Subnormal visual acuity after compliant amblyopia therapy: residual/refractory amblyopia or co-existing pathology? - a retrospective analysis.

Purpose: To assess the prevalence of alternate etiology/co-existing pathology among patients with amblyopia, and to characterize factors contributing to over-diagnosis of amblyopia. Methods: We retrospectively reviewed records of children (from 1 January 2016 to 31 December 2019) who were initially diagnosed as "amblyopia" but later an alternate diagnosis for subnormal vision was established. Patients who had a best corrected visual acuity (BCVA) of ≤20/32 (0.2 logMAR) after compliant amblyopia therapy were divided into 2 groups: those with refractory amblyopia (BCVA improvement from baseline <1 logMAR line) and residual amblyopia (BCVA improvement from baseline >1 logMAR line). Data was collected for presence/absence of amblyogenic risk factors, history, ocular examination, and investigations leading to the final alternate diagnosis. We analyzed the factors that contributed to the initial over-diagnosis of amblyopia using the diagnostic error evaluation and research (DEER) taxonomy tool. Results: During the study period, 508 children with an initial diagnosis of amblyopia met the study criteria. Among these 508 children, 466 were diagnosed to have amblyopia alone, while 26 children (5.1%, median age: 7 years, 17 boys: 9 girls) were revised to have an alternate diagnosis/co-existing pathology. These 26 patients comprised of 2 groups: children referred to us as amblyopia but rediagnosed to have an alternate diagnosis; and a second subset, initially diagnosed by us to have amblyopia, but later found to have alternate diagnosis/co-existing pathology. Subclinical optic neuritis (50%, 13 children), and occult macular dystrophy (OMD) (38.4%, 10 children) were the most frequent alternative diagnoses. Children with ametropic amblyopia (8/26, 30.7%) were most frequently misdiagnosed. Risk factors that led to an initial diagnosis of amblyopia were: high refractive error and heterotropia in 7 patients each (26.9%), anisometropia in 12 (46.1%), and prior pediatric cataract surgery in 4(15.3%). No improvement in BCVA in 21/26 (80.7%) children led to suspicion of co-existing etiology. Other clues were optic disc pallor (11), subnormal color vision (7), history of parental consanguinity in 7, and preceding febrile illness/rhinitis in 1 child. The DEER taxonomy tool suggested that the most common reasons for diagnostic errors were over-emphasis on amblyopia. Conclusion: Our study suggests that 5% of children diagnosed with amblyopia might have co-existing/alternate etiology. Most common co-existing etiologies were subclinical optic neuropathy, and OMD. No improvement in BCVA, subtle history and examination findings prompted further workup. Not considering co-existing etiologies was the most common reason for an initial overdiagnosis of amblyopia.

Clinical Profile, Neuroimaging Characteristics, and Surgical Outcomes of Patients With Acute Acquired Non-accommodative Comitant Esotropia.

PURPOSE: To report the clinical presentations, neuroimaging findings, and surgical outcomes in patients with acute acquired non-accommodative comitant esotropia (AACE). METHODS: A retrospective review of records of all patients diagnosed as having AACE between January 2011 and December 2019 across three tertiary eye care centers was done. Cases with AACE onset after age 1 year were included. Patients were divided into two groups based on age of onset of esotropia: childhood (16 years or younger) and adult (older than 16 years). Surgical success was defined as postoperative horizontal deviation of 8 prism diopters (PD) or less at the last follow-up visit. RESULTS: A total of 338 patients (220 males and 119 females; mean age at presentation: 12.60 ± 9.8 years) met the study criteria. The mean age at onset of esotropia in the childhood and adult onset groups was 3.61 ± 1.1 and 26.6 ± 8.7 years, respectively. There were significantly more individuals with myopia (30%) in the adult onset group compared to the childhood onset group (6%) (P = .004). Thirty-seven (16%) had positive neuroimaging findings (16.4% adult onset vs 9.4% childhood onset). A total of 148 (44%) patients underwent surgery for esotropia, and the overall success rate was 73%. Mean preoperative esotropia was comparable in either group (P = .20), but surgical success was better in the adult onset group (75.6% vs 66.3% in the childhood onset group). Mean duration of follow-up postoperatively was 13.6 ± 12 months. CONCLUSIONS: Two-thirds of the patients had childhood onset of AACE. Intracranial pathology was found in 1 of 6 patients. Surgical success was better in the adult onset group, which was not influenced by preoperative esotropia, neuroimaging findings, or refractive status, but was dependent on age at onset of esotropia and duration between onset and intervention. [J Pediatr Ophthalmol Strabismus. 2023;60(3):218-225.].

