RETINAL CHANGES IN PORETTI-BOLTSHAUSER SYNDROME: RETINA AS A WINDOW TO THE BRAIN.

PURPOSE: LAMA 1 gene as a pathologic variant leading to cerebellar dysplasia and cysts, nonprogressive ataxia, language, and motor developmental delay without any muscular involvement was recently described as Poretti-Boltshauser syndrome (PBS). Ocular involvement is a common associated feature in this neurodegenerative disorder. In this case report, we describe the retinal changes associated with Poretti-Boltshauser syndrome. METHODS, PATIENT, AND RESULTS: A 4-year-old female child presented with the progressive decreased vision for the past 6 to 8 months. Ophthalmic examination revealed mild myopia and ocular motor apraxia with retinal disruptions appearing as holes that were confined only to inner retinal layers. The child also had motor and speech developmental delays. Magnetic resonance imaging of the brain showed vermis hypoplasia with cerebellar dysgenesis and multiple cystic spaces in both cerebellar hemispheres. Whole exome sequencing revealed a homozygous pathogenic variant of exon 2-63 deletion in the LAMA 1 gene, which was confirmatory for Poretti-Boltshauser syndrome. CONCLUSION: Oculomotor apraxia and retinal changes can lead to visual disturbances in Poretti-Boltshauser syndrome. Identification of these features and prompt rehabilitative measures can improve the quality of life of these children.

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.].

From Scylla to Charybdis: Fixed drug combinations for tuberculosis and increased ethambutol-induced optic neuropathy in India.

Purpose: To describe the increase in prevalence of ethambutol-induced optic neuropathy (EON) in patients presenting to a single tertiary referral eye care center in India after introduction of weight-based fixed dose combinations and an increase in duration of ethambutol use from 2016 in the Revised National Tuberculosis Control Program. Methods: This was a retrospective, observational, referral hospital-based study of 156 patients with a diagnosis of EON presenting to a single tertiary referral eye care center between January 2016 and December 2019. The main outcome measure was to assess the increase in prevalence of EON cases presenting to our tertiary care institute. Results: During the 4-year study period, 156 new patients were diagnosed with EON. A total of 101 patients (64.7%) were males and 55 (35.3%) were females. The most common age group affected was 41-60 years. The significant complaint at presentation was decreased vision in all the patients. A rising trend in the number of patients diagnosed as EON was seen, with the prevalence increasing from 16 cases in 2016, 13 cases in 2017, and 31 cases in 2018 to 96 cases in 2019. Conclusion: The results of this study indicated an alarming increase in the trend of EON cases presenting to our tertiary care institute.

Virtual bedside clinics in pediatric ophthalmology and strabismus - An innovation in education and learning.

We piloted an innovation in teaching by conducting live virtual bedside clinics and evaluated the effectiveness compared to conventional bedside clinics. The purpose is to report the methodology and survey results of this innovation in teaching. A virtual bedside clinic was set up utilizing multiple audio-visual aids at a tertiary eye care facility. The bedside clinic was conducted and streamed live to pre-registered participants across the globe using the Zoom platform. The online survey was conducted comparing its effectiveness with conventional bedside clinics. A total of five sessions were conducted. A total of 2058 participants registered (411/session), of which 938 (45.57%) attended (187/session). A total of 287 participants (30.6%) responded to the survey. The respondents included ophthalmology residents (43.4%), fellows (19%), sub-specialty ophthalmologists (15.4%), general ophthalmologists (12%), and optometrists (9%). More than 95% of the respondents felt that these clinics were equally effective/better in imparting the following: physical examination 97%, clinical knowledge 99.3%, clinical reasoning 98.3%, procedural skills 95%, and communication skills 96.5%. Respondents suggested that these clinics were better/equally effective in the following techniques: general examination (96%), ocular motility (93.3%), nystagmus evaluation (93.3%), and anterior (80%) and posterior segment examination (73.3%). The hybrid mode presentation (97.3%) and discussion with the panel (100%) were reported to be equally effective/much better. Live virtual bedside clinics are a novel and effective way of continuing quality teaching and impactful learning. Most of the bedside manners, procedural skills, and examination techniques can be effectively taught through this virtual platform with a scope to improve anterior and posterior segment examination skills.

Congenital anomalies of inferior rectus and its surgical outcomes.

