Duane’s retraction syndrome,a case series from Iran

PURPOSE: To determine the relative prevalence of Duane's retraction syndrome (DRS) in a population of Iranian strabismus cases and to describe the clinical features. METHODS: Retrospectively, a population of 7,349 strabismus cases visited during 2000-2003 were evaluated for the diagnosis of DRS. Data regarding onset (noticed age), type, head turn, primary position deviation, narrowing of the palpebral fissure, leash phenomenon, refractive error, amblyopia, and history of surgery were collected. Chi-square test and Student's t-test were used. RESULTS: About 125 DRS cases were diagnosed (prevalence: 1.7%). The noticed age of the syndrome was at birth in 35.6%, during infancy in 25.4%, or childhood in 39.0%; the age at referral ranged from 3.5 months to 65.0 (median: 10.0) years. The female/male and left/right eye involvement ratios were 3:2 and 3.5:1, respectively. The syndrome was of type I in 87.0%, II in 6.5%, and III in 5.7%; 7.2% were bilateral. Horizontal deviations existed in 76.0% and vertical deviations in 12.8%. 26.8% had different degrees of amblyopia. Leash phenomenon was detected in 37.6% of cases. Head turn, primary position deviation (without turn), and orthophoria were observed in 71.2%, 13.6%, and 15.2%. Surgery had been performed in 71.2% of the cases. Vertical deviations and leash phenomenon were more common in types II and III and hypermetropia in type I (P values: 0.036, <0.001, and 0.025, respectively). CONCLUSION: Basic features of our series seem to be comparable with previous reports. The incidences of bilateral involvement and type III syndrome were lower. The proportion of cases with head turn was higher and surgery was performed more frequently.     

Bilateral retinal detachment in Hallermann-Streiff-François syndrome: a case report

We report the case of a patient with Hallermann-Streiff-Fran?ois syndrome, with typical presentation of white bilateral cataract. The surgical treatment showed morphological retinal abnormalities, which resulted in an exudative retinal detachment. This case underscores the importance of paying special attention during surgical treatment of cataract in this type of patient.     

Posner-Schlossman syndrome induced laser in situ keratomileusis keratectasia–a case report

Letter to the editor     

Bilateral optic neuropathy in a case of primary Gougerot Sjögren's syndrome

INTRODUCTION: Primary Sj?gren's syndrome is frequently characterized by a sicca syndrome without associated connective tissue disease. Association with an optic neuropathy is uncommon. CASE REPORT: We report a case of optic neuropathy in a 59-year-old woman known to have primary Sj?gren's syndrome confirmed clinically and histologically 2 years ago. She suddenly presented an initial bilateral visual loss. The ophthalmological exam noted a visual acuity of 1/10 in the right eye and limited to light perception in the left eye, with bilateral optic ischemic neuropathy more developed in the left eye. Fluorescein angiography showed, signs of ischemic neuropathy. The diagnosis of Horton disease was suspected, but subnormal blood velocity and a negative biopsy of the temporal artery confirmed the diagnosis of optic neuropathy associated with primary Sj?gren's syndrome. General steroid therapy improved optic neuropathy in the right eye but was ineffective in the left eye. CONCLUSION: Optic neuropathy associated with Sj?gren's syndrome is rare but must be considered the most common ophthalmological manifestation of the disease. Visual prognosis depends on the rapidity of diagnosis and therapy.     

Baerveldt glaucoma implant with Supramid© ripcord stent in neovascular glaucoma: a case series

AIM: To report the outcome of Baerveldt glaucoma implant (BGI) with Supramid© ripcord use in neovascular glaucoma (NVG). METHODS: We retrospectively evaluated the surgical outcome of the BGI with Supramid© 3/0 ripcord stent in patients with NVG. No tube ligation or venting slits were performed. Supramid was removed after 3mo if the target intraocular pressure (IOP) was not achieved. Surgical success was defined as IOP≤21 mm Hg with (qualified success) or without IOP-lowering medications (complete success). RESULTS: Twenty-six eyes from 24 patients were included in the study. The median duration of follow-up was 4 [interquartile range (IQR)=1-5]y, ranging from 0.5 to 5y. IOP decreased by a mean of 24.2 mm Hg (59.7%); from a mean of 40.5±12.6 mm Hg at baseline to 16.3±11.9 mm Hg, P≤0.001. The number of glaucoma medications reduced from a median of 5 (IQR=5-6) to 1 (IQR=0-2, P≤0.001) at the final follow-up. Overall success rates were 88.0% at 1y, 34.8% at 3y, 66.7% at 4y, and 50% at 5y. Hypertensive phase (HP) in the first 3mo occurred in 15/26 eyes (57.7%) with a mean IOP of 31.1 mm Hg. CONCLUSION: BGI with Supramid© ripcord stent gives close to 90% of the overall survival rate at the final follow-up without significant early hypotony. However, early HP is still a challenge.     

