Article

The Effect of Horizontal Rectus Muscle Surgery on Clinical and Eye Movement Recording Indices in Infantile Nystagmus Syndrome

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Abstract

To determine the relationship between horizontal rectus muscle surgery and eye movement recording in infantile nystagmus syndrome. In this prospective, interventional, non-randomized study, patients with infantile nystagmus syndrome were assigned to one of three treatment groups. Group I with head postures of less than 20 degrees , binocular visual acuity of less than 20/30, and heterotropia less than 30(Delta) underwent recessions of all 4 horizontal rectus muscles. Group II with head postures of less than 20 degrees , binocular visual acuity of less than 20/30, and heterotropia more than 30(Delta) underwent large recessions of two horizontal rectus muscles. Group III with head postures of more than 20 degrees underwent Kestenbaum-Anderson surgery. Baseline and follow-up evaluations included best corrected visual acuity for distance and near, cycloplegic refraction, head posture, and eye movement recordings. Fifty-eight (58)patients with a mean age of 18.7 (SD +/- 9.10 years) were enrolled. Four horizontal rectus muscle recessions were performed in 29 cases, 2 rectus recession in 23, and Kestenbaum-Anderson surgery in 6 cases. Mean follow-up period after surgery was 18 +/- 7.4 months. Distance visual acuity improved in all three groups, but only in the 2 rectus muscle surgery group was the change significant for both monocular and binocular vision (P < 0.001). In all 3 groups speed and amplitude of nystagmus waves were changed in positive or negative direction but only in 4-recti recession decrease was statistically significant (P = 0.02 & 0.04). Horizontal rectus muscle surgery in subjects with infantile nystagmus syndrome improves the visual acuity and nystagmus intensity.

