Novel application of Deep Anterior Lamellar Keratoplasty for retinal detachment repair in totally opaque cornea secondary to aniridia.

PURPOSE: We present a novel approach for managing retinal detachment in a patient with aniridia related corneal scarring precluding fundal view via a deep anterior lamellar keratoplasty. METHODS: A 30-year-old female patient who had bilaterally opaque cornea, due to advanced aniridia related keratopathy presented with, experiencing flashing of light in her right eye. Examination of the retinal fundus was not possible due to the dense corneal scarring and underlying cataract. A superior bullous macula off retinal detachment was diagnosed on ultrasonography. Visual acuity at presentation was hand motions. The corneal scarring extended to the mid to deep stroma. We performed a deep anterior lamellar dissection of the opacified corneal stroma, that allowed clear visualisation of a dense cataract. The cataract was removed by phacoemulsification and IOL inserted. This allowed to proceed with repair of retinal detachment, through pars plana approach. The surgery was completed by a donor deep lamellar keratoplasty. RESULTS: This achieved a good fundal view to proceed with phacovitrectomy visualised through the residual corneal layers. Uneventful pars plana vitrectomy, cryotherapy and SF6 gas were performed to reattach the retina. Post-operatively the graft remains clear at 6 months with a visual acuity of 20/160 in the right eye, a level of vision the patient had not experienced for decades. CONCLUSION: Deep anterior lamellar keratoplasty offered an alternative to temporary keratoprosthesis to achieve a clear view of the retinal fundus and perform pars plana vitrectomy and repair of the retinal detachment.

Periosteal fixation of the medial and lateral recti for large-angle incomitant exotropia.

PURPOSE: To describe the evolution of a surgical technique for the correction of large-angle incomitant exodeviations. METHODS: Retrospective review of an interventional case series from 2005 to 2019 in a single centre, with analysis of surgical procedure, prism diopter (PD) deviations and complications. RESULTS: Thirty-one patients underwent surgery at an average age of 42 years (range 4-75 years) for minimal medial rectus function, mostly from oculomotor nerve palsy (23/31; 74%). The mean pre-operative exodeviation was 75 PD (range 30-200PD). Sixteen patients (52%) had undergone previous strabismus surgery. Thirty-eight operations were performed in which the medial rectus insertion was anchored to the periosteum of the posterior lacrimal crest via a retrocaruncular transconjunctival approach. The ipsilateral lateral rectus (LR) was disinserted and fixed to lateral orbital tissue in 29/38 (76%) operations, injected with botulinum toxin in 5, recessed in 2 and had already undergone maximal LR recession in 2. In all but the first 8 operations, temporary limbal sutures were passed through the eyelids to maximally adduct the globe post-operatively. At last follow-up (mean 24 months; range 2-130), the mean reduction in exodeviation was 49PD (range 10-80) and overall residual deviation was 26PD (range 80PD base-in to 14PD base-out). The 5 LR toxin procedures had a mean reduction of 22PD (range 10-40). Seven patients had persistent diplopia, one a transient corneal erosion and one caruncle suture exposure 4 years after surgery. CONCLUSION: Large-angle exodeviations can be markedly improved by bi-rectus fixation. This approach is both safe and effective and can be performed in complex patients with multiple previous procedures.

Augmented medial rectus muscle recession versus medial rectus recession with posterior scleral fixation in partially accommodative esotropia: a randomized clinical trial.

PURPOSE: To compare the postoperative alignment after medial rectus muscle posterior scleral fixation versus augmented recession in children with partially accommodative esotropia (PAET) without convergence excess. METHODS: In this prospective study, 60 children with PAET without convergence excess were randomly assigned to either augmented medial rectus recession, based on the average of distance angle with correction and near angle without correction, or medial rectus recession, based on distance angle with correction, combined with posterior scleral fixation. The ductions, versions, angles of deviation, and difference between angles were analyzed before and 6 months after surgery. Complete success was defined as orthotropia to within 8Δ of esophoria/tropia for both distance and near, with spectacle correction, at 6 months. Patients who developed any exophoria/tropia with their original spectacles and needed reduction of their hyperopic correction to achieve successful outcome were defined as qualified successes. RESULTS: Of the 60 children, 25 in the augmented group and 28 in the posterior fixation group completed follow-up. The mean age at surgery was 4.90 ± 2.34 years. The complete success rate was 48% and 64% in the augmented and posterior fixation groups, respectively (P = 0.15); the qualified success rate was 80% and 92.9%, respectively (P = 0.17). Success rate was significantly higher (P = 0.04) in the posterior fixation group (56%) than in the augmented group (18%) when the preoperative angle disparity was ≥20Δ. CONCLUSIONS: Medial rectus posterior scleral fixation can improve the success rate in PAET with large angle disparity.

