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Purpose: This study was conducted to further define the specific clinical characteristics of patients with Brown syndrome and evaluate the outcomes of superior oblique tenotomy in its surgical management. Methods: A retrospective analysis of the medical charts of 45 patients with Brown syndrome was performed, which revealed that 11 patients underwent superior oblique tenotomy due to abnormal head posture and/or hypotropia and 1 patient underwent bilateral superior oblique tendon elongation with a silicone band due to abnormal head posture. In the last patient, silicone bands were removed at the postoperative 3rd month due to the lack of improvement in the abnormal head posture and the limitation of elevation in adduction. Simultaneous horizontal rectus muscle surgery was performed in four patients. Results: There was a predominance of female gender, right eye, congenital form, unilaterality, A-pattern, and an abnormal head posture type with a combination of chin up and head tilting. Bilateral form was observed only in female patients. Amblyopia was detected in two patients. Among patients aged >5 years, 40% had reduced stereopsis. Abnormal head posture was found in 60% of patients. More than half of them were diagnosed with a vertical and/or horizontal deviation. Tenotomy procedure eliminated the abnormal head posture in all patients and significantly improved the mean limitation of elevation in adduction and hypotropia (p=0.001, p=0.012). Two patients developed inferior oblique overaction in the operated eye. There was complete spontaneous resolution in two patients. Conclusions: The clinical features of patients with Brown syndrome in our study are considerably consistent with those of previous reports. The present study demonstrated the effectiveness of superior oblique tenotomy with less overcorrection in the surgical treatment of Brown syndrome. RESUMO Objetivo: Definir mais detalhadamente as características clínicas específicas de pacientes com síndrome de Brown e avaliar os resultados da tenectomia do músculo oblíquo superior no manejo cirúrgico da síndrome de Brown. Métodos: Prontuários de 45 pacientes com síndrome de Brown foram analisados retrospectivamente. Onze pacientes submetidos à tenectomia do músculo oblíquo superior devido a postura anormal da cabeça ou a hipotropia e um paciente submetido ao alongamento bilateral do tendão do oblíquo superior com uma faixa de silicone devido a postura anormal da cabeça. Neste último paciente, a faixa de silicone foi removida no terceiro mês pós-operatório devido à ausência de melhora na postura anormal da cabeça e à limitação da elevação em adução. Quatro pacientes submeteram-se simultaneamente à cirurgia do músculo reto horizontal. Resultados: Houve predominância de sexo feminino, olho direito, forma congênita, acometimento unilateral, padrão em “A” e um tipo de postura anormal da cabeça combinando queixo elevado e inclinação da cabeça. A forma bilateral foi vista apenas em pacientes do sexo feminino. Foi constatada ambliopia em 2 pacientes. Dentre os pacientes acima de 5 anos de idade, 40% tinham estereopsia reduzida. Postura anormal da cabeça estava presente em 60% dos pacientes. Mais da metade dos pacientes foi diagnosticada com um desvio vertical, horizontal ou ambos. O procedimento de tenectomia eliminou a postura anormal da cabeça em todos os pacientes e melhorou significativamente a limitação média da elevação em adução e a hipotropia (p=0,001 e p=0,012). Dois pacientes desenvolveram hiperação do músculo oblíquo inferior no olho operado. Resolução completa ocorreu espontaneamente em 2 pacientes. Conclusões: O quadro clínico dos pacientes com síndrome de Brown no nosso estudo é bastante consistente com os relatos iniciais na literatura. Este estudo mostrou a eficácia da tenectomia do oblíquo superior, com menor hipercorreção no tratamento cirúrgico da síndrome de Brown.

Background/Objective: To evaluate the success of combining ipsilateral inferior and superior oblique muscle surgery in young children with congenital unilateral superior oblique under action who present in infancy with a large socially noticeable head-tilt. Methods: A consecutive retrospective case series of young children was analysed. The success of surgery in eliminating the head-tilt was evaluated by pre- and postoperative ocular motility assessment focusing on the vertical misalignment in primary position and downgaze, the magnitude of the head-tilt in degrees and the status of the superior oblique tendon. Results: Five children had a mean age at first surgery of 41 (range 25–63) months, a mean primary position vertical deviation of 26 (25–30) prism dioptres, a head-tilt of 30 (20–35) degrees and a mean post-operative follow up of 24 (8–43) months. While there was a uniform surgical plan, nonetheless each operation required individualisation based on a spectrum of per-operative superior oblique tendon findings. The head tilt was eliminated in 40% and reduced in the remainder, to a mean of 7 (0–18) degrees and with a mean post-operative primary position vertical misalignment of 3 (range 0–10) and of 10 (range 0–40) prism dioptres in downgaze. Conclusion: Combined, ipsilateral oblique muscle surgery reduced the severe head tilt and primary position alignment to a psychosocially and functionally acceptable level. For the majority, the outcome was stable or associated with further decremental improvement. A persistent downgaze vertical tropia occurred in children with macroscopically abnormal superior oblique tendons but these cases were not identifiable clinically pre-operatively.

