Superior oblique muscle entrapment in orbital fracture presenting as acquired brown-like syndrome: a case report and review of literature.

Pediatric orbital trauma with fracture involving the junction of roof and medial wall leading to superior oblique entrapment is rare. Here the authors report a case of orbital fracture at the junction of roof and medial wall with entrapment of the superior oblique muscle presenting clinically as canine tooth syndrome which was surgically released. Postoperatively, the ocular motility improved, and the patient was relieved of diplopia. They recommend early surgical exploration in such cases, which lead to successful resolution of diplopia.

Surgical management of oculomotor nerve palsy: a comparison of lateral rectus deactivation combined with either medial rectus resection or medial rectus fixation to the medial palpebral ligament.

PURPOSE: To analyze and compare the outcome of two different surgical procedures in patients with complete oculomotor nerve palsy with large-angle exotropia. METHODS: The medical records of patients with total oculomotor nerve palsy and large-angle exotropia operated on at a single center from January 2006 to June 2020 were reviewed retrospectively. One group underwent lateral rectus deactivation with medial rectus resection (resection group); the other group underwent lateral rectus deactivation with medial rectus fixation to the medial palpebral ligament (fixation group). Surgical outcomes on the first postoperative day and at 6 months postoperatively were analyzed, including alignment and postoperative complications. All statistical analyses were performed using STATA version 14. A P value of <0.05 was considered significant. RESULTS: A total of 35 patients were included. There was a trend toward greater surgical success in the fixation group (93%) than in the resection group (65%), but these results were not statistically significant. Postoperative exotropic drifts were noted in both the procedures but tended to be more with patients in the resection group. Postoperative complications were noted only in the fixation group. CONCLUSIONS: Lateral rectus deactivation with medial rectus fixation to the medial palpebral ligament requires more time and greater surgical expertise but appears to better prevent postoperative exotropic drift compared with lateral rectus deactivation combined with medial rectus resection.

A Comparison of Chemodnervation to Incisional Surgery for Acute, Acquired, Comitant Esotropia: An International Study.

PURPOSE: To compare the efficacy of botulinum toxin injections to strabismus surgery in children with acute, acquired, comitant esotropia (ACE), and to investigate factors predicting success. DESIGN: International, multi-center nonrandomized comparative study METHODS: Setting: Cloud-based survey. STUDY POPULATION: Children aged 2 to 17 years who underwent a single surgical intervention for ACE. INTERVENTIONS: Botulinum toxin injection ("chemodenervation" group) or strabismus surgery ("surgery" group). MAIN OUTCOME MEASURES: Primary measure: success rate at 6 months in propensity-matched cohort, defined as total horizontal deviation of 10 prism diopters or less with evidence of binocular single vision. Secondary measure: Risk factors for poor outcomes in the full cohort. RESULTS: Surgeons from 19 centers contributed. There were 74 patients in the chemodenervation group and 97 patients in the surgery group. In the propensity-matched data (n = 98), success rate was not significantly different at 6 months (70.2% vs 79.6%; P = .2) and 12 months (62.9% vs 77.8%; P = .2), but was significantly lower in the chemodenervation group at 24 months (52% vs 86.4%; P = .015). Irrespective of treatment modality, treatment delay was associated with lower success rates at 6 months, with median time from onset to intervention 4.5 months (interquartile range (IQR): 2.1, 6.7) in the success group and 7.7 months (IQR: 5.6, 10.1) in the failure group (P < .001). CONCLUSIONS: In children with ACE, success rate after chemodenervation was similar to that of surgery for up to 12 months but lower at 24 months. Those with prompt intervention and no amblyopia had the most favorable outcomes, regardless of treatment modality.

Augmented loop myopexy with concurrent intraocular lens implantation for myopic strabismus fixus.

