Retraction Syndrome Research Articles

Superior Rectus Transposition and Medial Rectus Recession for Treatment of Duane Retraction Syndrome and Sixth Nerve Palsy

ABSTRACT Purpose: To evaluate the outcome of augmented superior rectus transposition (SRT) with medial rectus (MR) recession in patients with Duane Retraction Syndrome (DRS) and sixth nerve palsy. Methods: Twenty four patients (16 DRS and 8 sixth nerve palsy) that underwent the procedure were included. The superior rectus muscle was secured, detached, and re-attached to the sclera along the spiral of Tilaux, adjacent to lateral rectus insertion. A non-absorbable augmentation suture was passed through the sclera, 8 mm posterior to the insertion of the lateral rectus. Results: At the last follow-up, the effect of surgery in decreasing esotropia in both groups was significant (P = .001 for DRS group, P = .002 for sixth nerve palsy). In both groups, abduction deficit improved significantly (P < .001 for DRS and P = .008 for sixth nerve palsy). After the surgery, small, asymptomatic vertical deviation in primary position was induced in five patients (20.8%). Post-operatively, none of the patients complained of torsional diplopia. In the 6-month follow-up, compared with the first postoperative visit, an eso-drift at distance or near developed in 11 patients (45.8%). Of the 11 patients with eso-drift, overcorrection (exotropia of 3–14 PD) was present at the first post-operative visit in 5 cases. Four cases showed an exo-drift (2–5 PD) at distance or near over time. Conclusion: SRT with medial rectus recession improves esotropia and abduction limitation without inducing significant vertical deviations and torsional diplopia. Some of the cases that underwent SRT with MR recession may show an eso-drift. The eso-drift can correct initial exotropia in some cases.

CHN1 and duane retraction syndrome: Expanding the phenotype to cranial nerves development disease

Duane retraction syndrome is a congenital eye movement disorder characterized by a failure of abducens nerve to develop normally, resulting in restriction or absence of abduction, adduction, or both, and narrowing of the palpebral fissure and retraction of the globe on attempted adduction. There is a genetic heterogeneity in Duane retraction syndrome (DURS). DURS maps to chromosome 8q13 in some patients, and pathogenic variants in CHN1 and MAFB genes are known to lead to DURS. We report here a child and his father with Duane retraction syndrome, associated to swallowing difficulties and unilateral trapeze aplasia. A whole exome sequencing revealed a heterozygous missense variant in CHN1 gene. This gene encodes GTPase-activating protein and is involved in the assembly of neuronal locomotor circuits. A patient with a 8q deletion has previously been described with a Duane retraction syndrome associated to trapeze aplasia. We provide an additional description to support the role in cranial nerves development of the CHN1 gene.

Bilateral augmented superior rectus transposition with medial rectus recession for bilateral esotropic Duane retraction syndrome

Superior rectus transposition with medial rectus recession is commonly performed for unilateral esotropic Duane syndrome. For bilateral esotropic Duane syndrome, bilateral medial rectus recession is the most frequently performed surgery and usually provides satisfactory alignment in primary position; however, there is limited improvement in abduction. We report the outcomes of bilateral augmented superior rectus transposition and medial rectus recession in 4 patients with bilateral esotropic Duane syndrome. Postoperatively, abduction was improved in all patients; 3 were orthotropic in the primary position, and 1 had residual esotropia.