"Peek-a-boo" Sign in Pediatric Cataract Surgery.

The authors demonstrate a "peek-a-boo" sign for intra-operative evaluation of the posterior pole of the fundus during pediatric cataract surgery. [J Pediatr Ophthalmol Strabismus. 2022;59(5):e54.].

Superior Rectus Transposition in the Management of Duane Retraction Syndrome: Current Insights.

Various surgical approaches have been described for the management of Duane retraction syndrome (DRS), a type of congenital cranial dysinnervation disorder (CCDD), the goals of which include correcting the primary position deviation and abnormal head posture (AHP), minimizing globe retraction and overshoots and improving the ocular rotations. Vertical rectus transposition (VRT) is one such technique, found more effective in improving abduction and thereby expanding the field of binocular vision, as compared to horizontal muscle surgery. VRT, however, is associated with the risk of inducing vertical deviations and also poses a risk for development of anterior segment ischemia. To overcome these concerns, transposition of only the superior rectus to the lateral rectus was proposed and evaluated to reveal improvement in alignment, AHP and motility comparable to VRT but with lesser surgical time and fewer post-operative complications. With promising results in the management of DRS, superior rectus transposition (SRT) has been extensively studied and has evolved over the last decade with several modifications to further increase the efficiency and reduce the risk of post-operative complications. This article focusses on the pre-operative considerations while planning SRT in DRS, various approaches and surgical techniques described, and the outcomes and complications of SRT in DRS. The role of SRT in the management of other CCDDs may be explored with further studies.

Extraocular Muscle Transplantation for Primary Treatment of Large-Angle Exotropia.

AIM: To report the feasibility and outcomes of muscle transplantation combined with a unilateral recession-resection procedure for primary large-angle [≥60 Prism Diopters (PD)] exotropia. METHODS: We retrospectively reviewed medical records of patients from three tertiary eye-care institutes who underwent unilateral recess-resect with muscle transplantation for primary treatment of large angle exotropia between August 2017 and July 2020. Motor and restorative success were defined as alignment ≤10PD of orthotropia and ≤12PD of manifest deviation, respectively, at the final follow-up. Patients with at least 3 months of follow-up were included. RESULTS: Ten patients; six males and four females (median age 26 years) met the study criteria. The median follow-up was 12.89 months. The median preoperative exotropia reduced from 65PD (60 to 90PD) to 10PD (0 to 25PD) at final follow-up (p = .0019). The median correction obtained for the entire procedure (recession + resection + muscle transplantation) was 3.05 (1.8 to 4.3) PD/mm. Motor and restorative success was achieved in 60% and 70% of the patients, respectively. The median abduction limitation was -1 (0 to -1) at the final follow-up and there were no palpebral fissure changes beyond 6 weeks postoperatively. CONCLUSIONS: Unilateral muscle transplantation combined with the recession-resection procedure could be a useful alternative for the management of large-angle exotropia.

Tribal Odisha Eye Disease Study (TOES). Report # 8. Childhood cataract surgery and determinants of visual outcome in tribal districts.