Purpose: To evaluate safety profile and surgical outcomes of loop myopexy with concurrent intra-ocular lens implantation in cases of myopic strabismus fixus (MSF). Methods: A retrospective chart review of patients who underwent loop myopexy with concurrent small incision cataract surgery with intra-ocular lens implantation between January 2017 and July 2021 for MSF at a tertiary eye care centre was undertaken. A minimum of 6 months of follow-up after surgery was required for inclusion. The main outcome measures were improvement in alignment postoperatively, improvement in extra-ocular motility postoperatively, intraoperative and postoperative complications and post-operative visual acuity. Results: 12 eyes of 7 patients (male (6): female (1)) underwent modified loop myopexy at a mean age of 46.86 years (range 32-65 years). 5 patients underwent bilateral loop myopexy with intra-ocular lens implantation whereas 2 patients underwent unilateral loop with intra-ocular lens implantation. All eyes underwent additional medial rectus (MR) recession with lateral rectus (LR) plication. At the last follow-up, mean esotropia improved to 16 prism dioptres (PD) (Range: 10-20 PD) from 80 PD (Range:60-90PD), P = 0.016; and success (deviation ≤20PD) was achieved in 73% (95% CI 48 to 89%). Mean hypotropia at presentation was 10 PD (range 6-14 PD), which improved to 0 PD (range 0-9 PD), P = 0.063. Mean BCVA improved from 1.08 LogMar to 0.3 LogMar units. Conclusion: Loop myopexy combined with intra-ocular lens implantation is a safe and effective procedure in the management of patients who have Myopic Strabismus Fixus with visually significant cataract and improves both visual acuity and ocular alignment significantly.

An uncommon presentation of WAGR syndrome with persistent fetal vasculature.

Aniridia is an autosomal dominant congenital malformation associated with mutations in the PAX6 gene. It can be associated with deletion in the contiguous WT1 gene, leading to WAGR syndrome, characterized by Wilm tumor, aniridia, genitourinary anomalies, and mental retardation. Persistent fetal vasculature is a developmental malformation caused by incomplete regression of hyaloid vasculature. Most cases of persistent fetal vasculature occur sporadically; however, some inherited forms are described. We report a case of genetically confirmed WAGR associated with congenital cataract and persistent fetal vasculature.

The art of tackling strabismus skillfully by sparing vessels.

Background: Detaching a rectus muscle irreparably destroys its ciliary artery circulation which also supplies the anterior segment of the eye. Purpose: To educate strabismus surgeons about a method of detaching a muscle without compromising anterior segment circulation. Synopsis: A limbal based conjunctival incision is made. The muscle is identified, separated from its attachments and secured with 6-0 Vicryl. The anterior ciliary vessel supplying it is isolated by making a small snip incision in the muscle capsule with delicate blunt dissection parallel to the anterior ciliary artery The muscle is detached from its original insertion. The muscle is tied to sclera at the intended point of recession. The intact anterior ciliary artery, thus will continue to function, untouched. Highlights: We recommend pre-placing the sutures in the muscle and also in the sclera at the point of reattachment to avoid possible stretching and breaking of anterior ciliary vessels at the time of muscle detachment and also to dissect the artery free from muscle several millimeters more than the intended recession in order to spare the anterior ciliary circulation in strabismus surgery. Video link: https://youtu.be/Bn050Ihu9rU.

Comparison of measurements between manual and automated eyetracking systems in patients with strabismus - A preliminary study.

Purpose: The main objective is to test the measurements made by an automated eye-tracking system in the presence of strabismus and to compare the data with manual measurements of deviation. Methods: A prospective observational cross-sectional masked double-blinded study was conducted in a tertiary eye care center with 39 participants included in our study, aged 3-41 years. Initial screening of all participants was performed by an ophthalmologist. Ocular deviations were evaluated and compared between manual measurements and an automated eye-tracking system. The device is based on eye-tracking technology. The participants had either a congenital or acquired type of manifest or latent strabismus. Children less than 3 years of age, visual acuity <6/36, and abnormal configuration of the anterior segment were excluded from the study. Results: The prism alternate cover test (PACT) manual measurements and the automated alternate cover test for measuring horizontal deviation, the manual measurement, and the automated eye track system showed a highly positive correlation (r = 0.932, P < 0.001). The Bland Altman plot analysis shows good agreement between the two measurements, with the mean difference between the two measurements being 1.55 PD, and the 95% limit of agreement was ± 10 PD. Conclusion: The results obtained with an automated eye-tracking system correlate well with manual strabismus measurements with PACT in terms of diagnosis, precision, and accuracy, with an added benefit of lesser time consumption in performing the test in cooperative/motivated patients. Considering these aspects, patients above the age of 3 years could be assessed with the equipment.

Down syndrome with bilateral posterior lenticonus.

We present a case of bilateral posterior lenticonus in a young boy with Down syndrome. Association of posterior lenticonus in Down syndrome is rarely reported in the literature. We have discussed the clinical features and management of this patient at our hospital.

Intratrochlear steroid injections in acquired Brown syndrome-a case series.

