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Purpose: To compare 2 frequently used aqueous shunts for the treatment of glaucoma., Design: International, multicenter, randomized trial., Participants: Patients aged 18 years or older with uncontrolled glaucoma despite maximum tolerated medical therapy, many of whom had failed or were at high risk of failing trabeculectomy., Methods: Eligible patients were randomized to receive an Ahmed-FP7 valve implant (New World Medical, Inc, Rancho Cucamonga, CA) or a Baerveldt-350 implant (Abbott Medical Optics, Inc, Santa Ana, CA) using a standardized surgical technique., Main Outcome Measures: The primary outcome was failure, defined as intraocular pressure (IOP) outside the target range (5-18 mmHg) or reduced <20% from baseline for 2 consecutive visits after 3 months, severe vision loss, or de novo glaucoma surgery. Secondary outcomes measures included IOP, medication use, visual acuity, complications, and interventions., Results: A total of 238 patients were randomized; 124 received the Ahmed-FP7 implant, and 114 received the Baerveldt-350 implant. Baseline characteristics were similar between groups. Mean preoperative IOP was 31.4±10.8 mmHg on 3.1±1.0 glaucoma medications. At 5 years, the cumulative failure rate was 53% in the Ahmed group and 40% in the Baerveldt group (P = 0.04). The main reason for failure in both groups was high IOP, and the cumulative de novo glaucoma reoperation rate was 18% in the Ahmed group and 11% in the Baerveldt group (P = 0.22). Hypotony resulted in failure in 5 patients (4%) in the Baerveldt group compared with none in the Ahmed group (P = 0.02). Mean IOP was 16.6±5.9 mmHg in the Ahmed group (47% reduction) and 13.6±5.0 mmHg in the Baerveldt group (57% reduction, P = 0.001). Mean medication use was 1.8±1.5 mmHg in the Ahmed group (44% reduction) and 1.2±1.3 mmHg in the Baerveldt group (61% reduction, P = 0.03). The 2 groups had similar complication rates (Ahmed 63%, Baerveldt 69%) and intervention rates (Ahmed 41%, Baerveldt 41%). Most complications were transient, and most interventions were slit-lamp procedures., Conclusions: Both implants were effective in reducing IOP and the need for glaucoma medications. The Baerveldt group had a lower failure rate and a lower IOP on fewer medications than the Ahmed group, but had a small risk of hypotony that was not seen in the Ahmed group., (Copyright © 2016 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Objective: To compare 2 commonly used aqueous drainage devices for the treatment of refractory glaucoma., Design: International, multicenter, randomized trial., Participants: Patients aged 18 years or older with uncontrolled or high-risk glaucoma refractory to maximum medical therapy, many of whom had failed trabeculoplasty and trabeculectomy., Methods: Eligible patients were randomized to an Ahmed-FP7 valve implant (New World Medical, Inc., Rancho Cucamonga, CA) or a Baerveldt-350 implant (Abbott Medical Optics, Inc., Santa Ana, CA) using a standardized surgical technique., Main Outcome Measures: The primary outcome was failure, defined as intraocular pressure (IOP) outside of the target range (5-18 mmHg, with ≥20% reduction from baseline) for 2 consecutive visits after 3 months, vision-threatening complications, de novo glaucoma procedures, or loss of light perception. Secondary outcome measures include IOP, medication use, visual acuity, complications, and interventions., Results: A total of 238 patients were enrolled and randomized; 124 received the Ahmed implant and 114 received the Baerveldt implant. Baseline characteristics were similar in both groups. Half the study group had secondary glaucoma, and 37% had previously failed trabeculectomy. The mean preoperative IOP was 31.4±10.8 mmHg on 3.1±1.0 glaucoma medications. Median baseline Snellen visual acuity was 20/100. At 3 years, the cumulative probability of failure was 51% in the Ahmed group and 34% in the Baerveldt group (P = 0.03). Mean IOP was 15.7±4.8 mmHg in the Ahmed group (49% reduction) and 14.4±5.1 mmHg in the Baerveldt group (55% reduction; P = 0.09). Mean number of glaucoma medications was 1.8±1.4 in the Ahmed group (42% reduction) and 1.1±1.3 in the Baerveldt group (65% reduction; P = 0.002). There was a moderate but similar decrease in visual acuity in both groups (P< 0.001). The 2 groups had similar complication rates (52% Ahmed, 62% Baerveldt; P = 0.12); however, the Baerveldt group had a higher rate of hypotony-related vision-threatening complications (0% Ahmed, 6% Baerveldt; P = 0.005). More interventions were required in the Baerveldt group, although the difference did not reach statistical significance (38% Ahmed, 50% Baerveldt; P = 0.07). Most complications were transient, and most interventions were slit-lamp procedures., Conclusions: Both devices were effective in reducing IOP and glaucoma medications. The Baerveldt group had a lower failure rate and required fewer medications than the Ahmed group after 3 years, but it experienced more hypotony-related vision-threatening complications., (Copyright © 2013 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Objective: To report the design, baseline patient characteristics, and intraoperative complications of the Ahmed Versus Baerveldt (AVB) Study., Design: Multicenter, randomized, clinical trial., Participants: Patients were recruited from 7 international clinical sites and treated by 10 surgeons between 2005 and 2009. Inclusion