A Case of Superior Oblique Palsy without Brown Syndrome Induced by a Dog Bite.

BACKGROUND Pseudo-Brown syndrome is characterized by dysfunction of the superior oblique tendon-trochlear complex. Canine tooth syndrome, which involves superior oblique palsy with pseudo-Brown syndrome, results from damage to the trochlear and superior oblique tendon from dog bites around the eye. This report describes a variant of canine tooth syndrome without pseudo-Brown syndrome following a dog bite around the left upper eyelid. In this case, magnetic resonance imaging (MRI) facilitated early diagnosis and therapeutic intervention. CASE REPORT A 19-year-old man presented with torsional diplopia following a dog bite around the left upper eyelid and forehead. Five days after the injury, an alternate prism cover test revealed 6 prism diopters (Δ) exotropia and 5Δ left hypertropia. Ocular motility showed no significant limitation in elevation or depression during adduction. MRI performed on the same day showed a high-signal area extending from the superior oblique tendon to the trochlear region and the superior oblique muscle belly of the left eye. A diagnosis of canine tooth syndrome without pseudo-Brown syndrome was made and oral steroids were administered. Ocular alignment did not improve, so left inferior oblique myotomy was performed 7 months after the injury. The patient's cyclovertical diplopia resolved postoperatively. CONCLUSIONS Dog bites around the eye can result in abnormalities of the extraocular muscles. Early MRI may be useful for diagnosis and determining treatment strategies. This report has highlighted the importance of rapid assessment and management of patients with dog bites involving the eye.

Effect of vertical central plication on vertical deviations.

PURPOSE: To evaluate the surgical effect of the Wright central plication on vertical rectus muscles to correct vertical strabismus. METHODS: In this multicenter, retrospective, observational outcomes study, data were collected from two surgeons in different practice settings (2017-22). All patients who underwent vertical rectus central plication were included; those undergoing any concurrent strabismus surgery for vertical strabismus were excluded. Primary outcome was amount of strabismus correction in prism diopters per vertical rectus central plication. Secondary outcome was to determine factors associated with better or worse surgical outcomes and patient and patient responses. Data were analyzed using descriptive and bivariate statistics. RESULTS: A total of 36 patients were included. Mean age was 60 years. Mean follow-up was 8.4 months. Of the 36 patients, 11 (31%) had idiopathic strabismus, and 7 (19%) had congenital superior oblique palsy. The remainder had a history that included prior ocular surgery, trauma, and Brown syndrome; 16 (44 %) had prior strabismus surgery. Of 31 patients with preoperative diplopia, 23 (74%) had postoperative resolution of diplopia, and 10 of 16 patients with preoperative prisms (63%) no longer required prisms postoperatively. Mean vertical deviation change was 4.7Δ. Subgroup analysis removing patients with congenital superior oblique palsy showed a larger response of 5.5Δ. 78% of patients had a final deviation <5Δ. No complications or induced postoperative diplopia was reported. CONCLUSIONS: In our study cohort, vertical rectus central plication corrected approximately 5Δ (range, 4.5Δ-5.5Δ) of vertical strabismus due to a variety of causes.

Ophthalmic sequelae of prematurity in late childhood and adulthood: A review.

Advances in the care of premature infants have resulted in unprecedented rates of survival of these infants into adulthood, including those born at very low gestational ages. Ophthalmologists have historically followed premature infants to assess for the presence of and potential need for treatment of retinopathy of prematurity. However, a growing body of literature suggests that the ophthalmic consequences of prematurity extended beyond retinopathy of prematurity and that ophthalmic sequelae of prematurity can endure through adulthood even among formerly preterm adults who were never diagnosed with retinopathy of prematurity. These abnormalities can include a range of both anterior segment and posterior segment sequelae, including higher rates of corneal aberrations, ocular hypertension, strabismus, foveal anomalies, and retinal tears and detachments. This review aims to summarise this literature, underscoring the importance of lifelong examinations and regular monitoring for these complications among adults who were born prematurely.

The influence of orbital architecture on strabismus in craniosynostosis.