PURPOSE: To present our experience in the treatment of children with acquired Brown syndrome by means of intratrochlear injection of betamethasone. METHODS: The medical records of patients treated with intratrochlear betamethasone in 2016 at the Aravind Eye Hospital, Madurai, were reviewed retrospectively. The following data were collected: pre- and postoperative orthoptic work-up, blood work, and neuroimaging. Betamethasone injection was administered 2-8 weeks following onset of symptoms. RESULTS: Five children (4 girls), 1.5-15 years of age, were included. During the postoperative period, abnormal head posture and elevation in adduction improved in 4 subjects but did not resolve completely. The median vertical deviation was 11.5Δ preoperatively and reduced to 3.5Δ postoperatively. A significant reduction in deviation was demonstrable on diplopia and Hess charting in 2 of the older children. Subject 2, who did not show improvement after injection, was prescribed prism glasses and became diplopia free. CONCLUSIONS: In this case series, children with acquired Brown syndrome of idiopathic or presumed inflammatory etiology showed significant reduction in deviation and symptoms following intratrochlear injection of betamethasone. We recommend that this treatment be considered for children affected by acquired Brown syndrome, especially those in the amblyogenic age group.

Surgical outcome in monocular elevation deficit: a retrospective interventional study.

BACKGROUND AND AIM: Monocular elevation deficiency (MED) is characterized by a unilateral defect in elevation, caused by paretic, restrictive or combined etiology. Treatment of this multifactorial entity is therefore varied. In this study, we performed different surgical procedures in patients of MED and evaluated their outcome, based on ocular alignment, improvement in elevation and binocular functions. STUDY DESIGN: Retrospective interventional study. MATERIALS AND METHODS: Twenty-eight patients were included in this study, from June 2003 to August 2006. Five patients underwent Knapp procedure, with or without horizontal squint surgery, 17 patients had inferior rectus recession, with or without horizontal squint surgery, three patients had combined inferior rectus recession and Knapp procedure and three patients had inferior rectus recession combined with contralateral superior rectus or inferior oblique surgery. The choice of procedure was based on the results of forced duction test (FDT). RESULTS: Forced duction test was positive in 23 cases (82%). Twenty-four of 28 patients (86%) were aligned to within 10 prism diopters. Elevation improved in 10 patients (36%) from no elevation above primary position (-4) to only slight limitation of elevation (-1). Five patients had preoperative binocular vision and none gained it postoperatively. No significant postoperative complications or duction abnormalities were observed during the follow-up period. CONCLUSION: Management of MED depends upon selection of the correct surgical technique based on employing the results of FDT, for a satisfactory outcome.

Pigmented free-floating retrolental space cyst.

We report an unusual case of a free-floating pigmented retrolental cyst, which was diagnosed after examination by slitlamp, B-scan ultrasonography, and ultrasound biomicroscopy. Pigmented cysts, which may arise from the ciliary body epithelium, are embryologically and morphologically different from clear cysts.

Weill-Marchesani syndrome associated with retinitis pigmentosa.

Retinitis pigmentosa (RP) is associated with a wide variety of ocular and systemic disorders. The Weill-Marchesani syndrome is a multi-system disorder with microspherophakia as one of the common manifestations. A 14-year-old girl presented with short stature, short and stubby fingers, hypodontia and low-set ears. Slit-lamp examination revealed microspherophakia, with shallow anterior chambers with irido and phacodonesis. Ultrasonographic biomicroscopy confirmed the clinical findings and revealed hypoplastic ciliary body. Electroretinogram confirmed the diagnosis of RP. Though RP has been associated with ectopia lentis in earlier reports, this is, to the best of our knowledge, the first case report describing the association of RP and Weill-Marchesani syndrome.

Atypical association of Duane retraction syndrome and Bardet Biedl syndrome.

Duane's retraction syndrome (DRS) includes changes in palpebral fissure width along with restriction of ocular motility. Bardet Biedl syndrome (BBS) includes presence of retinitis pigmentosa (RP) with obesity, mental retardation, polydactyly and renal abnormalities. We report a case of rare association of DRS with BBS in a seven-year-old child. The ocular motility examination revealed left DRS with esotropia. Fundus examination revealed findings characteristic of an atypical retinitis pigmentosa. The electro-retinogram waveforms were extinguished both for rods and cones. He was diagnosed as a case of BBS on the basis of the ophthalmological findings plus association with the systemic features of obesity, polydactyly, hypogonadism, mental retardation and renal abnormalities. This case gives further evidence of the fact that BBS may be associated with abnormalities of eye movements.

Pupillary-iris-lens membrane with goniodysgenesis: a case report.

