Congenital fibrosis of extraocular muscles (CFEOM) syndrome is a genetically determined congenital disorder characterized by non-progressive ophthalmoplegia, restrictive ocular fixation, and ptosis. Its estimated incidence is approximately 1 in 230 000 to 250 000. This paper reports a family with type 3 CFEOM diagnosed at the Second Xiangya Hospital of Central South University. The proband was a 10-year-old female who presented with right esotropia and right upper eyelid ptosis. Whole-exome sequencing revealed a heterozygous c.904G>A mutation in the TUBB3 gene. Genetic testing of family members identified that the proband's mother carried the same mutation and exhibited left eyelid ptosis. The child underwent strabismus correction followed by ptosis repair, both of which led to marked postoperative improvement. For children presenting with congenital extraocular movement restriction and ptosis, genetic testing plays a crucial role in confirming the diagnosis and guiding family analysis. Additionally, individualized surgical intervention can significantly improve both ocular function and cosmetic appearance.
Humans ; Female ; Child ; Ophthalmoplegia/congenital* ; Fibrosis/congenital* ; Blepharoptosis/surgery* ; Mutation ; Tubulin/genetics* ; Pedigree ; Male ; Esotropia/genetics* ; Congenital Cranial Dysinnervation Disorders

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

Objective: To compare the postoperative alignment of patients surgically corrected for esotropia or exotropia 6 weeks and 6 months after surgery. Method: This retrospective study reviewed clinical records of patients who underwent horizontal muscle surgery at the University of the Philippines-Philippine General Hospital from 2010 to 2014. The changes in alignment from 1 week to 6 weeks and from 1 week to 6 months after surgery were compared for overcorrection, undercorrection, and adequate correction groups. ANOVA and Pearson correlation were used. Results: Twenty two esotropia and 10 exotropia patients were included. Overcorrected esotropia patients had an esotropic change in alignment (15.50+13.44 PD after 6 weeks, p=0.026; 25+18.38 PD after 6 months, p=0.008). Under corrected esotropia patients had an exotropic change in alignment (-1.25+5.91 PD after 6 weeks, p=0.026;-4.38+14.16 PD after 6 months, p=0.008). Undercorrected exotropia patients had an esotropic change in alignment (6.67+6.35 PD after 6 weeks, p=0.028; 6+9.85 PD after 6 months, p=0.024). The presence of vertical deviations in esotropia caused an exotropic change in alignment after 6 months (p=0.03). Conclusion Although an exotropic drift is more commonly reported, fusional vergences may account for postoperative alignment changes towards orthotropia.
Esotropia ; Exotropia

Cornelia de Lange syndrome (CdLS) is a rare multisystemic disorder that is characterized by mental retardation, prenatal and postnatal growth retardation, limb anomalies, and distinctive facial features, which include arched eyebrows that often meet in the middle (synophrys), long eyelashes, low-set ears, small and widely spaced teeth, and a small and upturned nose. Ophthalmic manifestations include long eyelashes, nasolacrimal duct obstruction, myopia, ptosis, and strabismus. There has been no report of surgical treatment for esotropia and unilateral ptosis in patients with CdLS in Korea. I report a patient with CdLS who underwent surgical treatment for esotropia and unilateral ptosis with a good surgical outcome.
De Lange Syndrome ; Ear ; Esotropia ; Extremities ; Eyebrows ; Eyelashes ; Humans ; Intellectual Disability ; Korea ; Myopia ; Nasolacrimal Duct ; Nose ; Strabismus ; Tooth