Purpose: Literature regarding different superior oblique (SO) weakening procedures showed variable results. Here, we aim to evaluate the effect of a novel superior oblique tendon suture lengthening (SOSL) procedure on weakening of SO in patients with A-pattern exotropia associated with dissociated vertical deviation and SO overaction (triad exotropia). Methods: The medical records of triad exotropia patients who underwent SOSL or SO tenotomy were reviewed. Surgical results of SOSL procedure mainly regarding the correction of A pattern, SO overaction, and fundus intorsion were analyzed and compared with those of SO tenotomy procedure. Results: SOSL demonstrated comparable efficacy in correction of A pattern (20.2△ ± 10.7△ vs 29.2△ ± 16.1△, p = 0.172), normalization of SO overaction (1.9 ± 0.9 vs 2.4 ± 1.5, p = 0.349), and conversion of fundus intorsion (11.1° ± 7.0° vs 11.3° ± 4.4°, p = 0.691) as SO tenotomy. Moreover, the success rate of A pattern collapse was significantly higher in the SOSL group than in the SO tenotomy group (86% vs 40%, p = 0.028). None of the patients in the SOSL group, but two in the SO tenotomy group, presented SO palsy postoperatively. In the SOSL group, the corrected magnitude of SO overaction strongly correlated with the dosage of suture lengthening ( p < 0.001). Conclusion: SOSL procedure could effectively eliminate the clinical manifestations associated with SO overaction. The graded dosage of SOSL leads to more controllable and predictable results compared to SO tenotomy, which makes SOSL a good alternative choice for SO weakening.

A 67-year-old woman presented with a left hypotropia and eye pain after a traumatic fall. She had multiple left orbital wall fractures and an acquired limitation to elevation in all gazes, worse in adduction, suggestive of Brown syndrome. During strabismus surgery, a white nodule on the superior oblique tendon was identified and excised. Histopathology of the nodule revealed densely aggregated inflamed fibrovascular and fibrocollagenous scar tissue. Superior oblique rupture with spontaneous reparative reapproximation resulting in nodular formation is uncommon. We speculate that resulting tendon shortening may have contributed to the apparent Brown syndrome in this patient.

Background Adjustable bilateral Harada-Ito procedures have been described, sometimes with asymmetric adjustment used to correct vertical misalignment when coexisting with torsional strabismus. We investigated the causes of significant postoperative torsional incomitance noted in some patients undergoing these procedures. Methods The medical records of patients who underwent bilateral Harada-Ito procedures for bilateral trochlear nerve palsy between 1980 and 2018 were reviewed retrospectively. Cases with simultaneous operation on any other oblique or vertical rectus muscle were excluded. Surgical results, especially using Lancaster red-green (Lan R-G) plots, were correlated with the procedures performed. Results A total of 17 patients were included. At their last follow-up visit (mean, 12 months after surgery), 9 were diplopia free. Of the 8 with continuing diplopia, 2 had undercorrection and 1 had Brown syndrome. In 5 patients with continuing diplopia, there was relative intorsion of the eye movement paths in upgaze and relative extorsion of these paths in downgaze, a type of torsional incomitance. Asymmetric adjustment with tightening of one superior oblique tendon, and often loosening of the contralateral superior oblique tendon, had been performed in those 5 cases. Only 1 of the successful cases had the same type of asymmetric adjustment. There was a positive association between the severity of the preoperative Lan R-G pattern and postoperative diplopia. Conclusions Asymmetric adjustment of bilateral Harada-Ito procedures when attempting to correct the coexisting vertical misalignment can cause significant torsional incomitance with incomplete correction of extorsion in downgaze and intorsion in upgaze. This pattern may result in postoperative diplopia that is surgically challenging to correct.

Brown syndrome is characterized by upward gaze impairment while the eye is in adduction. It is caused by abnormalities involving the superior oblique tendon-trochlea complex. Imaging can help confirm the diagnosis, shed light on its etiology, and determine the best course of treatment. However, reports of magnetic resonance imaging findings of acquired Brown syndrome are scarce in the literature. Here, we describe magnetic resonance imaging features of 2 cases of acquired Brown syndrome.

Purpose: To evaluate the efficacy of single or combined superior oblique tendon (SO) advancement for selected cases of unilateral superior oblique palsy (SOP).Methods: The medical records of 14 patients who underwent single or combined superior oblique tendon advancement in one institution from May 2017 to October 2018 were reviewed. All subjects with a diagnosis of unilateral SOP who underwent single or combined SO tendon advancement surgery were included. The goal of the surgery was to correct the hypertropia and head tilt. The single or combined SO tendon advancement surgery was selected based on the amount of hypertropia or head tilt and the Knapp classification of the SOP. The information recorded included pre- and postoperative deviation angle and ocular motility findings. The degree of upshoot in adduction was graded pre- and postoperatively.Results: Fourteen patients between the age of 3 and 52 years with unilateral superior oblique palsy were selected to undergo SO tendon advancement. Single SO tendon advancement was carried out in eight acquired Knapp class II or residual SOP patients. SO tendon advancement combined with contralateral inferior rectus recession was carried out in two acquired Knapp class II patients. SO tendon advancement combined with ipsilateral inferior oblique myectomy was carried out in four congenital Knapp class III patients. The changes in pre- and postoperative hypertropia at primary gaze in single SO tendon advancement patients were from 6.25 ± 2.12 prism diopters to 0.86 ± 1.46 prism diopters. The changes in degree of upshoot in adduction were from +1.86 to +0.21. Conclusions: SO tendon advancement worked well as single or combined muscle procedure to treat unilateral superior oblique palsy.