Purpose: The purpose of this study is to describe the demographic profile, clinical features, visual outcomes, and follow-up patterns after successful cataract surgery in children from the tribal community in Odisha, India. Methods: We retrospectively reviewed records of tribal children aged 4 months-16 years, who underwent public health financed cataract surgery at our institute from January 1, 2015, to December 31, 2019. Collected data included demographic profile, clinical features, outcomes, and follow-up. Univariate and multivariate linear regression identified factors affecting the visual outcome at a 6-week follow-up. Results: During this period, a total of 352 children [536 eyes; mean age: 9.11 ± 4.4 years, 219 boys (62%)] underwent cataract surgery. The most common etiology and presenting complaints were idiopathic congenital cataract and decreased vision, respectively. In 304 children (86%), presenting best-corrected visual acuity (BCVA) was <20/200 (1.0 LogMAR), 113 (32%) had associated strabismus, and 57 (16%) had associated nystagmus. The public health agency did not sponsor postoperative follow-up, and only 195 (56%) and 61 (17.3%) children completed a 6-week and a 3-month follow-up, respectively. Median BCVA at 6-week and 3-month review was 20/125 (0.8, interquartile range [IQR], 0.2-2 LogMAR) and 20/60 (0.5, IQR, 0.25-1.35 LogMAR), respectively. Conclusion: This study showed that children from the tribal community presented late with poor presenting VA and had suboptimal visual outcomes with inconsistent follow-ups. Greater advocacy, delivery of care closer to the place of residence, and financial support for follow-up care could improve early detection, regular evaluation, and outcomes.

ICO-OSCAR scoring for pediatric cataract surgeries performed by pediatric ophthalmology fellows in training-stage of fellowship and quality of surgery.

PURPOSE: To compare quality of surgical steps in pediatric cataract surgery performed by pediatric ophthalmology fellows in various stages of training by applying the International Council of Ophthalmology-Ophthalmology Surgical Competency Assessment Rubric (ICO-OSCAR). METHODS: Two experienced pediatric ophthalmologists analyzed video recordings of fellows performing pediatric cataract surgery at our institution between August 2019 and March 2020; fellows were scored according to the ICO-OSCAR with respect to the six key surgical step, namely, (1) wound construction, (2) anterior capsulorrhexis, (3) irrigation and aspiration, (4) intraocular lens implantation, (5) primary posterior capsulotomy (PPC)/anterior vitrectomy (AV), and (6) wound suturing. Cohen's kappa was used for inter-rater agreement. Fellows were categorized by months of training as stage 1 (first 6 months), stage 2 (7-18 months), and stage 3 (19-24 months). RESULTS: We analyzed 79 procedures performed by 11 pediatric ophthalmology fellows. The inter-rater agreement ranged from 85% to 96%; κ ranged from 0.64 to 0.91. Fellows in stages 2 and 3 of their training required less time and demonstrated superior technical proficiency in PPC and AV compared with fellows in stage 1 (median score, 4 vs 3). CONCLUSIONS: Objective scoring of cataract surgeries performed by fellows at various stages of training highlighted the steep learning curve for PPC and AV and confirmed that execution improves with experience.

Consecutive Esotropia with and without Abduction Limitation - Risk Factors and Surgical Outcomes of Lateral Rectus Advancement.

Purpose: To identify risk factors for abduction limitation in consecutive esotropia following surgical correction of exotropia. To study outcomes of lateral rectus advancement for consecutive esotropia correction.Methods: Patients with consecutive esotropia (>10PD) operated between 2007 and 2019 with a minimum follow-up of 2 months were reviewed retrospectively. Preoperative and postoperative alignment and ocular motility were recorded. Patients were classified into those with full abduction (group-A) and with abduction limitation (group-B). Success was defined as deviation ≤10 PD of esotropia or exotropia in the primary position.Results: Forty-cases fulfilled the inclusion criteria (group-A = 28 and group-B = 12). Median age at surgery was five years, median consecutive esotropia was 20PD and follow-up was 29.18 months. Abduction limitation (group-B) was associated with constant exotropia (p = .01) and larger bilateral lateral rectus recession (group-A = 13 mm, group-B = 15 mm; p = .04). Nineteen patients underwent lateral rectus advancement (group-A = 12, group-B = 7), one underwent medial-rectus recession and two were excluded due to lack of postoperative follow-up. Five patients had spontaneous resolution with good alignment (group-A = 3, group-B = 2), two refused surgery, three were observed and eight were lost to follow-up. Success following second surgery was similar in both groups (group-A = 86%, group-B = 77%)(p = 1).Conclusion: Constant exotropia and larger lateral rectus recession were associated with abduction limitation in consecutive esotropia. Lateral rectus advancement produced good outcomes irrespective of abduction limitation.