No abstract available.
Esotropia

BACKGROUND: To describe factors affecting the prognosis after operation for recurrent intermittent exotropia (X[T]) in children. METHODS: Clinical records of 50 patients who underwent operation for recurrent X(T) by a single surgeon were reviewed. The age at diagnosis of X(T), and first and second operations, deviation angle at distance and near, surgical method, concurrent vertical strabismus, stereoacuity, and Worth's Four Dot (W4D) examination before reoperation were analyzed, along with the postoperative deviation angle. A successful surgical outcome was defined as orthophoria, esodeviation ≤ 5 prism diopters, or exodeviation ≤ 10 prism diopters at distance. RESULTS: Among the 50 recurrent exotropes who underwent surgery and were followed up for more than 1 year postoperatively, 13 showed recurrent exotropia and 1 showed consecutive esotropia. The mean age at reoperation was 8.49 ± 2.19 years, and the mean duration of postoperative follow-up was 27.78 ± 12.02 months. Good near fusion before reoperation was a significant factor in the success of surgery (P = 0.006). Smaller postoperative deviation angle measured immediately and 2 months after surgery were related to smaller final deviation angle (P = 0.027 and P = 0.022, respectively). CONCLUSION: Peripheral suppression lowers the success rate of operation for recurrent X(T) in children. Overcorrection rather than orthotropia should be the target of immediate postoperative deviation angle. Peripheral suppression status and immediate and 2-month postoperative deviation angle may be important clues for predicting the final result of operation for recurrent X(T).
Child ; Diagnosis ; Esotropia ; Exotropia ; Follow-Up Studies ; Humans ; Methods ; Prognosis ; Reoperation ; Strabismus

PURPOSE: To evaluate the outcomes of modified medial rectus (MR) resection using a lowered amount of MR resection for recurrent exotropia after bilateral lateral rectus (LR) recessions. METHODS: Fifty-six patients, who underwent MR resection from 2003 to 2017 for recurrent exotropia after bilateral LR recessions, were included. MR resection was performed using modified MR resection with a smaller amount of resection than the standard of 4 mm resection at 20 prism diopters (PD) of exotropia. Postoperative surgical results at 1 month, 6 months, 1 year and 2 years were classified as a success (5 PD esotropia [ET]-10 PD exotropia [XT]), overcorrection (>5 PD ET), and undercorrection (>10 PD XT). The clinical factors affecting surgical results at postoperative 6 month and 2 years were also evaluated. RESULTS: The success rate was 78.2% at postoperative 1 month and 87.5% at postoperative 6 months. The overcorrection rate was 21.8% and the undercorrection rate was 0% at postoperative 1 month. At postoperative 6 months, the overcorrection rate decreased to 0% and the undercorrection rate was 12.5%. The success rate was 85.7% at postoperative 1 year and 66.6% at postoperative 2 years. There was no clinical factor affecting the surgical outcomes except the ocular alignment at postoperative 1 month. The deviation at postoperative 1 month was more esotropic in patients with success at postoperative 6 months and 2 years than that in patients with undercorrection (p < 0.05). CONCLUSIONS: The modified MR resection showed favorable results of 87.5% at postoperative 6 months and 85.7% at 1 year. The angle of deviation at postoperative 1 month was an indicator of subsequent surgical outcomes.
Esotropia ; Exotropia ; Humans

PURPOSE: To evaluate the clinical effects of medial orbital decompression in patients with thyroid orbitopathy. METHODS: Forty-three orbits of 28 patients who underwent medial orbital decompression for cosmetic purposes between January 2014 to January 2017 were retrospectively reviewed. Changes in visual acuity, intraocular pressure, exophthalmos, strabismus, and diplopia were checked before, 3 months, and 1 year after surgery. RESULTS: The average exophthalmos reduction was −2.99 ± 0.96 mm at postoperative 3 months and −3.07 ± 1.24 mm after 1 year (both, p < 0.001). In patients who underwent unilateral orbital decompression, the mean difference in exophthalmometry between the two eyes was significantly reduced from 3.06 ± 0.78 mm to 0.38 ± 0.44 mm after 3 months, and to 0.50 ± 0.46 mm after 1 year (p = 0.011 and p = 0.012, respectively). After surgery, the final postoperative intraocular pressure decreased significantly at postoperative 3 months and 1 year (both, p < 0.001). The mean preoperative horizontal deviation was 0.88 ± 4.85 prism diopters (PD) and 5.50 ± 6.74 PD at postoperative 3 months, which demonstrated significant esodeviation postoperatively (p = 0.007). Three patients had new onset esotropia (8.33%), but no surgical treatment was needed. CONCLUSIONS: Medial orbital decompression is a less invasive and safe surgical procedure for patients with asymmetric or mild thyroid-associated orbitopathy, which can be beneficial for reducing proptosis.
Decompression ; Diplopia ; Esotropia ; Exophthalmos ; Humans ; Intraocular Pressure ; Orbit ; Retrospective Studies ; Strabismus ; Thyroid Gland ; Visual Acuity

