[High-dose medialis recession in latent nystagmus]. / Hoch dosierte Medialisrücklagerung bei Nystagmus vom Latenstyp.

BACKGROUND: Large muscle recessions reduce anomalous head turn (AHT) in infantile nystagmus syndrome (INS). Their quantitative effect to reduce AHT in fusion maldevelopment nystagmus syndrome (FMNS) is unknown. AIM OF THE STUDY: To evaluate the reduction of AHT by large medial rectus muscle recession in patients with FMNS. METHODS: We analyzed data of 12 patients with pure FMNS who received large medial rectus muscle recessions on the sound eye or only eye between 2014 and 2019. Medians and ranges (min-max) were: age at surgery 14 years (3-43 years); decimal BCVA sound eye/fellow eye 0.56 (0.4-1.0)/0.01 (0-0.4); AHT at 5 m/0.3 m fixation distance 35° (15-45°)/20° (0-45°); amount of recession 13 mm (11.5-15 mm); follow-up 13 (4-39) months. RESULTS: At the final visit, AHT at 5 m/0.3 m was 10° (0-20°/0-15°). The individual reduction of AHT at 5 m amounted to 25° (8-35°) without any overcorrection. The available visual field of functionally monocular patients increased correspondingly. Slight overcorrection at 0.3 m occurred in 2 cases. Adduction of the sound eye was limited to 25e-40°). BCVA and anomalous head posture in vertical and frontal planes did not improve. CONCLUSION: Large medial rectus muscle recession on the sound eye improves AHT in FMNS by a similar amount compared to INS and the available visual field of functionally monocular patients.

Vertical transposition of the horizontal rectus muscles to correct head tilt because of infantile nystagmus syndrome - A case series.

Purpose: Head tilt associated with infantile nystagmus syndrome (INS) can be corrected by (a) operating the oblique muscles, (b) horizontally transposing the vertical rectus muscles, or (c) vertically transposing the horizontal rectus muscles. We report three cases of INS with head tilt corrected by vertically transposing the horizontal rectus muscles in both the eyes. Methods: Three cases of head tilt with INS from an institutional practice operated by a single surgeon were retrospectively reviewed and analyzed. The intervention included full tendon width transposition (upward or downward) of all four horizontal rectus muscles to induce cyclotorsion in the direction of head tilt. The primary outcome measure was the correction of head tilt in the primary position. Results: Three patients (boys) of ages ranging from 4 to 7 years with a pre-operative head tilt of 30° were operated upon. Although one patient's oblique muscles had been operated on to correct head tilt, another patient had an unmasked face turn after the surgery, which was corrected with a modified Anderson's procedure. Post-operatively, all patients had a reduction of head tilt to a range of 0-10°. Conclusion: Vertical transposition of horizontal rectus muscles is a simple surgical option to correct head tilt in INS. However, the results may vary based on individual cases.

Recession-Resection of the Vertical Rectus Muscles for Chin-up Vertical Abnormal Head Position Associated With Infantile Nystagmus Syndrome.

PURPOSE: To review the correction of chin-up abnormal head position (AHP) due to infantile nystagmus syndrome via surgery on the vertical rectus muscles, typically a combined recession-resection of these muscles. METHODS: This was a review of 6 patients who underwent surgical correction of chin-up vertical AHP in the context of infantile nystagmus syndrome at an academic institution. The correction of AHP, visual acuity, ductions, and ocular alignment were noted both preoperatively and postoperatively. The need for repeat surgery, induced strabismus, or correction of AHP were also noted. RESULTS: Six patients underwent surgery for chin-up AHP. A combined recession-resection of the vertical rectus muscles (bilateral inferior rectus muscle recession of 5 to 8 mm; bilateral superior rectus muscle resection of 7 to 8 mm) was performed in 4 of 6 patients, and isolated bilateral recession of the inferior rectus muscles was performed in the remaining 2 patients. Four of 6 patients (67%) achieved complete correction of their AHP at the last follow-up visit, with a mild residual chin-up AHP persisting in the other 2 patients. One patient developed large angle exotropia, one had restrictive hypertropia and horizontal plane null position, and another developed an incomitant horizontal strabismus with exotropia in right gaze. Reoperation was performed in the former 2 patients, with successful correction of the strabismus in each. CONCLUSIONS: Surgery on the vertical rectus muscles can reduce or eliminate a chin-up head position in patients with infantile nystagmus syndrome. Care should be taken to avoid producing a restriction of depression in abduction if the amount of resection is too large. [J Pediatr Ophthalmol Strabismus. 2022;59(6):410-415.].

