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To study the association between congenital aniridia and dry eyes, and the status of limbal stem cells.Single-center, retrospective, noninterventional, comparative case series.Twenty patients (36 eyes) diagnosed with congenital aniridia.Retrospective review of medical records at the King Khaled Eye Specialist Hospital in Riyadh, Saudi Arabia to identify all cases of congenital aniridia. Selected patients were requested to undergo further testing, especially for evaluation of dry eyes. Full ocular examinations were performed, including examination of the meibomian gland orifices and dry eye evaluation (by predetermined criteria of tear breakup time, tear meniscus level, presence of mucoid secretions, Schirmer's test with and without anesthesia, and fluorescein and rose Bengal staining patterns of the cornea and conjunctiva). Cytological evaluation was performed in 23 aniridic eyes by impression cytology to determine limbal stem cell status relative to control normal eyes.Abnormal tear film production and stability, meibomian gland dysfunction, severity of corneal involvement, and limbal stem cell deficiency.Twenty patients (36 eyes) diagnosed with congenital aniridia were classified into 3 groups according to the severity of corneal involvement: mild (2 eyes), moderate (21 eyes), and advanced (13 eyes) corneal changes. Tear breakup time was reduced by an average of 5 seconds in 90.7% of cases, tear meniscus levels were reduced to0.5 mm in 88.6% of cases, and stenosed meibomian orifices were found in 77.8% of patients. Dry eyes (94%) correlated with the severity of corneal involvement (P = 0.001). Impression cytology revealed metaplasia in all tested cases. Furthermore, 8 of 23 eyes (35%) and 15 of 23 eyes (65%) had early and advanced limbal stem cell deficiency, respectively.Abnormal tear film stability and meibomian gland dysfunction are newly identified factors in patients with aniridia. Both correlate to the severity of ocular surface disease. Impression cytology is informative in diagnosing various degrees of limbal stem cell deficiency, which is present in all tested aniridia eyes.

Objective To describe three cases of squamous cell carcinoma of the conjunctiva presenting with corneal and scleral inflammation, thinning, and perforation without any tumor formation. Design Three case reports. Participants Three male subjects aged 76, 66, and 61 years. Intervention The subjects had symptoms of external ocular inflammation with focal corneal or scleral thinning and inflammation, as well as interstitial keratitis in two cases. Initial diagnosis of sclerokeratitis with limbal thinning was made in all three in addition to a pterygium in case 2. Main outcome measures Diagnosis of the carcinoma was delayed for 2 to 24 months in two cases, whereas case 2 was diagnosed on excision of a pterygium from the involved area. Results All subjects developed intraocular extension after further scleral thinning and perforation without tumefaction. Conclusions Squamous cell carcinoma of the conjunctiva may be seen without a distinct mass and can masquerade as sclerokeratitis, scleromalacia, or interstitial keratitis. It seems that diffuse growth with inflammation leads to thinning, necrosis, and perforation of the ocular wall with resultant intraocular spread. Squamous cell carcinoma should be considered in the differential diagnosis of corneal and scleral thinning, perforation, and inflammation of unknown cause, especially in the older male subject.

The purpose of the study is to determine the outcome of penetrating keratoplasty in congenital hereditary endothelial dystrophy.Records of 40 patients (13 males, 27 females) who underwent penetrating keratoplasty (56 eyes) were reviewed. The mean age at surgery was 11.8 years (range, 2 months-35 years). The mean follow-up was 37 months (range, 6-136 months).In 35 (62.5%) of 56 eyes that underwent primary penetrating keratoplasty, the grafts survived. Graft survival analysis showed the probability of obtaining a clear graft is 92% at 1 year, 72% at 2 years, and 56.5% at 5 years. Graft survival was statistically better in eyes where onset of the disease is delayed (P = 0.02), if the graft donor age is between 5 and 30 years versus older than 30 years (P = 0.02), and for patients who kept follow-up appointments versus those who were delinquent (P0.03). Visual acuity was 20/40 in 1.9%, 20/50 to 20/80 in 18.9%, 20/100 to 20/300 in 49%, and less than 20/400 in 30.2%. The main causes of graft failure were graft rejection (six eyes) and bacterial keratitis (four eyes).Penetrating keratoplasty in congenital hereditary endothelial dystrophy is moderately successful, and graft survival is better in cases of delayed onset compared with that of congenital onset. Early surgical intervention is recommended to prevent development or progression of amblyopia.

