Journal of Current Ophthalmology
Volume:21 Issue: 1, Mar 2009

Behçet‘s syndrome, a major cause of posterior uveitis and visual loss in the Third World, has probably existed for more than 4000 years. It is a multisystem, inflammatory disorder whose principal manifestations are oral and genital aphthosis as well as inflammation of the eye, skin, and joints. The principal causes of death are from vascular and neurological involvement. No etiological agent has yet been identified for the disease; current evidence suggests that the normal flora of mucosal tracts induce immunological hyper-reactivity in genetically predisposed individuals. The visual prognosis in patients with Behçet‘s disease is poor, the principal cause of visual loss being consecutive inflammatory ischemic retinal vessel occlusions and macular edema, scar and optic atrophy. Treatment is directed at suppressing the inflammatory response using corticosteroids and a variety of second line immunosuppressive agents. Despite different treatment regimens used in different countries the visual prognosis is much the same, with a hard core of 15–20% of patients seemingly resistant to therapy and progressing relentlessly to blindness.1 In this issue of the Iranian Journal of Ophthalmology Davatchi and coworkers have presented an important review on "Longitudinal Study of Chlorambucil in Ocular Manifestations of Behçet’s Disease". The visual acuity of their 89 patients was improved in 68.4% of the eyes and the mean visual acuity was increased from 2.6 to 3.3 of Snellen chart after mean duration of treatment of 26.2±2.6 months by Chlorambucil. In the past mamo JG2 has reported that untreated ocular Behçet''s disease ends up in blindness in few years of time. Mamo and Azzam3 were the pioneers to treat the ocular Behçet''s disease by immunosuppressors and particularly by Chlorambucil and they reported successful results. Ever since, several immunosuppressors and new generations of drugs have been applied in treating this blinding disease and in many cases positive results have been reported. Despite all the efforts and progresses in treating ocular Behçet‘s disease in a recent international report by Kitaichi et al.4 The visual outcome of ocular Behçet‘s disease has been reported to be unfavorable. This report has been collected from 25 Behçet‘s disease centers including 1465 ocular Behçet‘s disease patients and the final visual acuity of 23% of the cases has been reported to be less than 0.1 despite the extensive treatments. Looking more carefully at the registry of Shariati hospital of Tehran (Behçet’s unit) in a survey of August 2008 among 6021 Behçet‘s disease patients only 4.3% of the eyes were legally blind unilaterally (vision=0.1 or less) and 3.8% were blind bilaterally and after 10.9±7.8 years of evolution of Behçet‘s disease which make us to postulate that the response of the Behçet‘s disease patients to the treatments is different at different regions of the world or the severity of the disease is variable at different regions and in different areas. In this present report of Davatchi et al although the visual acuity worsened in 31.6% of the eyes and among them 7.1% became blind (No-light-perception) the authors claim that the complications of the disease such as cataract, optic atrophy, macular scar, macular edema etc are responsible for this regression of visual acuity but the total adjusted disease activity index improved from 21.5 to 16.2 (P=0.0002) and 75.3% of patients had improvement of ocular inflammations. It is of interest to emphasize that 7.8% of the eyes in this presentation have had no-light-perception and they have gained some useful vision which could be explained by the very severe disc and optic edema and prevention of passage of axonic flow. Thanks to the effort and long time investigation of our colleagues nowadays the satisfactory results of cytotoxic drugs such as Chlorambucil have been proven and many new horizons are being opened in the treatment of ocular Behçet such as TNF, Interferon, Infliximab and Rituximab. Despite many advances in the understanding of the immunoregulatory mechanisms of uveitis, the ideal immunosuppressant has yet to be found. The three main classes of immunosuppressives that are widely used today in addition to glucocorticosteroids are antimetabolites, T cell inhibitors and alkylating agents. Antimetabolites include azathioprine, methotrexate and mycophenolate mofetil (MMF). T cell inhibitors include cyclosporine and tacrolimus. Alkylating agents include cyclophosphamide and chlorambucil. Cyclosporine and azathioprine have been found to be effective in the treatment of Behçet’s disease in randomized controlled trials, whereas the efficacy of other agents is shown by uncontrolled case series.5 References 1- Stanford MR. Behçet''s syndrome. Br J Ophthalmol 2003;87(4):381-2. 2- Mamo JG. The rate of visual loss in Behçet’s disease. Arch Ophthalmol 1970;84:451-2. 3- Mamo JG, Azzam SA. Treatment of Behçet’s disease with chlorambucil. Arch Ophthalmol 1970;84:446-50. 4- Kitaichi N, Miyazaki A, Stanford MR, et al. Ocular feature of Behçet’s disease: an international collaborative study. Br J Ophthalmol 2007;91:1579-82. 5- Jap A, Chee SP. Immunosuppressive therapy for ocular diseases. Curr Opin Ophthalmol 2008;19(6):535-40.