The presence of anomalous extraocular bands in Duane retraction syndrome

To determine the prevalence of anomalous extraocular bands in patients who underwent surgery for Duane syndrome and to compare the clinical findings in patients with and without bands. Thirty-one patients with Duane syndrome who had their first surgery on rectus muscles to correct the primary deviation and abnormal head posture were included in this retrospective study. Patients were divided into two groups depending on the identification of anomalous extraocular bands intraoperatively. Baseline clinical characteristics were compared between the groups. A total of 31 patients were included. Anomalous bands were found in 6 of 19 (32%) patients with esotropic Duane syndrome and 9 of 12 (75%) with exotropic Duane syndrome (P=0.02). In esotropic Duane syndrome, the bands were localized under the medial rectus muscle in 5 patients and under the lateral rectus muscle in 1 patient. All of the bands in patients with exotropic Duane syndrome were under the lateral rectus muscle. The amount of preoperative primary deviation, globe retraction, and up- or downshoot were similar between groups. All of the bands had distinct tight insertion on the sclera, requiring a sharp dissection for disinsertion. In 7 cases, the anomalous band was a translucent structure that could be identified under the surgical microscope as scleral indentation during forced duction testing. Histological examination of 6 cases revealed only fibrous tissue in 4 and accompanying striated muscle tissue in 2 patients. The present study highlights the incidence of anomalous bands in Duane syndrome.Repeating forced duction testing after disinsertion of the affected muscle and excision of the anomalous band is helpful for intraoperative identification of these structures.

Variant types of Duane retraction syndrome: synergistic divergences and convergences

To present the clinical features of 4 patients with Duane retraction syndrome characterized by synergistic divergence or convergence, abnormal vertical movements, and accessory fibrotic bands. The medical records of 4 patients were reviewed retrospectively for the following clinical manifestations: visual acuity, refraction, ocular alignments, ocular motility, head position, magnetic resonance imaging, surgical techniques, and outcomes. All 4 cases were diagnosed as variants of Duane retraction syndrome. Two cases (cases 1 and 2) had synergistic divergence with unilateral adduction deficit, and 2 (cases 3 and 4) had synergistic convergence with bilateral abduction deficit. Case 2 manifested abnormal vertical movements of the right eye, which goes down with adduction of the left eye and goes up oppositely with abduction of left eye. Accessory fibrotic bands were detected beside the medial rectus muscle of both eyes in case 3. Synergistic convergence in case 4 occurred only after the first strabismus surgery. Weakening of the misinnervated horizontal rectus muscle improved ocular alignment and head position in cases 1, 3, and4. Synergistic divergence and convergence are extremely rare and may present with a great diversity of clinical features. A good outcome is very difficult to achieve; however, weakening of the misinnervated horizontal rectus muscle was therapeutic in these patients.

Augmented superior rectus muscle transposition in management of defective ocular abduction

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.

Surgical Outcomes of Exotropic Duane Retraction Syndrome From a Tertiary Eye Care Center.

To report surgical outcomes of patients with exotropic Duane retraction syndrome. A retrospective review of the medical records from patients with exotropic Duane retraction syndrome who underwent strabismus surgery and had at least 2 months of postoperative follow-up was conducted. Surgical success was defined as a postoperative horizontal deviation within 8 prism diopters (PD), abnormal head posture (AHP) less than 5 degrees, and a two-step decrease in overshoots. Seventy-three patients with exotropic Duane retraction syndrome (38, 52% male, mean age 14 ± 7.9 years) met the study criteria. Unilateral type 3 Duane retraction syndrome was seen in 56 (77%) patients and type 1 in 14 (19%) patients. The mean follow-up period was 22 weeks (range: 8 to 209 weeks). Unilateral and bilateral lateral rectus recession were performed in 48 (65%) and 25 (34%) patients, respectively. In addition to recession, a lateral rectus Y-splitting was performed in 42 (56%) patients. Five patients underwent differential medial rectus and (larger) lateral rectus recession to alleviate severe globe retraction. The mean primary position reduced from 23 PD (CI: 19.6 to 26 PD) preoperatively to 9 PD (CI: 7.02 to 11.02 PD) postoperatively (P < .0001). Postoperatively 50% of patients were orthotropic in the primary position. Surgical success for primary position deviation, AHP, and overshoots were achieved in 74%, 81%, and 71% of patients, respectively. In the current study, type 3 Duane retraction syndrome was the most common subtype necessitating surgery for exotropic Duane retraction syndrome. Good surgical outcomes were obtained for various indications. Unilateral or bilateral lateral rectus recession was successful in improving motor alignment and AHP. The addition of lateral rectus Y-splitting corrected overshoots greater than grade 2 in most patients with exotropic Duane retraction syndrome. [J Pediatr Ophthalmol Strabismus. 2021;58(1):9-16.].

