Challenges in management of congenital enophthalmos due to anomalous accessory orbital extraocular muscle bands.

INTRODUCTION: Congenital enophthalmos is a rare condition characterized by posterior displacement of the globe, often associated with bony orbital anomalies or whole globe development defects. The purpose of this report is to present two unrelated cases of congenital enophthalmos secondary to anomalous accessory orbital bands and to describe characteristics of orbital imaging that differentiate this condition from the other causes. METHODS: The case records of two patients who presented with congenital enophthalmos and were discovered to have anomalous accessory orbital extraocular muscle bands were reviewed. The clinical features, initial diagnosis, high resolution magnetic resonance imaging (MRI) findings, and surgical outcomes were noted. A 3-dimensional reconstruction model was used to understand the approach and surgical management in one of the cases. RESULTS: Both patients presented with unilateral severe enophthalmos, globe retraction, and restricted ocular motility in all directions since birth. High-resolution MRI of the orbits revealed a short anomalous band, isointense to the muscle, arising from a rectus muscle belly and attaching to the posteroinferior part of the globe adjacent to the optic nerve. The caliber of the extraocular muscles and ocular motor nerves was normal. In one patient, surgery was not pursued due to the extreme posterior location of the band with proximity to the optic nerve. In the other patient, the deviation did not improve, despite successfully severing the accessory band, due to extensive scarring. CONCLUSION: Anomalous accessory orbital extraocular muscle bands are a rare and often overlooked cause of congenital enophthalmos when associated with limited ocular motility. Imaging the orbit can aid in diagnosis and help differentiate it from other causes. Safe surgical approaches to address the problem are limited, and available approaches may not be effective. These two cases highlight that the management of accessory extraocular muscle bands causing enophthalmos can be extremely challenging and difficult to improve even with intensive surgical intervention.

Accuracy of Intraocular lens power calculation in pediatric traumatic cataract.

PURPOSE: To evaluate the prediction error (PE) in pediatric traumatic cataract surgery involving primary or secondary intraocular lens implantation (IOL) and the factors affecting it. METHODS: Retrospective data of unilateral traumatic cataract eyes of children aged ≤16 years were collected between February 2019 and March 2022 at a tertiary eye care hospital. Absolute PE was calculated by deducting the target refraction from the observed refraction at 6 weeks postsurgery following suture removal. Simulated PE was calculated in eyes with corneal scar replacing the affected eye keratometry (K) with the K of the fellow eye and standard K (44D) and was then compared with absolute PE. RESULTS: Fifty children with a mean age of 9.5 years (2-16 years) were included. Mean absolute PE was 1.63 ± 1.8D (0 to 9D). Absolute PE was not affected by the age at surgery, method of biometry, duration of injury, type of cataract surgery, position of IOL, and preoperative keratometry. The absolute PE was affected by axial length (AXL) being <1.5D in AXL of 22.73 ± 0.84 mm, <1.5-2D in AXL of 22.07 ± 0.61 mm, and >2D in AXL of 22.01 ± 0.74 mm (P = 0.039) in univariate analysis. In multivariate analysis, none of the factors affected the absolute PE. In 34 eyes with corneal scar, higher variability in PE was observed. The standard K resulted in greater simulated PE as compared to the affected eye average K in eyes with scar involving the visual axis. CONCLUSION: Absolute PE following pediatric traumatic cataract was studied. It was higher in shorter AXLs. In corneal scar involving the visual axis, using the fellow eye K yielded lesser simulated PE as compared to standard K.

Efficacy of anti-suppression therapy in improving binocular vision in children with small-angle Esotropia.