Cytotoxic drugs are the gold standard and the first line treatment for ophthalmological manifestations of Behçet’s Disease (BD). The purpose of this study was to evaluate the outcome of eyes with chlorambucil on long-term follow-up, in a longitudinal study on 15 years. Chlorambucil was given as 0.2 to 0.3 mg per kilogram of body weight and per day. Prednisolone was concomitantly given as 0.5 mg per kilogram of body weight. Prednisolone was tapered later according to the improvement of inflammation. In refractory cases, the patient was switched to another cytotoxic drug. Visual acuity (VA) was calculated on a Snellen chart (on a scale of 10). A Disease Activity Index (DAI) was calculated for anterior uveitis (AU), posterior uveitis (PU), and retinal vasculitis (RV) according to Ben Ezra. The Total Inflammatory Activity Index (TIAI) and the Total Adjusted Disease Activity Index (TADAI) were also calculated. Results, before the treatment and after different intervals (from 3 months to 15 years), were compared together by the student paired t test. Confidence interval (CI) at 95% was calculated for percentages. Eighty nine patients received chlorambucil. The mean duration of treatment was 26.2 months ±2.6. Good results (stabilized or improved eyes from the baseline to last evaluation) were: VA 68.4%, PU 87.6%, RV 75.8%, TIAI 85.4%, TADAI 76.4%. The mean VA improved from 2.6 to 3.3, mean PU improved from 1.8 to 0.7, mean RV improved from 2.1 to 1.5, mean TIAI improved from 13.9 to 7.6, and mean TADAI improved from 21.5 to 16.2. The difference was statistically significant for all parameters. Longitudinal study showed maintenance of good results all over the study time. Chlorambucil with prednisolone was effective in ocular lesions of BD. Good results were maintained on long-term follow-up.

To review the visual outcome and complications of deep anterior lamellar keratoplasty (DALK) using the big-bubble technique in patients with corneal ectasia Eighteen consecutive patients with keratoconus or post-LASIK ectasia with poor spectacle-corrected visual acuity and/or contact lens intolerance were enrolled in this study between October 2005 and September 2006. DALK big-bubble technique was carried out in 17 eyes. In one eye, intraoperative conversion to penetrating keratoplasty (PKP) followed large Descemet''s membrane perforation. Visual and refractive outcomes and intraoperative and postoperative complications were assessed. Seventeen procedures (94%) were completed as DALK. The mean follow-up time was 9.5 months. At the last follow-up visit, mean best corrected visual acuity (BCVA) was 20/32 (0.20±0.14 LogMAR) and BCVA of 20/40 or better was achieved in 82% of the eyes. The mean postoperative topographic cylinder was 4.4±1.6 diopter (D), and the mean spherical equivalent was -3.3±6 D. Main postoperative complications included graft rejection (2/17, 12%), graft infection (1/17, 6%) and double anterior chamber (2/17, 12%). DALK is a valuable and successful alternative to PKP in patients with keratoconus. Graft rejection is a rare complication but is associated with good recovery because the endothelium is not involved.

