Project description:AIM:To identify ARIX gene polymorphisms in patients with congenital superior oblique muscle palsy and to find the relation between the ARIX gene and congenital superior oblique muscle palsy. METHODS:The three exons of the ARIX gene were sequenced by genomic DNA amplification with polymerase chain reaction (PCR) and direct sequencing in 15 patients with superior oblique muscle palsy (13 with congenital and two with acquired palsy) and 54 normal individuals. PCR products cloned into plasmids were also sequenced. A family with father and a daughter each having congenital superior oblique muscle palsy was also involved in this study. RESULTS:Four patients with congenital superior oblique muscle palsy carried heterozygous nucleotide changes in the ARIX gene. One patient with the absence of the superior oblique muscle had T7C in the 5'-UTR of the exon 1 and C-44A in the promoter region, both of which were located on the same strand. Another unrelated patient with congenital superior oblique muscle palsy had C76G in the 5'-UTR of the exon 1 and C-9A in the promoter region on the same strand. G153A in the 5'-UTR of exon 1 was found in common in two affected members of a family with congenital superior oblique muscle palsy. This G153A in the 5'-UTR of exon 1 was also present in four unrelated normal individuals. No other heterozygous nucleotide changes were found in normal individuals. CONCLUSIONS:The nucleotide change (G153A) in the 5'-UTR of exon 1 co-segregated with congenital superior oblique muscle palsy in one family. Four other nucleotide changes in the exon 1 or the promoter region were found only in patients with congenital superior oblique muscle palsy. These nucleotide polymorphisms may be one of the risk factors for the development of congenital superior oblique muscle palsy.
Project description:ObjectivesTo evaluate the effect of inferior oblique (IO) myectomy on ocular torsion according to the absence of the trochlear nerve in unilateral congenital superior oblique palsy (UCSOP).MethodsWe retrospectively reviewed the clinical data of patients who had been diagnosed with UCSOP and underwent ipsilateral IO myectomy (n = 43). Patients were classified into the present and absent groups according to the absence of the trochlear nerve and superior oblique hypoplasia on magnetic resonance imaging (MRI). For quantitative analysis of ocular torsion, disc-fovea angles (DFA) were collected in both eyes using fundus photographs taken within three months before surgery and one month after surgery.ResultsDFA of the paretic eye did not differ according to the absence of the trochlear nerve (9.4±5.6° in the present group vs. 11.0±5.4° in the absent group, p = 0.508). However, the present group had a larger DFA in the non-paretic eye than the absent group (14.1±6.7° in the present group vs. 8.0±5.0° in the absent group, p = 0.003). The change of ocular torsion after IO myectomy in the paretic eye was -5.3±3.7° in the present group and -4.8±3.5° in the absent group, respectively (p = 0.801). In the non-paretic eye, the change in DFA was -1.5±3.0° in the present group, which was larger than that in the absent group (0.7±2.6°, p = 0.047). In the multivariate analysis, the change in DFA was correlated with only the preoperative DFA (standardized β = -0.617, p<0.001 in the paretic eye, and standardized β = -0.517, p<0.001 in the non-paretic eye).ConclusionsIn the paretic eye, there was no significant difference in the change of ocular torsion between both groups, whereas in the non-paretic eye, the present group had a larger change in DFA after IO myectomy than the absent group. However, in the multivariable analysis, the change in ocular torsion was significantly correlated with preoperative excyclotorsion but not with the presence of the trochlear nerve itself.