PURPOSE: To evaluate the efficacy of anti-suppression exercises in children with small-angle esotropia in achieving binocular vision. METHODS: A retrospective review of patients aged 3-8 years who underwent anti-suppression exercises for either monocular or alternate suppression between January 2016 and December 2021 was conducted. Patients with esotropia less than 15 prism diopters (PD) and visual acuity ≥ 6/12 were included. Patients with previous intra-ocular surgery or less than three-month follow-up were excluded. Success was defined as the development of binocular single vision (BSV) for distance, near, or both (measured clinically with either the 4 prism base out test or Worth four dot test) and maintained at two consecutive visits. Qualified success was defined as the presence of diplopia response for both distance and near. Additionally, improvement in near stereo acuity was measured using the Stereo Fly test. RESULTS: Eighteen patients with a mean age of 5.4 ± 1.38 years (range 3-8 years) at the time of initiation of exercises were included in the study. The male female ratio was 10:8. The mean best corrected visual acuity was 0.18 LogMAR unit(s) and the mean spherical equivalent was +3.8 ± 0.14 diopters (D). The etiology of the esotropia was fully accommodative refractive esotropia (8), microtropia (1), post-operative infantile esotropia (4), partially accommodative esotropia (1), and post-operative partially accommodative esotropia (4). Patients received either office-based, home-based, or both modes of treatment for an average duration of 4.8 months (range 3-8). After therapy, BSV was achieved for either distance or near in 66.6 % of patients (95 % CI = 40.03-93.31 %). Binocular single vision for both distance and near was seen in 50 % of children. Qualified success was observed in 38.46% of patients. Persistence of suppression was observed in one patient (5.5 %). Near stereopsis improved to 200 s of arc or more in 60% of the patients. The mean esotropia reduced from 5.7 ± 4.0 PD for distance and 6.2 ± 4.66 PD for near to 2.7 ± 2.4 PD and 3.38 ± 4.7 PD respectively, at the last follow-up (p-value 0.004 and 0.006). Failure of therapy was noticed after six months of follow-up in the child with infantile esotropia. CONCLUSION: Anti-suppression exercises may be beneficial to improve binocular vision functions in children with small-angle esotropias of variable etiologies. Recurrence of suppression after cessation of therapy is possible, warranting regular follow up.

Acquired comitant esotropias - comparison of surgical outcomes of accommodative vs non-accommodative types.

Purpose: To compare the motor and sensory outcomes of strabismus surgery and the factors affecting surgical success in acquired acute non-accomodative esotropia (ANAET) and partially accommodative refractive esotropia (pARET). Methods: A retrospective chart review of patients with ANAET and pARET who underwent unilateral or bilateral horizontal rectus muscle surgery between January 2020 and December 2021 was conducted. Patients with postoperative follow-up of at least six weeks were included. Patients with pattern deviation, lateral incomitance, and near-distance disparity were excluded. Motor success was defined as a postoperative deviation within eight prism diopters of orthophoria. Sensory success was defined as presence of binocular single vision (BSV) for both distance and near (Worth four dot test). The effect of factors like age at onset, age at surgery, amblyopia before surgery, duration of squint before treatment, presence or absence and magnitude of vertical deviation, preoperative angle of deviation, and spherical equivalent on the motor and sensory success in each group were analyzed and compared. Results: 38 patients with ANAET and 33 patients with pARET were included. The mean age of onset of esotropia was 8.55 ± 4.65 years and 4.39 ± 2.27 years (p < .001) and the mean age at surgery was 10.62 ± 4.99 years and 7.89 ± 2.84 years (p = .006) in the ANAET and the pARET group respectively. The mean duration of the final follow-up was 38.51 weeks in the ANAET and 48.68 weeks in the pARET group (p = .089). Patients were successfully aligned for both distance and near in 81.5% of patients in the ANAET and 78.9% of patients in the pARET group at the final follow-up (p. 0.775). A BSV for both distance and near at the final follow-up was seen in 81.2% vs 66.6% of patients in the ANAET and the pARET group respectively (p = .25). A good near stereoacuity (<120 arcsecs) was seen in 60.6% and 41.9% of the ANAET and the pARET groups respectively (p = .175). The percentage of patients in the ANAET group who had orthophoria, any esodeviation, and any exodeviation for distance at the final follow-up was 63.1%, 34.2% and, 2.6%. The percentage of patients in the pARET group in similar categories was 36.3%, 42.4% and, 21.2%. None of the demographic and preoperative factors were found to affect the surgical outcomes in the two groups. Conclusions: The motor and sensory outcomes were similar in the two groups. A higher proportion of ANAET patients remained orthophoric during the follow-up. The patients in the pARET group showed a tendency toward exodrift.

Occurrence and subsequent development of vertical deviations in patients treated surgically for infantile esotropia.

Purpose: The purpose of this study was to evaluate the onset of dissociated vertical deviation (DVD) and inferior oblique overaction (IOOA), their subsequent development, and their correlation with pre and postoperative parameters. Methods: Medical records of patients with infantile esotropia who underwent surgery between 2005 and 2017 were retrospectively reviewed. DVD and IOOA were measured before and after surgery. Patients were divided into two groups based on horizontal and vertical deviation at the time of presentation: those with infantile esotropia only (group A) and patients with infantile esotropia who developed vertical deviation (group B). Results: Out of a total of 102 patients, DVD occurrence was seen in 53 patients (51.9%) and IOOA was seen in 50 patients (48.04%). DVD was seen in 22 patients at the time of initial examination and in 31 patients postoperatively. IOOA at presentation was seen in 45 patients (44.1%) and 5 patients (8.8%) postoperatively. No statistical difference was found in the age of surgery, angle of deviation, mean follow-up, and mean refractive error within both groups. The postoperative motor outcome was statistically comparable between the two groups (P = 0.29). Sensory outcomes of fusion (P = 0.048) and stereopsis (P-value = 0.00063) were better in group A. Conclusion: No correlation was found between the age of occurrence and development of vertical deviation with refractive error, angle of deviation, age, or type of surgery. We found that motor outcomes are not affected but sensory outcomes are affected in patients with vertical deviations. This indicates that DVD and IOOA are developed due to inherent disruption of fusion and stereopsis.

