ab externo have been the preferred choices of treatment for congenital glaucoma, and a variety of adult procedures adapted to children have been utilized for other types of pediatric glaucoma with variable results and complications. More recently, seton implantations of different types have become more popular to use in children, and newer techniques have become available including visualized cannulation and opening of Schlemm's canal, deep sclerectomy, trabectome, and milder more directed cyclodestructive procedures such as endolaser and transcleral diode laser cyclophotocoagulation. This paper reviews the different surgical techniques currently available, their indications, results, and most common complications to allow the surgeon treating these conditions to make a more informed choice in each particular case. Although the outcome of surgical treatment in pediatric glaucoma has improved significantly, its treatment remains challenging.

α-Amino adipic semialdehyde (α-AASA) accumulates in body fluids from patients with pyridoxine-dependent epilepsy because of mutations in antiquitin (ALDH7A1) and serves as the biomarker for this condition. We have recently found that the urinary excretion of α-AASA was also increased in molybdenum cofactor and sulfite oxidase deficiencies. The seizures in pyridoxine-dependent epilepsy are caused by lowered cerebral levels of pyridoxal-5-phosphate (PLP), the bioactive form of pyridoxine (vitamin B(6)), which can be corrected by the supplementation of pyridoxine. The nonenzymatic trapping of PLP by the cyclic form of α-AASA is causative for the lowered cerebral PLP levels. We describe 2 siblings with clinically evident pyridoxine-responsive seizures associated with increased urinary excretion of α-AASA. Subsequent metabolic investigations revealed several metabolic abnormities, all indicative for molybdenum cofactor deficiency. Molecular investigations indeed revealed a known homozygous mutation in the MOCS2 gene. Based upon the clinically evident pyridoxine-responsive seizures in these 2 siblings, we recommend considering pyridoxine supplementation to patients affected with molybdenum cofactor or sulfite oxidase deficiencies.

Primary Congenital Glaucoma (PCG) is an autosomal recessive disease caused by an abnormal development of the anterior chamber angle. Although, PCG has been linked to several genetic loci, the role that the genes at these loci or their encoded proteins play in the pathophysiology of PCG and development of the anterior chamber is not known. To identify proteins that may be altered in PCG and that may help in understanding the underlying pathophysiology of the disease, we took a global proteomics approach. Tryptic digests of the complex mixtures of proteins in aqueous humor were analyzed using Liquid Chromatography/Mass Spectrometry (LC-MS/MS). Proteins were identified by searching the data against the human subset of the UniProt database. The proteomes of aqueous humor in PCG (n = 7) and patients undergoing cataract surgery as control (n = 4) were compared based on the scan counts of comparable proteins. Using stringent filtering criteria, Apolipoprotein A-IV (APOA-IV), Albumin and Antithrombin 3 (ANT3) were detected at significantly higher levels in PCG AH compared to control, whereas Transthyretin (TTR), Prostaglandin-H2 D-isomerase (PTGDS), Opticin (OPT) and Interphotoreceptor Retinoid Binding Protein (IRBP) were detected at significantly lower levels. Many of these proteins play a role in retinoic acid (RA) binding/transport and have been implicated in the pathogenesis of neurodegenerative diseases such as Alzheimer's (AD). It is possible that similar to AD, the pathologic changes in PCG during development could be influenced by the availability of RA in the anterior chamber., (Copyright © 2010 Elsevier Ltd. All rights reserved.)

Ahmed valve implants are currently used to manage high-risk complicated adults and pediatric glaucoma when standard filtration surgery is unsuccessful. Despite its success, the Ahmed valve shunt has significant complications particularly in the anterior segment. We report an unusual case of transcorneal tube erosion of an Ahmed valve implant in an adult that resulted from long-standing tube-corneal touch. Periodic observation of tube position is recommended.

Purpose: To identify the microbiological spectrum and visual outcome of bleb-associated endophthalmitis., Methods: The medical records of patients treated for bleb-associated endophthalmitis were reviewed., Results: Seventy-five patients were identified. Vitreous cultures were positive in 48 (64%) patients. The most common isolates were Streptococcus species in 24 (50%) eyes, gram-negative bacteria in 10 (20.8%) eyes, and Staphylococcus epidermidis in 7 (14.6%) eyes. Good visual outcome was achieved in 33 (44%) patients. On univariate analysis, shorter interval from onset of symptoms and treatment (p = .005), better initial visual acuity (p = .004), clear cornea at presentation (p = .048), isolation of less virulent organisms (p < .001), and absence of diabetes mellitus (p = .048) were significant positive predictors for good visual outcome., Conclusions: Bleb-associated endophthalmitis is associated with severe visual impairment. Clinical features associated with better visual outcome include early presentation, better presenting visual acuity, clear cornea at presentation, culture of a less virulent organism, and absence of diabetes mellitus.