One-year Profile of Eye Diseases in Infants (PEDI) in secondary (rural) eye care centers in South India.

Purpose: The aim of this study was to report the proportion and patterns of eye diseases observed among infants seen at two rural eye care centers in South India. Methods: A retrospective review of case records of infants seen between January 1, 2017 and December 31, 2017 at two rural secondary eye care centers attached to L V Prasad Eye Institute, Hyderabad. Data were collected regarding their demographic profile, the pattern of eye problems observed, management at the facility itself, and need for referrals. Results: During this period, a total of 3092 children were seen. Among them, 141 were infants (4.56%, 71 boys: 70 girls, median age: 8 months). Twenty-five percent of infants were less than 6 months of age. The most common eye problem was congenital nasolacrimal duct obstruction (n = 76, 53.90%), followed by conjunctivitis (n = 33, 23.40%), retinopathy of prematurity (n = 4, 2.84%) and strabismus (n = 3, 2.13%). One case each of congenital cataract and suspected retinoblastoma were identified. Majority of the cases (58.8%) belonged to the oculoplastic and orbital surgery sub-specialty. Sixteen percent of the infants (n = 23) had sight-threatening eye problems. Twenty percent (n = 28) were referred to tertiary care hospital for further management. Conclusion: Profile of eye disease in infants in secondary or rural eye care centers ranged from simple to complex, including sight-threatening diseases. While our study concluded that nearly 4/5th of these eye problems were simple and could be managed by a well-trained comprehensive ophthalmologist, 20% of these cases required a referral to a tertiary care center.

Bilateral augmented superior rectus transposition with medial rectus recession for bilateral esotropic Duane retraction syndrome.

Superior rectus transposition with medial rectus recession is commonly performed for unilateral esotropic Duane syndrome. For bilateral esotropic Duane syndrome, bilateral medial rectus recession is the most frequently performed surgery and usually provides satisfactory alignment in primary position; however, there is limited improvement in abduction. We report the outcomes of bilateral augmented superior rectus transposition and medial rectus recession in 4 patients with bilateral esotropic Duane syndrome. Postoperatively, abduction was improved in all patients; 3 were orthotropic in the primary position, and 1 had residual esotropia.

Horizontal rectus muscle transplantation for recurrent and residual strabismus.

Purpose: To report outcomes of muscle transplantation for recurrent/residual strabismus. Retrospective, multicentric, observational study.  Methods: We retrospectively reviewed records of all patients operated at three institutes who underwent transplantation of the resected muscle for re-operations for residual/recurrent strabismus from January 1, 2018, to December 31, 2019. Data were collected regarding age, gender, visual acuity, type of strabismus, fixation preference, associated amblyopia, the surgical procedure performed, preoperative and postoperative primary position deviation, limitation of ductions, associated intra-operative and post-operative complications. Surgical success was defined as heterotropia less than or equal to 8 PD, and cosmetic success was defined as manifest deviation ≤12 PD (at 6 weeks follow up). Results: During this period, we identified records of seven patients (5 men: 2 women, median age: 24 years) who underwent muscle transplantation for residual/recurrent strabismus. Six patients underwent extraocular muscle surgery for residual/recurrent exotropia (XT) and one for residual esotropia (ET). In all the patients, median pre-operative primary position deviation reduced from 40 prism diopters (PD) (range: 30 to 55 PD) to 8 PD (range: 6 PD ET to 10 PD XT) at six-weeks follow-up. Four patients had residual exotropia (10-16PD), two had orthotropia and one patient had small-angle esotropia (6PD) at the last follow-up. Excluding two patients who underwent supramaximal re-recession of lateral rectus (≥11 mm from insertion) and re-resection of medial rectus, 3/5 (60%) patients achieved surgical success, and all (100%) achieved cosmetic success at six-weeks-follow-up. There was mild (up to -1) limitation of duction in all patients except one patient (-2 limitation of abduction) who underwent re-recession of lateral rectus to 11 mm from insertion along with medial rectus re-resection and muscle transplant procedure. No intra-operative or post-operative complications were encountered. No patient reported postoperative diplopia. Conclusions: This study describes the feasibility and successful use of muscle transplantation procedure to correct residual/recurrent strabismus, with or without re-recession, especially in patients planned for surgery in one eye. However, larger prospective studies with longer follow-ups will be needed to establish long-term outcomes and correction achieved from this procedure, and comparison with other approaches for reoperations.