Results of fusional vergence therapy in managing consecutive esotropia: A case series.

Purpose: To analyze the efficacy of fusional vergence therapy (FVT) in management of consecutive esotropia with diplopia after intermittent exotropia (IXT) surgery. The current study is carried on how FVT affects the duration of treatment, sensory fusion, and exotropic drift. Methods: This was a retrospective study for the medical record of 11 patients with consecutive esotropia after IXT surgery of 543 patients over the period of 5 year, with mean surgery age of 9.5 (range: 4-33 y). FVT was planned after minimum 6 weeks of surgery and was considered for maximum 24 weeks. Patients underwent a combination of office-based and home-based FVT. Successful outcome of therapy was considered where diplopia resolves in free space and achieves sensory fusion, stereopsis with no manifest deviation. Results: Record of 543 patients who had horizontal muscle surgery for IXT were identified and reviewed. Records of 11 patients who showed consecutive esotropia of 10 prism diopter (PD) or more with normal retinal correspondence, with or without diplopia complaint, after 6 week of surgery and had undergone vision therapy management were reviewed. A successful outcome of binocular single vision with good sensory and motor fusion with no manifest deviation or prism requirement was achieved with in the mean duration of 4.8 month of therapy. With a mean duration of 4 weeks of therapy, the mean angle of deviation reduced by 53% for distance (17 PD to 8 PD) and 27% for near (11 PD to 8 PD) and mean stereopsis improvement by 80% with 94% patients demonstrating sensory fusion on Bagolini test and 94% of patients having no symptoms of diplopia or squint. Conclusion: With nonsurgical management involving refractive error correction, FVT, and prism, consecutive esotropia was resolved in 74% cases. Management of consecutive esotropia with FVT can result in satisfactory sensory fusion and successful motor alignment.

Correction of horizontal and torsional compensatory head posture in infantile nystagmus syndrome using horizontal rectus muscle recession and resection with vertical transposition.

Infantile nystagmus syndrome (INS) is often characterized by an identifiable null zone. When the null zone is not in the straight-ahead gaze, a compensatory head posture (CHP) is adopted by the patient to achieve best possible vision. Various surgical procedures have been recommended to correct a CHP which is clinically predominant in one dimension of yaw (lateral rotation), pitch (anterior or posterior flexion/extension) or roll (lateral flexion). However, the presence of a complex CHP which is clinically evident in more than one dimension, warrants either a combination of multiple techniques or a stepwise approach. We report the case of a 26-year-old male with INS with an eccentric null and a multi-dimensional complex CHP of 30º left face turn, 20º right head tilt and 10º chin depression. The patient was managed by all four horizontal rectus muscle recession and resection with full tendon vertical transposition to address the face turn and head tilt. He underwent lateral rectus muscle (LR) recession with upward transposition and medial rectus muscle (MR) resection with downward transposition in the right eye. MR recession with upward transposition and LR resection with downward transposition were performed in the left eye. Postoperatively. the head posture improved significantly for both distance and near viewing. The chin depression also reduced after the procedure. He developed transient diplopia due to a small vertical deviation after the surgery, which was managed by prisms and fusional exercises. Thus, horizontal rectus muscle recession and resection combined with vertical transposition may be helpful to simultaneously improve the head tilt associated with the face turn, obviating the need for vertical rectus muscle or oblique muscle surgery.

Human (but not animal) motion can be recognized at first sight - After treatment for congenital blindness.