Climatic droplet keratopathy (CDK) is a degenerative disease of the cornea, highly prevalent in Saudi Arabia. It is characterized by aggregates of small, golden-yellow globules that accumulate in the subepithelial layers, Bowman's layer, and the superficial stroma of the cornea. The authors report on 22 patients (40 eyes) with CDK and lattice lines in the cornea. The condition occurred late in life (mean age, 67 years). It was bilateral in 82% of patients, with no family history of lattice dystrophy or systemic amyloidosis. These lines were found in all layers of the stroma. Results of histopathologic and electron microscopic examination of the lattice lines confirmed the presence of amyloid. Results of immunohistochemical examination showed evidence of deposition of AP protein. The cause of these deposits in the cornea is not known, but the authors speculate that environmental factors causing CDK also may play a role in the deposition of protein AP in the cornea. The contribution of trachoma to the degenerative changes in the cornea and secondary amyloidosis could not be ruled out, as all patients had inactive trachoma. Climatic droplet keratopathy with corneal amyloidosis, therefore, represents a form of "lattice degeneration" of the cornea.

Objective This study aimed to determine whether heparin surface-modified (HSM) intraocular lenses (IOLs) with a hydrophilic surface would reduce cell adherence and other postoperative changes compared with the conventional polymethylmethacrylate (PMMA) IOLs in patients with either diabetes mellitus or inactive uveitis. Design The study design was a randomized, double-masked, clinical trial. Participants Twenty-five patients with bilateral cataracts, 14 with inactive anterior uveitis and 11 with diabetes, with an age range of 11 to 81 years (mean, 52.8 years) participated. Intervention Bilateral cataract extraction with posterior chamber IOL implantation was measured, each patient receiving an HSM lens in one eye and a PMMA lens in the other. Pharmacia one-piece HSM and PMMA IOLs were used. Postoperative ocular changes were evaluated at regular intervals for 24 months in patients with inactive uveitis and for 6 months in patients with diabetes. Patients and physicians alike were unaware of which eye contained which lens until postoperative results were compiled. Records were kept by a study coordinator. Main outcome measures Comparisons of posterior synechiae, IOL cellular deposits, and posterior capsular fibrosis between PMMA and HSM IOLs were measured. Results Using the chi-square test, no statistically significant difference was found between the HSM and PMMA IOLs in the number of cellular deposits found on the anterior IOL surface, the number of adhesions between the iris and IOL, or the incidence of capsular opacification. Conclusion The HSM and PMMA IOLs showed similar postoperative results in patients with inactive uveitis or diabetes mellitus.

To determine the prognosis of penetrating keratoplasty (PK) for macular corneal dystrophy (MCD).Single-center, retrospective, interventional, noncomparative case series.One hundred forty-one patients (229 eyes) with MCD.Retrospective review of the medical record of every patient who underwent primary PK for histopathologically confirmed MCD at the King Khaled Eye Specialist Hospital between January 1, 1983 and December 31, 2002 and for whom at least 12 months of follow-up is available.Visual acuity (VA), graft survival, and postoperative complications.After a mean follow-up period of 5.9+/-3.8 years (range, 1-17), the mean best-corrected VA was 20/50. At the most recent visit, 206 (90.0%) grafts were clear, and 23 (10.0%) had failed. Probabilities of graft survival were 98.1% at 1 year, 89.8% at 5 years, 82.1% at 10 years, and 74.1% at 15 years. There was a statistically significant increased likelihood of graft failure if the patient was older than 40 years at the time of surgery (P0.00003). The differences in graft failure between patients older than 40 and those younger were not attributable to statistically significant differences in duration of follow-up, donor age, or donor endothelial cell counts. Corneal endothelial rejection episodes occurred in 47 (20.0%) grafts, but resulted in irreversible graft failure in only 8 (3.5%) eyes. Eighteen (27.3%) of 66 eyes with a recipient size ofor =7.5 mm developed a graft rejection episode, compared with 27 (16.6%) of 163 eyes with a recipient size of7.5 mm (P = 0.04). Microbial keratitis occurred in 14 (6.1%) grafts and was more likely to occur in patients over 40 (14.0% vs. 3.0%, P = 0.01). Clinically significant recurrence was observed in 12 (5.2%) grafts, after a mean interval of 84+/-48.2 months, and was directly related to patient age (P = 0.04) and inversely related to donor graft size (P = 0.04).Good visual results and excellent graft survival can be achieved after PK for MCD. The risk of graft failure is higher in patients older than 40 years.