Purpose: To determine the prevalence and risk factors for escalation of glaucoma therapy after penetrating keratoplasty (PK) and its impact on graft survival and visual outcome., Design: Retrospective case series., Participants: Seven hundred fifteen consecutive eyes of 678 patients undergoing PK., Methods: Retrospective review of every case of PK performed at King Khaled Eye Specialist Hospital between January 1, 2001 and December 31, 2002., Main Outcome Measures: Escalation of glaucoma therapy, graft survival, and visual outcome., Results: Escalation of glaucoma therapy occurred in 89 (12.4%) eyes of 715 PK procedures during a mean follow-up of 32.2 months. Medical escalation accounted for 73 (82.0%) cases, whereas surgical escalation occurred in 16 (18.0%) eyes. The following were significantly associated with an increased risk of escalation of glaucoma therapy: surgical indication for PK (P<0.001), increasing patient age (P<0.001), preexisting glaucoma (P<0.001), recipient trephination < 7.0 mm (P = 0.02), and pseudophakia or aphakia (P<0.001). Eyes with escalation of glaucoma therapy had significant reduction in graft survival compared with eyes in which this did not occur (52.8% vs. 82.9%, P<0.001). Escalation of glaucoma therapy was associated with a significant reduction in the percentage of eyes achieving visual acuity of 20/40 or better (9.0% vs. 42.1%, P<0.001) and a significant increase in those obtaining 20/200 or worse (70.8% vs. 26.7%, P<0.001)., Conclusions: Escalation of glaucoma therapy is a serious sequela of PK that is significantly associated with an increased risk of graft failure and poor visual outcome.

Background and Objective: To highlight the occurrence of scleral thinning after transcleral diode laser cycloablation therapy., Patients and Methods: A retrospective chart review was done of consecutive patients from the glaucoma and pediatric ophthalmology clinics of a tertiary eye care center in whom the presence of scleral thinning was noted after transcleral diode laser cycloablation therapy., Results: Eleven eyes of 9 patients with various types of glaucoma (traumatic, congenital, juvenile, steroid induced, uveitic, and secondary angle closure) were found to have scleral thinning after undergoing transcleral diode laser cycloablation therapy for refractory glaucoma. Seven patients had undergone only 1 session before new scleral thinning was noted. Two patients had bilateral transcleral diode laser cycloablation therapy and subsequent bilateral thinning., Conclusions: Scleral thinning may occur after diode laser cycloablation therapy even with only 1 treatment session and intact sclera, and young patients may be more susceptible to thinning.

We report a case of traumatic globe rupture following blunt trauma in the left eye of a 20-year-old male who had undergone deep anterior lamellar keratoplasty for keratonus. Prolapse of both, the crystalline lens and the vitreous was present through the inferior 180 degrees donor host junction. After repositioning, anterior vitrectomy and repair of graft host junction, the graft regained clarity in eight weeks and there was good visual recovery.

Purpose: To study the long-term outcomes of surgical revision of leaking blebs after trabeculectomy and identify possible risk factors for failure., Patient and Methods: A retrospective, nonrandomized, noncomparative interventional study of 34 eyes with late bleb leaks after trabeculectomy that underwent bleb excision with conjunctival advancement. The primary outcome measure was successful repair and control of intraocular pressure (IOP). The other measures evaluated included change in visual acuity from baseline and complications such as recurrence of bleb leak, endophthalmitis or the need for additional antiglaucoma medication to control IOP., Results: After a mean follow up of 36.2+/-23 months, the mean IOP at the last visit was 14.5+/-7.6 mm Hg. IOP in 58.8% of eyes was controlled without medication. Complications included early leaks (7/34), late recurrent or persistent leaks (1/34), and endophthalmitis (1/34). In all, 41.2% patients required additional medication at the last visit. Survival analysis at 22 months the probability of total and qualified success was 52% and 72%, respectively. However, this dropped to 10% and 15%, respectively, at 5 years. The probability of total and qualified success further dropped to 2.5% and 5% at 5 years when the IOP cut-off was lowered from 21 to 15 mm Hg. Cox regression analysis failed to identify risk factors for bleb failure., Conclusions: Surgical bleb revision seems to be effective in treating late bleb leaks with few postoperative complications. However, patients should be followed carefully as late failure of bleb function beyond 2 years is a significant possibility.

Corticosteroid-induced glaucoma is a well-recognized phenomenon in adults, but not children. We describe an infant who developed juvenile glaucoma with buphthalmos while receiving systemic steroid treatment. The intraocular pressure normalized several months following discontinuation of treatment.

Purpose: To investigate surgical outcomes after deep anterior lamellar keratoplasty (DALKP) in eyes with keratoconus., Methods: Retrospective review of 127 eyes of 118 keratoconus patients who underwent DALKP at King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia between January 1, 1998 and November 30, 2001. The mean age of all patients was 25.9 years (range 11-70 years). The mean follow-up for all patients was 10.9 months (range 4-61 months). Baring of Descemet's membrane (DM) during DALKP was achieved in 47 (37%) eyes; some stroma was left during operation in the remaining 80 (63%) eyes. Snellen visual acuity, intraoperative complications, and postoperative graft status were assessed., Results: At the last follow-up visit, 74% of all eyes were able to achieve a best-corrected visual acuity of 20/50 or better. This level of improvement was more statistically significant with baring of DM (P = 0.005). Intraoperative perforation of DM occurred in 16 (13%) cases and was statistically significant in eyes with deep corneal scars (P = 0.012). However, this perforation did not seem to have an impact on the final visual acuity (P = 0.48). Main graft-related complications included graft-host vascularization (7/127), stromal graft rejection (4/127), graft infection (1/127), and persistent epithelial defect (1/127)., Conclusions: Deep lamellar keratoplasty provides a safer and successful alternative to penetrating keratoplasty for keratoconus patients but remains a challenging procedure. Endothelial graft rejection is absent after DALKP. However, stromal graft rejection, although very rare, does occur.