criteria required that patients be at least 18 years of age and have uncontrolled glaucoma refractory to medicinal, laser, and surgical therapy., Methods: Eligible patients were randomized to undergo implantation of an Ahmed-FP7 valve (New World Medical, Inc., Rancho Cucamonga, CA) or a Baerveldt-350 implant (Abbott Medical Optics, Inc., Santa Ana, CA) using standardized surgical technique, to be followed for 5 years., Main Outcome Measures: The primary outcome measure was failure, defined as intraocular pressure (IOP) out of target range (5-18 mmHg with ≥ 20% reduction from baseline) for 2 consecutive visits after 3 months, vision-threatening complications, additional glaucoma procedures, or loss of light perception. Secondary outcome measures included IOP, medication use, visual acuity, complications, and interventions., Results: A total of 238 patients were enrolled in the study; 124 received the Ahmed-FP7 valve implant and 114 received the Baerveldt-350 implant. The 2 treatment groups did not differ in any baseline characteristics with the exception of sex. The mean age of the study group was 66 ± 16 years, and 55% were women, with a greater proportion in the Baerveldt group (P=0.01). The mean baseline IOP of the study group was 31.4 ± 10.8 on a mean of 3.1 ± 1.0 glaucoma medications. The median Snellen visual acuity was 20/100, mean number of previous laser therapies was 0.9 ± 1.1, and mean number of previous surgeries was 1.7 ± 1.2. Five (4%) patients in the Ahmed group and 4 (4%) patients in the Baerveldt group experienced significant intraoperative complications., Conclusions: Aqueous drainage devices are being increasingly used for glaucoma refractory to conventional treatment, and the AVB Study compares the 2 most commonly implanted devices. The 2 groups had similar baseline characteristics, and there were a similar number of intraoperative complications for both devices., Financial Disclosure(s): Proprietary or commercial disclosure may be found after the references., (Copyright © 2011 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Objective: To report the 1-year treatment outcomes of the Ahmed Versus Baerveldt (AVB) Study., Design: Multicenter randomized clinical trial., Participants: A total of 238 patients were enrolled in the study, including 124 in the Ahmed group and 114 in the Baerveldt group., Methods: Patients aged 18 years or older with uncontrolled glaucoma refractory to medicinal, laser, and surgical therapy were randomized to undergo implantation of an Ahmed-FP7 valve (New World Medical, Inc., Rancho Cucamonga, CA) or a Baerveldt-350 implant (Abbott Medical Optics, Inc., Santa Ana, CA), to be followed for 5 years., Main Outcome Measures: The primary outcome measure was failure, defined as intraocular pressure (IOP) out of target range (5-18 mmHg with ≥ 20% reduction from baseline) for 2 consecutive visits after 3 months, vision-threatening complications, additional glaucoma procedures, or loss of light perception. Secondary outcome measures included IOP, medication use, visual acuity, complications, and interventions., Results: There were no significant differences in baseline ocular or demographic characteristics between the study groups with the exception of sex. Preoperatively, the study group had a mean IOP of 31.4 ± 10.8 mmHg on a mean of 3.1 ± 1.0 glaucoma medications with a median Snellen acuity of 20/100. The cumulative probability of failure a 1-year was 43% in the Ahmed group and 28% in the Baerveldt group (P = 0.02). The mean IOP at 1 year was 16.5 ± 5.3 mmHg in the Ahmed group and 13.6 ± 4.8 mmHg in the Baerveldt group (P < 0.001). The mean number of glaucoma medications required was 1.6 ± 1.3 in the Ahmed group and 1.2 ± 1.3 in the Baerveldt group (P = 0.03). Visual acuity was similar in both groups at all visits in the first year (P = 0.66). In the first year after surgery, there were a similar number of patients who experienced postoperative complications in the 2 groups (45% Ahmed, 54% Baerveldt, P = 0.19), but a greater number of patients in the Baerveldt group required interventions (26% Ahmed vs. 42% Baerveldt, P = 0.009)., Conclusions: The Baerveldt-350 group had a higher success rate than the Ahmed-FP7 group after 1 year of follow-up, but required a greater number of interventions., Financial Disclosure(s): Proprietary or commercial disclosure may be found after the references., (Copyright © 2011 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Purpose: To discuss the clinical problem of exposed anterior segment tubes, to review the literature on revision surgery, and to present my method of revision for tube exposure., Methods: Review of the pertinent literature and tabulation of complications from the author's cases of revision surgery., Results: The risk of endophthalmitis requires that all cases of exposed anterior segment tube undergo revision with a patch graft. Simple conjunctival closure is inadequate. A patch graft of collagenous human autograft or allograft material is necessary to prepare the eye for complete healing and resolution of the exposure. Methods of revision from the literature are reviewed and presented, along with the author's method of revision. Complications, reported and potential, are discussed., Conclusions: All exposed anterior segment tubes should be revised with patch graft to resolve the exposure and avoid endophthalmitis. Several methods of revision are available, and the rate of complications is low.