Optic atrophy in children: Current causes and diagnostic approach.

BACKGROUND/AIMS: Optic atrophy is one of the leading causes of sight impairment in children. It frequently poses a diagnostic challenge, as it can be caused by many ocular and systemic conditions. We aimed to determine the current causes of optic atrophy at our centre and to describe the use of investigations, including molecular genetic testing. METHODS: We reviewed the medical records of children with optic atrophy seen at Moorfields Eye Hospital between 2010 and 2015. We recorded demographic data, reason for referral, history, investigations and diagnosis. RESULTS: We studied 143 cases aged below 16 years. A cause could be identified in all cases. Taking a full history was the most important part of the diagnostic workup, identifying a cause in 96 (67%) children. A developmental disorder of the brain and/or optic nerve, sometimes with retinal involvement, was the commonest cause (n = 33, 23%), followed by inheritable optic neuropathies (n = 27, 19%). Other causes included perinatal insults (n = 18, 13%), post-infectious or post-inflammatory conditions (n = 18, 13%), accidental or abusive trauma (n = 14, 10%) and inheritable retinal dystrophies (n = 13, 9%). Rare conditions included neurodegenerative disorders (n = 7, 5%), skeletal developmental disorders such as rickets (n = 4, 3%), tumours (n = 4, 3%), ischaemic events including large optic nerve head drusen (n = 4, 3%) and toxic events/metabolic conditions (n = 1, 0.7%). CONCLUSION: In this series, an underlying cause could be identified in all cases. Taking a comprehensive antenatal, perinatal, postnatal and family history will indicate a probable diagnosis in two-thirds of children, and targeted ancillary tests may identify the cause in most remaining cases.

Automated Ocular Surface Image Analysis and Health-Related Quality of Life Utility Tool to Measure Blepharokeratoconjunctivitis Activity in Children.

PURPOSE: To explore the tolerability of automated conjunctival hyperemia quantification in children with blepharokeratoconjunctivitis (BKC) and its agreement with clinical activity grading and to explore the Children's Health Utility 9D (CHU9D) as a measure of health-related quality of life in children with BKC. METHODS: We enrolled 63 children, 31 with BKC and 32 without ocular surface inflammation, with a median [interquartile range (IQR)] age of 10.6 (7.2-13.9) years for BKC and 11.4 (9.5-13.8) years for healthy volunteers. Two masked observers graded the ocular surface images. The children indicated discomfort during imaging on a 5-point Likert scale. Using nonparametric tests, we explored the interobserver agreement and the agreement of automated redness index (RI) measurements of limbal and bulbar conjunctival hyperemia with clinician assessment. The children also completed the 9-item CHU9D. RESULTS: The children tolerated imaging well: median (IQR) Likert value of 0 ("comfortable") (0-0) in healthy volunteers and 1 ("a little bit uncomfortable") (0-2) in mild/moderate BKC. In children with BKC, the median (IQR) bulbar RI was 1.3 (0.8-1.6) and the median limbal RI was 0.7 (0.3-1.1). In healthy volunteers, the median bulbar RI was 0.8 (0.55-1.1; P = 0.162) and the median limbal RI was 0.3 (0.2-0.4; P = 0.02). The agreement between RI and clinical grading was high. There was no significant difference between the mean CHU9D utility score between the 2 groups [0.89 (SD 0.08) vs. 0.92 (SD 0.07); P = 0.15]. CONCLUSIONS: Automated conjunctival hyperemia quantification is feasible in children with ocular surface inflammation and may prove useful for long-term monitoring and as an objective outcome measure in clinical trials.

Meibography and corneal volume optical coherence tomography to quantify damage to ocular structures in children with blepharokeratoconjunctivitis.