PURPOSE: To evaluate the clinical features of children with high hyperopia weaning with age. METHODS: The medical records of 203 children wearing spectacles due to hyperopia of +4.00 diopters (D) or greater in at least one eye based on the cycloplegic refraction and with follow-up for 3 years or more were reviewed. The patients were divided into those who showed a decrease in the spherical equivalent (SE) of 1.50 D or greater and those who maintained. The age of wearing spectacles, the magnitude of hyperopia, the angle of deviation, the ratio of accommodative-convergence to accommodation (AC/A), and the frequency of amblyopia and anisometropia were compared. RESULTS: Forty seven patients with decreased hyperopia and 156 patients with sustained hyperopia were included. The decreased-group started to wear spectacles later than the sustained-group (5.0 ± 2.3 years vs. 4.1 ± 2.4 years). The mean SE of the hyperopic eye in the decreased-group was significantly greater at the initial visit than in the sustained-group (6.29 ± 2.18 D vs. 5.47 ± 1.38 D); was identical at the 1 year follow-up (4.83 ± 1.72 D vs. 4.89 ± 1.55 D); and significantly lower at the last follow-up (3.15 ± 1.72 D vs. 4.65 ± 1.56 D). In the decreased-group, the mean hyperopia of 3.14 ± 2.02 D decreased during a mean period of 3.9 years, especially during the first year after spectacle correction. At baseline, the frequency and angle of esotropia at both distant and near with/without hyperopic correction was significantly larger in the sustained-group. The frequency of amblyopia and anisometropia and the AC/A were identical between the two groups, while the frequency of amblyopia at the last follow-up was significantly lower in the decreased-group. CONCLUSIONS: Some patients with hyperopia of +4.00 D or greater who had none or a small angle of esotropia and improved amblyopia showed a decrease in hyperopia with age.
Amblyopia ; Anisometropia ; Child ; Emmetropia ; Esotropia ; Eyeglasses ; Follow-Up Studies ; Humans ; Hyperopia ; Medical Records ; Weaning

PURPOSE: We report a patient with delayed-onset abducens nerve palsy and Horner syndrome after endovascular treatment of traumatic carotid-cavernous fistula (CCF). CASE SUMMARY: A 68-year-female visited our ophthalmic department complaining of gradual-onset ptosis of the left eye and horizontal diplopia. She had undergone endovascular treatment to treat left-sided traumatic CCF after a car accident 10 years before; she had been told at that time that the treatment outcome was favorable. The left-sided ptosis gradually developed 6 years after the procedure, accompanied by diplopia. The left eye exhibited miosis and the extent of anisocoria increased in dim light. An extraocular examination revealed 30 prism diopters of left esotropia in the primary gaze and a −4 abduction limitation of the left eye. CCF recurrence was suspected; however, magnetic resonance imaging with magnetic resonance angiography of brain did not support this. The esotropia did not improve during the 6-month follow-up and strabismus surgery was performed. CONCLUSIONS: Delayed-onset abducens nerve palsy and Horner syndrome can develop even after successful endovascular treatment of CCF. Strabismus surgery should be considered in patients whose diplopia does not spontaneously improve.
Abducens Nerve Diseases ; Abducens Nerve ; Anisocoria ; Brain ; Carotid-Cavernous Sinus Fistula ; Diplopia ; Esotropia ; Fistula ; Follow-Up Studies ; Horner Syndrome ; Humans ; Magnetic Resonance Angiography ; Magnetic Resonance Imaging ; Miosis ; Recurrence ; Strabismus ; Treatment Outcome