The transcription factor FKHL7 gene has recently been associated with the anterior segment dysgenesis disorder of the eye known as Axenfeld-Rieger anomaly (ARA). A growing body of evidence indicates that mutations in FKHL7 cause not only defects in the anterior segment of the eye but defects in the heart valves and septa as well. In order to evaluate its contribution to normal heart septation and valve formation, expression of the mouse homologue Mf1 in embryonic hearts was analyzed by in situ hybridization. A weak but significant level of Mf1 expression could be detected in the endocardium of mouse embryos as early as day 8.5 post-conception (p.c.). Mf1 expression was undetectable in the hearts of day 9.5 p.c. embryos, but by day 10.5-11 p.c., Mf1 transcripts could be found again in the endocardium of both the atrium and ventricle and a relatively strong signal was observed in the dorsal portion of the septum primum, in what appeared to be the spinal vestibule. At day 13 p.c. when aortic and pulmonary trunks are separated, relatively more Mf1 transcripts were detected in the leaflets of aortic, pulmonary, and venous valves, the ventral portion of the septum primum, as well as in the single layer of cells on the edges of the atrioventricular cushion tissues. Surprisingly, there was no signal detected in the developing interventricular septum. At day 15 p.c., overall Mf1 signals were greatly decreased. However, significant levels of expression could still be observed in the atrial septum, the tricuspid valve, the mitral valve, and in the venous valve but not in the interventricular septum. The temporal and spatial expression patterns of the Mf1 gene in developing mouse hearts suggest that Mf1 may play a critical role in the formation of valves and septa with the exception of the interventricular septum. This is further supported by our studies showing that mutations in the FKHL7 gene were associated with defects in the anterior segment of the eye as well as atrial septal defects or mitral valve defects. Dev Dyn 1999;216:16-27., (Copyright 1999 Wiley-Liss, Inc.)