Coats’ disease and bilateral cataract in a child with Turner syndrome: a case report

Purpose To report the first case in which Coats’ disease was observed with infantile cataract in a girl with Turner syndrome (TS). Materials and methods We examined a 4-year-old female infant with TS who was referred with a diagnosis of leukocoria in the left eye. Results and discussion Examination under anaesthesia revealed a bilateral punctate cataract and left eye fundus showed vascular retinal abnormalities typical of Coats’ disease. Cryotherapy was performed on the telangiectatic vessels and the child was followed up for a period of 12 months. Despite cryotherapy resulting in regression of the peripheral exudates, an exudative maculopathy persisted with poor visual outcome. We suggest that Coats’ disease should be considered as a rare ocular manifestation in TS.     

Ranibizumab as adjuvant in the treatment of rubeosis iridis and neovascular glaucoma—results from a prospective interventional case series

Background

To evaluate the capability of adjuvant intraocular ranibizumab (Lucentis®) injections in the treatment of rubeosis and intraocular pressure in patients with rubeosis and neovascular glaucoma.

Methods

Ten eyes with rubeosis (R) and ten eyes with neovascular glaucoma (NVG) received Lucentis® injections (ranibizumab 0.5 mg/0.05 ml) in this prospective, monocenter, 12-months, interventional case series. The primary efficacy outcome measure was the change of degree of iris rubeosis as documented by iris fluorescein angiography measured after 12 months. Secondary outcomes were intraocular pressure (IOP), best-corrected visual acuity (BCVA, logMAR), numbers of additional interventions or antiglaucoma medications administered after injection, the gonioscopic status of the anterior chamber angle, and central retinal thickness.

Results

In the R group, 3.6 injections and in the NVG group 2.3 injections of Lucentis® were administered. Additional treatments were photocoagulation (n?=?19), cyclodestructive procedures (n?=?9), cryopexy (n?=?3), and vitrectomy (n?=?1). The mean stage of rubeosis was 3.4?±?0.7 in the R group and 3.6?±?0.8 in the NVG group at baseline. At month 12, the rubeosis was almost resolved in the R group (0.1?±?0.3, p?<?0.001), and decreased significantly in the NVG group (0.7?±?1.1, p?<?0.001). In the NVG subgroup, mean IOP was 41.4?±?13.4 mmHg at baseline, which decreased rapidly (18.2?±?12.3, day-14, p?=?0.005) and stabilized during the follow-up (15.6?±?2.0 mmHg, p?<?0.05). BCVA improved significantly in both groups (p?<?0.05, at month 12).

Conclusions

Injection of 0.5 mg ranibizumab appears to be beneficial as an adjuvant treatment in neovascular glaucoma and rubeosis due to its anti-angiogenic properties and its ability to prevent establishment or progression of anterior chamber angle obstruction. Conventional therapeutic procedures addressing the retinal ischemia are still required in a stage-wise treatment approach.     

Corneal changes in ectrodactyly–ectodermal dysplasia–cleft lip and palate syndrome: case series and literature review

The aim of this study is to describe the corneal changes in three unrelated patients with ectrodactyly-ectodermal dysplasia-cleft lip and palate (EEC) syndrome and review the literature on the possible etiology and clinical presentation of similar cases. Case 1 is an 18-year-old female with cleft lip and palate, syndactyly, and bilateral corneal pannus superiorly and inferiorly. She was initially diagnosed and treated as herpes simplex virus keratitis. Case 2 is a 3-year-old female born with cleft lip and palate, absent radial digits in both hands, and bilateral lacrimal stenosis. She developed progressive stromal scarring and neovascularization in both eyes. Her cornea perforated after developing infectious ulceration. Case 3 is a 49-year-old male with cleft palate, claw-hand deformities, absent meibomian glands and lacrimal duct, right ankyloblepharon, and a superior wedge-shaped opacity in the left cornea. The clinical findings demonstrated the different spectrum of keratopathy seen in patients with EEC. All patients were treated medically and without any surgical intervention. Limbal stem cell deficiency (LSCD) is presumed to be the cause in all three cases. Corneal changes in EEC can have variable presentation. LSCD seems to be the etiology of such keratopathy. Recurrent infection from lacrimal drainage obstruction and tear film instability are other risk factors for disease severity and progression.     

A case of ectopic cilia in nail–patella syndrome

Both ectopic cilia and nail–patella syndrome (NPS) are rare entities. To our knowledge we report the first case of the two anomalies coexisting in one patient. We present the case of a 2-year-old girl, with no other ophthalmic complication of NPS, who had an excellent cosmetic outcome and no lesion recurrence following surgical excision of ectopic cilia.     

Occurrence of motility disorders in patients undergoing tube surgery—consecutive,retrospective follow-up case series analysis

Graefe's Archive for Clinical and Experimental Ophthalmology - The two glaucoma drainage devices (GDD) Ahmed and Baerveldt tubes are most commonly used for the treatment of refractory glaucoma....