The long-standing nativist vs. empiricist debate asks a foundational question in epistemology - does our knowledge arise through experience or is it available innately? Studies that probe the sensitivity of newborns and patients recovering from congenital blindness are central in informing this dialogue. One of the most robust sensitivities our visual system possesses is to 'biological motion' - the movement patterns of humans and other vertebrates. Various biological motion perception skills (such as distinguishing between movement of human and non-human animals, or between upright and inverted human movement) become evident within the first months of life. The mechanisms of acquiring these capabilities, and specifically the contribution of visual experience to their development, are still under debate. We had the opportunity to directly examine the role of visual experience in biological motion perception, by testing what level of sensitivity is present immediately upon onset of sight following years of congenital visual deprivation. Two congenitally blind patients who underwent sight-restorative cataract-removal surgery late in life (at the ages of 7 and 20 years) were tested before and after sight restoration. The patients were shown displays of walking humans, pigeons, and cats, and asked to describe what they saw. Visual recognition of movement patterns emerged immediately upon eye-opening following surgery, when the patients spontaneously began to identify human, but not animal, biological motion. This recognition ability was evident contemporaneously for upright and inverted human displays. These findings suggest that visual recognition of human motion patterns may not critically depend on visual experience, as it was evident upon first exposure to un-obstructed sight in patients with very limited prior visual exposure, and furthermore, was not limited to the typical (upright) orientation of humans in real-life settings.

Development of Visual Memory Capacity Following Early-Onset and Extended Blindness.

It is unknown whether visual memory capacity can develop if onset of pattern vision is delayed for several years following birth. We had an opportunity to address this question through our work with an unusual population of 12 congenitally blind individuals ranging in age from 8 to 22 years. After providing them with sight surgery, we longitudinally evaluated their visual memory capacity using an image-memorization task. Our findings revealed poor visual memory capacity soon after surgery but significant improvement in subsequent months. Although there may be limits to this improvement, performance 1 year after surgery was found to be comparable with that of control participants with matched visual acuity. These findings provide evidence for plasticity of visual memory mechanisms into late childhood but do not rule out vulnerability to early deprivation. Our computational simulations suggest that a potential mechanism to account for changes in memory performance may be progressive representational elaboration in image encoding.

A rare association of blepharophimosis-ptosis-epicanthus inversus case with congenital nasolacrimal duct obstruction.

PURPOSE: The aim of reporting this case is to describe a rare combination of blepharophimosis-ptosis-epicanthus inversus syndrome with congenital nasolacrimal duct obstruction. A variety of lacrimal anomalies have been seen in blepharophimosis-ptosis-epicanthus inversus syndrome but the occurrence of nasolacrimal duct obstruction is rare. METHOD: The blepharophimosis-ptosis-epicanthus inversus syndrome is an autosomal dominant rare genetic defect with clinical manifestation of dysplasia of the eyelids, palpebral fissures, flat nasal bridge, and ptosis. A 20-month-old boy was referred with the complaints of watering and discharge from his right eyes since birth. On examination, the child had all the features of blepharophimosis-ptosis-epicanthus inversus syndrome with right congenital nasolacrimal duct obstruction in line with the published reports. RESULT: On endoscopic probing and irrigation, the probe could not be visualized into the inferior meatus. On dacryoendoscopy, the membranous part of the nasolacrimal duct was found to be completely obliterated with no light transmission into the nose indicating a malformed nasolacrimal duct. The child was managed by endoscopic dacryocystorhinostomy. We could find only one case report published so far on the combination of congenital nasolacrimal duct obstruction with blepharophimosis-ptosis-epicanthus inversus syndrome. This study adds one more case of blepharophimosis-ptosis-epicanthus inversus syndrome with congenital nasolacrimal duct obstruction and adjuvant use of dacryoendoscopy.

Lateral Rectus Muscle Injury During Double-Headed Pterygium Surgery: A Rare Complication.

A 43-year-old man developed double vision, esotropia, and abduction limitation after excision of a doubleheaded pterygium. Exploration during strabismus surgery 4 months later revealed a partially severed lateral rectus muscle and extensive conjunctival scarring. Direct injury to the lateral rectus muscle during pterygium surgery is extremely rare. Prevention and management strategies are discussed. [J Pediatr Ophthalmol Strabismus. 2020;57:e96-e99.].

Necrotizing scleritis after strabismus surgery in Treacher Collins syndrome.

Objective: To describe a case of surgically induced scleral necrosis in Treacher Collins syndrome after strabismus surgery. Methods: A 19-year-old girl underwent bilateral squint surgery. Two weeks postoperatively, she presented with subconjunctival abscess in the left eye. The surrounding conjunctiva was markedly inflamed with raised edges. Surgical debridement, microbiological evaluation and medical management were started immediately. Screening for autoimmune and vasculitic conditions did not provide any positive results. Results: On subsequent follow-up, conjunctival retraction and an area of scleral necrosis with thinning was noted. Significant healing with antibiotics and steroids was noted within one week. The integrity of the globe was well maintained and no further procedure for tectonic support was performed. Conclusion: Surgically induced scleral necrosis can be immune-mediated or following surgical site infection. Pre-existing scleral thinning due to neuroectodermal apoptosis in Treacher Collins syndrome remains a possible explanation for the accelerated necrotising scleritis in our case.