Titanium T-Plate as a Stabilizing Platform in the Management of Acquired Nystagmus and Oscillopsia Without a Null Zone.

The primary goal of nystagmus treatment is to improve visual function and quality of life. Current surgical interventions are limited by regression of effect, serious complications, and reliance on a null zone. Tse and colleagues in 2017 reported the use of a T-plate/suture fixation platform for globe stabilization to treat acquired nystagmus and oscillopsia without a null zone in a patient with bilateral internuclear ophthalmoplegia. However, the improvement in nystagmus was not objectively quantified. This case series reports 2 patients who underwent bilateral T-plate placement which resulted in immediate and sustained improvement of nystagmus objectively measured by videonystagmography.

Clinical and Electrophysiological Outcomes After Eye Muscle Surgery in 81 Adults With Infantile Nystagmus Syndrome.

PURPOSE: To characterize the effects of eye muscle surgery on patients older than 18 years with infantile nystagmus syndrome (INS) who have had only optical treatment. METHODS: This was a prospective, single-center, interventional case series analysis of clinical and electro-phyisological data before and after surgery. Outcome measures included: clinical characteristics, surgical procedure, and preoperative and postoperative binocular best corrected visual acuity (BCVA) in the null position, anomalous head posture (AHP), contrast sensitivity, strabismic deviation, and nystagmus acuity function (NAFX). Postoperative data used were collected for a minimum of 12 months after surgery. Parametric and non-parametric statistical analysis of the outcome measures was performed. RESULTS: Ages ranged from 18 to 72 years (average: 36 years) and follow-up from 12 to 74 months (average: 26 months). A surgical algorithm of nine separate procedures involving at least two recti muscles on each eye was used for each patient. Most patients had associated systemic and/or ocular diagnoses, including albinism (35%), amblyopia (23%), optic nerve or retinal disorders (48%), refractive error (80%), AHP (44%), aperiodicity (27%), and strabismus (69%). There were no serious surgical complications, with a reoperation rate of 12%. There were significant group mean increases in BVCA, AHP, contrast sensitivity, strabismic deviation, and NAFX after surgery. Sixty percent of patients who were legally ineligible for driving prior to surgery became eligible after eye muscle surgery. CONCLUSIONS: Adult patients with INS showed sustained improvement in many afferent and efferent measures of visual function after eye muscle surgery. [J Pediatr Ophthalmol Strabismus. 2021;58(2):93-104.].

Surgical interventions for infantile nystagmus syndrome.