Purpose To assess the surgical effect of single-stage superior oblique recession with intraoperative suture adjustment under topical anesthesia and sedation in terms of A-pattern correction, vertical alignment, and superior oblique overaction. Methods The medical records of patients who underwent superior oblique weakening (recession with adjustable suture) for superior oblique overaction from 2015 to 2018 were reviewed retrospectively. Preoperative, pre- and postadjustment, and 6-week follow-up data were assessed and compared for A pattern, primary position hypertropia, superior oblique overaction scale and objective fundus torsion. Results A total of 29 patients (17-42 years of age) were included. Of 51 operated eyes, 37 underwent intraoperative adjustment (further recession of 1–4 mm) after superior oblique recession of 8 mm. Mean decrease in primary position hyperdeviation after adjustment was 3.6Δ ± 2.7Δ (range, 0Δ-8Δ); in A-pattern deviation, 5.5Δ ± 4.8Δ (range, 0Δ-16Δ). At 6 weeks’ follow-up, A pattern had either resolved completely or became clinically insignificant ( 10Δ. Conclusions In our study cohort, single-stage, unilateral superior oblique tendon recession with adjustable suture under topical anesthesia and sedation was well tolerated and resulted in good postoperative outcomes at 6 weeks’ follow-up, effectively correcting primary position vertical deviation of

BACKGROUND: We describe successful surgical treatment of superior oblique myokymia, which had recurred after superior oblique tenectomy. METHODS: Single case report. RESULTS: The 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. CONCLUSIONS: Complete 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.

Purpose To evaluate the results of a superior oblique thinning technique for the treatment of congenital Brown syndrome. Methods The medical records of consecutive patients at a single institution who were diagnosed with congenital Brown syndrome and underwent superior oblique thinning were retrospectively reviewed. In all cases, the prism cover test was used to assess ocular alignment. Standardized nine-gaze photographs were used to evaluate ocular rotations. Ocular torsion was measured using fundus retinography. Intraoperatively, all patients showed a positive forced duction test for elevation from adduction that became negative after a strongly maintained traction test maneuver. Results A total of 21 eyes of 20 consecutive patients (mean age, 5.8 ± 2.5 years) were included. Preoperatively, 16 patients were orthotropic in primary position; 4, hypotropic. Postoperatively, all patients were orthotropic in primary position. Limitation of elevation in adduction improved from −3.4 ± −0.6 (range, −2 to −4) to 0.6 ± 1 (range, 0 to −3; P 1° change in ocular torsion after surgery in 3 of 6 patients (P > 0.5). Superior oblique underaction or hypertropia did not occur. No superior oblique palsy appeared in any patient. There were no changes beyond 3 months postoperatively except for 1 patient who relapsed 9 months after surgery and 1 patient who improved after 4 years. Conclusions Superior oblique thinning is effective in improving elevation in adduction and hypotropia in primary position in patients with Brown syndrome associated with a thickened superior oblique tendon.

Purpose To report the dose-response relationship of adjustable Harada-Ito surgery, and postoperative stability of cyclodeviation. Design Retrospective cohort study. Methods Twenty patients underwent a unilateral adjustable Harada-Ito advancement of the anterior fibers of the superior oblique tendon, with or without resection. The surgical dose was calculated as resection plus advancement plus adjustment. Cyclodeviation was measured using double Maddox rods at preoperative and postoperative time points. We assessed dose effect by calculating change in cyclodeviation from preoperatively to postadjustment and evaluated the dose-response relationship by Spearman rank methods. Postoperative stability of cyclodeviation was assessed by comparing postadjustment measurements with 1-day and 6-week measurements, and also 1- and 5-year measurements, when available. Results Change in cyclodeviation from preoperatively to postadjustment ranged from 7 to 20 degrees of incyclo effect. There was a dose effect of 1.3 degrees per mm (r s = 0.52, P = .019). Postoperative incyclodeviation unwound a mean of 3.6 degrees ± 2.5 degrees between postadjustment and day 1. Overall change between postadjustment and the 6-week window was 6.5 ± 2.9 degrees in an excyclo direction. Unwinding continued with a mean change of 0.2 ± 2.6 degrees between the 6-week and 1-year windows, and of 0.4 ± 2.1 degrees between 1-year and 5-years windows. Conclusions Adjustable Harada-Ito advancement of the anterior fibers of the superior oblique tendon has a dose response of 1.3 degrees per millimeter, but its effect regresses over time. Based on our data, an immediate postoperative target incyclodeviation of 7 degrees is reasonable.

Brown syndrome is known as limited elevation of the affected eye during adduction. It is caused by a disorder of the superior oblique tendon, which makes it difficult for the eyeball to look upward, especially during adduction. It is classified into congenital true sheath Brown syndrome and acquired simulated Brown syndrome. Acquired simulated Brown syndrome can be caused by trauma, infection, or inflammatory conditions. The surgical restoration of blowout fractures can also lead to limitations of ocular motility, including Brown syndrome. We report on three patients with acquired simulated Brown syndrome, who complained of diplopia and limitation of ocular motility after operations to treat blowout fractures.

