Boston type I keratoprosthesis for treatment of gelatinous drop-like corneal dystrophy after repeated graft failure.

PURPOSE: To describe the use of a Boston type I keratoprosthesis as a secondary penetrating procedure to treat gelatinous drop-like corneal dystrophy (GDLD), with presentation of pathologic findings, genetic analysis, and discussion of other surgical options. METHODS: A 43-year-old woman with GDLD in both eyes, best corrected visual acuity (BCVA) of counting fingers in both eyes, and recurrent corneal opacification following two penetrating keratoplasties presented for visual rehabilitation. A Boston type I keratoprosthesis was implanted in her left eye after extracapsular clear lens extraction. RESULTS: The surgery was uneventful and one month after surgery, best corrected vision improved to 20/30, which has been maintained for a period of more than nine months. At the 12-month visit, her vision was noted to be diminished to 20/200 due to a retroprosthetic membrane and improved to 20/25 two weeks after a Yag capsulotomy. Histopathologic examination of the corneal specimen disclosed predominantly subepithelial amyloid deposition. Genetic analysis is presented. CONCLUSIONS: GDLD is a rare disorder of primary corneal amyloidosis. Recurrence of this condition following surgery is very common. Boston type I keratoprosthesis as a secondary procedure can be successful in restoring vision in affected patients.

[GDx-VCC NFI analysis in Lebanese glaucoma patients]. / Analyse de l'indice NFI du GDx VCC dans le glaucome au Liban.

PURPOSE: To describe the Nerve Fiber Indicator (Nerve Fiber Index; NFI) parameter findings and progression in Lebanese glaucoma patients or suspects. STUDY DESIGN: Retrospective, observational study. PATIENTS AND METHODS: A review was conducted of the GDx VCC examinations performed between January 2003 and December 2008 in an ophthalmological diagnostic center, in 1063 Lebanese subjects referred for this testing by their ophthalmologists. This group of subjects included confirmed glaucoma patients and glaucoma suspects. GDx VCC examination was repeated one or more times, at a one-year interval, in only 136 patients. Since we only had access to their GDx VCC examinations, the number of subjects in each category was unknown prior to the exam. After GDx VCC examination, we divided the subjects into three categories according to their NFI score. Subjects were considered to be normal when the NFI score was below 30, glaucoma suspects when the score was between 30 and 40, and confirmed glaucoma patients when the score was above 40. RESULTS: Among the 1063 patients studied, 525 were female and 538 male, with a mean age of 56.8 ± 14.2 years at the time of examination. The mean NFI score was 27.01 ± 14.23, higher in males than females in the various age groups, and higher in older than in younger subjects, the greatest difference being between the 11- to 20-year and the 81- to 90-year age groups (P=0.015). Eight hundred and eighty patients had an NFI score within normal limits, the score was consistent with glaucoma suspect in 280 patients and with confirmed glaucoma in 103 patients. Among the 136 patients who underwent multiple GDx VCC examinations, 69 were initially classified as normal. Upon repeat GDx VCC examinations over a five year period, 24 of these 69 patients (34.78%) presented with scores consistent with glaucoma suspect or confirmed glaucoma. CONCLUSION: This study demonstrates that glaucoma was detected or suspected in at least one of three subjects referred for GDx VCC examination (383/1063). NFI scores were higher in males than females and in older compared to younger subjects.

Genetics of autoimmune thyroid disease in the Lebanese population.

This study aims to investigate the association of human leukocyte antigen (HLA) class II genes and cytotoxic T lymphocyte-associated antigen-4 (CTLA-4) with autoimmune thyroid diseases in the Lebanese population. A total of 128 patients with autoimmune thyroid disease (55 with Graves' disease (GD) and 73 with Hashimoto's thyroiditis (HT)) were typed for HLA DQA1 (0301 and 0501) and DQB1 (0201, 0302, and 0303) and for 49A/G CTLA-4 using PCR-based sequence-specific priming methods. A total of 186 matched controls were typed for the same alleles and compared to the diseased population. Results showed no significant differences in HLA DQB1*0201 or DQB1*0301 allelic frequencies or CTLA-4 polymorphisms between patients and controls. For GD, there was a weak association with HLA DQB1*0302 [34.6% (19 of 55) vs. 21.5% (40 of 186), P = 0.048, odds ratio (OR) = 1.926, confidence interval (CI) = 0.999-3.715] and HLA DQB1*0302-DQA1*0501 haplotype [56.36% (31 of 55) vs. 40.8% (76 of 186), P = 0.042, OR = 1.870, CI = 1.018-3.433]. For HT, the frequencies of DQB1*0302-DQA1*0501 haplotype [28.8% (21of 73) vs. 14.5% (27 of 186), P = 0.008, OR = 2.378, CI = 1.241-4.558] and DQB1*0302-DQA1*0301 haplotype [60.2% (44 of 73) vs. 38.7% (72 of 186), P = 0.002, OR = 2.402, CI = 1.381-4.180] were significantly higher in patients. On the other hand, weak association was found between HT and DQA1*0301 allele [32.9% (24 of 73) vs. 20.9% (39 of 186), P = 0.044, OR = 1.846, CI = 1.011-3.373]. Findings show that DQB1*0302-DQA1*0501 and DQB1*0302-DQA1*0301 haplotypes may play a role in the pathogenesis of HT in the Lebanese population. For the 49A/G CTLA-4 polymorphism, no significant difference was found between patients and controls.

[Keratoconus screening in a Lebanese students' population]. / Dépistage du kératocône dans une population universitaire au Liban.

OBJECTIVE: Screening for keratoconus and potential risk factors in a medical student population in Lebanon using anterior topography. POPULATION AND METHODS: We randomly selected 110 medical students doing rotations in the Hôtel-Dieu de France hospital in Lebanon between November 2009 and February 2010. Ninety-two students agreed to participate in the screening protocol, which included a questionnaire and an anterior topography. The topography was performed using the placido disk Tomey(®) Topographic Modeling System TMS-4. The results were interpreted using the analysis programs supplied with the machine and by an experienced ophthalmologist. RESULTS: Ninety-two students participated in the study (83.6%): 49 males (53.2%) and 43 females (46.8%). The mean age was 23.6±1 year. Fifty-eight students were ametropic (63.1%): 44 participants were myopic (47.8%), 40 had astigmatism (43.4%), and six were hyperopic (6.5%). Three students (six eyes) were diagnosed with keratoconus. The prevalence of keratoconus was 3.3%, of which 2.2% were already known cases. The prevalence of forme frustre keratoconus was 1.1%. Eleven students (12.1%) had a family history of keratoconus, only one was diagnosed with the disease. Atopy and eye rubbing were not found significantly related to keratoconus in this population. CONCLUSION: Keratoconus is a prevalent disease among our population of Lebanese medical students, which confirms the clinical impression that keratoconus is relatively frequent in Lebanon. Further studies need to be conducted on a larger and more representative sample to assess keratoconus prevalence and risk factors in Lebanon.