Lateral rectus recession for consecutive exotropia in esotropic Duane syndrome: A case report

Consecutive exotropia is considered as a rare complication after medial rectus recession that is performed for cases of esotropic Duane syndrome. Here, we present a case of 5-year-old female initially presented with the limitation of abduction, narrowing of the palpebral fissure, and globe retraction of the left eye along with a mild left face turn. She was diagnosed with Duane syndrome Type 1. She was treated with the medial rectus recession (6.5 mm). Postsurgery, she developed a consecutive exotropia. This was managed successfully with the ipsilateral lateral rectus recession (8 mm). Following this surgery, the patient was orthoptic in the primary position and her abnormal head posture was markedly improved.

Improvement of surgical treatment of Duane syndrome in children

To analyze the use of bifurcation of the lateral rectus muscle (LRM) in the surgical treatment of Duane syndrome (DS) in children. The analysis of surgical treatment involved 12 children with paralytic strabismus at the age of 8 to 17. The following research methods were conducted for all patients: the study of eye motility in different directions of gaze, the study of eyes position and the degree of deviation in different positions of gaze, the Bielschowsky test, the Park's three-step test, determination of the nature of torticollis, the forcing test - intraoperative forceps test, multislice computed tomography (MSCT) of the head and orbit, ultrasonography of the oculomotor muscles. All study children underwent bifurcation (Y-cleavage procedure) of LRM with recession in exo-DS in 17% of cases, without recession in eso-DS - 83% of cases, respectively. In all operated patients with Duane syndrome, the direction of LRM was compensated (downshoot and upshoot were eliminated). In a direct gaze, the correct position was achieved in 10 (83%) cases, in 2 (17%) the residual angle of up to 10 PD remained, and torticollis was eliminated in 9 (75%) patients. The method of LRM bifurcation - the procedure of dividing the muscle into two portions and changing its primary localization, accompanied by abnormalities (presence of deviation, retraction, anomalous vertical movements, limited ocular motility, etc.), - helps compensate for them, and provides cosmetic as well as functional improvement.

Ophthalmic presentations and long-term outcomes of subconjunctival and atypical orbital myocysticercosis.

Purpose:To study the clinical presentation, radiological features, diagnosis, and treatment response in subconjunctival and atypical orbital myocysticercosis.Methods:Retrospective analysis of diagnosed subconjunctival and atypical (strabismus, diplopia, and blepharoptosis) orbital myocysticercosis was performed. A diagnostic criterion (2 of the 3) among clinical features, radiological findings, and treatment response was used in our study. A minimum of “post-treatment” follow-up of 12 months was observed.Results:Thirty-five patients were included with a mean age of 16 years having male predominance (n = 22, 62.8%). All had a unilateral presentation, with 24 (68.6%) patients having subconjunctival cysticercosis, of which 22 were located in close proximity of the rectus muscle insertion. At presentation, 10 patients had diplopia, 7 had strabismus, and 6 had face turn. Pseudo Duane’s and pseudo-Brown’s syndrome were noted in 5 and 4 patients, respectively. Radiologically, single muscle myositis without scolex was seen in 12 (34.3%) cases. All patients first received medical treatment, and surgical intervention (cyst removal) was attempted after treatment failure. Complete resolution of symptoms was noted in 22 (after medical treatment only) and in 8 (after surgery).Conclusion:In the majority, the subconjunctival cysticercosis is found in proximity to the rectus muscle insertion, as a part of orbital myocysticercosis. In atypical scenarios, a satisfactory response to medical treatment can be considered as diagnostic of cysticercosis.