We report two patients, one with congenital dystrophic medial rectus muscles and one with absence of the medial rectus muscles; in addition, one of them had absence of the lateral rectus muscles. While absence of the superior oblique and superior rectus has been more commonly reported in literature, especially with craniofacial syndromes, our patients were nonsyndromic. Considering the risk of anterior segment ischemia, correction of the large-angle exotropia was performed by horizontal rectus muscle surgery where possible, along with transfer of the superior oblique tendon to the superior part of the normal medial rectus muscle insertion area to create a tethering effect with a good outcome.

We present a 47-year-old male with superior oblique (SO) tendon rupture that developed due to a broken metal hook while installing snow chains on his car. The patient presented to our clinic as an emergency for the right eye trauma and pain. The examination revealed a small cut in the right upper eyelid and an edematous tendinous structure that extended downwards through the conjunctival cut at the upper fornix. This structure was thought to be the SO tendon. Emergency exploration and SO tendon repair were performed under general anesthesia. The SO tendon was sutured to its own insertion region 5 mm in width and 5 mm behind a point 3 mm temporal to the superior rectus muscle. The conjunctiva was also repaired. There was 6 PD (prism diopter) right hypertropia and vertical diplopia on postoperative follow-up. The diplopia symptoms improved in the postoperative 3rd month. The patient had no diplopia at the 6th month follow-up but 4 PD hypertropia continued. [Med-Science 2016; 5(4.000): 1008-10]

Introduction: Undercorrections have been reported after a number of surgical procedures for Brown’s syndrome. The reason for late undercorrections is not clear but may be related to the heterogeneous etiology of Brown’s syndrome.Purpose: We report a patient with late undercorrection after superior oblique tendon recession for Brown’s syndrome that was partially relieved by a silicone spacer placed nasally.Results: We noted on surgical exploration that the superior oblique tendon had attached to the sclera under the nasal border of the superior rectus. Further weakening of the tendon was achieved by a No. 240 band expander that resulted in improvement of adduction in elevation.Conclusion: Undercorrections following surgery for Brown’s syndrome can be relieved by further weakening the superior oblique muscle. However, as late undercorrections have been reported, the optimal timing of surgery is not certain.

A 67-year-old white man with a history of right orbital trauma and restrictive strabismus underwent right superior oblique tenectomy for superior oblique restriction. Bleeding was noted from the superior rectus intraoperatively, despite the fact that the muscle was neither exposed nor hooked for the procedure. Subsequent exploration of the muscle revealed a full-width tear across the superior rectus muscle approximately 5 mm posterior to its insertion. The remaining residual distal muscle was removed from the insertion and the proximal end of the torn superior rectus muscle was identified and reattached to the insertion. We postulate that shearing forces exerted by a taut superior oblique tendon were sufficient to tear the overlying superior rectus muscle.

Brown syndrome is characterised by impaired supraduction worse in adduction due to a restricted superior oblique tendon passing through the trochlea. A few reports have previously described Brown syndrome after upper eyelid surgery, including blepharoplasty and ptosis repair. The authors describe two additional cases of Brown syndrome following ptosis repair. The first case is a 65-year-old woman with new-onset vertical binocular diplopia following bilateral levator advancement surgery. Ocular motility examination demonstrated moderate impairment of elevation in adduction. The second case is a 35-year-old woman who presented with new-onset intermittent binocular diplopia following right upper lid ptosis repair. Examination revealed large vertical fusional amplitudes and a large left intermittent hyperphoria in an alignment pattern consistent with Brown syndrome. Despite presenting after surgery, these cases differ in mechanism. The first case likely occurred due to intraoperative impairment of the superior oblique tendon sheath or trochlea, whereas the second case represented an unmasking of a long-standing, previous vertical strabismus that was consistent with a Brown syndrome pattern.

Bilateral fourth nerve palsy may be symmetric or asymmetric with combined vertical and excylotropic deviations and so there may be an advantage to independent adjustment of vertical and torsional components. We report a surgical technique that allows such independent adjustment.Retrospective interventional case series.Fifteen patients, aged 17-73 years, underwent adjustable bilateral superior oblique tendon advancements for bilateral fourth nerve palsy: 11 symmetric (≤2 prism diopters [pd] hyperdeviation in straight-ahead gaze) and 4 asymmetric. Motor alignment was assessed with double Maddox rods and prism and alternate cover tests preoperatively, pre- and postadjustment, and 6 weeks postoperatively.Preoperative torsion ranged from 7 to 30 degrees excyclotropia (mean 17 ± 7 degrees) and hyperdeviation from 0 to 10 pd. Preadjustment torsion ranged from 5 degrees excyclotropia to 40 degrees incyclotropia, and hyperdeviation from 0 to 8 pd. Twelve of the 15 patients (80%) were adjusted to a target of 0 pd hyperphoria and 10 degrees incyclotropia (actual mean 9 degrees incyclotropia, range 2-13 degrees incyclotropia). At 6 weeks postoperatively there was expected excyclodrift (to mean 4 degrees excyclotropia, range 0 degrees incyclotropia to 15 degrees excyclotropia), but 13 (87%) had 5 degrees or less excyclotropia and 14 (93%) had 2 pd or less hyperdeviation. Mean torsional correction from preoperative to preadjustment was 31 ± 14 degrees (P.0001), and from preoperative to 6 weeks was 13 ± 6 degrees (P.0001).Adjustable bilateral superior oblique tendon advancement allows independent control of torsional and vertical components of the deviation, and therefore may be useful in cases of bilateral superior oblique palsy.