To assess and to compare stromal and endothelial changes at the cellular level in patients who had photorefractive keratectomy (PRK) and laser in situ keratomileusis (LASIK) using in vivo confocal microscopy In this semiexperimental study, 32 eyes of 16 patients (4 males and 12 females) with low to moderate myopia [-1.00 to -4.50 diopters (D)] or low to moderate myopic astigmatism (a total below 4.50 D and a max astigmatism of 1.50 D) were investigated. All participants had the history of PRK or LASIK surgery and were examined with Confoscan 3 confocal microscope (Nidek, Japan) before and 6 months after surgery. The densities of the keratocytes in the anterior and posterior stroma and the endothelial cell count (ECC) were measured. Finally the collected data was analyzed by SPSS16 and T-test. The mean ablation depth in LASIK and PRK groups were 61.00±17.17 and 62.13±15.4 μm, respectively. Six months after surgery, the mean cell density change in anterior and posterior stroma in LASIK group was 367.12±103.35 cells/mm2 (34.7% reduction) and 9.25±28.28 cells/mm2 (1.31% reduction) respectively. In the PRK group, these values were respectively 319.71±83.45 cells/mm2 (31.13% reduction) and 0.17±38.97 cells/mm2 (0.02% reduction). The ECC increased by 0.27% and 1.39% in LASIK and PRK groups, respectively. At retroablation zone in LASIK group, the mean number of keratocytes decreased by 37.2%. By Confoscan application, after both PRK and LASIK, a decrease in the number of stromal keratocytes which was more significant in the anterior stroma was found. On the other hand, there was a slight increase in the ECC compared to preoperative counts which may be attributed to counting errors. There was not any significant difference in stromal cell densities and the endothelial cell counts between LASIK and PRK groups.

To compare laser interferometry (LI) and ultrasound A-scan in measurement of axial length (AL) in eyes with asteroid hyalosis Twenty three patients with unilateral moderate to severe asteroid hyalosis determined by B-scan ultrasonography images participated in the study. The AL measurements were obtained by LI and ultrasound A-scan in both asteroid hyalosis and noninvolved eyes. The measurements were performed by the two methods as well as for affected and contralateral normal eyes and were statistically compared using the paired T-test. A P-value less than 0.05 was considered significant. The ultrasound AL measurements were taken in every case but measurement with the LI method was not possible in 4 eyes with asteroid hyalosis (17.3%). There was a high correlation between LI and ultrasound A-scan measurements in both asteroid hyalosis and noninvolved eyes (r2=0.97, P<0.001 and r2=0.95, P<0.001 respectively). There was no significant difference between AL taken by either method in asteroid hyalosis and noninvolved eyes. The failure rate of AL measurement using LI was relatively high in eyes with asteroid hyalosis. There was a high correlation between AL measured by ultrasound A-scan and LI method in both asteroid hyalosis and noninvolved eyes.

To evaluate the role of orthoptic treatments in the management of intermittent exotropia In a retrospective study, clinical records of patients diagnosed with intermittent exotropia were reviewed. Patients with basic, convergence insufficiency (CI) and divergence excess (DE) types of intermittent exotropia who underwent orthoptic treatments were enrolled. Office treatments included prism exercises and pencil push-ups, and home exercises included pencil push-ups, 3D tests and dominant eye occlusion. Clinical evaluation of symptoms, binocular orthoptic status and maximum angle of deviation was done before treatment and at 8 weeks after the beginning of the treatment and at the time of last examination. Seventy four patients with a mean age of 18.4±12.2 years and mean follow-up of 13.5±10.1 weeks were included in the study. Forty three (58.1%) patients had a basic type of exodeviation, 22 (29.7%) had a CI type of exodeviation and 9 (12%) had a DE type of exodeviation. The treatment was successful in 88.3% of patients in basic type, all patients in CI type and 88.8% in DE group. Success rate was not significantly different between the three groups (P=0.25). Strabismus surgery was performed in one patient in basic type and one in DE group due to the lack of improvement. Orthoptic treatment seems to be effective in reducing symptoms and improving signs of intermittent exotropia.