Project description:ImportanceBoth vertical rectus belly transposition (VRBT) and superior rectus transposition (SRT) can be performed simultaneously with ipsilateral medial rectus recession (MRc) and have been shown to be effective for chronic sixth nerve palsy. However, it is unclear whether VRBT is superior to SRT in correcting esotropia.ObjectiveTo compare the effectiveness of modified VRBT plus MRc (mVRBT-MRc) vs augmented SRT plus MRc (aSRT-MRc) in Chinese patients with chronic sixth nerve palsy.Design, setting, and participantsThis parallel-design, double-masked, single-center, randomized clinical trial was conducted from January 15, 2018, to May 24, 2021. The follow-up visits were scheduled at 1 month and 6 months. Eligible Chinese participants with unilateral chronic sixth nerve palsy were randomly assigned to receive either mVRBT-MRc (VRBT group) or aSRT-MRc (SRT group).InterventionsmVRBT-MRc or aSRT-MRc.Main outcomes and measuresChange of horizontal deviation in primary position from baseline to 6 months.ResultsOf the total 25 eligible participants, the mean (SD) age was 45.4 (12.6) years, with 10 male participants (40%) and 15 female participants (60%). Thirteen participants (52%) were randomly assigned to the VRBT group, and 12 (48%) were randomly assigned to the SRT group. At baseline, the mean (SD) horizontal deviation was 65.7 (10.8) prism diopters (Δ) in the VRBT group and 60.5Δ(14.1Δ) in the SRT group. Similar amounts of MRc were performed in both groups. At 6 months, the horizontal deviation changed from baseline by 66.3Δ in the VRBT group and by 51.5Δ in the SRT group. The adjusted group difference was 10.9Δ (95% CI, 5.3Δ-16.6Δ), favoring the VRBT group (P = .001). Four times as many participants corrected more than 60Δ with mVRBT-MRc compared with aSRT-MRc. The group difference of the improvement of abduction limitation was -0.2 (95% CI, -0.8 to 0.5; P = .64). Although there was a higher proportion of undercorrection in the SRT group (difference, 45%; 95% CI, 16%-75%; P = .01), no differences were identified for other suboptimal outcomes between groups.Conclusions and relevanceCompared with aSRT-MRc, mVRBT-MRc showed better effect in correcting esotropia with no differences detected for other suboptimal outcomes. mVRBT-MRc may be a promising alternative surgical procedure for chronic sixth nerve palsy, particularly for large esotropia of more than 60Δ, if these results are confirmed in larger, diverse cohorts with longer follow-up.Trial registrationChiCTR Identifier: ChiCTR-INR-17013705.
Project description:PURPOSE:Superior rectus transposition has been popularized for the treatment of abduction deficiencies. Potential complications include induced vertical deviation and torsion. A new procedure, the inferior rectus transposition (IRT), may be similarly beneficial for patients at risk for postoperative vertical deviation or incyclotropia. The purpose of this study is to describe the outcomes of patients undergoing IRT. DESIGN:Prospective, interventional case series. METHODS:Five patients in an academic pediatric ophthalmology and strabismus practice with a complete lateral rectus palsy who underwent IRT were studied. Changes in anomalous head posture, ocular rotations, ocular alignment, and torsion preoperatively to postoperatively were compared. RESULTS:The patients ranged in age from 19-89 years. There was a significant correction in the angle of esotropia (ET) from 39±17? (14-55?) to 12 ± 9.8? (0-22?) postoperatively (P = .02). Two of 5 patients had preoperative hypertropia of the affected eye (1.4 ± 2.2?; range, 2-5?). One of those had no vertical deviation postoperatively and 1 patient resulted in 2? hypotropia. One patient without vertical misalignment preoperatively developed a small postoperative vertical deviation. Torticollis significantly improved from 31.4 ± 11.6° to 5 ± 5.8° (P = .004). All patients improved abduction, with a mean of -4.4 ± 0.5 preoperatively to -3.4 ± 0.9 postoperatively (P = .07). CONCLUSION:Initial postoperative follow-up in patients with abducens palsy undergoing IRT shows a significant improvement in ocular alignment and torticollis. In patients with preoperative hypertropia, IRT resulted in a downward shifting effect on the operated eye. IRT may be a beneficial procedure for patients with preoperative hypertropia or intorsion requiring transposition procedures. Future studies with larger populations and longer durations of follow-up will be required before this procedure can be recommended.