PURPOSE: To better characterize the correlation of bony orbital dysmorphology with strabismus in craniosynostosis. METHODS: The medical records of patients with craniosynostosis with and without strabismus seen at Rady Children's Hospital (San Diego, CA) from March 2020 to January 2022 were reviewed retrospectively in this masked, case-control study. Computed tomography scans of the orbits were analyzed to obtain dimensions of the orbital entrance and orbital cone. Primary outcome was correlation of strabismus with orbital measurements. RESULTS: A total of 30 orbits from 15 patients with strabismus and 15 controls were included. Craniofacial disorders included in the study were nonsyndromic craniosynostosis (63%), Crouzon syndrome (13%), Apert syndrome (13%), and Pfeiffer syndrome (10%). Orbital index (height:width ratio) (P = 0.01) and medial orbital wall angle (P = 0.04) were found to differ significantly between the strabismus and control groups. CONCLUSIONS: In our small cohort, bony orbital dimensions, including the ratio of orbital height to width and bowing of the medial orbital wall, were associated with strabismus in craniosynostosis.

An unexpected turn: a case of torsional anomalous retinal correspondence after myectomy of previously anteriorized inferior oblique muscles in a child.

We report a case of torsional diplopia caused by presumed torsional anomalous retinal correspondence after myectomy of previously asymmetrically anteriorized inferior oblique muscles for inferior oblique overaction. Given this patient's experience, it may be prudent to operate with caution on previously anteriorized inferior oblique muscles, especially when anteriorization is performed at a very young age.

"Strabismus in children" is not simple - bilateral persistent hyperplasia primary vitreous: a case report.

Persistent hyperplastic primary vitreous (PHPV) is a rare congenital developmental ocular disorder caused by incomplete regression of the embryonic hyaloid vasculature; bilateral presentations are even rarer. We report a 6-year-old child with bilateral PHPV who visited our hospital for strabismus, without exhibiting leukocoria, microphthalmia, and systemic diseases. These unique characteristics distinguish our case from other cases of PHPV. It is crucial to increase awareness of congenital eye disease in children and the importance of performing fundus examination with the pupils dilated.

The Frequency and Manifestations of Ocular Causes of Abnormal Head Posture.

PURPOSE: To determine the frequency and manifestations of different ocular causes of abnormal head posture (AHP). METHOD: This prospective, consecutive case series study was performed on 149 patients with ocular AHP at Farabi hospital, Iran, from February 2020 to June 2021. All patients underwent routine ophthalmic examinations. The manifestation of AHP was determined by direct observation from three viewing angles, while the patient read the smallest line on the vision chart that they could see. In front, above, and lateral gazes, observations were performed to find head tilt, head turn, and chin abnormal position, respectively. A picture with habitual AHP was taken from all patients. The amount of head tilt was measured by calculating the angle between the line that connects the lips center to the center of the eyebrows and the vertical line using the Corel Draw X7 computer software. RESULTS: The mean age of 149 patients with ocular AHP [101 (67.8%) males and 48 (32.2%) females] was 16.2 ± 12.2 (range, 2-57) years. The most common ocular sources of AHP were found to be superior oblique palsy (SOP) in 66 (44.3%) patients, 54 (36.2%) cases with Duane's retraction syndrome (DRS), and 12 (8.1%) patients with nystagmus. Other frequent causes of ocular AHP were dissociated vertical deviation (DVD) in 5 (3.4%), A and V pattern strabismus in 3 (2.0%), and 2 cases (1.3%) in each of Brown syndrome, inferior rectus (IR) palsy, and congenital fibrosis of the extraocular muscles (CFEOM). The most common manifestations of AHP in all cases were "pure head turn" (48.3%), followed by "pure head tilt" (24.8%), "simultaneous head tilt and head turn" (20.8%), and "chin up" (6.0%). The mean head tilt among all patients with head tilt was 10.4° ± 8.9° (range, 5.0°-31.7°). CONCLUSION: The most frequent ocular sources of AHP were SOP, DRS, and nystagmus, followed by DVD, A and V pattern strabismus, IR palsy, CFEOM, and Brown syndrome. In addition, pure head turn and pure head tilt were the most common manifestations of ocular AHP but were not always seen in the same direction or combination as previously reported with these etiologies.