To evaluate outcome of surgically slanting extraocular muscle insertions in A and V-pattern strabismus In this prospective non-randomized interventional case series, we created a slanting surgical reinsertion line with a 3- 4 mm difference between upper and lower corners of the muscles in 16 patients with A or V strabismus (V-Exotropia in 10 patients, A-Exotropia in 1 patient, V-Esotropia in 5 patients). Cases with moderate or marked overaction of oblique muscle were excluded. All patients were followed for 6 months. V-Exotropia: the average amount of V-pattern was 29.5 ∆ before and 9.2 ∆ after surgery. Slanting surgery reduced the V-pattern in 10 of 10 patients. The mean amount of reduction was 20.3 ∆ (P<0.0001). All anatomical muscle insertions were normal. V-Esotropia: the average amount of the V-pattern was 22.4 ∆ before and 13.2 ∆ after surgery. Slanting surgery reduced V-pattern in 4 of 5 patients. The mean reduction was 9.2 ∆ in the V-pattern (P<0.067) and 28 ∆ in down gaze (P<0.003) and 22 ∆ in primary position (P<0.014). A-Exotropia: (1 patient) A-pattern was 20 ∆ before and 6 ∆ after surgery. Amount of pattern reduction was 14 ∆. Slanting muscle insertion for correction of A and V strabismus is suitable procedure for reducing A and V-patterns.

The aim of this study is to evaluate the effectiveness of intraoperative botulinum toxin-A (BTA) injection as an adjunct to the surgical treatment of large-angle esotropia or exotropia. Thirteen patients were included in this interventional case series study. Mean age of the patients was 23.31±13.29 years (range: 5-43 years). Of these 13 patients, 6 were exotropic and 7 were esotropic. The average preoperative esodeviation was 79.29±16.44 prism diopters (PD) (range: 60-100 PD) and exodeviation was 75.83±11.14 PD (range: 65-90 PD). Depending on the degree of deviation ten or twenty units of Dysport were injected intraoperatively into the recessed horizontal rectus muscles in all of the patients. The average follow-up was 7±1 months (range: 6-8 months). The average final deviation in the esotropia group was 8.57±10.69 PD of esotropia. The average final deviation in the exotropia group was 14.17±12.00 PD of exotropia. The final deviation of the 69% patients was within 15 PD of esotropia or exotropia. This study shows that the combination of BTA injection with recession may be a useful treatment option in cases of large angle horizontal deviation.

To assess the results of 1-minute and 5-minute Schirmer''s test (ST) when the eyes are open (STo) and closed (STc) in normal participants. In a comparative, observational case series study, 34 normal volunteers were included in the study. Inclusion criteria were normal ophthalmologic findings except for refractive errors, no use of contact lenses, no history of previous medical or ophthalmic problems, and no current use of medications. STo and STc in 1 and 5 minutes time were performed separately for all subjects with an interval of at least 24 hours using Whatman No. 41 (5x60 mm) with bended end of the paper inserted into the lateral side of the lower conjunctival fornix. There were 19 females and 15 males with a mean age of 20.8 years (range: 18-23). Mean value of STc was significantly less than STo in both 1 minute and 5 minute tests. One-minute STo and STc showed a significantly less wetting than 5-minute test. Normal distribution was observed for all values. A significant correlation were found between 1-minute and 5-minute tests in both STo and STc; therefore, two equations were proposed to calculate the 5-minute from 1-minute ST. Statistical analysis did not provide a reliable equation for calculating the standard ST (5-minute STo) from the most comfortable state (1-minute STc). Faster and more comfortable ST (1-minute) is a reliable test to calculate the 5-minute ST in both open and closed eye, using the provided equations. The 1-minute STc is not a reliable test to calculate the 5-minute STo.

