Large, Spontaneous Macular Hole with Posterior Pole Detachment in a Patient with Best Vitelliform Macular Dystrophy.

PURPOSE: To describe the visual and anatomical outcomes in a patient with a full-thickness macular hole and Best vitelliform macular dystrophy. METHODS: The authors present a case of a large spontaneous macular hole with associated posterior pole detachment in a patient with a history of Best vitelliform macular dystrophy including clinical course and surgical outcome. PATIENT: The patient presented with a history of blurred central vision. He was known to have Best vitelliform macular dystrophy. Examination revealed BCVA 6/36 (0.78 logMAR) and a full-thickness macular hole (1,102 µm) with a shallow posterior pole detachment extending to the vascular arcades. He underwent phacovitrectomy with silicone oil tamponade. Internal limiting membrane (ILM) peel was prohibited due to a very adherent posterior hyaloid membrane (PHM). RESULTS: The patient developed type 2 closure. He had oil removal in 14 months combined with PHM and ILM peel. Two months postoperatively, he had further reduction of the foveal defect and the retina remained flat. Final BCVA was 6/24 (0.60 logMAR). CONCLUSION: Macular holes with Best disease are rare and are thought to be due to rupture of a cyst in the vitelliform stage or atrophy in later stages. This case outlines that closure of the macular hole, flattening of the detachment, and improvement in visual acuity is possible with vitrectomy and ILM peeling.

Ocular manifestation, complications and aetiological factors in Stevens-Johnson syndrome/toxic epidermal necrolysis.

OBJECTIVE: To describe the ocular manifestations of Stevens-Johnson Syndrome/Toxic Epidermal Necrosis among inpatients at a tertiary care hospital. METHODS: The retrospective observational descriptive study was carried out at the Aga Khan University Hospital, Karachi, and comprised data on age, gender, aetiology and ocular findings related to patients diagnosed with Stevens-Johnson Syndrome/Toxic Epidermal Necrosis between January 2000 and December 2011. SPSS 19 was used for statistical analysis. RESULTS: There were 87 patients; 48(55.2%) of them being males.The overall mean age was 33.2±22.2 years, ranging from 1 month to 84 years. The most common aetiology was idiopathic 20(23%) followed by non-steroidal inflammatory drugs and anti-epileptics 11(12.6%) each. Besides, 84(96.6%) patients had oral mucosal involvement whereas 45(51.7%) had ocular and 27(31.0%) had genital-mucosal involvement. Glassroding was performed in 16(18.4%) patients due to minor conjunctival adhesions. CONCLUSION: Ocular manifestations of varying severity were frequent, with drugs being the most common aetiology.

Retinopathy of prematurity: an evaluation of existing screening criteria in Pakistan.

AIM: To evaluate if broadening the criteria for retinopathy of prematurity (ROP) screening to include babies with gestational age ≥32 weeks and/or birth weight ≥1500 g, would have an impact on the number of babies diagnosed as having ROP. METHODS: A prospective cohort study was carried out at the Aga Khan University Hospital, Karachi, Pakistan. Infants with gestational age ≤35 weeks or birth weight ≤2000 g born in this hospital from May 2010 to December 2012 were screened for the presence of ROP 4-6 weeks after birth. Subsequent examinations were performed at intervals based on the findings of initial eye examinations. Infants diagnosed as having ROP were treated with argon laser therapy. Neonatal risk factors were also assessed. Cumulative incidence of ROP was calculated for babies falling within and outside current screening criteria. Multivariate logistic regression analysis was performed to examine the predictors of ROP. RESULTS: A total of 301 infants were screened: 27 (9%) babies developed ROP, of which 19 had stage 3 ROP or worse. None of the babies falling outside the current screening criteria developed ROP. The incidence of ROP in the infants meeting the current screening criteria was 11.5%. Multivariate logistic regression analysis showed that only gestational age (adjusted relative risk 0.774, 95% CI 0.603 to 0.994) was independently associated with the development of ROP. CONCLUSIONS: In our population ROP was not seen to occur in infants older than 32 weeks gestational age and/or weighing more than 1500 g.

Long-term outcome of primary external dacryocystorhinostomy.

OBJECTIVE: To assess the long-term functional outcome of external dacryocystorhinostomy (DCR) in terms of epiphora. STUDY DESIGN: Single-group cohort study. PLACE AND DURATION OF STUDY: Section of Ophthalmology, The Aga Khan University Hospital, Karachi, from January 2000 to June 2010. METHODOLOGY: This study included adults who underwent external DCR surgery at the AKUH during January 2000 to June 2010. The main outcome measure was the proportion of patients reporting to have developed epiphora after surgery. During the telephonic interviews participants were asked if they had a recurrence (symptoms such as watering or discharge) after surgery. Individuals answering in affirmative were asked when the symptoms started. Data on age at surgery, gender and pre-operative symptoms were collected from medical records. Recurrence-free curves were calculated according to the Kaplan-Meier method. RESULTS: A total of 44 persons who underwent DCR surgery were contactable by telephone in 2011 and all agreed to participate in the study. The mean age of the patients at the time of surgery was 48.0 ± 15.8 years. Overall, 38.6% (17/44) participants reported having developed epiphora after surgery. The overall median recurrence-free time was 1.7 years; there was no statistically significant difference in the median recurrence-free time between men and women. CONCLUSION: The long-term functional outcome of external DCR surgery does not appear to be optimal and, in fact, far worse than the short-term results reported in the international literature.

A case of orbital apex syndrome in a patient with malignant otitis externa.

A rare case of skull base infection with a rapid clinical course leading to visual loss and eventually death in a poorly-controlled diabetic patient is presented. A 37-year-old woman presented with a history of visual loss and painful protrusion of the right eye for the preceding 3 days.This was accompanied by vertigo and right-side facial weakness. The patient had perception of light in her right eye and 20/25 (presenting visual acuity) in the fellow eye, accompanied by right-side proptosis, total ophthalmoplegia, optic disc pallor and central retinal artery occlusion. Magnetic resonance imaging showed the presence of oedema with soft-tissue thickening in the right external auditory canal along with evidence of fluid within the middle ear cavity and mastoid air cells on the right side. A diagnosis of Orbital Apex Syndrome was made and treatment initiated.

Visual outcome of cataract surgery.

The aim of this study was to assess whether the visual outcome of cataract surgery in the institution was in accordance with the World Health Organization recommendations. In this retrospective case series, all patients who underwent cataract surgery by a single surgeon from January 2009 till June 2011 were included. Date were collected from medical records on age, gender, visual acuity and causes of sub-optimal outcome. The main outcome was best-corrected visual acuity in the operated eye, measured 4 - 6 weeks after surgery. Data on visual outcome was grouped using WHO's classification. Of the 495 eyes that underwent cataract surgery, 58% were female. Overall, 93.3% of the operated eyes had good visual outcome, while 4.4% and 2.2% had borderline and poor outcomes, respectively. Pre-existing diseases accounted for 93.9% of the borderline/poor outcome. The study showed good visual outcome of cataract surgeries performed using phacoemulsification with intraocular lens (IOL) insertion.