Congenital inferior rectus hypoplasia and aplasia is a rare abnormality of the extraocular muscles, and it is the second most common after superior oblique muscle aplasia. It has been reported either in isolation or with coexisting ocular or systemic associations. We describe here cases of inferior rectus hypoplasia/aplasia, their clinical features, and surgical approach to achieve satisfactory outcomes. We retrospectively reviewed medical records from January 2009 to December 2020 of patients with vertical strabismus due to inferior rectus hypoplasia/aplasia at a tertiary eye care center. Those who underwent surgical intervention with an adequate follow up postoperatively were included in the study. Three patients who presented with congenital hypertropia and diagnosed of inferior rectus aplasia/hypoplasia and who underwent strabismus surgery were identified. Diagnosis was based on clinical examination and orbital imaging. Coexisting horizontal deviation was present in two patients. None of the patients had any other ocular, cranial, or systemic anomalies. All patients had isolated inferior aplasia/hypoplasia with normal other extraocular muscles on orbital imaging. Intraoperatively, ipsilateral superior rectus was tight in all patients. All underwent ipsilateral superior rectus recession, whereas two patients with large vertical strabismus required additional inferior oblique anteronasal transposition in the same eye. Satisfactory outcomes were achieved in all three patients without the need for resurgery. Moderate to large vertical A pattern strabismus and limitation of infraduction are common clinical findings. Orbital imaging helps not only in diagnosis but also in surgical planning. Weakening of antagonist superior rectus with anteronasal transposition of inferior oblique not only gives good surgical outcomes but also avoids potential complications related to vertical transposition of either horizontal rectus muscle.

Clinical profile, diagnostic challenges, and outcomes in subacute/chronic cerebral sinus venous thrombosis.

PURPOSE: To report clinical profile, diagnostic challenges, and outcomes in cases of subacute/chronic cerebral sinus venous thrombosis (CSVT) presenting to neuro-ophthalmologists/neurologists. METHODS: This was a multicentric, retrospective, observational study. Records of patients with neuroimaging proven subacute/chronic CSVT seen the from January 1, 2016 to March 31, 2020 were analyzed. Data collected included duration of symptoms, diagnosing physician, ophthalmological vs. focal/generalized neurological symptoms, optic disc examination, perimetry, and neuroimaging findings. Statistical analysis was performed using STATA software. RESULTS: Forty-three patients with subacute (30)/chronic (13) CSVT were identified (32 males, 11 females). Median age was 37 (IQR 27-47) years. The presenting complaints were blurred vision 34 (79%), headaches in 25 (58%), vomiting 12 (28%), and diplopia 11 (26%). Eleven patients had associated sixth cranial nerve palsy. All but two patients had either disc edema/optic atrophy; four had unilateral disc edema at presentation. Ophthalmologists and neurologists diagnosed/suspected CSVT correctly in 13/29 (45%) and 11/14 (78.5%) patients, respectively. Most common initial alternate diagnosis was idiopathic intracranial hypertension in 12 (28%). Female gender, age ≤36, unilateral papilledema, not obtaining venogram at initial workup increased chances of initial alternate diagnosis. Median follow-up duration was 21 days. Average visual function remained stable in majority of patients at last follow-up. In total, 47.6% of patients had best-corrected visual acuity ≥20/30 at the final follow-up. CONCLUSION: In our series, subacute or chronic CSVT presented presented primarily with symptoms of intracranial hypertension. Unilateral papilledema, middle-aged patients, female gender, lack of focal/generalized neurological symptoms created diagnostic dilemma. Visual function remained stable in majority of patients.

Ocular ischaemic syndrome following coil embolisation for direct carotid cavernous fistula.

A 36-year-old man presented with proptosis and external ophthalmoplegia of the left globe following road traffic injury. Cerebral angiogram revealed moderate flow direct carotid cavernous fistula on left side for which coil embolisation was done repeatedly. Subsequently, the patient developed decreased vision in left eye and developed features of left-sided ocular ischaemic syndrome. The patient was treated conservatively with spontaneous reversal of ocular ischaemic syndrome and complete regain of visual function.

Oculomotor synkinesis: an uncommon sequela of paediatric cavernous sinus thrombosis.

We report an unusual case of acquired oculomotor synkinesis as a sequela of cavernous sinus thrombosis in a child. A 4-year-old male child presented to our emergency services with sudden onset periorbital swelling with complete ptosis of the left upper eyelid. This was preceded by a febrile episode and a furuncle at the tip of the nose. Computerised axial tomography of the orbit revealed orbital cellulitis in the left eye. The child was started on systemic antibiotics followed by a short course of systemic steroids. MRI of the brain with contrast revealed left cavernous sinus and superior ophthalmic vein thrombosis. Following administration of systemic anticoagulants and antibiotics, the ocular motility and ptosis improved remarkably. However, 6 months post-treatment, the child developed signs of aberrant regeneration of the third cranial nerve (oculomotor synkinesis).

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.

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.].

Pivoting to teleconsultation for paediatric ophthalmology and strabismus: Our experience during COVID-19 times.

