Ophthalmic manifestations of 107 cases with hemolysis, elevated liver enzymes and low platelet count syndrome.

OBJECTIVE: To present various ophthalmologic disorders in a clinical series of hemolysis, elevated liver enzymes and low platelet count (HELLP) syndrome cases. METHODS: This is a prospective clinical study performed between 2002 and 2005. One hundred seven HELLP patients attended in either Departments of Ophthalmology or Obstetrics and Gynecology, Medical School, Gaziantep University, Gaziantep, Turkey were evaluated. RESULTS: Mean age was 25.5 (22-36 years). Mean levels were 2.5 gravidity, 1.3 parity, 55,200/mm3 platelet counts, 308.7 U/l aspartate transaminase, 255.4 U/l alanine transaminase, and 1711.6 U/I lactate dehydrogenase. Four patients died (3.7%) despite the proper treatments. Cortical blindness was observed in 3 cases (2.7%), serous retinal detachments in 4 (3.7%), and mild hypertension changes in 18 (16%). CONCLUSION: Ophthalmic complications are possible during and after this syndrome. Almost all ophthalmologic changes recover after delivery by cesarean section, nevertheless, it is essential that ophthalmologists should be aware of retinal disorders when this fatal complication of pregnancy is encountered.

Angiotensin-converting enzyme insertion-deletion polymorphism in primary open-angle glaucoma.

PURPOSE: To investigate the hypothesis that primary open-angle glaucoma (POAG) is associated with a common insertion-deletion (I/D) polymorphism in the angiotensin-converting enzyme (ACE) gene. METHODS: ACE I/D polymorphism was investigated in a control group of healthy subjects (n = 101) and in a group of patients diagnosed with POAG (n = 104). Polymerase chain reaction detection of I/D polymorphism was used to determine the presence of the two ACE alleles in the groups. RESULTS: Neither the I/D genotype distributions nor the allele frequencies differed significantly between POAG and control subjects (DD genotype 34.6 vs. 39.6%; ID genotype 53.9 vs. 40.6%; II genotype 11.5 vs. 19.8%, p = 0.1; D allele 61.5 vs. 60%; I allele 38.5 vs. 40%, p = 0.8). CONCLUSION: We could not identify a possible association of the I/D polymorphism in the ACE gene with POAG, however further studies with larger patient numbers in different populations are required to clarify the role of ACE gene in susceptibility to POAG.

Effectiveness of retroequatorial recession surgery in congenital nystagmus.

OBJECTIVE: The aim of this retrospective study was to evaluate the effectiveness of the retroequatorial recession technique in the surgical treatment of nystagmus and associated strabismus. METHOD: The effect of large recessions of four horizontal extraocular muscles was analyzed qualitatively in seven patients with congenital nystagmus. RESULTS: The ages of the patients ranged between 12 and 42 years (mean, 21.5 years). Of the seven patients, four had motor and three had sensory nystagmus. Visual acuity increased in five (71.4%) patients. Planned recovery of associated strabismus was attained in six patients (85.7%). In five patients who had abnormal head position, the head positions and nystagmus intensities were found to be decreased. No postoperative complication, such as diplopia, was encountered. CONCLUSION: The retroequatorial recession technique is preferable in patients who have congenital nystagmus, especially in the presence of horizontal squint associated with low visual acuity, because of its success in increasing visual acuity and decreasing the amplitude of nystagmus, its reversibility, making posssible new operations in the future, as well as the absence of any significant complications.

Comparison of secondary implantation of flexible open-loop anterior chamber and scleral-fixated posterior chamber intraocular lenses.

PURPOSE: To analyze and compare the outcomes and complication rates of secondary implantation of flexible, open-loop, anterior chamber intraocular lenses (AC IOLs) and single-piece, scleral-fixated, posterior chamber IOLs (PC IOLs). SETTING: Departments of Ophthalmology, Gaziantep University Medical Faculty, Research Hospital, Gaziantep, and Inönü University Medical Faculty, Research Hospital, Malatya, Turkey. METHODS: This study comprised 124 eyes of 113 aphakic patients (61 men, 52 women) with insufficient capsule support who had secondary IOL implantation from January 1997 to June 2001. In Group 1 (n = 73 eyes), a flexible, open-loop AC IOL was implanted and in Group 2 (n = 51 eyes), a single-piece, scleral-fixated PC IOL. The mean follow-up was 34 months (range 6 to 53 months) and took place at several different clinical settings. The mean interval between the initial cataract operation and secondary IOL implantation was 63 months (range 6 months to 12 years). The postoperative outcomes, safety, efficacy, and complication rates were analyzed, and the preoperative and postoperative best spectacle-corrected visual acuities (BSCVAs) were compared. RESULTS: The postoperative mean BSCVA was 20/34.8 +/- 45.2 (SD) in Group 1 and 20/32.1 +/- 33.7 in Group 2; the difference was not significant (P =.718). A BSCVA of 20/40 or better was achieved in 62 eyes (84.9%) in Group 1 and 45 eyes (88.2%) in Group 2. A BSCVA of 20/25 or better was achieved in 40 eyes (54.8%) and 33 eyes (64.7%), respectively. The difference between the 2 groups was not significant (P =.472). Complications occurred in 25 eyes (34.2%) in Group 1 and 13 eyes (25.5%) in Group 2 (P >.05). The most frequent complications in Group 1 were early transient corneal edema, intraocular pressure elevation, cystoid macular edema, hyphema, secondary glaucoma, and iris capture or pupil decentration and in Group 2, suture erosion, a tilted or decentered IOL, fibrin reaction, and vitreous prolapse into the anterior chamber. CONCLUSIONS: The AC IOLs and PC IOLs were safe and effective for secondary implantation to correct aphakia. Secondary implantation of the scleral-fixated PC IOL seemed to provide a more favorable outcome and a lower complication rate than the open-loop AC IOL in complicated cataract cases with inadequate capsule and zonular support. As scleral-fixated PC IOL implantation is technically more difficult than AC IOL implantation, the decisive factor in choosing a secondary IOL is surgical experience. Long-term comparison of both techniques is required.