The Roko Syndrome: A Mirror of Koro Syndrome

It is well established that 1 in 50 individuals receives a diagnosis of body dysmorphic disorder (BDD). Within body image disorders, there is genital retraction syndrome also known as Koro. A unique syndrome in which there is a heightened belief that one’s genitals will diminish in size, retract into the abdomen and ultimately lead to death. However, we have recently discovered a separate form of BDD that is directly opposite of Koro, in which the patient presents a strong belief that his penis will enlarge and extend out of their body. We present a unique case report of a counter-Koro syndrome. This syndrome is characterized by the delusion that one’s penis is growing larger and that it will result in its extreme protrusion from the abdomen and a consequent fear of recurring and visible erections. Given its mirror-like presentation and uniqueness to occurring only in males, we refer to it as Roko Syndrome. To our knowledge, this syndrome has not yet been reported in the literature and requires further study to understand whether it fits as a separate syndrome or falls along the spectrum of body dysmorphia. Thus, assessments used to identify body image disorders can be broadened to include items representing the behavior and presentation of Roko that we delineate in comparison of Koro. The new syndrome is also easily distinguishable from a priapism which is a urological emergency.

MANAGEMENT OF DUANE RETRACTION SYNDROME

Purpose: To determine the clinical profile of various subtypes of Duane retraction syndromepatients and to evaluate different surgical strategies in correction of different clinical signs.Patients and Methods: This was a hospital-based prospective interventional study conductedat the Ophthalmology department, Al-Azhar university hospital in Assiut over a duration of twoyears from October 2017 to October 2019. Thirty one patients of DRS were scheduled to beincluded in this study. They were divided into three groups; Group I: 21 patients of DRS type I,Group II: 4 patients of DRS type II and Group III: 6 patients of DRS type III. Age of patientsranged from 9 months to 28 yrs old. Postoperative results were evaluated over 6th monthsfollow-up period. Results: Thirteen patients were left without surgical intervention, they wereorthophoric or with minimal misalignment in PP with mild retraction of the globe on attemptedadduction and none of them had any vertical shoot nor abnormal head position, eighteen patients ofDRS had surgical intervention. Nine cases were exotropic, 8 cases were esotropic & 1 case wasorthophoric. Horizontal deviations were managed with unilateral or bilateral muscle recession&simultaneous recession of MR & LR. Retraction of the globe was managed with single musclerecession or simultaneous recession of both MR & LR. Vertical shoots were managed with LRY-splitting, IO recession& simultaneous recession of both MR& LR. Conclusion: Every patientof Duane retraction syndrome has an individual story, with good diagnosis & accurate grading todifferent signs we can determine the best surgical technique for the correctable signs. Singlemuscle recession & simultaneous recession of both MR & LR can correct horizontal strabismusin PP, the retraction of the globe on attempted adduction & the abnormal head position effectively.Overshoots can be corrected by LR Y-splitting, simultaneous recession of both MR& LR IOanteriorisation surgery is the best choice in cases with HT in PP & slow upshoot. Small LR resectioncan be done in esotropic cases with mild retraction (grade 1).

Hummelsheim procedure combined with medial rectus recession in complete sixth nerve palsy and esotropic Duane Retraction Syndrome.

To evaluate the effect of half tendon vertical rectus muscle transposition (HVRT) combined with medial rectus muscle recession (MRrec) in complete sixth nerve palsy (CSNP) and esotropic Duane Retraction Syndrome (eDRS). A retrospective study of patients with unilateral CSNP or eDRS who underwent HVRT combined with medial rectus recession over the course of 18 years. The patients with previous strabismus surgery and follow up less than 3 months were excluded. Overcorrection was defined as any amount of exotropia, and undercorrection was defined as residual esotropia of ⩾10 prism diopter (PD). A total of 39 patients were enrolled including 22 CSNP and 17 eDRS patients. Esotropia improved from 45.8 ± 22 and 22.5 ± 6.4 PD to 0.8 ± 2.5 and 0.3 ± 1 PD in CSNP group and eDRS group respectively. The angle of face turn improved from 34.3 ± 8.4° and 26.5 ± 9° to 0.6 ± 2.4° and 0.8 ± 1.6° in the CSNP group and eDRS group respectively. Abduction limitation improved from -4.5 ± 0.5 and -4 ± 0 units to -2.9 ± 0.5 and -2.7 ± 0.5 units in CSNP group and eDRS group respectively. No patient developed a new vertical deviation. An overcorrection occurred in one patient of the eDRS group who improved after botulinum toxin injection in the ipsilateral lateral rectus muscle. Undercorrection was seen in two patients. HVRT combined with MRrec is an effective procedure to improve esotropia, face turn, and abduction limitation in CSNP and eDRS.