Project description:PurposeAlthough the Parks-Bielschowsky three-step test is the cornerstone of cyclovertical strabismus diagnosis, it has not been validated against an external benchmark. We evaluated the test's sensitivity in clinical diagnosis of superior oblique palsy in patients with unequivocal magnetic resonance imaging (MRI) evidence of superior oblique atrophy.MethodsA total of 73 strabismic patients were selected from a prospective MRI study because they exhibited superior oblique atrophy indicative of superior oblique denervation and thus confirmatory of superior oblique palsy. Of these, 50 patients who had no confounding factors were included for detailed study. Ocular motility data were evaluated to determine sensitivity of single and combined clinical findings in diagnosis of superior oblique palsy.ResultsMaximum mean ipsilesional superior oblique cross section was reduced to 9.6 ± 0.6 mm(2) (mean ± standard error) in superior oblique palsy, representing 52% of the 18.5 ± 0.6 mm(2) contralesional superior oblique maximum cross section and 52% of the 18.4 ± 0.4 mm(2) control maximum superior oblique cross section (P < 0.001). Of the 50 patients, 35 (70%) with superior oblique atrophy fulfilled the entire three-step test. In 14 (28%) patients two steps were fulfilled; in 1 patient (2%), only one step. Affected superior oblique cross section was similar in orbits that fulfilled the three-step test (9.8 ± 0.9 mm(2)) and those that did not (9.1 ± 0.7 mm(2); P = 0.58).ConclusionsThe complete three-step test fails to detect 30% of cases of superior oblique atrophy. Often only two of three steps are positive in superior oblique palsy.
Project description:PurposeTo present the results of bilateral superior rectus transposition with medial rectus recession in a case of chronic bilateral sixth nerve palsy.ObservationBilateral superior rectus transposition with medial rectus recession resulted in full correction of esotropia with resolution of horizontal diplopia, improvement in abduction, and regain of stereoacuity in our case. There was minimal limitation of adduction, with no abnormal vertical or torsional changes.Conclusion and importanceBilateral superior rectus transposition with medial rectus recession appears to be a useful procedure for surgical treatment of bilateral sixth nerve palsy with minimal side effects. Given its potential for reduced risk of anterior segment ischemia (ASI), it may have especially good value in the select group of patients at risk for ASI. Studies with larger sample size and longer follow up are needed to further evaluate this procedure and elucidate the variables in surgical technique for superior rectus transposition.
Project description:BackgroundSuperior rectus muscle transposition (SRT) is one of the proposed transposition techniques in the management of defective ocular abduction secondary to chronic sixth nerve palsy and esotropic Duane retraction syndrome (Eso-DRS). The aim of the current study is to report the outcomes of augmented SRT in treatment of Eso-DRS and chronic sixth nerve palsy.Methodsa retrospective review of medical records of patients with Eso-DRS and complete chronic sixth nerve palsy who were treated by augmented full tendon SRT combined with medial rectus recession (MRc) when intraoperative forced duction test yielded a significant contracture. Effect on primary position esotropia (ET), abnormal head posture (AHP), limitation of ocular ductions as well as complications were reported and analyzed.Resultsa total of 21 patients were identified: 10 patients with 6th nerve palsy and 11 patients with Eso-DRS. In both groups, SRT was combined with ipsilateral MRc in 18 cases. ET, AHP and limited abduction were improved by means of 33.8PD, 26.5°, and 2.6 units in 6th nerve palsy group and by 31.1PD, 28.6°, and 2 units in Eso-DRS group respectively. Surgical success which was defined as within 10 PD of horizontal orthotropia and within 4 PD of vertical orthotropia was achieved in 15 cases (71.4%). Significant induced hypertropia of more than 4 PD was reported in 3 patients (30%) and in 2 patients (18%) in both groups, respectively.Conclusionaugmented SRT with or without MRc is an effective tool for management of ET, AHP and limited abduction secondary to sixth nerve palsy and Eso-DRS. However, this form of augmented superior rectus muscle transposition could result in high rates of induced vertical deviation.
