OBJECTIVE: Intermittent exotropia (IXT) is the most common form of strabismus that warrants standardized management for better and predictable outcomes. Currently there are no preferred practice guidelines on the non-surgical management of IXT. Among the aforementioned methods, orthoptics is the most favored by optometrists and ophthalmologists based in China and Israel. However, consensus statements on the use of such modality are yet to be established. This study aimed to identify preferred non-surgical management strategies for intermittent exotropia (IXT) among members of the Philippine Society of Pediatric Ophthalmology and Strabismus (PSPOS). METHODS: The survey collected data on respondent and patient demographics, perceived causes of IXT, clinical course, and management preferences. Incomplete responses were excluded, and data were analyzed using frequency distribution and the Fisher test. RESULTS: Forty of 54 (74%) PSPOS members participated, predominantly from urban settings (34 respondents). Most IXT cases were children aged 4 to 11 years. The proportion of pediatric patients was not significantly influenced by respondents’ age (p=0.1628) or practice region (p=0.451). Twenty-one (52.5%) respondents cited fusion defects as the primary cause of IXT. While twenty-one noted an unpredictable course, 14 identified it as a progressive course. Overminus lenses were preferred for younger patients (1-4 years), with an increasing preference for orthoptics in older age groups (5 to>18 years). Pediatric ophthalmologists aged 30 to 40 years (p=0.005) and those with 1 to 5 years of experience (p=0.020) favored overminus lenses for 5-10-year-olds. Preference for overminus lenses was stronger outside the NCR (p=0.044). Furthermore, 90% of respondents said surgery shouldn’t be performed at diagnosis, and 42.5% of respondents were against it for younger ages. CONCLUSION The findings revealed a strong preference for overminus lenses among PSPOS members, with increasing use of orthoptics for older patients. Factors influencing management decisions included respondent age, practice region, and perceptions of IXT’s cause and course. Future randomized controlled trials are essential to evaluate non-surgical interventions and develop comprehensive treatment guidelines.
Human ; ophthalmology ; strabismus ; exotropia

BACKGROUND AND OBJECTIVE

There is no strict by-the-book rule as to which approach is the best strabismus surgery for patients with sensory exotropia. More commonly, a monocular lateral rectus recession and a medial rectus resection (monocular R & R; MRR) is performed in the eye with a poorer prognosis. Rarely, for larger deviations, a third or fourth horizontal muscle in the better eye is added. This study aimed to determine the outcomes of strabismus surgery performed for sensory exotropia in a tertiary hospital in the Philippines.

The medical records of all patients with sensory exotropia who underwent strabismus surgical correction from January 2015 to December 2019 were retrospectively reviewed.

A total of 29 medical records satisfied the inclusion criteria. Mean age at diagnosis and at the time of surgery were 7.5 ± 11.6 (range: 1 to 68 years) and 12.4 ± 16.2 years (range: 1 to 68 years) years old, respectively, with a mean follow-up of 6.2 months (range: 3 to 24 months). After a mean follow-up of 6.2 months, the overall success (alignment in primary position is within 10 prism diopters of orthotropia) was relatively low, where 34% were successful, 65.5% developed recurrence, and none had overcorrections. Survival plots of both surgeries revealed a decline in success probability in achieving desired alignment six months after surgery.

We reported the surgical outcomes of 29 patients with sensory exotropia. The general trend realized was that the decline in success rates of good alignment was evident beginning six months post-operatively. The retrospective design serves as a limitation and hence, readers should treat results with caution.

Silver-Russell Syndrome (SRS) is a rare disorder associated with prenatal and postnatal growth retardation with associated characteristic facial and ocular features including strabismus. We report the outcome of strabismus surgery performed for exotropia in a 4-year-old patient with SRS. The patient presented with decreased visual acuity and constant exotropia of the right eye noted since 3 months of age. Systemic SRS characteristics consisted of relative macrocephaly, short stature, forehead prominence and stunted growth pattern. An X-pattern exotropia is consistent with bilateral tight lateral recti muscles with overelevation in adduction of the left eye was present. Patient underwent unilateral right lateral rectus recession and right medial rectus resection for a 50-prism diopter constant exotropia. Patient had a favorable outcome of within 8 prism diopters from orthotropia at 1st, 3rd-, 6th- and 12th-month post-operatively.
Russell Syndrome ; exotropia ; macrocephaly