Purpose: To determine whether autosomal dominant iris hypoplasia is caused by mutations in the newly described gene for Rieger syndrome (RIEG/PITX2)., Method: Mutation screening and sequence analysis was performed in a single family., Results: A novel mutation in the RIEG/PITX2 gene was found in all affected but no unaffected individuals. This mutation would be expected to result in an arginine to tryptophan amino acid change in the homeodomain of solurshin, the RIEG/ITX2 gene product., Conclusion: Autosomal dominant iris hypoplasia is caused by a defect in the same gene that is defective in many cases of Rieger syndrome.

Glaucoma is a major cause of blindness and is characterized by progressive degeneration of the optic nerve and is usually associated with elevated intraocular pressure. Analyses of sequence tagged site (STS) content and haplotype sharing between families affected with chromosome 1q-linked open angle glaucoma (GLC1A) were used to prioritize candidate genes for mutation screening. A gene encoding a trabecular meshwork protein (TIGR) mapped to the narrowest disease interval by STS content and radiation hybrid mapping. Thirteen glaucoma patients were found to have one of three mutations in this gene (3.9 percent of the population studied). One of these mutations was also found in a control individual (0.2 percent). Identification of these mutations will aid in early diagnosis, which is essential for optimal application of existing therapies.

Objectives: To assess retinal complications and to identify risk factors for retinal complications following aqueous shunt procedures., Materials and Methods: Records of 38 consecutive aqueous shunt procedures that were performed on 36 patients at the Eye Institute of the Medical College of Wisconsin, Milwaukee, from June 1993 to March 1995 (minimum follow-up, 6 months) were reviewed. The mean +/- SD follow-up was 11.4 +/- 5.2 months (median, 10.5 months)., Results: Twelve patients (32%) had the following retinal complications: 4 serous choroidal effusions (10%) that required drainage, 3 suprachoroidal hemorrhages (8%), 2 vitreous hemorrhages (5%), 1 rhegmatogenous retinal detachment (3%), 1 endophthalmitis (3%), and 1 scleral buckling extrusion (3%). Surgical procedures for retinal complications were required in 8 (67%) of these 12 patients. Visual acuity decreased 2 lines or more in 9 (75%) of these 12 patients. The median onset of a postoperative retinal complication was 12.5 days, with 10 patients (83%) experiencing complications within 35 days. Serous choroidal effusions developed in 10 other patients (26%), and these effusions resolved spontaneously. Visual acuity decreased 2 lines or more in 2 (20%) of these additional 10 patients. Patients who experienced serious retinal complications were significantly older, had a higher rate of hypertension, and postoperative ocular hypotony. Serious retinal complications were distributed evenly among patients with Krupin valves with discs and Molteno and Baerveldt devices. Experience with the Ahmed glaucoma valve implant was limited., Conclusion: Aqueous shunt procedures may be associated with significant retinal complications and subsequent visual loss.

Purpose: Studies of twins may help to establish the importance of hereditary and environmental determinants of chronic diseases. Intraocular pressure (IOP) is a risk factor for glaucoma. The authors hypothesized that IOPs would be more highly correlated between monozygotic (MZ) than between dizygotic (DZ) twins., Methods: This was a concurrent, case-control study. Volunteer pairs of twins were recruited at a twins' festival. Zygosity was determined by a questionnaire that correlates well with blood-typing classification. Intraocular pressure was measured with a Tonopen six times within 5 minutes (3 times per eye) and averaged., Results: Mean IOP was 14.4 +/- 2.7 mmHg for 61 pairs of MZ twins and 13.9 +/- 2.6 mmHg for 32 pairs of DZ twins. The difference in IOP between the members of a pair was abbreviated delta IOP. The authors found a significant difference in median delta IOP between the MZ and DZ groups (1.2 versus 2.3 mmHg; P = 0.0014). Intraocular pressures were more highly correlated when considering right versus left eyes (intraclass correlation, r1 = 0.853; P < 0.001) than when considering the members of pairs of MZ twins (r1 = 0.735; P < 0.001), which in turn were more highly correlated than pressures of the members of pairs of DZ twins (r1 = 0.407; P = 0.006)., Conclusions: The intraclass correlation for IOP was greater for MZ than for DZ twins. In addition, the median intrapair difference (median delta IOP) was less for MZ than for DZ twins. These results suggest that hereditary factors may play some role in the determination of IOP.