Surgical Outcomes of Exotropic Duane Retraction Syndrome From a Tertiary Eye Care Center.

PURPOSE: To report surgical outcomes of patients with exotropic Duane retraction syndrome. METHODS: A retrospective review of the medical records from patients with exotropic Duane retraction syndrome who underwent strabismus surgery and had at least 2 months of postoperative follow-up was conducted. Surgical success was defined as a postoperative horizontal deviation within 8 prism diopters (PD), abnormal head posture (AHP) less than 5 degrees, and a two-step decrease in overshoots. RESULTS: Seventy-three patients with exotropic Duane retraction syndrome (38, 52% male, mean age 14 ± 7.9 years) met the study criteria. Unilateral type 3 Duane retraction syndrome was seen in 56 (77%) patients and type 1 in 14 (19%) patients. The mean follow-up period was 22 weeks (range: 8 to 209 weeks). Unilateral and bilateral lateral rectus recession were performed in 48 (65%) and 25 (34%) patients, respectively. In addition to recession, a lateral rectus Y-splitting was performed in 42 (56%) patients. Five patients underwent differential medial rectus and (larger) lateral rectus recession to alleviate severe globe retraction. The mean primary position reduced from 23 PD (CI: 19.6 to 26 PD) preoperatively to 9 PD (CI: 7.02 to 11.02 PD) postoperatively (P < .0001). Postoperatively 50% of patients were orthotropic in the primary position. Surgical success for primary position deviation, AHP, and overshoots were achieved in 74%, 81%, and 71% of patients, respectively. CONCLUSIONS: In the current study, type 3 Duane retraction syndrome was the most common subtype necessitating surgery for exotropic Duane retraction syndrome. Good surgical outcomes were obtained for various indications. Unilateral or bilateral lateral rectus recession was successful in improving motor alignment and AHP. The addition of lateral rectus Y-splitting corrected overshoots greater than grade 2 in most patients with exotropic Duane retraction syndrome. [J Pediatr Ophthalmol Strabismus. 2021;58(1):9-16.].

Validation of visual acuity applications for teleophthalmology during COVID-19.

PURPOSE: The objective of this study was to identify and validate smartphone-based visual acuity (VA) apps that can be used in a teleophthalmology portal. METHODS: The study was conducted in three phases: A survey to investigate if the SmartOptometry App was easy to download, understand and test (phase I), an in-clinic comparison of VA measured in a random testing order with four tools namely COMPlog, Reduced Snellen near vision, Peek Acuity (Distance VA) and SmartOptometry (Near VA) (phase II) and a repeatability study on these 4 tools by measuring VA again (phase III). The study recruited the employees of our institute and adhered to the strict COVID-19 protocols of testing. RESULTS: Phase I Survey (n = 40) showed 90% of participants used android phones, 60% reported that instructions were clear, and all users were able to self-assess their near VA with SmartOptometry App. Phase II (n = 68) revealed that Peek Acuity was comparable to COMPlog VA (P = 0.31), however SmartOptometry was statistically significantly different (within 2 log MAR lines) from Reduced Snellen near vision test, particularly for young (n = 44, P = 0.004) and emmetropic (n = 16, P = 0.04) participants. All the 4 tests were found to be repeatable in phase III (n = 10) with a coefficient of repeatability ≤0.14. CONCLUSION: Smartphone-based apps were easy to download and can be used for checking patient's distance and near visual acuity. An effect of age and refractive error should be considered when interpreting the results. Further studies with real-time patients are required to identify potential benefits and challenges to solve.