BACKGROUND: Infantile nystagmus syndrome (INS) is a type of eye movement disorder that can negatively impact vision. Currently, INS cannot be cured, but its effects can potentially be treated pharmacologically, optically, or surgically. This review focuses on the surgical interventions for INS. Despite the range of surgical interventions available, and currently applied in practice for the management of INS, there is no clear consensus, and no accepted clinical guidelines regarding the relative efficacy and safety of the various treatment options. A better understanding of these surgical options, along with their associated side effects, will assist clinicians in evidence-based decision-making in relation to the management of INS. OBJECTIVES: To assess the efficacy and safety of surgical interventions for INS. SEARCH METHODS: We searched CENTRAL, MEDLINE Ovid, Embase Ovid, ISRCTN registry, ClinicalTrials.gov, and the World Health Organization (WHO) International Clinical Trials Registry Platform (ICTRP) to 3 July 2020, with no language restrictions. SELECTION CRITERIA: We included randomised controlled trials (RCTs) studying the efficacy and safety of surgical options for treating INS. DATA COLLECTION AND ANALYSIS: Our prespecified outcome measures were the change from baseline in: binocular best-corrected distance visual acuity; head posture; amplitude, frequency, intensity, and foveation period durations of the nystagmus waveform; visual recognition times; quality of life and self-reported outcome measures; incidence of adverse effects with a probable causal link to treatment; and permanent adverse effects after surgery. Two review authors independently screened titles and abstracts and full-text articles, extracted data from eligible RCTs, and judged the risk of bias using the Cochrane tool. We reached consensus on any disagreements by discussion. We summarised the overall certainty of the evidence using the GRADE approach. MAIN RESULTS: We only identified one eligible RCT (N = 10 participants), undertaken in India. This trial randomised participants to receive either a large retro-equatorial recession of the horizontal rectus muscle of 9 mm on the medial rectus and 12 mm on the lateral rectus, or a simple tenotomy and resuturing of the four horizontal rectus muscles. We did not identify any RCTs comparing a surgical intervention for INS relative to no treatment. In the single eligible RCT, both eyes of each participant received the same intervention. The participants' age and gender were not reported, nor was information on whether participants were idiopathic or had sensory disorders. The study only included participants with null in primary position and did not explicitly exclude those with congenital periodic alternating nystagmus. The study did not report funding source(s) or author declaration of interests. The evaluation period was six months. We judged this study at low risk for sequence generation and other sources of bias, but at high risk of bias for performance and detection bias. The risk of bias was unclear for selection bias, attrition bias, and reporting bias. There is very uncertain evidence about the effect of the interventions on visual acuity and change in amplitude, frequency, and intensity of the nystagmus waveform. We were unable to calculate relative effects due to lack of data. None of the participants in either intervention group reported adverse effects at six-month follow-up (very low-certainty evidence). There was no quantitative data reported for quality of life, although the study reported an improvement in quality of life after surgery in both intervention groups (very low-certainty evidence). Change in head posture, foveation period durations of the nystagmus waveform, visual recognition times, and permanent adverse effects after surgery were not reported in the included study. We judged the certainty of the evidence, for both the primary and secondary efficacy outcomes, to be very low. Due to a lack of comprehensive reporting of adverse events, there was also very low-certainty of the safety profile of the evaluated surgical interventions in this population. As such, we are very uncertain about the relative efficacy and safety of these interventions for the surgical management of INS. AUTHORS' CONCLUSIONS: This systematic review identified minimal high-quality evidence relating to the efficacy and safety of surgical interventions for INS. The limited availability of evidence must be considered by clinicians when treating INS, particularly given these procedures are irreversible and often performed on children. More high-quality RCTs are needed to better understand the efficacy and safety profile of surgical interventions for INS. This will assist clinicians, people with INS, and their parents or caregivers to make evidence-based treatment decisions.

Horizontal Transposition of the Vertical Rectus Muscles to Correct a Head Tilt in 5 Patients With Idiopathic Nystagmus Syndrome.

PURPOSE: Patients with idiopathic nystagmus syndrome often develop an abnormal head position. A horizontal face turn can be treated with the augmented Kestenbaum procedure, while patients with a chin up or chin down position can be treated with surgery on the vertical recti and/or oblique muscles. Although rare, some patients may have a head tilt with no face turn. We report 5 patients who underwent horizontal transposition of the vertical rectus muscles to correct a head tilt. DESIGN: Retrospective case series. METHODS: Five cases from 2 different tertiary referral eye centers and 3 different surgeons were reviewed and analyzed. Surgery for the patients consisted of either a 7-mm or full tendon-width transposition of the vertical rectus muscles of each eye to induce cyclotorsion in the direction of the head tilt. The presenting clinical histories, measurements, and surgical outcomes were reviewed. The primary outcome measure was correction of head tilt. RESULTS: Of 5 patients, 2 had previous horizontal face turns that were corrected with an augmented Kestenbaum procedure and later developed a head tilt, while 3 patients presented primarily with a head tilt. Age at surgery ranged from 5 to 8 years. Initial tilts were measured between 20-45°. Surgery was performed bilaterally except in 1 patient with history of morning glory disc anomaly and therefore transposition surgery was only performed on fixating eye for that patient. Postoperatively, 3 of 5 patients had near complete (0-5°) resolution of their tilt. One patient had a persistent 5-degree head tilt and a small chin up that was nullified with vertical prisms in spectacles. CONCLUSION: Transposition of the vertical rectus to induce cyclotorsion in the direction of the head tilt improves abnormal head titling in patients who have idiopathic nystagmus syndrome. This procedure was safely performed in patients with previous augmented Kestenbaum procedures with no incidence of anterior segment ischemia in our cases.