Intussusception refers to the invagination (telescoping) of a part of the intestine into itself. Intussusception occurs primarily in infants and toddlers.1 The peak incidence is between 4 and 36 months of age, and it is the most common cause of intestinal obstruction in this age group. 2 Approximately 1 percent of cases are in infants younger than three months, 30 percent between 3 and 12 months, 20 percent between one and two years, 25 percent between two and three years, and 10 percent between three and four years.3 Although intussusception is most common in infants and young children, it is important to consider this diagnosis in children outside this age range. Approximately 10 percent of cases are in children over five years, and 3 to 4 percent in those over 10 years.3,4 We share here images from an actual case of a 15-year-old Filipino male with an ileo-ileal intussusception that is beyond the typical age range, with an incidental finding of an intraluminal mass that was histomorphologically diagnosed as Meckel’s diverticulum (MD). The diagnosis of intussusception is relatively rare in the patient’s age and the diagnosis of MD in the presence of intussusception is sparsely reported in the Philippines. Intussusception refers to the invagination (telescoping) of a part of the intestine into itself. Intussusception occurs primarily in infants and toddlers.1 The peak incidence is between 4 and 36 months of age, and it is the most common cause of intestinal obstruction in this age group. 2 Approximately 1 percent of cases are in infants younger than three months, 30 percent between 3 and 12 months, 20 percent between one and two years, 25 percent between two and three years, and 10 percent between three and four years.3 Although intussusception is most common in infants and young children, it is important to consider this diagnosis in children outside this age range. Approximately 10 percent of cases are in children over five years, and 3 to 4 percent in those over 10 years.3,4 We share here images from an actual case of a 15-year-old Filipino male with an ileo-ileal intussusception that is beyond the typical age range, with an incidental finding of an intraluminal mass that was histomorphologically diagnosed as Meckel’s diverticulum (MD). The diagnosis of intussusception is relatively rare in the patient’s age and the diagnosis of MD in the presence of intussusception is sparsely reported in the Philippines.
Meckel Diverticulum ; Strabismus ; Adolescent

In this study, we explored the neural mechanism underlying impaired stereopsis and possible functional plasticity after strabismus surgery. We enrolled 18 stereo-deficient patients with intermittent exotropia before and after surgery, along with 18 healthy controls. Functional magnetic resonance imaging data were collected when participants viewed three-dimensional stimuli. Compared with controls, preoperative patients showed hypoactivation in higher-level dorsal (visual and parietal) areas and ventral visual areas. Pre- and postoperative activation did not significantly differ in patients overall; patients with improved stereopsis showed stronger postoperative activation than preoperative activation in the right V3A and left intraparietal sulcus. Worse stereopsis and fusional control were correlated with preoperative hypoactivation, suggesting that cortical deficits along the two streams might reflect impaired stereopsis in intermittent exotropia. The correlation between improved stereopsis and activation in the right V3A after surgery indicates that functional plasticity may underlie the improvement of stereopsis. Thus, additional postoperative strategies are needed to promote functional plasticity and enhance the recovery of stereopsis.
Humans ; Exotropia/surgery* ; Depth Perception/physiology* ; Strabismus/surgery* ; Oculomotor Muscles/surgery*

Humans ; Botulinum Toxins, Type A/therapeutic use* ; Esotropia/drug therapy* ; Neuromuscular Agents ; Injections ; Treatment Outcome ; Oculomotor Muscles

More than 100 genes located on the X chromosome have been found to be associated with X-linked intellectual disability (XLID) to date, and NEXMIF is a pathogenic gene for XLID. In addition to intellectual disability, patients with NEXMIF gene mutation can also have other neurological symptoms, such as epilepsy, abnormal behavior, and hypotonia, as well as abnormalities of other systems. Two children with intellectual disability and epilepsy caused by NEXMIF gene mutation were treated in the Department of Pediatrics, Xiangya Hospital, Central South University from March 8, 2017 to June 20, 2020. Patient 1, a 7 years and 8 months old girl, visited our department because of the delayed psychomotor development. Physical examination revealed strabismus (right eye), hyperactivity, and loss of concentration. Intelligence test showed a developmental quotient of 43.6. Electroencephalogram showed abnormal discharge, and cranial imaging appeared normal. Whole exome sequencing revealed a de novo heterozygous mutation, c.2189delC (p.S730Lfs*17) in the NEXMIF gene (NM_001008537). During the follow-up period, the patient developed epileptic seizures, mainly manifested as generalized and absent seizures. She took the medicine of levetiracetam and lamotrigine, and the seizures were under control. Patient 2, a 6-months old boy, visited our department due to developmental regression and seizures. He showed poor reactions to light and sound, and was not able to raise head without aid. Hypotonia was also noticed. The electroencephalogram showed intermittent hyperarrhythmia, and spasms were monitored. He was given topiramate and adrenocorticotrophic hormone (ACTH). Whole exome sequencing detected a de novo c.592C>T (Q198X) mutation in NEXMIF gene. During the follow-up period, the seizures were reduced with vigabatrin. He had no obvious progress in the psychomotor development, and presented strabismus. There were 91 cases reported abroad, 1 case reported in China, and 2 patients were included in this study. A total of 85 variants in NEXMIF gene were found, involving 83 variants reported in PubMed and HGMD, and the 2 new variants presented in our patients. The patients with variants in NEXMIF gene all had mild to severe intellectual disability. Behavioral abnormalities, epilepsy, hypotonia, and other neurological symptoms are frequently presented. The phenotype of male partially overlaps with that of female. Male patients often have more severe intellectual disability, impaired language, and autistic features, while female patients often have refractory epilepsy. Most of the variants reported so far were loss-of-function resulted in the reduced protein expression of NEXMIF. The degree of NEXMIF loss appears to correlate with the severity of the phenotype.
Child ; Epilepsy/genetics* ; Female ; Humans ; Intellectual Disability/genetics* ; Male ; Muscle Hypotonia/complications* ; Mutation ; Phenotype ; Seizures/genetics* ; Strabismus/complications*