We describe a rare case of pupillary-iris-lens membrane with goniodysgenesis, a unilateral neurocristopathy. The membrane represents ectopic iris on the lens with abnormal iris stroma and anterior chamber angle from aberrant induction, migration or regression of neural crest cells. The membrane can be progressive. Catastrophic vision loss from angle closure can occur and may be controlled with surgery. This subject needed treatment for amblyopia.

Bilateral spontaneous hyphema in juvenile xanthogranuloma.

This report describes a rare occurrence of bilateral, spontaneous, nontraumatic hyphema in a 6 weeks old infant, associated with a small, multiple skin lesions. The diagnosis of juvenile xanthogranuloma was confirmed by histopathological examination of the cutaneous lesions. The hyphaema cleared gradually in 2 weeks time with conservative management.

Double Augmented Vertical Rectus Transposition for Large-Angle Esotropia Due to Sixth Nerve Palsy.

PURPOSE: To study the binocular alignment and ocular motility in patients with large-angle esotropia due to sixth nerve palsy treated with double augmented vertical recti transposition. METHODS: This was a prospective interventional study. Fifteen patients with non-resolving sixth nerve palsy who underwent surgical correction were included in the study. Fourteen patients also underwent an additional medial rectus recession. Two patients with an associated small vertical deviation had a selective augmentation of one vertical rectus muscle. Binocular alignment, ocular motility, duction limitation, improvement in head posture, induced vertical deviations, and field of diplopia-free binocular single vision (when possible) were analyzed. Successful outcome was defined as a residual horizontal deviation of 10 prism diopters (PD) or less with no vertical deviation at final follow-up (6 months). RESULTS: The double augmented Hummelsheim procedure improved esotropia from 58.3 ± 10.8 PD preoperatively to 7.2 ± 5.1 PD postoperatively (P = .001). Three (20%) patients had residual deviation of greater than 10 PD, of which 1 patient had diplopia and was treated with prisms. Postoperative binocular field of vision was performed in 6 patients, the mean of which was 20° for abduction and 45° for adduction. Three of 6 patients had elimination of face turn and the rest had residual head posture of less than 5°. Two patients had an induced vertical deviation of less than 4 PD. In patients who had selective augmentation, the vertical deviation was completely corrected. CONCLUSIONS: The patients operated on with double augmentation of the Hummelsheim procedure combined with medial rectus recession had reduced mean primary esotropia and improved diplopia-free field of vision postoperatively. [J Pediatr Ophthalmol Strabismus. 2016;53(6):369-374.].

Congenital oculomotor nerve palsy due to effects of carotid artery agenesis.

Isolated carotid artery agenesis is not generally recognized as a cause of congenital oculomotor nerve palsy. We report this rare association in 2 children and examine the underlying mechanism.

Mutational screening of LCA genes emphasizing RPE65 in South Indian cohort of patients.

BACKGROUND: Leber congenital amaurosis (LCA) is the most severe form of inherited retinal visual impairment in children. So far, mutations in more than 20 genes have been known to cause LCA and among them, RPE65 is a suitable candidate for gene therapy. The mutational screenings of RPE65 and other LCA genes are requisite in support of emerging gene specific therapy for LCA. Therefore, we have carried out a comprehensive LCA genes screening using a combined approach of direct sequencing and DNA microarray based Asper chip analysis. METHODOLOGY/PRINCIPAL FINDINGS: Thirty clinically diagnosed index LCA cases from Southern India were screened for coding and flanking intronic regions of RPE65 through direct sequencing. Among thirty, 25 cases excluded from RPE65 mutations were subjected to Asper chip analysis, testing 784 known pathogenic variations in 15 major LCA genes. In RPE65 screening, four different pathogenic variations including two novel (c.361insT & c.939T>A) and two known (c.394G>A & c.361delT) mutations were identified in five index cases. In the chip analysis, seven known pathogenic mutations were identified in six index cases, involving genes GUCY2D, RPGRIP1, AIPL1, CRX and IQCB1. Overall, 11 out of 30 LCA cases (36.6%) revealed pathogenic variations with the involvement of RPE65 (16.6%), GUCY2D (10%), RPGRIP1 (3.3%), AIPL1 (3.3%) and CRX & IQCB1 (3.3%). CONCLUSIONS/SIGNIFICANCE: Our study suggests that such combined screening approach is productive and cost-effective for mutation detection and can be applied in Indian LCA cohort for molecular diagnosis and genetic counselling.