Consecutive strabismus after infantile nystagmus syndrome surgery and potential risk factors.

PURPOSE: The aim of this study is to evaluate the incidence of consecutive strabismus after infantile nystagmus surgery and its potential risk factors. METHODS: A retrospective study including 89 patients was conducted. Patients presented infantile nystagmus (idiopathic or ocular disease-associated nystagmus) without previous or coincidental strabismus. Sex, age at surgery, amblyopia, botulinum toxin (BT) injection before surgery, spherical equivalent, anisometropia, surgery procedure (Anderson's or retroequatorial recessions of four horizontal recti), and follow-up were analyzed. Kaplan-Meier and univariate Cox regression were performed. RESULTS: The median age at surgery was 5 years. The median follow-up was 36 months. The incidence of consecutive strabismus was 11.2%. There were eight patients with exotropia and two patients with esotropia. Consecutive strabismus was associated with severe bilateral amblyopia (p = 0.036), previous treatment with BT injection (p = 0.025), and large recessions of the four horizontal muscles (p = 0.001). The hazard ratio for patients with severe bilateral amblyopia was 5.4 (95% CI 1.1-25.8), and for patients previously treated with BT was 6.1 (1.3-29.3). The survival rate was 95.4% at 6 months and 88.5% at 3 years. CONCLUSION: Severe bilateral amblyopia, previous BT treatment, and type of surgery seem to be associated with consecutive strabismus after infantile nystagmus surgery. Most cases appear within the first months after surgery.

A Case of Infratentorial Meningioma Causing Spontaneous Downbeat Nystagmus: Case Report and Review of the Literature.

BACKGROUND: Spontaneous downbeat nystagmus is a finding that raises suspicions of a central nervous system disorder. Vermis and lower brainstem lesions are considered to be responsible, but the exact mechanism is still controversial. We observed a rare case of spontaneous downbeat nystagmus caused by an infratentorial meningioma. CASE DESCRIPTION: A 50-year-old woman was incidentally diagnosed with infratentorial tumor. Later, she suffered from oscillopsia and the symptom disturbed her daily life especially while driving. Magnetic resonance imaging showed a tumor of approximately 30 mm in diameter at the dorsal midline of the posterior fossa. The tumor was compressing the cerebellar vermis and was apparently responsible for the symptoms that affected her daily life; therefore, we decided to perform tumor removal. The postoperative course was uneventful, and the spontaneous downbeat nystagmus completely disappeared. CONCLUSIONS: Vertical nystagmus is a finding that raises suspicion of a central nervous system disorder, and requires detailed examination. In addition, in case of vertical nystagmus because of tumor compression of the vermis, removal of the tumor can be an effective treatment.

Long-term Outcomes Following Surgery for Infantile Nystagmus Syndrome With Abnormal Head Positioning.