Objective To report ocular complications of Rift Valley fever (RVF) during its first reported outbreak in southwest Saudi Arabia in autumn 2000. Design Cross-sectional study of patients in a referral hospital. Participants One hundred forty-three consecutive patients with confirmed RVF serologic test results and ocular lesions were enrolled in the study. Methods Hospitalized patients (n = 30) and outpatients (n = 113) with clinical symptoms consistent with RVF, positive RVF serologic test results, and ocular abnormalities were studied. Ophthalmologic examinations, including fundus photography and fluorescein angiography, were performed. Patients were followed up at regular intervals to determine the prognosis and outcome of identified ocular abnormalities. Main outcome measures Visual acuity at initial presentation and course of anterior and posterior segment complications. Results Among 143 patients (78% males; mean age, 53.2 years), 212 eyes were affected, comprising 47 eyes in 30 inpatients and 165 eyes in 113 outpatients. The mean interval between the onset of RVF and visual symptoms ranged from 4 to 15 days (mean, 8.8 days). Macular or paramacular retinitis was identified in all the affected eyes (n = 212) at the time of initial assessment. Lesions included retinal hemorrhages (40%), vitreous reactions (26%), optic disc edema (15%), and retinal vasculitis (7%). Anterior uveitis was present in 31% of outpatients. Fluorescein angiography of the retinitis showed early hypofluorescence with late staining of retinal lesions and blood vessels. Initial visual acuity was less than 20/200 in 80% of eyes in the outpatient group; their vision improved, deteriorated, or remained the same in 13%, 15%, or 72%, respectively. Evaluation at the last follow-up showed macular (60%) or paramacular (9%) scarring, vascular occlusion (23%), and optic atrophy (20%) in the outpatient group. Conclusions Rift Valley fever was associated with major ocular morbidity. Ocular manifestations of RVF occurred with a relatively higher frequency than reported up to now and were not limited to severe infections. Rift Valley fever affects the uvea and posterior chorioretinal area and is associated with permanent visual loss resulting from macular and paramacular scarring, vascular occlusion, and optic atrophy. The study demonstrated for the first time that transient nongranulomatous anterior uveitis is associated with RVF.

Background: Eyelids of patients with trachoma may be thickened. This thickening could be attributed to trachomatous changes in the conjunctiva and tarsus. Methods: Biopsies of tarsal plates and palpebral conjunctivae were obtained from 17 upper eyelids of 11 patients with inactive trachoma who underwent posterior tarsotomy procedures for entropion repair. Results: Light microscopy studies showed a thick and compact subepithelial fibrous membrane adherent to the tarsal plate. This membrane caused apparent thickening of the tarsus when measured intraoperatively (range, 1.25–2.00 mm). Other histopathologic findings include atrophy of the meibomian glands with thickening of the acinar basement membrane, loss of goblet cells, retention cysts, and hyaline degeneration of the tarsal plate with focal replacement by adipose tissue. Conclusion: The contraction of the subepithelial fibrous membrane formed by vertically oriented parallel collagen fibers is one of the main factors contributing to the entropion formation.

Acute angle-closure glaucoma can be triggered by a mature or intumescent cataract, and occurs in eyes with open angles, as well as in those with narrow, occludable angles. This is encountered more in developing countries, where patients present late. They tend to wait until the cataract becomes mature, because it is common belief among these patients that cataracts should not be operated on until vision drops to the level of hand movements or light perception. The authors reviewed the charts of 10 patients who were treated for phacomorphic glaucoma, by first undergoing neodymium: YAG (Nd:YAG) laser iridotomy. In all patients, the acute angle-closure glaucoma attack could be reversed or prevented by the iridotomy, before subsequent cataract extraction. The value of Nd:YAG laser iridotomy in the initial management of phacomorphic glaucoma is discussed.

The type of corneal ectasia, the presence of breaks in Descemet's membrane, and the success with contact lens wear or penetrating keratoplasty were studied in 61 patients with corneal ectasia and vernal keratoconjunctivitis. There were 53 patients with keratoconus, 5 with pellucid marginal corneal degeneration, 2 with keratoglobus, and 1 with superior corneal thinning. The high rate of hydrops and the corneal ectasia itself may be related to excessive eye rubbing. Success with contact lens wear or penetrating keratoplasty in vernal keratoconjunctivitis patients with keratoconus is less than in patients with keratoconus alone.