Objective: To describe the clinical profile of patients with amblyopia seen at a Philippine tertiary hospital. Methodology: This was a cross-sectional study utilizing a chart review of children ages 6 months to 18 years old diagnosed with amblyopia at a Philippine tertiary hospital. Records with complete entry of history and ocular examinations were included. Verbal children with best-corrected visual acuity in both eyes of less than 6/15 or a 2 line difference between eyes were considered amblyopic. Response to alternate occlusion or refixation patterns was used as a basis for diagnosing amblyopia in pre-verbal children. Patients with visual deprivation amblyopia such as from cataract who had undergone lens extraction and optical correction were included. Results: There were 334 patients included in the study. The mean age was 5±3 years with 52% being male. Deprivation type of amblyopia was present in 192 (58%) patients, strabismic type in 81 (24%), and refractive type in 61 (18%). No sex predilection was observed. Majority of the patients were 3-6 years old on initial consult. Cataract was the most common cause of deprivation amblyopia affecting 127 (66%) patients. Strabismic amblyopia was present in 58 patients with 74% being esotropic. Anisometropic refractive amblyopia was more common at 37 (58%) than isometropic refractive amblyopia. Conclusion Visual deprivation amblyopia was the most common cause of amblyopia in a Philippine tertiary hospital.
Amblyopia ; Cataract ; Anisometropia ; Strabismus

PURPOSE: We sought factors affecting amblyopia recurrence after successful treatment.METHODS: We included 117 patients with amblyopia. Patients were divided into recurrence and non-recurrence groups. We analyzed sex, age, amblyopia type, treatment duration, visual acuity, and binocular status.RESULTS: Of the 117 patients, 25 (21.4%) experienced recurrences. In that group, 60.0% of patients (compared to 14.1% of the no-recurrence group) exhibited high-frequency strabismus (p < 0.001). The recurrence group were younger than the no-recurrence group at both the beginning and end of treatment (p < 0.05). None of visual acuity at treatment commencement or end, stereoacuity, or suppression affected amblyopia recurrence.CONCLUSIONS: Recurrent amblyopia is frequent after initial successful treatment if the patient exhibits strabismus or is young.
Amblyopia ; Humans ; Recurrence ; Risk Factors ; Strabismus ; Telescopes ; Visual Acuity

PURPOSE: We used a questionnaire to explore perceptions and clinical practice patterns of Korean pediatric ophthalmologists in terms of amblyopia.METHODS: From September to November 2018, we conducted a web-based questionnaire survey of 99 specialists of the Korean Association for Pediatric Ophthalmology and Strabismus who operated ophthalmology clinics in Korea. We received 56 responses (56.57%) and retrospectively analyzed the data.RESULTS: The average specialist age was 44.0 ± 9.7 years. The mean age of treated amblyopia patients was 3 to 5 years (69.6%); the most common amblyopia was refractive anisometropic amblyopia (75.0%). On average, treatment commenced at 4 years of age (53.6%); child and parent co-operation most significantly influenced treatment success (46.4%). The preferred test was cycloplegic refraction (96.4%) and the preferred treatment occlusion therapy (100%) with glasses correction (98.2%). Occlusion therapy was most commonly performed for 2 hours/day (69.6%); the minimum age for eyeglasses prescription was 2.10 ± 1.18 years. Only three respondents (5.36%) prescribed contact lenses and only one (1.79%) performed refractive surgery.CONCLUSIONS: In Korea, amblyopia treatment is based on occlusion therapy and glasses correction. However, the time of treatment commencement, the duration of occlusion therapy, and the glasses used for correction varied. It is necessary to develop guidelines for amblyopia treatment; these should reflect current medical conditions.
Amblyopia ; Child ; Contact Lenses ; Eyeglasses ; Glass ; Humans ; Korea ; Ophthalmology ; Parents ; Practice Patterns, Physicians' ; Prescriptions ; Refractive Surgical Procedures ; Retrospective Studies ; Specialization ; Strabismus ; Surveys and Questionnaires