PURPOSE: To evaluate long-term outcomes of surgical treatment for abnormal head positioning (AHP) associated with infantile nystagmus syndrome (INS). DESIGN: Retrospective observational case series. METHODS: Review of 150 patients who underwent surgery for AHP associated with nystagmus. Outcomes included head positioning, duction limitations, and strabismus, and were evaluated several times postoperatively. Successful collapse of AHP was defined as being ≤10°. RESULTS: Thirty-one patients had surgery for AHP in the pitch (chin up/down) position, whereas 119 had surgery for a horizontal AHP. In addition, 54 underwent 50%-60% augmentation, 19 underwent 40% augmentation, 5 underwent less than 40% augmentation. Thirty-eight had surgical dose modified to correct strabismus, and 3 underwent surgery different from standard Kestenbaum procedures. Collapse of AHP: At the 1-3-week follow-up (n = 131), 125 patients (95%) had collapse of AHP. The percentage trended down at the 2-5-month (91%, n = 106) and 2-year follow-ups (83%, n = 57). However, at 5 and 10 years, it was 93% (n = 42) and 93% (n = 14), respectively, due to reoperation in a small minority. Over- and undercorrection: At 1-3 weeks, 5% of patients were overcorrected whereas 0% were undercorrected. Over- and undercorrection rates peaked at 2 years postoperatively. Ten years out, there were no overcorrections and 7% undercorrections. Four percent of patients required reoperation for overcorrection (mean 2.7 years) and 5% did for undercorrection (mean 3.9 years). CONCLUSION: Surgery for the head positioning associated with INS produces excellent outcomes throughout 10 years postoperatively. Overcorrection presents early and resolves either over time or with additional surgery. Undercorrection develops later and can persist despite reoperation.

High-dose Anderson operation for nystagmus-related anomalous head turn.

PURPOSE: To evaluate the effectiveness of a high-dose Anderson procedure (AP) to correct infantile nystagmus-related anomalous head turn (HT). METHODS: Twenty-nine consecutive orthotropes with infantile nystagmus with and without associated sensory defect received high-dose AP. HT was measured while the patient tried to read letters at best-corrected visual acuity (BCVA) level at 5 m and 0.3 m. BCVA, binocular vision (BV), and alignment (prism and cover test) were measured. High-dose AP with recessions of 9-16 mm was performed. All measures were taken before and 3-6 and ≥ 8 months post surgery. Success was defined by postoperative HT ≤ 10°/HT ≤ 15°. RESULTS: Medians and ranges (minimum-maximum) were:. Age at surgery was 7 years (4-44). HT at 5 m and HT at 0.3 m were 35° (20-40) and 20° (0-35), respectively. After 4 months (3-6), HT was 10° (- 3-20) and 5° (- 5-20); success rates were 74%/96% and 83%/96%. After 15 months (8-45), HT was 12° (0-20) and 6° (0-15); success rates were 46%/75% and 92%/100%; residual HT > 15° occurred in 5/9 cases with recessions < 13 mm and 1/15 cases with recessions ≥ 13 mm. With recessions ≥ 13 mm, 60% (95% confidence intervals (C.I.), 33-83%) achieved HT ≤ 10° and 93% (95% C.I. 66-99%) achieved HT ≤ 15°. Overcorrection did not occur. Anomalous head posture components in vertical and frontal planes did not improve. Residual motility was 30° (10-45). The mean BCVA improved by only 0.037 logMAR (p = 0.06). BV and ocular alignment were constant, except in 2 patients whose exophoria decompensated. CONCLUSIONS: Kestenbaum surgery is a common procedure to correct infantile nystagmus-related HT. Anderson surgery is confined to bilateral yoke muscle recession; hence, less invasive but nevertheless comparably effective, high dosage is provided.

Small-incision lenticule extraction in a patient with high astigmatism and nystagmus.

Small-incision lenticule extraction (SMILE) was performed in a patient with high astigmatism and nystagmus. The patient presented for a refractive surgery evaluation with a manifest refraction of -0.50 -6.00 × 137 (20/40-1) in the right eye and -2.25 -2.25 × 30 (20/40-1) in the left eye. The patient was deemed suitable for corneal laser refractive surgery. Consideration was given to SMILE, laser in situ keratomileusis, and photorefractive keratectomy. It was decided SMILE was the best option given that the refractive portion of the procedure is performed while the cornea is immobilized by suction. The treatment was uneventful, and the final manifest refraction was +0.25 -0.25 × 130 (20/40) in the right eye and +0.25 diopter sphere (20/40) in the left eye. Topography was normal, showing a well-centered treatment zone. This case highlights an additional use for SMILE, given its ability to immobilize the cornea during treatment.