A patient with congenitally restrictive strabismus and contralateral secondary muscle enlargement.

AIM: To report the case of a congenital restrictive strabismus with a contralateral enlargement of extraocular muscles. CASE: The clinical presentation, findings, and postoperative outcomes of a 4 years old boy referred to evaluation for presenting a left eye deviation since birth are detailed. CONCLUSION: A unilateral congenital restrictive strabismus (congenital hypotropia and esotropia) can be the cause of contralateral secondary enlarged extraocular muscle and It must be included in the differential diagnosis.

The effect of previous orbital decompression on outcomes of rectus muscle recession surgery in patients with thyroid ophthalmopathy.

PURPOSE: To evaluate the effect of previous orbital decompression on outcomes of rectus muscle recession surgery in patients with thyroid-associated ophthalmopathy. METHODS: This retrospective study enrolled 55 eyes of 33 patients treated in our hospital for restrictive strabismus caused by thyroid-associated ophthalmopathy. We performed muscle recession for the obviously restricted extraocular muscles, with 6 weeks of follow-up. Surgical outcomes were compared between the orbital decompression group (DG, n=15) and non-orbital decompression group (NDG, n=18). RESULTS: A total of 33 patients with Graves' ophthalmopathy who underwent rectus muscle recession surgery were included. Of these, 15 patients had undergone orbital decompression prior to strabismus surgery, and 18 had not. The two groups did not differ in terms of the preoperative horizontal or vertical ocular deviation, degree of restriction of eye movement, degree of diplopia, or mean number of muscles that underwent surgery (P>0.05). There was no significant difference in the preoperative horizontal or vertical ocular deviation, level of eye movement restriction, degree of diplopia and the success rate of the surgery (P>0.05). CONCLUSION: Rectus muscle recession surgery in patients with thyroid-associated ophthalmopathy during the quiescent period could improve the ocular deviation and diplopia, and orbital decompression performed before strabismus surgery had no significant effect on surgical technique or outcomes of rectus muscle recession surgery.

Relationship between strabismus associated with Angelman syndrome and orbital anomaly.

PURPOSE: To investigate the relationship between the details of strabismus and orbital abnormalities determined by ocular motility tests and orbital imaging examinations in 9 cases with Angelman syndrome (AS). STUDY DESIGN: A retrospective, clinical report. METHODS: The 9 AS cases (mean age at initial visit: 4.6 ± 8.0 years) were confirmed by genetic diagnosis of the chromosome 15q11-13 region. In all cases, axial imaging of the orbit in the transverse plane of the horizontal extraocular muscles was obtained. The opening angle between both lateral walls of the orbit (greater wing of sphenoid) was measured as the biorbital angle, and compared with the 95% confidence interval of the orbital angle in normal children. RESULTS: All cases had exotropia with means of the distance and near of angle 32.2 prism diopters (Δ) ± 9.7Δ and 32.8Δ ± 8.3Δ. The mean of the biorbital angle was 107.7° ± 7.6°, greater than the biorbital angle of 94.3° ± 5.1° previously reported in 129 normal children (P < 0.0001, t-test). Except for one biorbital angle of 93° in the 25-year-old patient, all the biorbital angles in the 8 children were larger than the upper 95% confidence interval in normal children. Astigmatic and hyperopic ametropic amblyopia were detected in 3 cases and 1 case, respectively. CONCLUSIONS: The frequency of exotropia in AS is higher than previously reported, with our results strongly suggesting that the enlarged biorbital angle is related to the pathogenesis of exotropia in AS.

Yokoyama procedure for cyclic strabismus with axial high myopia:a case report.