PURPOSE: To evaluate non-contact infrared meibography and anterior segment optical coherence tomography (AS-OCT) to detect meibomian gland (MG) and corneal changes in children with blepharokeratoconjunctivitis (BKC). METHODS: We acquired infrared meibography images of upper and lower lids and AS-OCT corneal scans. One masked observer graded meiboscore, full/partial MG dropout, and measured total corneal volume and differential corneal volume per quadrant and central corneal thickness (CCT). RESULTS: We enrolled 63 children, 31 with BKC and 32 without ocular surface inflammation; median (interquartile range) age BKC 10.6 (7.2-13.9) years, healthy volunteers (HV) 11.4 (9.5-13.8) years. Likert scale scores for meibography and OCT indicate no to low discomfort. Meiboscores for upper and lower lids as well as the total meiboscore were significantly higher in children with BKC than in HV. Subscores for full and partial MG dropout were also significantly higher in children with BKC than in healthy volunteers. There was no statistically significant difference between upper and lower lid for meiboscore nor full/partial MG dropout scores. The corneal volume in the superior quadrant was significantly higher in children with BKC than in HV, whereas the corneal volume in the nasal and inferior quadrants was significantly lower. CONCLUSIONS: Non-contact imaging technologies objectively demonstrate damage to meibomian glands and changes in corneal volume secondary to BKC. The tests are well tolerated by children with mild/moderate ocular surface inflammation and can detect changes without the requirement for routine eversion of the upper lid. These parameters may be useful both for clinical follow-up and clinical trials.

Dynamic Changes in Schlemm Canal and Iridocorneal Angle Morphology During Accommodation in Children With Healthy Eyes: A Cross-Sectional Cohort Study.

Purpose: The purpose of this study was to explore changes in Schlemm canal (SC), trabecular meshwork (TM), and iridocorneal angle (ICA) morphology during accommodative effort in children and young adults. Methods: We acquired anterior segment optical coherence tomography images (AS-OCT) of the ICA and ciliary muscle (CM) of both eyes of 50 children age 4 to 16 years with healthy eyes, at two levels of accommodation: 2.5 and 15 diopters (D). Semiautomated nasal ICA measurements were as follows: angle opening distance at 500/750 µm (AOD-500, -750), trabecular iris space area at 500/750 µm (TISA-500, -750), and trabecular iris angle at 500/750 µm (TIA-500, -750). Manual measurements were as follows: anteroposterior and radial SC diameter (SC-APD, SC-RD), cross-sectional area of SC (SC-CSA) and TM height (TMH), TM length (TML), and TM density (TMD). CM width was measured at 1, 2, and 3 mm from the scleral spur (CM-1, CM-2, CM-3). For each parameter, a three-level random-effects model was fitted to estimate differences between the two levels of accommodation. Results: With accommodative effort, SC diameters and CSA increase significantly, as do TM length and iridocorneal angle parameters. With increasing age, SC dimensions reduce. Angle parameters are smaller in eyes with greater spherical equivalent (hypermetropia). Conclusions: AS-OCT can be used to visualize dynamic morphologic changes in outflow structures with physiologic accommodation. The increase in SC dimensions with accommodative effort may contribute to the regulation of IOP in children.

Retrospective cohort study shows that the risks for retinopathy of prematurity included birth age and weight, medical conditions and treatment.

AIM: This study described the characteristics and risk factors of neonates who developed retinopathy of prematurity (ROP) and severe treatable ROP in two Egyptian neonatal intensive care units (NICUs). METHODS: This retrospective cohort study comprised 108 preterm neonates who were screened for ROP after being admitted to the two NICUs run by Cairo University Hospital from June 2014 to May 2015. Patients were examined using digital fundus photography and indirect ophthalmoscopy was performed if ROP was detected. RESULTS: Retinopathy of prematurity occurred in 75 patients. Late-onset sepsis, ventilation and hypercapnia were independently associated with ROP. Patients who developed severe treatable ROP had a younger gestational age (GA) than patients who did not develop ROP or developed mild or moderate ROP (29 weeks, range 27-33 weeks versus 32 weeks, range 28-36 weeks, p = 0.002) and a lower birthweight (1200 g, range 980-1590 g versus 1460 g, range 770-2475 g, p = 0.029). The risk factors associated with severe treatable ROP included the duration of admission, the duration of incubator oxygen, late-onset sepsis, intraventricular haemorrhage, total parenteral nutrition and the duration of caffeine citrate therapy. CONCLUSION: This study showed that the risks for ROP were wide-ranging and included GA and weight, medical conditions and treatment.