Rapidly progressive vision loss due to fulminant idiopathic intracranial hypertension: a diagnostic and management dilemma.

A 44-year-old obese woman presented with decrease in vision in the right eye (RE) for 3 days. She reported a simultaneous onset of holocranial headache that worsened on bending forward. She denied eye pain, pain on eye movements, and other ocular or neurological complaints. On examination, her distance best-corrected visual acuity was counting fingers at 1 m in the RE and 20/20 in the left eye (LE). Colour vision was subnormal in both eyes (BE). There was grade II relative afferent pupillary defect in the RE. Fundus examination showed disc oedema in BE . Visual fields in the LE showed central scotoma extending nasally. A provisional diagnosis of papillitis was considered. However, contrast-enhanced MRI of the brain and orbits showed evidence of elevated intracranial pressure. Cerebrospinal fluid (CSF) opening pressure was 42 cm H2O while rest of the CSF analysis was normal. Diagnosis was revised to fulminant idiopathic intracranial hypertension. Management with medical therapy and urgent thecoperiteoneal shunt improved visual function in BE.

Pituitary adenoma presenting as acute onset isolated complete third cranial nerve palsy without vision changes.

We report a case of isolated unilateral complete pupil involving third cranial nerve palsy due to pituitary adenoma with parasellar extension into the right cavernous sinus. The patient was referred to us from neurosurgery with sudden onset binocular vertical diplopia with complete ptosis, and mild right-sided headache of 5-day duration. Ocular examination revealed pupil involving third cranial nerve palsy in right eye while rest of the examination including automated perimetry was normal. MRI brain with contrast revealed a mass lesion with heterogenous enhancement in the sella suggestive of a pituitary macroadenoma with possible internal haemorrhage (apoplexy). In addition, the MRI showed lateral spread to the right cavernous sinus which was causing compression of the right third cranial nerve. The patient was systemically stable. This report highlights a unique case as the lesion showed a lateral spread of pituitary adenoma without compression of the optic chiasm or other cranial nerves.

Long-term outcomes following primary intraocular lens implantation in infants younger than 6 months.

Purpose: To study the long-term safety profile and visual outcomes of primary intraocular lens (IOL) implantation in infants <6 months of age. Methods: This was a retrospective observational study conducted at a tertiary eye care center in South India. Infants under 6 months meeting the selection criteria who underwent cataract surgery (lens aspiration, primary posterior capsulorhexis, and anterior vitrectomy) with primary IOL implantation between January 2008 and December 2011 and minimum 3-year follow-up were included. Patient demographics, serial refractions, visual acuity, complications, and associated amblyopia/strabismus were reviewed. Visual acuity, myopic shift, and complications were the outcome measures. Results: Sixty-nine eyes of 38 infants (31 bilateral; mean age: 4.6 months) were reviewed. Mean follow-up was 51 months (range: 36-84). Median logMAR best-corrected visual acuity at the final visit was 0.74 (interquartile range [IQR]: 0.50-0.98) in eyes with bilateral cataracts and 0.87 (IQR: 0.60-1.14) in eyes with unilateral cataracts with an average myopic shift of 6.7 diopters over 4.2 years. Most common postoperative complication was visual axis opacification (VAO) (13 eyes, 18%), necessitating membranectomy followed by pigmentary IOL deposits (11 eyes, 15%), and IOL decentration and glaucoma in four eyes each (5.6%). Mixed linear effect model found no significant association of age, gender, laterality, and postoperative complications with final visual acuity (P ≥ 0.05). Eyes with unilateral cataracts had a greater myopic shift than bilateral cases (P = 0.03). Conclusion: Primary IOL implantation in infants <6 months is reasonably safe in appropriately selected infants. VAO was the most common postoperative complication, and a large myopic shift was observed.