Background: Bilateral fourth nerve palsy is characterised by excyclotorsion, which can be corrected by reinforcement of the anterior tendon fibres of the superior oblique muscle. Patients and Methods: A retrospective study of 40 consecutive patients with bilateral acquired fourth nerve palsy operated by a selective tuck of the anterior portion of the superior oblique tendon between 1994 and 2012 was undertaken. Horizontal, vertical and torsional deviations were measured in 9 diagnostic positions of gaze and the field of binocular single vision was evaluated with the Harms tangent screen. Postoperative follow-ups took place at 1 week, 6 months, and ≥ 3 years. Results: Preoperative mean excyclotorsion was 9° in the primary position and 15° in downgaze. These values decreased to 2° and 5° 6 months after surgery, and 2.5° and 6° at ≥ 3 years. Immediate post-operative incyclotorsion in upgaze (28 patients) and Brown syndrome (15 patients) regressed spontaneously. The median score of field of binocular single vision improved from 4 % preoperatively to 76 % postoperatively. Conclusions: The selective tuck of the anterior tendon fibers of the superior oblique tendon enables an efficient and long-lasting correction of the ocular torsion induced by bilateral trochlear palsy.

To report the surgical outcome of superior oblique tendon split lengthening for management of patients with severe type of congenital Brown syndrome. Brown syndrome is characterized by hypotropia in primary position and limitation of elevation in adduction more than -4.Fourteen consecutive patients with severe congenital Brown syndrome underwent superior oblique split lengthening surgery (10 mm). The amount of hypotropia in primary gaze and the degree of elevation in adduction were compared before and after the surgery. Any surgical complications were also recorded.Surgery was performed on 15 eyes in 11 female and 3 male subjects. Mean postoperative follow-up time was 12.93 ± 1.79 months (ranging from 10 to 16 months). Average hypotropia in primary gaze improved from 16.2 ± 5.5 prism diopters (range: 10-25 prism diopters) preoperatively to 5.9 ± 4.0 prism diopters (range: 0-18 prism diopters) postoperatively recorded at final follow-up examination (p 0.001). The limitation in adduction improved from -7.2 ± 4.5 (range: -6 to -8) preoperatively to -1.8 ± 1.3 (range: zero to -5) postoperatively (p 0.001). No surgical complications were noted.Superior oblique split lengthening has a significant effect on reducing primary gaze hypotropia and improving elevation in adduction. This technique should be considered for the treatment of patients with severe congenital Brown syndrome.

To determine the position of rectus muscle pulleys in Japanese eyes and to evaluate the effect of oblique muscle surgery on rectus muscle pulleys. Quasi-coronal plane MRI was used to determine area centroids of the 4 rectus muscles. The area centroids of the rectus muscles were transformed to 2-dimensional coordinates to represent pulley positions. The effects of oblique muscle surgery on the rectus muscle pulley positions in the coronal plane were evaluated in 10 subjects with cyclovertical strabismus and, as a control, pulley locations in 7 normal Japanese subjects were calculated. The mean positions of the rectus muscle pulleys in the coronal plane did not significantly differ from previous reports on normal populations, including Caucasians. There were significant positional shifts of the individual horizontal and vertical rectus muscle pulleys in 3 (100%) patients with inferior oblique advancement, but not in eyes with inferior oblique recession and superior oblique tendon advancement surgery. The surgical cyclorotatory effect was significantly correlated with the change in the angle of inclination formed by the line connecting the vertical rectus muscles (p = 0.0234), but weakly correlated with that of the horizontal rectus muscles. The most important factor that affects the pulley position is the amount of ocular torsion, not the difference in surgical procedure induced by oblique muscle surgery.

To report the results of treatment of Brown syndrome using Achilles tendon allograft as a superior oblique tendon expander.Achilles tendon allografts were prepared from the tissue bank. Five eyes of 3 patients with congenital Brown syndrome (mean age, 6 years) were enrolled in the study. Surgery was performed with a superotemporal conjunctival fornix approach. The superior oblique tendon was exposed and severed; then a 2- to 3-mm-wide strip of Achilles tendon (with a thickness of 1-2 mm) was trimmed and sutured between the cut ends using double-armed 5-0 Mersilene sutures. The length of the expander ranged from 7 mm to 8 mm, depending on the severity of the disease.The preoperative hypotropia in primary position ranged from 15(Δ) to 30(Δ), with abnormal head position in 1 patient. Nine months after the operation, 2 eyes with moderate Brown syndrome showed an improvement in the hypotropia to3(Δ), and 2 eyes with severe disease had residual hypotropia of 5(Δ). Another eye with severe Brown syndrome had a residual hypotropia of 20(Δ). The elevation in adduction improved by 1 grade in 1 eye and resolved in the other 4 eyes. The amount of correction in the deviation improved with time. None of the patients developed secondary superior oblique palsy, rejection, infection, extrusion, prolonged ocular inflammation, or foreign body sensation.Superior oblique tendon elongation with Achilles tendon allograft had acceptable short-term results in 4 of 5 eyes treated for Brown syndrome.