Modified Y Splitting of Lateral Rectus in Treatment of Abnormal Vertical Eye Movements Combined with Globe Retraction in Duane Retraction Syndrome

Duane retraction syndrome is the most frequently seen restrictive ocular motility disorders. It is clinically presented with limitation of horizontal movement, variable amounts of upshoots or downshoots and globe retraction combined with narrowing of the palpebral aperture on attempted adduction. An 8-year-old patient presented with severe restriction of abduction, reciprocal upshots or downshoots, and globe retraction combined with the palpebral fissure narrowing of on adduction. After the modified Y splitting of LR and recession of both horizontal rectus operation, all cosmetically disfiguring clinical features disappeared. In this case report modified Y splitting procedure and its long-term efficacy is presented.

Effect of lateral rectus muscle resection on abduction in Duane retraction syndrome type 1.

To assess the effect of lateral rectus muscle resection on abduction in Duane retraction syndrome (DRS) type 1. The medical records of patients with DRS type 1 were reviewed retrospectively. Fifteen patients who underwent lateral rectus resection were included. Prism and cover test and the Krimsky test were used to detect deviations. Ocular ductions, abnormal head position (AHP), and globe retraction were recorded. Nine (60.0%) patients were female. The mean age was 13.1 ± 2.3 (range, 2-34) years. Left eyes were included in ten (66.7%) patients. Mean follow-up time was 37.6 ± 16.6 (range, 6-70) months. All patients had AHP, 13 patients had mild globe retraction, and 2 patients had no globe retraction preoperatively. Mean lateral rectus resection was 3.1 ± 0.7 (range, 2.0-4.5) mm, and the mean medial rectus recession was 4.4 ± 0.6 (range, 3.0-5.0) mm. The mean preoperative deviation decreased from 23.3 ± 6.9 (range, 14-35) prism diopters (pd) to 2.2 ± 4.1 (range, 0-10) pd at near, and from 23.6 ± 7.1 (range, 14-35) pd to 1.8 ± 3.5 (range, 0-10) pd at distance, at 6months postoperatively (p = 0.01). The mean limitation in abduction decreased from -3.2 ± 0.9 to -1.3 ± 1.1 postoperatively (p < 0.001). AHP resolved in all patients. There was not a limitation in adduction or a worsening of globe retraction in any patient. Lateral rectus resection can be used to improve abduction in patients with DRS type 1 who have mild globe retraction. We assume that this procedure has no worsening effect on globe retraction in appropriate cases.

Outcomes of symmetric bilateral medial rectus recession in large-angle esotropic Duane syndrome.

To evaluate the efficacy of symmetric bilateral medial rectus recession in large-angle esotropic Duane retraction syndrome (DRS) with moderate to severe globe retraction. In a retrospective study, medical reports of 30 patients with esotropia of equal or more than 20 prism diopters (pd) and moderate to serve globe retraction due to unilateral DRS who underwent symmetric bilateral medial rectus recession were reviewed. Age, gender, laterality, amblyopia, length of follow-up, pre- and postoperative measurements of primary position deviation, ocular ductions and severity of globe retraction and abnormal head posture were evaluated. A successful result was defined as decreasing esotropia to equal or less than 8 pd or equal or less than 8 pd of consecutive exotropia. The mean age of patients at surgery was 13.7 ± 8.5 years old (range: 3-38). The mean preoperative esotropia measured 28.9 ± 9.1 pd in distance and 25.7 ± 7.2 pd in near, which decreased to 4.9 ± 6.1 pd in distance and 3.9 ± 8.8 pd in near postoperatively. The mean bilateral medial rectus recession was 4.9 ± 0.9 mm (range: 3-6 mm). The mean abnormal head posture improved from 19.1 ± 6.9 degrees (range: 10-30 degrees) to 3.3 ± 4.7 degrees (range: 0-15 degrees) postoperatively. At the last follow-up visit, 23 patients (76.7%) had a successful outcome. In the large-angle esotropic DRS patients with moderate to severe globe retraction, symmetric bilateral medial rectus recession, can be conducted to successfully resolve primary position deviation and abnormal head posture.