BACKGROUND: It is a very rare form of ocular motility characterized by alternating strabismus and orthotropia. We report a patient with a 48-h cycle of esohypotropia associated with axial high myopia that resolved by Yokoyama procedure. CASE PRESENTATION: A 43-year-old female patient who underwent left medial rectus muscle recession and lateral rectus muscle resection elsewhere due to highly myopic strabismus 2 years ago. The patient experienced a recurrence of left esohypotropia 12 months after undergoing surgery, exhibiting a 48-hour cycle. The cycle is one full day of esohypotropia and one day of orthotropia. The patient exhibited a case of high myopia in the left eye, characterized by a diopter measurement of -24.00DS and an eye axis measurement of 28.43 mm. Orbital CT showed supertemporal dislocation of the posterior portion of the elongated globe out from the muscle cone. Based on these observations, we performed Yokoyama procedure by uniting the muscle bellies of the superior rectus(SR) and lateral rectus (LR) muscles to restoring the dislocated globe back into the muscle cone. CONCLUSIONS: When cyclic strabismus is combined with axial high myopia, the Yokoyama procedure was effective and cycles are successfully terminated without overcorrection on no squint days. The purpose of this procedure is to put the dislocated globe back into its muscle cone by uniting the muscle bellies of the superior rectus and lateral rectus.

Ocular abnormalities in children with developmental delay.

Purpose: To describe the presence of ocular abnormalities in children with developmental delay (DD) and compare with normal children; to analyze associated risk factors, systemic problems, and the possible treatment that can be delivered. Methods: This was a cross sectional, observational study. We included children between one and 18 years, diagnosed as developmental delay in DD group, and next immediate age- and sex-matched children without developmental delay on the same day or during the same period in the control group. Detailed history and neuroimaging findings were noted. Uncorrected visual acuity, best-corrected visual acuity for distance and near, cycloplegic refraction, anterior, and posterior segment examination was carried out. Various ocular problems, delayed visual maturation (DVM), and cortical visual impairment (CVI) were diagnosed based on examination. Data were analyzed statistically, and P value <0.05 was considered as statistically significant. Results: Ninety-four children were included in each group. Mean age was 4.97 ± 3.84 years, and 64.89% were males. In DD group: Most common abnormal neuroimaging finding was gliotic changes; systemic associations: 39 children; 83 children had ocular problems: refractive error-70, strabismus-39, cataract-five, amblyopia-16; DVM-13; CVI-13 children; glasses and vision stimulation were advised in 39 and 65 children, respectively; whereas, in control group: refractive error-36, strabismus-15, cataract-two; amblyopia-20 children. Conclusion: 88.29% of developmental delay children had ocular abnormalities, commonest was refractive error (74.47%); these values were higher than in control group; common risk factors were low birthweight and consanguineous marriage; epilepsy was the most common systemic association.

Inferior rectus muscle Y splitting procedure for near-distance hypotropia disparity: an alternative to the Faden procedure.

A young male patient with unilateral pathological myopia complained of selective hypotropia for distance with exophoria. Due to large globe, a notable amount of inferior scleral show was evident. In this scenario, extensive dissection along inferior rectus muscle to perform a Faden procedure would have worsened the inferior scleral show. Thus, as an alternative, the anterior procedure, that is, "inferior rectus muscle Y-splitting procedure with 4-mm recession" was undertaken. At the end of 3 months follow up, the troublesome selective hypotropia for distance disappeared significantly with very little changes along the lower lid position. Therefore, in presence of specific anatomical limitations, the inferior rectus muscle Y splitting procedure can be considered in lieu of Faden procedure for near-distance hypotropia disparity.

Strabismus in Retinopathy of Prematurity: Risk Factors and the Effect of Macular Ectopia.

Objectives: This study aimed to examine factors associated with strabismus in patients with retinopathy of prematurity (ROP) and the relationship between strabismus and macular ectopia. Materials and Methods: Patients with ROP were divided into three groups: Group 1, patients with spontaneous regression (n=45); Group 2, patients who received laser treatment (n=70); and Group 3, patients who underwent surgical treatment (n=91). Rates of anisometropia, amblyopia, nystagmus, macular ectopia, and retinal pathologies were evaluated and their impacts on strabismus development were determined. Disc-to-fovea distance (DFD) was measured from color fundus photographs and the correlation of macular ectopia with severity of strabismus was evaluated. Results: A total of 206 patients were included. Rates of anisometropia, amblyopia, nystagmus, macular ectopia, retinal pathologies causing vision loss, and strabismus were higher in Group 3 (p=0.0001) and correlated with higher stages of ROP (p=0.0001). Macular ectopia (p=0.005), retinal pathologies (p=0.005), and amblyopia (p=0.012) had the strongest impact on strabismus development in ROP patients. DFD and strabismus severity were not significantly correlated (p=0.364). Mean visual acuity (VA) was significantly higher in orthophoric patients compared to those with esotropia and exotropia (p=0.027). Esotropic patients had lower VA compared to patients with exotropia, but this finding was not statistically significant (p=0.729). Conclusion: Presence of macular ectopia, retinal pathologies, and amblyopia were the most strongly correlated risk factors for strabismus development in ROP patients. DFD was not associated with severity of strabismus. Exotropia was mostly related to higher DFD and a possible relationship between esotropia and lower VA was observed.