To evaluate the outcomes of a superior oblique tendon spacer procedure using nonabsorbable adjustable sutures in patients with Brown syndrome.This noncomparative interventional case series includes 25 eyes of 25 patients with Brown syndrome. In all patients the superior oblique tendon was exposed; two nonabsorbable polyester sutures were placed 4 mm apart, and the tendon was cut. With the use of a slipknot, the cut ends of the tendon were separated 5 to 8 mm. Tendon separation was adjusted intraoperatively according to the exaggerated traction test and indirect ophthalmoscopy.Overall 25 eyes of 25 patients with mean age of 8.00 +/- 4.62 years were operated and followed for a mean period of 13.2 +/- 7.6 months (range, 3 to 30 months). Mean elevation in adduction improved from -3.96 before surgery to -0.67 (p0.001); mean hypotropia improved from 11.08(Delta) to 0.32(Delta) (p0.001). Two patients developed overcorrection, but recurrence was not observed in any case. The patients continued to improve over the follow-up period.The adjustable superior oblique tendon suture spacer procedure has favorable results and seems to be technically easier than a silicone expander procedure for Brown syndrome.

Purpose of review This review discusses recent advances in our understanding of the pathology, diagnosis, and treatment of congenital fourth nerve palsies. Recent findings High-definition MRI, which can now more accurately image the trochlear nerve, has increased our understanding of the pathology of fourth nerve palsies. A more sensitive two-step test is proposed to replace the three-step Parks-Bielschowsky test. Procedures strengthening the superior oblique tendon can be performed with a low incidence of secondary Brown syndrome. Summary Recent MRI studies have shown two pathological mechanisms associated with congenital superior oblique palsies. Superior oblique tuck combined with inferior oblique recession is a very effective way of eliminating head tilt secondary to congenital fourth nerve palsies.

Purpose To evaluate the efficacy of superior oblique muscle split Z-tendon lengthening in the treatment of superior oblique muscle overaction with respect to normalization of superior oblique ductions and versions, reduction of A -pattern strabismus and correction of vertical deviations. In addition, to determine the incidence of complications of the procedure including overcorrection and the effect of the procedure on horizontal deviation. Methods A retrospective review was performed of 19 patients who underwent superior oblique muscle split Z-tendon lengthening for A -pattern strabismus or significant vertical deviation associated with superior oblique overaction between June 1980 and September 1999. Ten males and 9 females were included, ranging in age from 2 to 45 years (mean: 9 years). Patients underwent either a unilateral or bilateral superior oblique tendon lengthening based on the patient’s clinical picture. Results Ten of 11 patients (91%) who underwent unilateral tendon lengthening and 10 of 16 eyes (63%) that underwent bilateral superior oblique tendon had complete normalization of superior oblique action. Seven of 10 patients (70%) who underwent unilateral or bilateral tendon lengthening for a clinically significant A -pattern had the A -pattern completely eliminated or brought to a clinically insignificant level. All 8 patients who underwent a unilateral tendon lengthening for a clinically significant vertical deviation had reduction of the vertical deviation. There was no statistically significant effect of either the unilateral or bilateral procedure on horizontal deviation. Conclusions The superior oblique muscle split Z-tendon lengthening procedure is highly effective for normalization of superior oblique muscle function and reduction of A -pattern strabismus and vertical deviations associated with superior oblique muscle overaction.

Introduction To evaluate the efficacy of superior oblique Z-tenotomy in the treatment of overdepression in adduction (superior oblique overaction) with respect to collapse of A-pattern strabismus and normalization of versions. Methods The medical records of patients who underwent bilateral superior oblique Z-tenotomy for mild to moderate overdepression in adduction and A-pattern strabismus between June 2004 and August 2010 were retrospectively reviewed. Surgical indications included mild to moderate overdepression in adduction (+2 to 3) and an A pattern of 10 Δ to 40 Δ between upgaze and downgaze. A monopolar electrode microdissection needle was used to make two tenotomies, 60% to 80% in width and 8 mm apart, perpendicular to the axes of the tendon in opposite directions. Surgical success included a postoperative A pattern of ≤10 Δ and overdepression in adduction of ≤1. Results A total of 20 patients (mean age, 9.8 years; age range, 3-34) underwent bilateral superior oblique Z-tenotomy during the study period: 2 patients (10%) with esotropia and 18 with exotropia (90%). Average decrease in pattern was 16 Δ (range, 0 Δ -32.5 Δ ). Success rate for pattern collapse was 78%, with resolution of overdepression in adduction of 90%. Conclusions Z-tenotomy of the superior oblique tendon collapsed A-pattern strabismus and normalized versions.

The superior oblique tendon can be functionally divided into the anterior one third, which is responsible for intorsion, and the posterior two thirds, which is responsible for depression and abduction (Fig. 18.1). Superior oblique tightening procedures are based on this physiologic division and either tighten the whole tendon (i.e., full tendon tuck) or tighten the anterior tendon fibers (i.e., the Harada-Ito procedure).

Superior oblique tendon weakening procedures are used in the management of various types of strabismus, including superior oblique overaction, inferior oblique palsy, and Brown’s syndrome (see Chap. 7). Most procedures weaken the superior oblique muscle by slackening the tendon. Uncontrolled procedures include tenotomy and tenectomy, in which the tendon is cut and the cut ends are free to widely separate or scar back together. These uncontrolled procedures should not be used in patients with binocular fusion, as the incidence of consecutive superior oblique palsy is more than 50 %.

