Eibhlin McLoone

Long term functional and structural outcomes of laser therapy for retinopathy of prematurity.

Aims: To assess the long term functional and structural outcomes of premature babies who received diode laser photocoagulation for threshold retinopathy of prematurity (ROP). Methods: 25 patients (43 eyes) treated with laser were recalled for assessment at a mean follow up of 11 years. A further seven patients (14 eyes) with subthreshold ROP, which had regressed spontaneously without laser treatment, were also examined. All children underwent distance acuity, near acuity, contrast sensitivity (CS), and colour vision assessments followed by a dilated fundal examination and cycloplegic autorefraction. Results: The laser treated eyes had a mean distance visual acuity of 0.37 logMAR, a mean near visual acuity of 0.39 logMAR, a mean contrast sensitivity of 1.49 log CS units, and a mean spherical equivalent of −2.10D. An unfavourable distance visual acuity outcome occurred in five eyes (13.5%). An unfavourable near visual acuity outcome was also noted in the five eyes (13.5%) with poor distance visual outcome. 7% had an unfavourable structural outcome. On comparison with the control group, there was no significant difference in near acuity, CS, refraction, or colour vision between the two groups. However, there was a statistically significant difference in terms of distance visual acuity (p = 0.03). Conclusions: Laser treated eyes with favourable structural outcome have a good visual outcome. The results show a long term benefit from diode laser photocoagulation in preserving distance and near vision in eyes with threshold ROP.

RetCam image analysis of optic disc morphology in premature infants and its relation to ischaemic brain injury

Aims: To assess optic disc characteristics in premature infants with and without ischaemic brain injury and to evaluate the role of optic disc morphology in dating the injury. Methods: RetCam fundal images, cranial ultrasounds and magnetic resonance imaging (MRI) of 109 premature infants were analysed. The study cohort was divided into subgroups depending on the presence or absence of periventricular leucomalacia (PVL) and intraventricular haemorrhage (IVH). The control group consisted of infants with normal neuroimaging at term and 2 years of age. Using the image analysis software of the RetCam, optic disc diameter (ODD), optic disc area (ODA), and optic cup area (OCA) were measured at 33–34 weeks gestational age. As serial cranial ultrasonography had been performed, it was possible to date the brain injury in those infants with periventricular white matter (PVWM) damage. Results: Although there was a trend towards reducing ODD, ODA, and OCA with increasing severity of IVH, only the IVH 4 group differed significantly from the controls for these parameters (p = 0.002, p = 0.02, and p = 0.04, respectively). 44.4% of infants with grade 4 IVH had small discs. Only one patient had a large cup in a normal sized disc; this patient had IVH 4. In patients with PVWM damage, the median time of insult was 27 weeks in those with small discs and 28 weeks in those with normal discs. This difference was not significant (p = 0.23). Conclusions: Premature infants with IVH 4 have an increased incidence of optic nerve hypoplasia. We found no association between disc morphology and timing of brain injury.

Effect of diode laser retinal ablative therapy for threshold retinopathy of prematurity on the visual field : Results of goldmann perimetry at a mean age of 11 years

PURPOSE To assess the peripheral visual field in premature children who received diode laser photocoagulation for threshold retinopathy of prematurity (ROP) and in a comparison group with subthreshold ROP that had regressed spontaneously without laser treatment. PATIENTS AND METHODS Eleven patients (18 eyes) were treated with laser, and nine patients (16 eyes) with subthreshold untreated ROP were recalled for assessment at a mean follow-up of 11 years. All children underwent Goldmann visual field testing using the II4e and V4e stimuli. Distance visual acuity measurement, dilated fundal examination, and cycloplegic autorefraction were also performed. RESULTS The visual field extent in treated eyes was 3% to 4% smaller for the V4e target and 7% to 10% smaller for the II4e target than in the comparison eyes. For the II4e stimulus, the right eye visual field extent of the laser-treated children did not differ significantly from that of the untreated comparison group (P = .11), but the left eyes showed a borderline significant reduction (P = .046). For the larger V4e stimulus, no significant differences were noted (right eye, P = .41; left eye, P = .30). CONCLUSIONS This is the first study to assess the effect of diode laser therapy on the peripheral visual field in eyes with threshold ROP using Goldmann perimetry. Laser-treated eyes showed a slight constriction of peripheral visual fields compared with untreated subthreshold eyes. It is uncertain whether this was due to the laser treatment itself or to the more severe ROP in the laser group. However, the limited reduction in visual field extent is comparable to that reported for cryotherapy and is unlikely to be of functional significance.

Fundus Fluorescein Angiographic Characterization of Diffuse Choroidal Hemangiomas

PURPOSE To evaluate the clinical usefulness of fundus fluorescein angiography (FFA) in characterizing diffuse choroidal hemangiomas in Sturge-Weber syndrome. METHODS A retrospective case review of children attending a university hospital clinic with a diagnosis of Sturge-Weber syndrome was undertaken. The FFA findings of seven patients who had undergone angiography were reviewed. RESULTS FFA confirmed the presence of choroidal hemangioma in each of the seven cases and delineated the extent of the choroidal lesions. No adverse effects of fluorescein injection were encountered. CONCLUSIONS FFA is effective in diagnosing and characterizing diffuse choroidal hemangiomas, and may be performed safely in children.

Amniotic band syndrome associated with an atypical iris and optic nerve defect

We report the case of an 8-year-old boy who presented with right cleft lip and palate, left Tessier number 3 and 11 clefts, and limb abnormalities because of amniotic band syndrome. He was found to have an atypical iris and optic disk nasal defect and a right-sided ptosis, which have not been previously reported with amniotic band syndrome.