Among the three forms of anthrax in human (cutaneous, inhalational, and gastrointestinal), cutaneous anthrax is the most common form with prevalence of 95%. The cutaneous anthrax is a rare cause of periorbital (preseptal) cellulitis that may remained without correct diagnosis and appropriate treatment. This may result in severe local complications such as blindness and airway obstruction or dissemination of the disease like as bacteremia and meningitis. We report a child with periorbital anthrax to present the clinical manifestation and progression of the disease and remind physicians the interesting contagious cause of periorbital cellulitis. Case report: A 2.5-year-old boy from a rural area of Guilan province in Iran was seen to have a small erythematous papule in his right lower eyelid 5 days prior to admission. Then, the lesion progressed to a necrotic ulcer with a black central scar and severe surrounding edema and erythema. Severe swelling of the eyelids and edema all over the face occurred as well. Fever was absent. The child had repetitious contacts with domestic herbivores. The CT scan of the orbit and paranasal sinuses showed soft tissue swelling only. Gram-positive rods were seen in the smear of exudate taken from cutaneous lesion but culture result showed negative. Treatment with intravenous ciprofloxacin, penicillin, and clindamycin resulted in clinical improvement but due to cicatricial ectropion formation, oculoplastic intervention was considered to be done. Physicians must consider a cutaneous lesion to be anthrax if any of the following exists: a history of contact with domestic herbivores and animals, similar lesions in the family members, cutaneous necrotic ulcer with the characteristic black scar and peripheral edema, and observing gram-positive rods in scraped material taken from beneath the scar edge or exudate of cutaneous lesions. Although anthrax of the eyelids is not common, it must be included in the differential diagnosis of periorbital cellulitis.

Among the three forms of anthrax in human (cutaneous, inhalational, and gastrointestinal), cutaneous anthrax is the most common form with prevalence of 95%. The cutaneous anthrax is a rare cause of periorbital (preseptal) cellulitis that may remained without correct diagnosis and appropriate treatment. This may result in severe local complications such as blindness and airway obstruction or dissemination of the disease like as bacteremia and meningitis. We report a child with periorbital anthrax to present the clinical manifestation and progression of the disease and remind physicians the interesting contagious cause of periorbital cellulitis. Case report: A 2.5-year-old boy from a rural area of Guilan province in Iran was seen to have a small erythematous papule in his right lower eyelid 5 days prior to admission. Then, the lesion progressed to a necrotic ulcer with a black central scar and severe surrounding edema and erythema. Severe swelling of the eyelids and edema all over the face occurred as well. Fever was absent. The child had repetitious contacts with domestic herbivores. The CT scan of the orbit and paranasal sinuses showed soft tissue swelling only. Gram-positive rods were seen in the smear of exudate taken from cutaneous lesion but culture result showed negative. Treatment with intravenous ciprofloxacin, penicillin, and clindamycin resulted in clinical improvement but due to cicatricial ectropion formation, oculoplastic intervention was considered to be done. Physicians must consider a cutaneous lesion to be anthrax if any of the following exists: a history of contact with domestic herbivores and animals, similar lesions in the family members, cutaneous necrotic ulcer with the characteristic black scar and peripheral edema, and observing gram-positive rods in scraped material taken from beneath the scar edge or exudate of cutaneous lesions. Although anthrax of the eyelids is not common, it must be included in the differential diagnosis of periorbital cellulitis.

the literature focused on clinical manifestations and differential diagnoses. The patient had ‘mild’ involvement of the ophthalmic apparatus: absent foveal reflex, cataract, and abnormal retinal vessels, which in the context of similar reports on ocular manifestations is an indication of wide polymorphism of the condition. Dermatologists, geneticists, and ophthalmologists should be aware of this disorder when they are consulted for the diagnosis of hamartomatous dysplasia conditions.

To report a case of purtscher retinopathy associated with valsalva retinopathy after car accident A 54-year-old man presented with a sudden visual loss in his left eye after car accident. Fundus examination of the left eye showed cotton-wool spots and retinal hemorrhages in the posterior pole associated with subhyaloid hemorrhage in macula. Fundus examination showed typical findings for both purtscher retinopathy and valsalva retinopathy. These findings suggest the purtscher retinopathy features has occurred as a result of valsalva maneuver in our case.