[Comprehensive analysis of the effect of null zone shifting surgery treatment on patients with infantile nystagmus syndrome].

Objective: To characterize the postoperative change of eyes related parameters of patients with infantile nystagmus syndrome(INS), so as to provide a reference for the clinical evaluation of postoperative effect and the rational arrangement of patients' follow-up time after operation. Methods: A retrospective study. Clinical and follow-up data of 17 patients diagnosed with INS at Department of Ophthalmology in Xinhua Hospital, School of Medicine, Shanghai Jiaotong University from June 2014 to December 2016 were collected. All patients with abnormal head posture (AHP) underwent null zone shift surgery. The operative methods were Parks 5-6-7-8, Anderson, Kestenbum 5-5-6-4,null zone shift combined with strabismus correction and vertical null zone transposition. Ophthalmological examination and eye movement were recorded, including best corrected binocular visual acuity (BCBVA), position of the null zone, expanded nystagmus acuity function (NAFX) and foveation time. Single factor repeated analysis of variance, independent sample t test and rank sum test were used for statistical analysis. Results: Among the 17 children, 6 were females and 11 were males. The age at surgery of the patients was 5-11 years.The follow-up time was (14.8±6.0) months. Preoperative BCBVA was 0.382±0.147 (corrected posture), 0.300±0.056 (AHP); foveation time was (0.594±0.011)s;position of null zone was 23.570°±0.118°. The BCBVA at three months after operation was 0.318±0.044 (corrected posture), 0.260±0.045 (AHP); foveation time was (0.950±0.146)s; position of null zone was 5.360°±1.107°. The BCBVA at six months after operation was 0.264±0.039 (corrected posture), 0.230±0.037 (AHP);foveation time was (1.496±0.233) s; position of null zone was 6.070°±1.303°. The BCBVA at twelve months after operation was 0.309±0.039 (corrected posture), 0.250±0.045 (AHP);foveation time was (1.455±0.201) s; position of null zone was 9.290°±8.520°. There was statistical difference between the data of pre-operation and post-operation(all P<0.05). Change of null zone position was identified in six patients after six months.The preoperative NAFX of patients with presence of change of null zone positon was 0.308±0.063 (the primary position), 0.393±0.210 (null zone); BCBVA was 0.450±0.043 (corrected posture), 0.417±0.031 (AHP); foveation time was 0.122 (0.080-1.014)s. The postoperative NAFX of those patients was 0.430±0.090(the primary position), 0.471±0.140 (null zone); foveation time was 0.438(0.170-1.450) s. The data above were lower than that of patients with no regression of null zone[0.523±0.142,0.601±0.110,0.200±0.063,0.250±0.076,0.725(0.230-1.440)s,0.610±0.160,0.680±0.120,0.975(0.380-2.000)s]. The difference was statistically significant(all P<0.05). Conclusions: Null zone shifting surgery is an effective approach for treating INS. Reduction in the null zone position can be observed in some patients at 6 months after operation, which was related to NAFX, BCBVA and foveation time. It is recommended to extend the follow-up time to at least 6 months after the operation. (Chin J Ophthalmol, 2019, 55:13-19).

Clinical and electrophysiological results of eye muscle surgery in 17 patients with downbeat nystagmus.