Iris hypoplasia is an autosomal dominant disorder which is frequently associated with glaucoma. This glaucoma is usually resistant to medical therapy and can lead to blindness. A large family of Scandinavian descent with a five generation history of iris hypoplasia was studied. Fifteen individuals were found to have iris hypoplasia, nine of whom had associated glaucoma. In an attempt to identify the chromosomal location of the disease-causing gene, this family was genotyped with short tandem repeat polymorphisms (STRPs) known to map to loci previously associated with glaucoma. The juvenile glaucoma locus at 1q25 and a congenital glaucoma locus on 6p were both statistically excluded. However, significant linkage was demonstrated at the Rieger syndrome locus at 4q25. The highest observed LOD score was 3.70 (theta = 0) and was obtained with marker D4S1616. Three recombination events were observed in affected individuals that together demonstrate that the disease-causing gene lies between markers ACT3E03 and D4S1611, an interval of approximately 7 cM. These results suggest that autosomal dominant iris hypoplasia and Rieger syndrome are allelic.

African Americans have a higher prevalence of elevated blood pressure and a higher prevalence of increased intraocular pressure (IOP). The blood pressure of African Americans has been found to be related to skin color. This study evaluated whether IOP was related to skin color. We measured IOP using a Tonopen and skin darkness using a spectrocolorimeter in 213 African Americans. Seventy patients were identified as systemic hypertensives. Spearman correlation coefficients were calculated to compare IOP and skin darkness. Mean IOP among hypertensives was 17.7 +/- 3.6 mmHg and among normotensives was 17.7 +/- 3.8 mmHg. Mean IOP for the whole sample was 17.7 +/- 3.7 mmHg. No significant correlations were found between skin darkness and IOP among the normotensive and hypertensive groups (p = 0.52 and 0.44) nor for the sample as a whole (p = 0.33). Skin darkness as a measure of skin color in this sample population did not predict those subjects with higher IOPs.

Purpose: To determine the incidence of, visual loss from, and perioperative risk factors for suprachoroidal hemorrhage (SCH) occurring during or after glaucoma filtering surgery., Methods: Contact B-Scan ultrasonography was used to evaluate at a median of 15 days postoperatively, one eye of 158 patients who underwent various glaucoma filtering procedures during an 18 month period., Results: Ultrasonography detected SCH in 13 patients (8.2%). SCH was recognized during surgery in two cases; 11 were detected postoperatively. Preexisting aphakia (odds ratio 12.9, 95% confidence interval 3.6 to 46.2) and intraoperative anterior vitrectomy (odds ratio 5.2, 95% confidence interval 1.2 to 22.4) were significantly associated with SCH. A significant negative association was found for combined cataract/glaucoma procedures with posterior chamber intraocular lens implantation (odds ratio 0.08, 95% confidence interval 0.01 to 0.69). No significant association between SCH and age, sex, race, diabetes, obesity, systemic hypertension, right versus left eye, type of glaucoma, surgeon, number of preoperative antiglaucoma medications, 5-fluorouracil/mitomycin-C therapy-or previous vitrectomy was found. For some risk factors the power of the study may not be sufficient to establish a correlation: with SCH. Two patients with SCH had serious visual acuity loss and 2 had mild visual acuity loss. Eyes of three patients were surgically drained of SCH. Most patients with SCH did not experience pain, and only one presented-with elevated intraocular pressure at the time SCH was recognized., Conclusion: Pre-existing aphakia and concurrent vitrectomy were significant risk factors identified. Combined cataract and glaucoma filtering procedures correlated negatively with suprachoroidal hemorrhage. Most patients with suprachoroidal hemorrhage experienced little or no visual loss, pain, or intraocular pressure elevation.