A mutation in the Norrie disease gene (NDP) associated with familial exudative vitreoretinopathy.

A mutation in the Norrie disease gene ( NDP ) associated with familial exudative vitreoretinopathy

Congenital nasolacrimal duct obstruction: Comparison of two different treatment algorithms

PURPOSE To clarify the most appropriate treatment regimen for congenital nasolacrimal duct obstruction (CNLDO). METHODS A retrospective observational analysis was performed of patients undergoing probing with or without intubation to treat CNLDO in a single institution (Royal Victoria Hospital, Belfast) from 2006 to 2011. RESULTS Based on exclusion criteria, 246 eyes of 177 patients (aged 0 to 9.8 years with a mean age of 2.1 years) were included in this study: 187 (76%) eyes had successful outcome at first intervention with primary probing, whereas 56 (23%) eyes underwent secondary intervention. There were no significant differences by gender, age, or obstruction complexity between the successful and unsuccessful patients with first intervention. For those patients requiring secondary intervention, 16 of 24 (67%) eyes had successful probing, whereas 22 of 24 (92%) had successful intubation. Patients with intubation as a secondary procedure were significantly more likely to have a successful outcome (P = .037). Statistical analysis was performed using the Fishers exact test and Barnards exact test. CONCLUSIONS Primary probing for CNLDO has a high success rate that is not adversely affected by increasing age. This study also indicates that if initial probing is unsuccessful, nasolacrimal intubation rather than repeat probing yields a significantly higher success rate. [J Pediatr Ophthalmol Strabismus. 2016;53(5):285-291.].

Biotinidase Deficiency, Bilateral Optic Atrophy, and a Visual Field Defect

Abstract We present an update on the ophthalmic status of a female patient with biotinidase deficiency who has attended our clinic for over 30 years. She is the only reported case of juvenile-onset glaucoma associated with biotinidase deficiency. Her intraocular pressure, optic nerve appearance, and visual field defect have, however, remained stable throughout her follow-up. We feel that this patient’s visual field defect is a result of optic atrophy due to biotinidase deficiency in early life rather than glaucomatous damage.

Pseudotumour Cerebri Syndrome in Two Children on Systemic Steroid Therapy for Uveitis

An 8-year-old girl with antinuclear antibody positive oligoarticular juvenile idiopathic arthritis was diagnosed at 18 months of age, with uveitis onset at 2.5 years old. She was examined at the clinic in July 2013 at the age of 6 years and 4months. Shewas 111 cm in height, 19.5 kg inweight; her BMI was 15.8 and she had a Z-score of 0.47. Her treatment for uveitis was Pred Forte drops three times a day to both eyes and methotrexate 15 mg orally, weekly. She had been on systemic steroids for 4 years, which had recently been tapered to stop over 3 months following commencement of methotrexate. There was no tetracycline use or Vitamin A. Her visual acuity was 6/9 in her right eye and 6/24 in her amblyopic left eye. Therewas 1+ cellular activity in the anterior chamber of each eye (Standardization of Uveitis Nomenclature, SUN, criteria). There was no vitreous activity. There was bilateral optic disc swelling (Figure 1A). Her disc appearance was normal in both eyes at all examinations for 4 years prior to this attendance, the last ofwhichwas 1month earlierwhen she was still on oral prednisolone. She denied any symptoms; in particular, she did not complain of headache. There were no optic disc drusen on ultrasonography of her eyes. CT scanning of her head revealed no structural abnormality. Lumbar puncture was performed with the patient in a lateral decubitus position and 15 mL of cerebral spinalfluidwasdrainedwhen an openingpressure of 41 cmCSFwas recorded. Cerebrospinal fluid constituents were normalwith glucose 3.3mmol/Landprotein 0.15 g/ L. Acetazolamide was commenced but not tolerated and changed to furosemide. There was complete resolution of the disc swelling after 2 months, at which point her visual acuity was 6/7.5 in the right eye and 6/15 in the left eye (Figure 1B). She remained stable off furosemide and there was no recurrence of the disc swelling.

Torpedo maculopathy: disease spectrum and associated choroidal neovascularisation in a paediatric population

PurposeTorpedo maculopathy is a rare lesion of the retinal pigment epithelium. This study set out to look at these lesions in the paediatric population and determine the spectrum and features of the disease.MethodsThe paediatric ophthalmology database was used to identify eight children with torpedo maculopathy between 2012 and 2017. Fundal images and optical coherence tomography (OCT) was used to analyse the cases.ResultsEight patients with torpedo maculopathy were identified, making the prevalence approximately 2 per 100,000 population under 16 years old. The OCT images were classified using the previously described subtypes: Type 1 in patients 5 and 6 and Type 2 in patients 1, 2, 3, 4 and 8. The average age of presentation of Type 1 and Type 2 lesions was 8 and 7 years old respectively. We also report patient 8, who is the youngest reported case of choroidal neovascular membrane associated with torpedo maculopathy. Good anatomical response to a single injection of anti-Vegf was shown.ConclusionsThis study is the first case series of torpedo maculopathy in the paediatric population. Contrary to previous reports of two distinct types of lesion on OCT representing different stages of the same disease, our case series indicates that Type 1 and Type 2 lesions are in fact different phenotypic entities both of which can occur at a young age. We also present the associated risk of choroidal neovascular membrane formation which is an important consideration for long term follow-up.