Central corneal thickness is lower in osteogenesis imperfecta and negatively correlates with the presence of blue sclera.

BACKGROUND: Osteogenesis imperfecta (OI) is a rare, autosomal-inherited, connective tissue disorder characterised by bone fractures, deafness and blue sclera. Additional ocular findings are decreased ocular rigidity, myopia, glaucoma, keratoconus, corneal opacity, small corneal diameter and congenital Bowman's layer agenesis. PURPOSE: This cross-sectional, masked, case-control study aimed to assess whether central corneal thickness (CCT) is affected in patients with OI and to focus on the clinical significance of scleral blueness. MATERIALS AND METHODS: Twenty-three children with OI (13 boys, 10 girls) and 15 age-, sex- and refraction-matched healthy control subjects (eight boys, seven girls) were assessed for CCT by ultrasound pachymetry. The CCT was compared between two different patient subgroups (type-I OI with blue sclera, n = 12; type-IV OI without blue sclera, n = 11). Mann-Whitney U-test or analysis of variance was used as indicated and only right eyes of each subject were included in statistical analysis. Results were expressed as mean +/- S.D. and statistical significance was taken as p < 0.05. RESULTS: Mean age and sex distribution was similar between the groups (10.1+/-2.5 vs 9.8+/-1.8 years, p > 0.05). Patients with OI had significantly lower CCT (459.5+/-24.6 microm) than in control subjects (543.6+/-21.4 microm; p < 0.001). The CCT was below 500 microm in 22 of 23 children (95.6%) with OI, 15 of which (65.2%) were below 450 microm. In contrast, CCT was over 500 microm in all eyes in the control group. Type-I OI eyes with blue sclera had significantly (p = 0.005) lower CCT readings (446.5+/-16.3 microm) than type-IV OI eyes without blue sclera (473.6+/-25.0 microm). Mean keratometric values were similar between the groups (44.2+/-1.7 vs 43.8+/-1.6 dioptre, p > 0.05). Mean cycloplegic refraction was similar between the groups (-0.32+/-0.5 vs -0.18+/-0.4 dioptre; p > 0.05), although five of 23 OI patients had myopia, and mean intraocular pressure was lower in OI patients than controls (12.7+/-1.8 mmHg vs 15.6+/-1.9 mmHg; p < 0.001). CONCLUSIONS: The CCT is thinner and negatively correlated with the blueness of the sclera in patients with OI. The CCT readings may therefore be of utmost importance in the diagnosis of OI. An ophthalmologist should be aware of an artificially low intraocular pressure measurement in such patients. In addition, when considering a keratorefractive treatment, CCT must be evaluated carefully to avoid unexpected results or complications. Sturdy protective spectacles should be prescribed to those who are not bed bound. Possible correlation of low CCT with biochemical changes in scleral collagen or systemic parameters awaits further investigation.

Decreased central corneal thickness in children with Down syndrome.

PURPOSE: A cross-sectional, masked, case-control study was undertaken to investigate whether central corneal thickness is affected in children with Down syndrome and to focus on its clinical significance. PARTICIPANTS AND METHODS: Twenty-eight children with Down syndrome (15 boys and 13 girls) aged 5 to 15 years and 20 age-matched and gender-matched healthy control subjects (11 boys and 9 girls) from a similar ethnic background were enrolled in the study. Central corneal thickness was measured by ultrasound pachymetry. Only the right eye of each child in each group was included in the statistical analysis. The Mann-Whitney U test was used and a P value of less than .05 was considered significant. RESULTS: The mean ages of the children with Down syndrome (9.28 +/- 3.47 years) and the healthy control subjects (8.75 +/- 3.30 years) were comparable (P > .05). Central corneal thickness values were below 500 microm in 19 (67.8%) of the 28 children with Down syndrome, 4 of which were less than 450 microm. However, all central corneal thickness measurements in the control eyes were more than 500 microm. The mean central corneal thickness in the children with Down syndrome was significantly (P < .001) less (488.39 +/- 39.87 microm) than that in the healthy control subjects (536.25 +/- 20.70 microm). Mean keratometric values were significantly (P < .001) higher in the eyes of the children with Down syndrome (46.35 +/- 1.28 D) than in the eyes of the control subjects (43.32 +/- 1.15 D). CONCLUSIONS: Children with Down syndrome had a decreased central corneal thickness compared with healthy control subjects. Decreased central corneal thickness may give an artificially low intraocular pressure measurement by applanation tonometry. Central corneal thickness must be considered when developing approaches for keratorefractive treatment of patients with Down syndrome.