Identification of a novel CHN1 p.(Phe213Val) variant in a large Han Chinese family with congenital Duane retraction syndrome

Duane retraction syndrome (DRS) is a neuromuscular dysfunction of the eyes. Although many causative genes of DRS have been identified in Europe and the United States, few reports have been published in regard to Chinese DRS. The aim of the present study was to explore the genetic defect of DRS in a Chinese family. Exome sequencing was used to identify the disease-causing gene for the two affected family members. Ophthalmic and physical examinations, as well as genetic screenings for variants in chimerin 1 (CHN1), were performed for all family members. Functional analyses of a CHN1 variant in 293T cells included a Rac-GTP activation assay, α2-chimaerin translocation assay, and co-immunoprecipitation assay. Genetic analysis revealed a NM_001822.7: c.637T > G variant in the CHN1 gene, which resulted in the substitution of a highly conserved C1 domain with valine at codon 213 (NP_001813.1: p.(Phe213Val)) (ClinVar Accession Number: SCV001335305). In-silico analysis revealed that the p.(Phe213Val) substitution affected the protein stability and connections among the amino acids of CHN1 in terms of its tertiary protein structure. Functional studies indicated that the p.(Phe213Val) substitution reduced Rac-GTP activity and enhanced membrane translocation in response to phorbol-myristoyl acetate (PMA). Together with previous studies, our present findings demonstrate that CHN1 may be an important causative gene for different ethnicities with DRS.

Incidence of symptomatic vertical and torsional diplopia after superior rectus transposition for esotropic Duane syndrome and abducens nerve palsy

To report the incidence of symptomatic vertical and torsional diplopia after superior rectus transposition (SRT) for esotropic Duane syndrome and abducens nerve palsy. The medical records of patients with esotropic Duane syndrome or abducens nerve palsy seen at Boston Children's Hospital (2006-2018) and treated with unilateral SRT with or without augmentation was performed. The primary outcome was incidence of postoperative vertical or torsional diplopia in primary position. The secondary outcome was induced vertical deviation in affected side gaze. A total of 69 patients met inclusion criteria: 32 with abducens nerve palsy and 37 with esotropic Duane syndrome. Vertical alignment changed in both hyper- and hypotropic directions. Median pre- and postoperative vertical deviation in primary gaze was 1.1Δ (10th-90th percentile, 0Δ-6Δ hypertropia) and 0.4Δ (10th-90th percentile, 6Δ hypotropia to 8 Δ hypertropia), respectively. Postoperative vertical diplopia occurred in 7%, including 4 of 49 treated with loop myopexy (8%), 1 of 13 without augmentation (8%), and 0 of 7 treated with sclera-fixated augmentation. All but one was successfully treated with prism or secondary surgery. Intorsional change predominated, but no patient had torsional diplopia post adjustment. Vertical misalignment in affected side gaze increased from 19% to 45% after SRT (P=0.01). In this largest-to-date review of patients treated with SRT, with or without MR recession, no patient developed persistent torsional diplopia, while 7% developed symptomatic vertical diplopia in primary position, similar to the reported incidence after balanced vertical rectus transposition. Vertical misalignment in affected side gaze increased, however fusion is already limited by unresolved esotropia in this field.