Extra-large V pattern in exotropia: a rare case and its management.

V pattern arises when the eyes diverge more in up-gaze, as compared to down-gaze, leading to vertical gaze incomitance of a horizontal strabismus. In most cases, the magnitude of V pattern is small to moderate, and a single surgical procedure for the same is enough to correct it. We describe a rare case where an extra-large V pattern (>60 Prism Dioptres (PD)) was present along with large exotropia (70 PD). Craniosynostosis or facial asymmetry was not present. The extra-ocular motility evaluation revealed mild over-elevation in adduction and extreme divergence in up-gaze. Examination of the fundus showed ex-cyclotorsion of both the globes. The V pattern was managed by bilateral inferior oblique muscle recession and full thickness upshift of both lateral rectus muscles. The exotropia was treated by bilateral lateral rectus recession and right eye medial rectus resection. Postoperatively, both the exodeviation and V pattern reduced to less than 10PD. The ex-cyclotorsion was also reduced after the surgery. There was restoration of binocularity and the near stereopsis improved to 170 secs of arc. Thus, extra-large V pattern strabismus may need a combination of procedures to achieve a good motor and sensory outcome.

Subconjunctival silicone oil complicating strabismus surgery.

Purpose: To report a case of an otherwise simple strabismus surgery that became complex due to the presence of extensive subconjunctival silicone oil. Methods: A 15-year-old boy underwent strabismus surgery for sensory exotropia. He had undergone two prior vitreoretinal surgeries for traumatic endophthalmitis and retinal detachment, respectively. The second procedure involved injection of silicone oil as an endotamponade. During the strabismus surgery, subconjunctival nodules filled with silicone oil were noted. This made the surgery difficult due to episcleral fibrosis. The multiple silicone oil cysts were removed by rupturing or resecting them along with the tenon's fascia. The extraocular muscles were meticulously isolated and operated using adjustable suture technique. Results: Following surgery, the strabismus was corrected satisfactorily and the patient was relieved of his ocular discomfort and congestion. Conclusions: Subconjunctival leakage of silicone oil can lead to capacious inflammation and complicate strabismus surgery manifold.

Bilateral Symmetric and Asymmetric Superior Rectus Recession for Patients with Dissociated Vertical Deviation.

PURPOSE: To evaluate the outcome of bilateral symmetric and asymmetric superior rectus (SR) recessions in patients with bilateral dissociated vertical deviation (DVD) without oblique dysfunction and determine factors that might influence the surgical outcome. DESIGN: Retrospective study. METHODS: All patients who underwent bilateral SR recession for bilateral DVD from January 2012 to December 2016 at an eye hospital in New Delhi, India were included. Indication for surgery was decompensated DVD in 1 or both eyes. Symmetric SR recession was performed for symmetric DVD and asymmetric SR recession was performed for asymmetric DVD of 10 prism diopters (PD) or more. Patients with a minimum follow-up of 6 months were included. Success was defined as absence of manifest DVD in both eyes at the final postoperative visit. RESULTS: Medical records of 27 patients were analyzed. Their median age at surgery was 8 years [interquartile range (IQR), 6-10 years] and mean follow-up was 16.3 months (range, 6-48 months). Symmetric and asymmetric surgeries were performed in 19 and 8 patients, respectively. The amount of SR recession performed ranged from 5 to 10 mm. The median DVD reduced from 9 PD (IQR, 6-14 PD) to 5 PD (IQR, 3-8 PD) in the right eye (P = 0.015) and 9 PD (IQR, 7-12 PD) to 6 PD (IQR, 3-10 PD) in the left eye (P = 0.016) after surgery. Successful outcome was seen in 63% of patients. There was no difference in successful outcome with respect to age, sex, preoperative horizontal deviation, preoperative vertical deviation, postoperative residual horizontal deviation, surgical technique, asymmetry of SR recession, and magnitude of preoperative DVD. Patients with a preference for fixation were more likely to have a successful outcome. CONCLUSIONS: Bilateral SR recession resulted in a success rate of 63% after single operation for bilateral DVD without oblique dysfunction. We found that the probability of a successful outcome did not depend on age at surgery, sex, preoperative horizontal or vertical deviation, magnitude of preoperative DVD, symmetry of SR recession, or surgical technique.