Project description:PURPOSE:To determine whether the inferior oblique (IO) muscle weakening procedure combined with exotropia surgery affects the surgical correction of exotropia. DESIGN:Institutional, retrospective study. METHODS:We retrospectively reviewed the medical records of 310 patients who had undergone exotropia-correcting surgery combined with IO weakening (group A, 64 patients) or without IO weakening (group B, 246) with a postoperative follow-up of 6 months or more. The main outcome measures were the postoperative mean angle of horizontal deviation, the success rate, and the overcorrection rate. Surgical success was defined as an alignment between 10 prism diopters (PD) of exodeviation and 5 PD of esodeviation. RESULTS:The postoperative mean angles of exodeviation, throughout the follow-up period, did not significantly differ between the groups. Although the surgical success rate was higher in group B at postoperative 1 month (p = 0.035), there was no statistical difference between the 2 groups from postoperative 6 months.: The final success rates were 56.3 and 51.6% (p = 0.509). The overcorrection rate was significantly higher in group A at postoperative 1, 6 and 24 months (p = 0.017, p = 0.028, p = 0.030, respectively); however, at the final follow-up, there was no overcorrection in either group. CONCLUSION:The overcorrection rate was higher in group A until postoperative 2 years, even though the mean angles of exodeviation and the success rates did not significantly differ between the 2 groups. Surgeons should be mindful of overcorrection when planning exotropia surgery combined with the IO weakening procedure.
Project description:BackgroundV pattern identification is essential for proper strabismus management. Graded recession is a tailored approach to treat inferior oblique overaction (IOOA). The aim is to evaluate the efficacy of graded recession of inferior oblique muscle for correction of different grades of V pattern.MethodsForty patients from 3 to 18 years old with V pattern strabismus and primary IOOA were evaluated by prism cover test to assess the grade of IOOA and amplitude of V-pattern. Graded recession of IO muscle depends on the amplitude of the V-pattern and degree of IOOA. Eight mm recession for amplitude 15 PD to 20 PD and mild IOOA (10 PD-15 PD or + 1) ,10 mm recession for amplitude 20-30 PD and moderate IOOA (15-25 PD or + 2) and maximum recession for amplitude more than 30 PD and marked IOOA (≥ 25 PD or + 3). Simultaneous correction of the horizontal deviation was performed. Follow up after I week,1 month ,3 month and 6-month. Trial Registration Number (TRN) (NCT05786053) on 23/3/2023.ResultsThe mean age of the study patients was 9 ± 4.261. Twenty patients (50%) had V-pattern esotropia, 12 (30%) exotropia, 4 (10%) orthotropic and four (10%) had Dissociated vertical deviation (DVD). Four cases 10% were of grade 1, 20 cases (50%) grade 2 and 16 cases (40%) were of grade 3. Of eighty eyes, 66 eyes (82.5%) were fully corrected with no residual IOOA, and 14 eyes (17.5%) were under corrected. V-pattern was corrected in 28 cases 70% and only 12cases (30%) had residual V-pattern grade 1.ConclusionsGraded recession is an effective procedure for correction of V pattern strabismus with various grades of primary inferior oblique overaction. It can be tailored according to the the degree of IO overaction which is significantly related to the grade of V pattern. The 8 mm recession for IO was significantly related to recurrence or inadequate break of the V pattern in our studied cases. The grade of IOOA correlates with the amplitude of V-pattern. The amount of recession was planned according to preoperative IOOA and grade of V-pattern with frequent undercorrections obtained by the standard 8 mm recession. A + 2 overaction merits a 10-mm recession of the inferior oblique. A + 3 or + 4 overaction merits a 14-mm maximal recession.
Project description:BackgroundWe describe successful surgical treatment of superior oblique myokymia, which had recurred after superior oblique tenectomy.MethodsSingle case report.ResultsThe distal stump of the superior oblique tendon was extirpated by stripping it from the globe. The ipsilateral superior rectus muscle also was recessed, to correct a hypertropia that had resulted from the original superior oblique tenectomy.ConclusionsComplete removal of the distal superior oblique muscle tendon provided definitive relief of superior oblique myokymia. Superior rectus muscle recession, combined with previous inferior oblique myectomy, compensated effectively for loss of superior oblique function.