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: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:Purpose:To present the results of bilateral superior rectus transposition with medial rectus recession in a case of chronic bilateral sixth nerve palsy. Observation:Bilateral 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 importance:Bilateral 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: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: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.
Project description:Reconstruction of the superior capsule is a valuable intervention for some patients in whom massive, irreparable posterosuperior rotator cuff tears are identified. Superior capsular reconstruction techniques most commonly use either tensor fascia lata autograft or dermal allograft. As an alternative to more traditional superior capsular reconstruction, we commonly transpose and incorporate the biceps tendon into the rotator cuff repair construct in such patients instead. This biological superior capsular reconstruction biceps transposition technique ("bio-SCR"), described herein, provides increased structural integrity and supplemental tendon graft using viable cells while potentially serving the role of the superior capsule. As such, transposition of the biceps tendon may offer unique and significant advantages over other techniques and can be an effective and valuable alternative in appropriate cases.
Project description:PurposeTo investigate changes in vertical strabismus and extorsion in patients with intermittent exotropia and mild unilateral inferior oblique muscle overaction (IOOA) who underwent horizontal muscle surgery without vertical or oblique muscle surgery.MethodsThe medical records of 41 patients were retrospectively analyzed. The patients were followed up for at least 6 months after surgery. Fundus photography was performed before and after surgery, and the sum of the angles of torsion in both eyes was used to measure changes in extorsion using ImageJ software. The enrolled patients were divided into two groups according to the degree of IOOA: patients with grade 1 IOOA were placed in +1 IOOA group and those with grade 2 IOOA in +2 IOOA group. The pre- and postoperative angles of horizontal and vertical strabismus and extorsion were compared between the two groups.ResultsThe +1 IOOA and +2 IOOA groups included 24 and 17 patients, respectively. The angle of preoperative exotropia did not differ significantly: 25.54 ± 5.68 prism diopters (PD) and 25.65 ± 8.11 PD in the +1 IOOA and +2 IOOA groups, respectively. In the +1 IOOA and +2 IOOA groups, hypertropia was 2.67 ± 1.52 PD and 2.82 ± 1.13 PD, respectively, and extorsion angles were 7.14 ± 2.77° and 7.94 ± 2.87°, respectively. As the IOOA degree increased, the extent of hypertropia and extorsion also increased. However, there were no significant differences between the two groups. Postoperative angles of hypertropia and extorsion significantly decreased in both groups (p < 0.001) after surgery. The degree of change in hypertropia and extorsion was not significantly different between the two groups (p = 0.563 and p = 0.354, respectively).ConclusionsHypertropia and extorsion improved significantly after horizontal muscle surgery in patients with mild unilateral IOOA and intermittent exotropia. There was no significant difference in the improvement in hypertropia or extorsion between IOOA grades I and II.
Project description:PURPOSE: To report the postoperative results of full-tendon vertical rectus transposition (VRT) augmented with lateral fixation suture for the treatment of type 1 Duane syndrome and sixth nerve palsy and to determine whether there was a decrease in the effect of the Foster suture over time. METHODS: This retrospective, consecutive case series included patients who underwent a full-tendon VRT transposition with lateral fixation for type 1 Duane syndrome or sixth nerve palsy. The primary outcome measures included deviation, abnormal head posture(AHP), abduction deficiency, and postoperative binocular single visual field (BSVF). RESULTS: Eighty-seven patients (87 eyes: 40 eyes with Duane syndrome and 47 eyes with sixth nerve palsy) were included in this study. In Duane syndrome patients, the deviation was reduced by a mean of 95%, the AHP was eliminated in 86% of patients, the abduction was improved by 42%, and a useful BSVF of ≈ 67% of normal was achieved at 1 year post operation. In sixth nerve palsy patients, the deviation was reduced by 99%, the abduction was improved by 59%, and a useful BSVF of ≈ 71% of normal was achieved at 1 year post operation. In both groups, the improvements in deviation angle and abduction were stable postoperatively. Sixteen patients needed reoperation for undercorrection. CONCLUSION: VRT surgery with posterior fixation is an effective treatment method for complete sixth nerve palsy and Duane syndrome with esotropia, AHP, and abduction deficiency. The procedure carries a small risk of reoperation for undercorrection. The effect of the Foster suture did not decline over time.
Project description:Stem cell competition controls organ size, maintains tissue homeostasis and optimizes long-term tissue function through eliminating cells less fit than their neighbors, but whether competitive interaction occurs between neural stem cells in the developing brain and its mechanistic drivers remains enigmatic. We applied single-cell RNA sequencing of mouse E11.5 medial cortex and bulk RNA sequencing of AnnexinV positive, PI negative cells to identify the loser cell in the natural state.