Adjustable graded augmentation of superior rectus transposition for treatment of abducens nerve palsy and Duane syndrome

To report the results of adjustable graded augmentation of superior rectus transposition, a novel modification of superior rectus transposition (SRT) designed to reduce postoperative vertical or torsional diplopia. The medical records of patients who underwent adjustable graded augmentation of SRT with or without adjustable medial rectus recession (MRc) from February 2017 to December 2019 were reviewed retrospectively. A Mendez ring was used to monitor torsional change after transposition of the superior rectus muscle to the lateral rectus muscle and after sequential placement of 2 or 3 augmentation sutures by superior rectus-lateral rectus loop myopexy. If excessive mechanical intorsion was induced, the responsible augmentation suture was severed intraoperatively. If torsional or vertical diplopia was noted after recovery, the distal-most augmentation suture was cut. Exotropia was managed by severing the distal-most augmentation suture or by medial rectus adjustment. A total of 8 patients who underwent adjustable graded augmentation of SRT were included (6 using the 3-suture technique): 3 for esotropic Duane syndrome, 2 for abducens nerve palsy, 1 for Moebius syndrome, and 2 for combined trochlear and abducens nerve palsies. Of the 8 patients, 4 had prior strabismus surgery, and 1 patient had previously undergone treatment with botulinum toxin. Severing one augmentation suture in 3 cases resolved vertical (n=2) or torsional (n=1) diplopia and consecutive exotropia (n=2), resulting in excellent alignment and reduction of torticollis to ≤4° in 7 cases. The technique proved insufficient in 1 patient, who had undergone 3 prior strabismus procedures. In this study cohort, adjustable graded augmentation of SRT effectively managed the risk of postoperative vertical or torsional diplopia.

Tendon elongation with bovine pericardium in strabismus surgery\u2014indications beyond Graves\u2019 orbitopathy

BackgroundFor some patients with complex ocular motility disorders, conventional strabismus surgery is insufficient. Surgery with tendon elongation allows correction of larger angles and maintains a sufficient arc of contact for rectus muscles. This study reports results for tendon elongation with bovine pericardium (Tutopatch®) in indications other than Graves’ orbitopathy in which it is already widely used.MethodsWe reviewed the records of all patients who underwent surgery with Tutopatch® in our institution. Angles of squint and head postures were analyzed preoperatively, on the first postoperative day, and in the long term (median 9 weeks after the operation). Patients with Graves’ orbitopathy were excluded.ResultsFrom 2011 to 2018, the procedures on 58 eyes of 54 patients (35 females, median age 35 years (3–75)) met the inclusion criteria. Horizontal rectus muscle surgery (53 eyes) was conducted on patients with residual strabismus (13), Duane’s retraction syndrome with eso- (type I: 16)/exodeviation (type II: 2, type III: 1), 6th (7)/3rd nerve palsy (7), Möbius syndrome (2), congenital fibrosis of the extraocular muscles type 3A (CFEOM3A, TUBB3 mutation) (4), and orbital apex syndrome (1). Vertical rectus muscle surgery (5 eyes) was conducted on patients with myasthenia (1), vertical tropia after orbital floor fracture (1), CFEOM1 (2), and Parry-Romberg syndrome (1). 42 eyes had prior eye muscle surgery (1–5 procedures, median 1). Out of 45 patients with postoperative long-term data, 43 showed an angle reduction. Fifty-one percent had an angle of 10Δ (prism diopter) or less, one had a significant over-effect, and 10 had revision surgery. For the heterogeneous group of residual eso- and exotropias, the median absolute horizontal angle was reduced from 35Δ (16 to 45Δ) to 9Δ (0 to 40Δ), for Duane’s retraction syndrome from 27.5Δ (9 to 40Δ) to 7Δ (0 to 40Δ), and for sixth and third nerve palsies from 43Δ (20 to 75Δ) to 18Δ (4 to 40Δ). For 3 patients with vertical rectus muscle surgery, the median absolute vertical angle was reduced from 30Δ (20 to 45Δ) to 4Δ (1 to 22Δ). The motility range was shifted in the direction contrary to the elongated muscle in all subgroups. A considerable reduction of the excursion into the field of action of the elongated muscle had to be registered.ConclusionsStrabismus surgery with bovine pericardium introduces new surgical options for complicated revisions and for rare and complex oculomotor dysfunctions. Yet, it has to be recognized that this type of surgery aiming at maximum effects, despite preservation or restitution of the arc of contact, leads to reduction of the excursion into the field of action of the elongated muscle. Furthermore, dose finding can be difficult depending on the underlying pathology and more than one intervention might be necessary for optimal results.