We report two patients who presented with Brown’s syndrome. The first is a 7-year-old boy who at the time of his diagnosis was also found to have enthesitis and HLA-B27 positivity. The second patient was diagnosed with bilateral Brown’s syndrome at 13 months of age. At age 7 she developed a persistent oligoarticular arthritis and unilateral anterior iritis consistent with the oligoarticular Juvenile Idiopatic Arthritis (JIA) phenotype. These cases highlight ophthalmologic findings and diagnostic considerations with respect to Brown’s syndrome and associated childhood onset rheumatologic disease.

Purpose: Philip Knapp described a method, sometimes referred to as the "chicken suture,"of securing a loose nonabsorbable suture to the cut ends of the superior oblique tendon to facilitate future reversal. The purpose of this study is to describe a modification of Knapp's technique to achieve partial, reversible, and intraoperatively adjustable superior oblique weakening. Methods: The superior oblique tendon was exposed, 2 polyester nonabsorbable sutures were placed 4 mm apart, and the tendon was cut. With the use of a slip knot, the cut ends of the tendon were separated by 2 to 8 mm. Tendon separation was adjusted intraoperatively according to the exaggerated traction test and, in some cases, fundus torsion. Medical records of all patients who underwent surgery with this technique were reviewed and the outcomes tabulated. Results: Twelve patients (16 eyes) were treated for superior oblique overaction and 3 patients (3 eyes) for Brown syndrome. Follow-up was 2 to 46 months (mean, 17 months). Mean superior oblique overaction improved from +1.3 before surgery to +0.3 after surgery, mean A pattern improved from 20 PD to 2 PD, and fundus intorsion improved from +1.2 to +0.3. In Brown syndrome, the mean elevation in adduction improved from −3.8 to −1.0. One patient from each group developed an overcorrection. None of the patients developed recurrence. The patients with Brown syndrome continued to improve over a 1-year period. Conclusions: The superior oblique tendon suture spacer is effective, intraoperatively adjustable, and technically easier to perform than a silicone expander procedure. This technique should be considered as an alternative for patients requiring superior oblique weakening. (J AAPOS 2001;5:164-71)

Brown syndrome is characterized by restricted elevation in adduction. Congenital Brown syndrome usually is diagnosed during early childhood. It is believed to be caused by an abnormality of the superior oblique tendon as it passes through the trochlea. The natural history of Brown syndrome is poorly characterized. Many patients with congenital Brown syndrome undergo strabismus surgery during childhood in an attempt to correct the problem surgically. This report describes spontaneous regression of congenital Brown syndrome in an adult.

Purpose: We describe 6 cases of a previously unreported variation of bilateral Brown's syndrome that presented in congenital form in one eye and developed later in the fellow eye with no underlying cause. Methods: We reviewed the clinical records of 6 patients from 6 separate practices to determine whether there were any common clinical features on presentation or in their clinical courses. Results: All 6 patients were diagnosed with unilateral congenital Brown's syndrome at the first ophthalmologic assessment but showed no evidence of the syndrome in the fellow eye. In 5 cases the contralateral syndrome developed in the second eye after surgery was performed on the first eye, and in 1 case it developed before any surgery was done. The ages at onset of the syndrome in the second eye ranged from 2 to 8 years. None of the children had any evidence of systemic illness or local orbital disease to explain an acquired Brown's syndrome. Conclusion: To our knowledge, this is the first reported series of cases of bilateral Brown's syndrome that manifested sequentially in the eyes with no known causes for an acquired syndrome in the second eye. This finding supports the premise that congenital and acquired Brown's syndrome are on a continuum with a common pathophysiology of restriction of free movement of the superior oblique tendon in the trochlea. (J AAPOS 2000;4:158–63) J AAPOS 2000;4:158–63

This case report describes a child who developed an acquired bilateral sequential Brown’s syndrome, confirmed each time intraoperatively, that is likely the result of a new mechanism not previously described in the literature. Arguments are provided to support the premise of a new mechanism and a new measure of relative trochlear position is suggested to further elucidate the role of “desagittalization” of the superior oblique tendon in the spectrum of superior oblique dysfunction.

Brown's syndrome is characterized by a limitation of elevation in adduction, slight or normal limitation of elevation in abduction, divergence in straight upgaze (V-pattern), intorsion in upgaze and positive forced duction. It is caused by a tight or inelastic superior oblique tendon. Benign joint hypermobility is a hereditary disease of the connective tissue characterized by an increase of mobility in diverse joints. Its prevalence is very changeable regarding age range, sex and ethnicity, varying from 2 to 35% in men and 5 to 57% in women. In this case the authors describe a case of Brown's syndrome associated with Benign joint hipermobility and call attention to a little described association in the literature. J.C.S, male, 6 years old, mulatto, student, was referred to the University of Santo Amaro with complaint of exotropia for 2 years that it increased in supraversion. The patient with the diagnosis of bilateral Brown's syndrome, was diagnosed as having benign joint hipermobility by the reumatologist. The patient with benign joint hipermobility can develop symptoms such as arthralgia caused by a joint inflammation. We believe in the possibility that Brown syndrome has occurred, caused by an inflammatory process in the trochlea that started because of the benign joint hypermobility.