Supraequatorial displacement with lateral rectus myopexy for treatment of myopic sagging/heavy eye.

PURPOSE: To describe the outcome of the patients diagnosed of sagging/heavy eye associated to myopia, that were operated on with the supra-equatorial displacement with LR myopexy. METHODS: A retrospective study of 9 cases between 2017-2023. The following data were analyzed: horizontal and vertical deviation, diplopia, amblyopia, ductions, ocular torsion, sensorial test, macular pathology, and the orbital magnetic resonance. Treatment was considered Successful if the diplopia was improved or eliminated and a final vertical deviation (VD) ≤5 prism diopters (PD). RESULTS: The mean age (SD) was: 62.11 (4.6) years (100% women). A total of 88.88% presented diplopia. The mean preoperative hypotropia was: 11.33 PD (SD 3.16), and the mean final VD 3.44 PD (SD 3.05). After surgery, the hypotropia was overcorrected in one case, under corrected in 5, and orthophoria was achieved in another three. The technique was associated with surgery of another rectus muscle in 4 subjects. The mean follow-up time after surgery was 34 months (SD 34.62). Six of the 9 patients improved with a vertical deviation ≤5 PD. In 3 patients, the diplopia was eliminated, while in 5 it remained intermittent (three with macular pathology). CONCLUSION: Supra-equatorial displacement with LR myopexy for treatment of myopic sagging/heavy eye, is a therapeutic option if hypotropia is less than 12 PD or the Yokoyama technique is not indicated. A good result was obtained in most cases, although diplopia could only be totally suppressed in three, and another five remained intermittent.

Extraocular muscle enlargement of indeterminate cause in pediatric patients: case series and literature review.

PURPOSE: To specify the clinical characteristics of enlarged extraocular muscles of indeterminate cause in pediatric patients based on a case series and literature review. METHODS: The medical records of pediatric patients who presented between January 2019 and January 2022 with enlarged extraocular muscles, where the underlying cause could not be determined, were retrospectively reviewed. RESULTS: Four patients were included. The main reason for presentation was evaluation of abnormal head posture. Head tilt or turn with duction deficit was observed in all patients. The age of onset ranged from 6 months to 1 year. Two patients presented with esotropia and hypotropia; the other 2 patients, with large-angle esotropia. Orbital imaging was performed in all cases and revealed unilateral rectus muscle enlargement sparing the muscle tendon. All 4 patients were found to have an enlarged medial rectus muscle. In the 2 patients with hypotropia, the inferior rectus muscle was also involved. No underlying systemic or orbital disease was found. There were no changes in the orbit or extraocular muscles on follow-up imaging test. The intraoperative forced duction test revealed severe restriction in the direction of gaze opposite to the primary field of action of the enlarged muscles. CONCLUSIONS: Enlargement of extraocular muscles should be considered in the differential diagnosis when large-angle incomitant vertical or horizontal misalignment and abnormal head posture are observed in infancy.

Vision outcomes in children with fetal alcohol spectrum disorders.