PURPOSE: To test the hypothesis that eye muscle surgery in treatment of patients with acquired downbeat nystagmus results in improvement measures of visual and ocular motor function. METHODS: This is a prospective, interventional case series analysis of clinical and electrophyisological data before and after eye muscle surgery in 17 patients with acquired downbeat nystagmus who did not respond to medical treatments. Outcome measures included: 1) routine demography and clinical characteristics, 2) subjective oscillopsia (SO), 3) binocular best-corrected visual acuity in the null position (BVA), 3) primary position strabismic deviation (SD), 5) anomalous head posture (AHP), 6) contrast sensitivity function (CS), and 7) nystagmus slow phase velocity (SPV). All patients were followed at least 12 months. Parametric and non-parametric statistical analysis of outcome measure data above pre- and post-treatment were perfomed using standard software on grouped data using computerized software. RESULTS: Patients' age ranged from 5 to 85 years (average 27 years). About 59% were male. Follow up ranged from 1-10 years (average 2.0 years). Around 70% had an associated central nervous systemic diagnosis, 100% had an AHP, oscillopsia and decreased CS, 53% had other eye disease, and 59% had strabismus. There were no complications from surgery. There were signficant post-treatment improvements in mean/median group BVA, SO, SD, AHP, CS, and SPV. CONCLUSION: This study supports the hypothesis that eye muscle surgery as treatments for patients with acquired downbeat nystagmus can result in improvements in multiple aspects of ocular motor and visual functions.

Long-term follow-up after vertical extraocular muscle surgery to correct abnormal vertical head posture.

PURPOSE: To report outcomes of vertical extraocular muscle surgery to correct abnormal vertical head posture in children with horizontal infantile nystagmus. METHODS: Retrospective case series of seven patients evaluated at one institution with abnormal vertical head position (chin-up or chin-down) in the setting of horizontal infantile nystagmus. All patients underwent bilateral vertical extraocular muscle surgery with the same surgeon. Pre- and postoperative vertical head position under binocular viewing conditions was the primary outcome measure. Secondary outcomes included visual acuity, stereopsis, degree of cyclotorsion, and residual ocular alignment. RESULTS: Seven children with infantile nystagmus syndrome, ranging in age from 4 months to 5 years at presentation, underwent extraocular muscle surgery to correct chin-down or chin-up head position associated with a null position in the setting of horizontal nystagmus. Five children had other ocular pathology (albinism, n = 4; cone-rod dystrophy, n = 1). Five of the seven patients had combined vertical recti and oblique muscle surgery. Three of the patients had additional extraocular muscle surgery to correct horizontal misalignment (exotropia, n = 2; esotropia, n = 1). One patient underwent a separate Kestenbaum procedure (bilateral horizontal resection/recession) to correct concomitant horizontal face turn. Median postoperative follow-up was 8.0 years (range 1-9.5 years). Postoperatively, three patients had complete resolution of their abnormal head position, and three had an improvement in their head posture, with a mean reduction in original vertical head position of 25 degrees. One of these patient had a reversal of their head position from a chin-up to a small chin-down position. Only one patient had no clinically significant improvement in their head position. No patients had signs or symptoms of cyclotorsion postoperatively. CONCLUSION: Combined vertical recti and oblique muscle surgery can successfully improve and even resolve vertical head posture, and prevents cyclorotary disorders postoperatively.

The Role of Superior Oblique Posterior Tenectomy Along With Inferior Rectus Recessions for the Treatment of Chin-up Head Positioning in Patients With Nystagmus.

PURPOSE: To evaluate the clinical outcomes of bilateral superior oblique posterior 7/8th tenectomy with inferior rectus recession on improving chin-up head positioning in patients with horizontal nystagmus. METHODS: Medical records were reviewed from 2007 to 2017 for patients with nystagmus and chin-up positioning of 15° or more who underwent combined bilateral superior oblique posterior 7/8th tenectomy with an inferior rectus recession of at least 5 mm. RESULTS: Thirteen patients (9 males and 4 females) were included, with an average age of 7.3 years (range: 1.8 to 15 years). Chin-up positioning ranged from 15° to 45° degrees (average: 30°). Three patients had prior horizontal muscle surgeries, 1 for esotropia and 2 for horizontal null zones causing anomalous face turns. Ten patients underwent other concomitant eye muscle surgery: 3 had esotropia, 1 had exotropia, and 2 had biplanar nystagmus null point requiring a horizontal Anderson procedure. Four patients underwent simultaneous bilateral medial rectus tenotomy and reattachment. All patients had improved chin-up positioning. Eight patients had complete resolution, whereas 5 had minimal residual chin-up positioning. Three patients developed an eccentric horizontal gaze null point with compensatory anomalous face turn with onset 2 weeks, 2 years, and 3 years postoperatively. Average follow-up was 42.7 months. No postoperative pattern deviations, cyclodeviations, or inferior oblique overaction were seen. No surgical complications were noted. CONCLUSIONS: Bilateral superior oblique posterior 7/8th tenectomy in conjunction with bilateral inferior rectus recession is a safe and effective procedure for improving chin-up head positioning in patients with horizontal nystagmus with a down gaze null point. [J Pediatr Ophthalmol Strabismus. 2018;55(4):234-239.].