Purpose: To investigate the efficacy and safety of isolated superior oblique tucking in the treatment of congenital superior oblique palsy. Methods: Twenty-one patients with unilateral congenital superior oblique palsy and lax superior oblique tendon underwent isolated superior oblique tucking and retrospective analysis of the amount of tucking procedure, preoperative and postoperative vertical deviation in the primary position and reading position, abnormal head posture, ocular motility, and corrected objective torsion. Patients were evaluated before and 6 to 12 months after surgery. Results: The average vertical deviation in the primary position was 10.9 ± 3.3 prism diopters (PD) before surgery and 1.5 ± 2.6 PD after surgery (n = 21, P < .05). The average vertical deviation in the reading position was 15.6 ± 3.6 PD before surgery and 2.6 ± 3.1 PD after surgery ( P < .05). The average corrected vertical deviation was 9.9 ± 3.2 PD in the primary position and 13.0 ± 3.4 PD in the reading position ( P < .05). The amount of tucking ranged from 5 to 12 mm (mean: 8.3 ± 2.3 mm), which was not related to the corrected vertical deviation in the primary and reading positions, the preoperative vertical deviation in the reading position, or the corrected objective torsion. After surgery, all patients had head posture that was normal or less than 5°. Unremarkable superior oblique underaction and negative head tilt test were found in all patients after surgery. All patients had mild Brown syndrome after surgery, but none were symptomatic. Conclusions: Isolated superior oblique tucking is an efficient and safe procedure for treatment of superior oblique palsy with hyperdeviation less than 15 PD in the primary position and remarkable superior oblique underaction. The superior oblique forced duction test was the most important criterion for planning surgery. [J Pediatr Ophthalmol Strabismus 2014;51(4):249–254.]

The authors describe two cases with unilateral aplasia of the superior oblique tendon. The incomitance pattern could not be explained solely by the lacking torque of the superior oblique muscle. Rather, a considerable adaptation of the central nervous system had to be invoked resulting in compensatory innervation of the remaining seven vertical muscles of both eyes.

Proteus syndrome is a disorder of patchy, or mosaic, postnatal overgrowth. Mosaic somatic mutation of the AKT1 gene has been identified in over 90% of individuals meeting the diagnostic criteria for Proteus syndrome. Onset occurs in infancy and can involve any tissue of the body. The connective tissue and bone, skin, central nervous system, and the eye are commonly involved. Epibulbar tumors, strabismus, and posterior segment involvement have previously been reported. However, there have not been any reports of lesions affecting the extraocular tendons. We report a case of Proteus syndrome patient presenting with vertical strabismus secondary to a fibrous tumor within the superior oblique tendon. The tumor was successfully excised with complete resolution of the strabismus.

Diplopia in the reading position developed in two patients with unilateral Brown syndrome after a 7-millimeter section of #240 silicone retinal band was sewn between the cut ends of the superior oblique tendon at tenotomy. In both cases, forced ductions were positive, indicating a restrictive downgaze deficit. Surgical exploration revealed adhesions that prevented the normal sliding of the superior oblique tendon beneath the superior rectus muscle. Forced ductions became normal and downgaze improved after removal of the silicone band. Secondary superior oblique muscle paresis was also evident at the time of reoperation, requiring recession of the contralateral inferior rectus muscle (cases 1 and 2) and recession of the ipsilateral inferior oblique muscle (case 1). A restrictive downgaze deficit and a paretic overcorrection must be recognized as possible complications of the superior oblique tendon silicone "expander" operation.

Purpose To compare the surgical results in patients with congenital superior oblique palsy treated with inferior oblique recession (IOR) versus those treated additionally with superior oblique tendon tuck (IOR+SOT). Methods A consecutive, nonrandomized series of patients with congenital superior oblique palsy with vertical deviations in adduction between 10° and 20° were retrospectively reviewed. In the first series, patients were treated with IOR; in the second series, patients were treated with IOR+SOT. We examined the effects of surgery on binocular alignment and on the field of binocular single vision. Results Each group consisted of 20 patients. Preoperative ocular deviations were similar in both groups. Postoperative residual vertical deviations were not different in the primary position or in downgaze; however, significantly better alignment was achieved in the IOR+SOT group in adduction and downgaze in adduction. Consecutive Brown pattern occurred in 18 of 20 patients who underwent IOR+SOT versus 5 of 20 who underwent IOR. Residual torsion was not statistically different. The field of binocular single vision improved to 92% (range, 28%-100%) after IOR and to 96% (range, 64%-100%) after IOR+SOT ( P = 0.20). Conclusions IOR alone is a suitable procedure for most congenital superior oblique palsies with a moderate-to-large vertical deviation in adduction, resulting in a lower incidence of consecutive Brown pattern than with IOR+SOT.

A 5-year-old girl with Noonan-neurofibromatosis syndrome was diagnosed with bilateral superior oblique palsy. At surgery, the right superior oblique tendon was absent, and further exploration revealed abnormal tissue inserting into Tenon's capsule. Orbital imaging was not performed. Congenital absence of the superior oblique tendon was diagnosed. Although Noonan syndrome is known to have many ocular manifestations, absence of the superior oblique tendon has not been previously reported.