PURPOSE: The objective is to analyse and report the data of teleconsultations provided to paediatric ophthalmology and strabismus patients during COVID-19 times and to elaborate our experience for guiding future teleconsultation practices to General, paediatric Ophthalmologists and Strabismologists. METHODS: Retrospective analysis of electronic medical record data of teleconsultations provided in the department of Strabismus, Paediatric and Neuro-ophthalmology was done. Patients with optic nerve related disorders were excluded. Study period was one month. Statistical analysis of collected data was done using Microsoft excel. RESULTS: A total of 198 patients were provided teleconsultations (an average of seven teleconsultations/day). The final analysis included 161 patients after excluding optic nerve related disorders. The median age was seven years. We had a near equal gender distribution (53% males and 47% females) of whom a third were new cases. Video calling was used in 14%, review of clinical photos shared was used in 53%. Rest of the 33% were given telephonic advice. Allergic conjunctivitis (14%), pseudophakia (9%), strabismus (12%), status post strabismus surgery (8%), cranial nerve palsies (11%) were common diagnoses. 19% (n = 30) were advised/needed visit in emergency services on same or next day. CONCLUSION: Our experience of teleconsultation during COVID-19 times for paediatric ophthalmology and strabismus patients was very encouraging. Pivoting teleconsultation platform can provide primary eye care to most of the patients and work as essential forward triage for rest. As we continue to further explore the currently available avenues in multimedia such as video conferencing and web/mobile based applications, we believe that tele-ophthalmology platform can provide a reliable service in patient care.

Oral versus single intravenous bolus dose antibiotic prophylaxis against postoperative surgical site infection in external dacryocystorhinostomy for primary acquired nasolacrimal duct obstruction - A randomized study.

PURPOSE: To compare the efficacy of a single perioperative bolus dose of intravenous antibiotic versus postoperative oral antibiotic prophylaxis for prevention of surgical site infection (SSI) in external dacryocystorhinostomy (DCR) for primary acquired nasolacrimal duct obstruction (PANDO). METHODS: This was a prospective randomized controlled study with a noninferiority design. Patients undergoing external DCR surgery were randomized into two groups A and B. Patients in group A received a single bolus dose of intravenous cefazolin 1 g at surgery, whereas those in group B received oral cephalexin 500 mg postoperatively twice a day for 5 days. Allocation concealment was ensured by sequentially numbered opaque sealed envelopes (SNOSEs). Both groups were advised identical postoperative local wound care regimens. Any clinical evidence of SSI at 4 weeks of follow-up in either group was the main outcome measure. RESULTS: In all, 338 patients randomized into two groups of 169 patients each participated in this study. At follow-up of 4 weeks, only one patient in group B developed postoperative SSI. None in group A developed postoperative SSI. Other potential risk factors for postoperative SSI were also analyzed by univariate and multivariate analyses but none achieved statistical significance in either group. CONCLUSION: Our results demonstrate that a single bolus dose of perioperative intravenous antibiotic offers adequate prophylaxis against postoperative SSI and compares favorably with the more commonly used oral antibiotic prophylaxis in external DCR for PANDO in our population and our practice scenario.

Bilateral macular infarction after gemcitabine and carboplatin chemotherapy.

PURPOSE: To report a case of macular infarction after doublet chemotherapy with gemcitabine and carboplatin. METHODS: A middle-aged lady presenting with bilateral macular infarction post-chemotherapy for metastatic cervical malignancy was investigated for thromboembolic risks and treated. RESULTS: The macular perfusion and edema improved with control of hypertension and treatment with pentoxifylline. Visual improvement was satisfactory, and the possible associated risk such as hypertension was noted. CONCLUSION: This case underscores the need for active screening of patients on chemotherapy with gemcitabine and carboplatin for retinal vascular occlusive changes when hypertension is associated.

Comparative Evaluation of the Ostium After External and Nonendoscopic Endonasal Dacryocystorhinostomy Using Image Processing (Matlabs and Image J) Softwares.

PURPOSE: The purpose of this study was to compare the characteristics of the ostium after external dacryocystorhinostomy and nonendoscopic endonasal dacryocystorhinostomy (NEN-DCR). METHODS: This cross-sectional study included patients who underwent a successful external dacryocystorhinostomy or NEN-DCR and had ≥1 month follow up. Pictures of the ostium were captured with a nasal endoscope (4 mm, 30°) after inserting a lacrimal probe premarked at 2 mm. Image analyses were performed using Image J and Contour softwares. RESULTS: Of the 113 patients included, external dacryocystorhinostomy group had 53 patients and NEN-DCR group had 60 patients. The mean age of patients in the NEN-DCR group (38 years) was significantly (p < 0.05) lower than the external dacryocystorhinostomy group (50 years). There was no statistically significant difference (2 sample t test, p > 0.05) in mean follow up (6 vs. 4 months), maximum diameter of ostium (8 vs. 7 mm), perpendicular drawn to it (4 vs. 4 mm), area of ostium (43 vs. 36 mm), and the minimum distance between common internal punctum and edge of the ostium (1 vs. 1 mm) between the external and NEN-DCR groups. CONCLUSIONS: Image processing softwares offer simple and objective method to measure the ostium. While ostia are comparable in size, their relative position differs with posteriorly placed ostia in external compared with inferior in NEN-DCR.