Visual and ocular motor outcomes in children with posterior fossa tumors.

PURPOSE: To describe the clinical characteristics and visual and ocular motor outcomes of a large cohort of pediatric patients treated for tumors of the posterior cranial fossa. METHODS: The medical records of all patients with posterior fossa tumors evaluated by the ophthalmology services at two large tertiary care academic hospitals between 2005 and 2011 were reviewed retrospectively. Data abstracted for each study patient included demographic information, presenting signs and symptoms, pathologic diagnosis, and results of the most recent ophthalmology examination. RESULTS: A total of 139 patients were included. Visual outcomes were categorized as "good" (bilateral acuity of 20/20-20/40) in 101 patients (72.7%), "fair" (<20/40-20/200 in one or both eyes) in 12 patients (8.6%), or "poor" (<20/200 in one or both eyes) in 9 patients (6.5%). Patients with medulloblastoma and ependymoma had a significantly greater risk of a poor or fair visual outcome than those with juvenile pilocytic astrocytoma (both P < 0.05), independent of age and sex. Thirty-two patients (23.0%) developed nystagmus, and 59 patients (42.4%) developed strabismus. Twenty-four patients (17.3%) underwent eye muscle surgery for persistent strabismus. CONCLUSIONS: The majority of patients had good visual outcomes, although ocular motor abnormalities were common. Tumor type was a significant risk factor for permanent vision loss.

Magnetic Oculomotor Prosthetics for Acquired Nystagmus.

PURPOSE: Acquired nystagmus, a highly symptomatic consequence of damage to the substrates of oculomotor control, often is resistant to pharmacotherapy. Although heterogeneous in its neural cause, its expression is unified at the effector-the eye muscles themselves-where physical damping of the oscillation offers an alternative approach. Because direct surgical fixation would immobilize the globe, action at a distance is required to damp the oscillation at the point of fixation, allowing unhindered gaze shifts at other times. Implementing this idea magnetically, herein we describe the successful implantation of a novel magnetic oculomotor prosthesis in a patient. DESIGN: Case report of a pilot, experimental intervention. PARTICIPANT: A 49-year-old man with longstanding, medication-resistant, upbeat nystagmus resulting from a paraneoplastic syndrome caused by stage 2A, grade I, nodular sclerosing Hodgkin's lymphoma. METHODS: We designed a 2-part, titanium-encased, rare-earth magnet oculomotor prosthesis, powered to damp nystagmus without interfering with the larger forces involved in saccades. Its damping effects were confirmed when applied externally. We proceeded to implant the device in the patient, comparing visual functions and high-resolution oculography before and after implantation and monitoring the patient for more than 4 years after surgery. MAIN OUTCOME MEASURES: We recorded Snellen visual acuity before and after intervention, as well as the amplitude, drift velocity, frequency, and intensity of the nystagmus in each eye. RESULTS: The patient reported a clinically significant improvement of 1 line of Snellen acuity (from 6/9 bilaterally to 6/6 on the left and 6/5-2 on the right), reflecting an objectively measured reduction in the amplitude, drift velocity, frequency, and intensity of the nystagmus. These improvements were maintained throughout a follow-up of 4 years and enabled him to return to paid employment. CONCLUSIONS: This work opens a new field of implantable therapeutic devices-oculomotor prosthetics-designed to modify eye movements dynamically by physical means in cases where a purely neural approach is ineffective. Applied to acquired nystagmus refractory to all other interventions, it is shown successfully to damp pathologic eye oscillations while allowing normal saccadic shifts of gaze.