BACKGROUND: Previous studies demonstrated that children with Fetal Alcohol Spectrum Disorders (FASD) are more likely to have vision impairments. However, existing human clinical and epidemiological investigations are few and include limited sample sizes. This study aimed to explore the association between ophthalmologic abnormalities and FASD in a sample of 5-7 year old children in the general population. METHODS: This was a cross-sectional study nested in a larger study intended to estimate the prevalence of FASD in San Diego, California, conducted between 2012 and 2014. Prenatal exposure to alcohol, dysmorphology examinations, and a neurobehavioral testing battery were collected for each child and an FASD diagnosis was assigned. Parents of participating children were asked to release their child's vision screening or diagnostic records. RESULTS: Vision records were obtained for 424 participants in the larger prevalence study. Of these, 53 children were classified as having FASD. A statistically significant association was found between FASD and a diagnosis of strabismus; 5/42 (11.9%) of children who were classified as having FASD had strabismus compared to 6/290 (2.1%) of children who were not classified as having FASD (p = .01). All five cases of strabismus in the FASD group occurred in 19 children classified as having partial fetal alcohol syndrome (pFAS). No association was found between FASD and vision impairment (p = .23), refractive errors (p = .66), glasses/contact lens prescription (p = .30), or having one or more ophthalmological abnormalities (p = .97). CONCLUSIONS: An association between strabismus and FASD, specifically partial FAS, suggests that the effect of alcohol exposure on risk of strabismus must be severe enough to result in facial features consistent with FASD. This emphasizes the importance of vision screening in children with FASD.

Pulled-in-two syndrome in strabismus surgery for congenital fibrosis of the extraocular muscles.

Purpose: The aim of this study is to present six cases of pulled-in-two syndrome (PITS) in strabismus surgeries and to discuss our prevention and management strategies. Methods: This is a retrospective study presenting cases of PITS during strabismus operations. The medical records of the subjects who underwent operation in the strabismus unit of Beyoglu Eye Training and Research Hospital were reviewed retrospectively, from January 2000 till March 2022. Detailed ophthalmological examination records and angle of deviation were noted. Results: A total of six cases (four males and two females) with a mean age of 37.2 ± 28.0 (min 9-max 71) years were included in this study. All of the cases had congenital fibrosis of extraocular muscles (CFEOM). The most commonly involved muscle was medial rectus (83%). Majority of cases (67%) were adults. In all cases, the muscle was found and reattached to the globe. No patients had diplopia after surgery. Conclusion: PITS is a rare complication that can be seen during strabismus surgeries, and CFEOM patients are in the risk group. Therefore, surgeons should be cautious and prepared in risky patients to be able to manage this complication.

Insights Regarding Optometric Findings of CHARGE Syndrome in a Pediatric Low Vision Clinic.

SIGNIFICANCE: CHARGE, named for common findings-coloboma, heart defects, atresia of choanae, retardation of growth and development, genital hypoplasia, and ear anomalies-is a frequent etiology of deaf-blindness. A retrospective review in a pediatric low vision clinic presented the opportunity to investigate ocular findings in this syndrome with variable clinical presentations. PURPOSE: This retrospective study reviewed ocular findings and visual function measures from low vision evaluations of patients with CHARGE syndrome, which may influence their multidisciplinary management. METHODS: A retrospective chart review was conducted by three examiners of 60 patients presenting with CHARGE syndrome at a pediatric low vision clinic. Visual acuity and contrast sensitivity were obtained using standard measures. Ocular alignment and cycloplegic refractive error measurements were recorded. Refractive findings were analyzed using vector analysis. Anterior and posterior segment findings were recorded. RESULTS: Patients ranged in age from 1 to 29 years and were followed up for a mean of 4.3 years. Best-corrected visual acuity ranged from no light perception to 20/20 Snellen equivalent. Characteristics of strabismus, occurring in 82% of patients, were reported. Contrast sensitivity was reduced in 52% of patients. Chorioretinal colobomas were reported in 88% of patients. The most common ocular findings included nystagmus (43%), microphthalmia (27%), iris coloboma (27%), and facial nerve palsy (23%). Refractive vector analysis revealed significant myopic progression of the spherical equivalent with age and a tendency for with-the-rule astigmatism and minimal obliquity. CONCLUSIONS: This retrospective review of a relatively large sample size for this rare condition outlined the most common ocular manifestations of CHARGE syndrome. Decreased visual acuity, myopic refractive error, strabismus, and reduced contrast sensitivity were common. Thus, careful optometric evaluation in this population is required, as these findings must be considered in appropriate clinical and habilitative management.