We compared 23 patients (ages 4 to 18 years) who were receiving long-term oral prednisone therapy with 31 normal controls (ages 7 to 16 years). Indications for corticosteroid treatment included renal transplant in 11 patients, nephrotic syndrome in 7, glomerulonephritis in 4, and vasculitis without renal disease in 1. The mean duration of prednisone therapy was 5.0 +/- 3.5 years, and the mean dose at the time of examination was 0.29 +/- 0.18 mg/kg/day. Mean intraocular pressure was 16.0 +/- 3.0 mm Hg (range, 12 to 25 mm Hg) in the prednisone group and 15.4 +/- 2.1 mm Hg (range, 12 to 20 mm Hg) in the control group. The difference between the means was 0.6 mm Hg (P = 0.35). Posterior subcapsular cataracts were present in seven (30%) of the prednisone patients, but in none of the controls (P = .001). None of the cataracts were visually significant. We found no evidence that pediatric patients on long-term, low-dose prednisone have higher intraocular pressures (IOPs) than normal children, although they are more likely to develop posterior subcapsular cataracts.

We describe a patient with a preexisting, functioning Molteno implant in whom acute endophthalmitis developed following cataract extraction. The condition was treated successfully without removing the implant.

A 66-year-old man developed progressive visual field loss in the inferior arcuate region of the right eye with normal central visual acuity. Intraocular pressures were normal on all but one occasion. The right optic disk showed extensive glaucomalike cupping; the left optic disk was normal. Magnetic resonance imaging revealed a tumor of the right medial sphenoid wing impinging upon the intracranial portion of the right optic nerve. It was resected via a frontotemporal craniotomy. Histopathologic examination revealed a meningothelial meningioma. The visual field and optic disk cupping remained unchanged postoperatively. To our knowledge, this is the first report in which both glaucomalike cupping and visual field loss occurred as the result of a compressive lesion of the anterior visual pathway.

Several reports have suggested that nonarteritic anterior ischemic optic neuropathy (AION) may be related to increased intraocular pressure. We reviewed the records of 45 patients aged 48 through 86 years with nonarteritic AION (10 patients had bilateral AION) for intraocular pressure measurements and the diagnosis of glaucoma or suspected glaucoma. This group was compared with 45 age- and sex-matched patients with normal eye examination results. The mean +/- SD intraocular pressures were 16.3 +/- 3.3 mm Hg for 45 eyes of the 45 patients with AION and 16.1 +/- 2.8 mm Hg for 45 eyes of the 45 control patients (paired t test, P = .70). Among patients with unilateral AION, intraocular pressure was not greater in the involved eye than in the uninvolved eye. Three patients with AION had a previous diagnosis of glaucoma, while three of the control patients were being followed up with suspected glaucoma. We found no evidence in our series to support the hypothesis that AION is associated with increased intraocular pressure.

To determine the efficacy and safety of the procedure, we reviewed the records of 25 eyes of 25 patients who had received noncontact transscleral Nd:YAG laser cyclocoagulation for a variety of uncontrolled glaucomas. Thirteen to 35 pulses (mean, 22.7 pulses) had been placed 1 mm to 2 mm behind the limbus with an energy per pulse of 3.4 J to 8.2 J (mean 4.5 J). Follow-up ranged from 6 months to 23 months, with the exception of one eye, which had to be enucleated. Nine of the 25 eyes (36%) required one or two repeat treatments. Twelve (48%) had intraocular pressures at last follow-up of less than 21 mm Hg but greater than 4 mm Hg. Ten eyes (40%) developed moderate to severe pain transiently, and 10 (40%) developed substantial, but transient, inflammation. Transscleral Nd:YAG laser cyclocoagulation appears to be relatively effective and well tolerated.