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.

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.

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.

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.

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.

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.

Improvement in visual acuity following surgery for correction of head posture in infantile nystagmus syndrome.

PURPOSE: To report the effect of the abnormal head posture (AHP) correcting procedures on the visual acuity improvement in patients with infantile nystagmus syndrome (INS) and the visual acuity improvement outcomes in different AHP correcting surgeries in INS. METHODS: This was a prospective, non-randomized, interventional study. Twenty-eight patients underwent the Anderson-Kestenbaum procedure or the modified Anderson procedure with or without tenotomy of at least one horizontal recti for correction of AHP. Best-corrected binocular null zone acuity and degree of AHP was recorded preoperatively and compared with those done 1 month postoperatively. RESULTS: The average null zone logarithm of the minimum angle of resolution acuity was 0.42 preoperatively, which improved significantly to 0.33 postoperatively (P = .002). The AHP ranged from 10° to 40° (mean: 20.89°), which improved significantly to a mean of 3.21° (P = .000). No significant difference (P = .65) was found in the visual acuity improvement among patients who underwent the Anderson-Kestenbaum procedure or the modified Anderson procedure with or without tenotomy. No significant difference in the visual acuity improvement was seen in patients who underwent tenotomy of at least one horizontal rectus muscle along with the modified Anderson procedure compared to those who underwent the modified Anderson procedure alone (P = .28). CONCLUSION: The procedures used mainly for correction of AHP in INS do yield significant improvement in the visual acuity. This improvement is seen in patients undergoing surgery for both horizontal and vertical AHP.

Bilateral persistent fetal vasculature: a study of 11 cases.

PURPOSE: To describe the clinical and radiologic features of patients with bilateral persistent fetal vasculature (PFV). METHODS: All patients diagnosed with symmetric, bilateral PFV over a 3.5-year period were included in this representative, observational case series. Only patients with more or less symmetrical disease in both eyes were included. Examination under anesthesia was performed in all but 1 case. B-scan ultrasonography of the eye was performed in all cases. A computed tomographic or magnetic resonance imaging scan was done if the ocular ultrasonography was inconclusive. A systemic workup was performed to rule out associated systemic anomalies. RESULTS: The study group included 11 patients. Of the 22 eyes studied, 20 (91%) showed features of both the anterior and the posterior forms of PFV. Two children tested positive for IgM rubella antibodies. Ten eyes (45.5%) had a horizontal corneal diameter of less than 10 mm. Fifteen eyes (68.2%) had cataract at presentation. Intraocular pressure was >21 mm Hg in 17 eyes (77.3%). CONCLUSIONS: Microcornea and cataract were common findings. Bilateral PFV cases were more commonly associated with combined anterior and posterior PFV. Bilateral PFV, although infrequent, should be considered in the differential diagnosis of bilateral leukocoria.

Wildervanck syndrome associated with cleft palate and short stature.

We report a case of Wildervanck syndrome exhibiting Klippel-Feil anomaly, Duane retraction syndrome and deafness. Since the first case was reported in 1952, there have been more reports describing this triad, either complete or incomplete. Our patient had the complete triad of the syndrome along with cleft palate and short stature. Also, a review of the literature regarding this syndrome is presented here.

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.

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.