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... Múltiples trabajos con ejemplos de niveles II y III de evidencia sugieren que la cirugía de los músculos mejora el nistagmos y la función visual. [18][19] Hertle, 20 en un editorial de la revista Ophthalmology del año 2009, señalaba que pacientes con nistagmos a los que se les realizaba cirugía de los músculos oculares, obtenían una visión más útil por unidad de tiempo y reconocían los objetos más rápidamente, tenían menos movimientos de la cabeza, mejores movimientos y mejor sensibilidad de contrastes, y por consecuencia un mejor funcionamiento. 21 Señala que una hipótesis actual es que la interferencia de la cirugía con las terminaciones nerviosas propioceptivas periféricas del tendón o del músculo extraocular, influye en las vías oculares motoras centrales, lo que resulta en una mejoría de las oscilaciones en el SNI. ...
... Although the etiology of INS is unknown, it seems an interruption between the developing sensory and motor systems interferes with ocular motor calibration (neural "cross talk") and results in involuntary oscillations. 3,4 It is hypothesized that tenotomy probably changes proprioceptive inputs which control resting tension of the EOMs. 7,11 So, it can reduce the slow-phase gain of nystagmus. ...
Article
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Article
Infantile nystagmus syndrome (INS) is an important clinical diagnosis because it is a common presenting sign of many ocular, neurologic, and systemic diseases. Although INS has been studied for more than a century, its diagnosis and treatment remains a challenge to clinicians because of its varied manifestations and multiple associations, and its pathogenesis continues to rouse considerable scientific debate. Fueled by these challenges, recent basic research and clinical investigations have provided new insights into INS. New genetic discoveries and technological advances in ocular imaging have refined our understanding of INS subtypes and offer new diagnostic possibilities. Unexpected surgical outcomes have led to new understanding of its pathogenesis based on novel hypothesized pathways of ocular motor control. Comparative studies on nonhuman visual systems have also informed models of the neural substrate of INS in humans. This review brings together the classic profile of this disorder with recent research to provide an update on the clinical features of INS, an overview of the current theories on how and why INS develops, and a practical approach to the diagnosis and management of INS.
... From a motor point of view, many studies have involved operations on extraocular muscles. 8,9,23,24 However, the effect of keratorefractive surgeries on motor indices of nystagmus has not been previously studied. To the best of our knowledge, the current study is the first to quantify the improvement in motor parameters of nystagmus following keratorefractive surgery. ...
Article
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... In another prospective study, 58 patients with infantile nystagmus were assigned to one of three groups based on preoperative measures of AHP, visual acuity, and heterotropia. 70 Each group underwent a different variation of recession and resection procedure to correct the AHP. Overall, they saw significant improvement in distance monocular visual acuity in all groups. ...
Article
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Abstract: Nystagmus refers to involuntary, typically conjugate, often rhythmic oscillations of the eyes. The most common cause of nystagmus in children is infantile nystagmus syndrome (INS). INS presents within the first few months of life and is sometimes accompanied by an ocular condition associated with sensory impairment. Because this condition affects a person throughout life, it is important to understand the options available to manage it. This review focuses on the underlying nystagmus etiology, psychosocial and functional effects of nystagmus, as well as current principles of management, including optical, pharmacological, surgical, and rehabilitative options. Currently, the neural mechanisms underlying INS are not fully understood. Treatment options are designed to increase foveation duration or correct anomalous head postures; however, evidence is limited to mainly pre- and post-study designs with few objective comparisons of treatment strategies. Management of INS should be individualized. The decision on which treatment is best suited for a particular patient lies with the patient and his/her physician.
... Because nystagmus associated with albinism also leads to deterioration in vision because of motion blur, these findings indicate that OCT has a clinical use in recognizing patients who have potential for visual improvement as a result of treatments to reduce nystagmus intensity, such as pharmacologic interventions. 24,25 With the rapid development of OCT technology, it is likely that high-resolution hand-held OCT devices soon will be available clinically. These may allow the visualization of photoreceptor morphologic features in neonates, possibly enabling us to assess the future potential for vision and the need for early intervention. ...
Article
The relationship between foveal abnormalities in albinism and best-corrected visual acuity (BCVA) is unclear. High-resolution spectral-domain optical coherence tomography (SD OCT) was used to quantify foveal retinal layer thicknesses and to assess the functional significance of foveal morphologic features in patients with albinism. Cross-sectional study. Forty-seven patients with albinism and 20 healthy control volunteers were recruited to the study. Using high-resolution SD OCT, 7×7×2-mm volumetric scans of the fovea were acquired (3-μm axial resolution). The B scan nearest the center of the fovea was identified using signs of foveal development. The thickness of each retinal layer at the fovea and foveal pit depth were quantified manually using ImageJ software and were compared with BCVA. Total retinal thickness, foveal pit depth, photoreceptor layer thickness, and processing layer thickness in relation to BCVA. Total photoreceptor layer thickness at the fovea was correlated highly to BCVA (P = 0.0008; r = -0.501). Of the photoreceptor layers, the outer segment length was correlated most strongly to BCVA (P<0.0001; r = -0.641). In contrast, there was no significant correlation between either total retinal thickness or pit depth and BCVA (P>0.05). This was because of an inverse correlation between total photoreceptor layer thickness and total processing layer thickness (P<0.0001; r = -0.696). Neither the total retinal thickness nor the pit depth are reliable indicators of visual deficit, because patients with similar overall retinal thickness had widely varying foveal morphologic features. In albinism, the size of the photoreceptor outer segment was found to be the strongest predictor of BCVA. These results suggest that detailed SD OCT images of photoreceptor anatomic features provide a useful tool in assessing the visual potential in patients with albinism. The author(s) have no proprietary or commercial interest in any materials discussed in this article.
Article
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Article
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Article
Purpose: To report the comparative clinical findings for two nystagmus procedures in two consecutive case series: the Sinskey anterior extirpation procedure (SAEP) and a modification, myectomy without reattachment (MWR). Methods: Twenty consecutive patients underwent the SAEP and 19 underwent MWR. The SAEP is a snare-assisted unmeasured deep myectomy, whereas MWR is a measured myectomy at posterior Tenon's fascia. All patients underwent comprehensive ophthalmologic and orthoptic examination with infrared videonystagmography. Binocular best corrected visual acuity, nystagmus amplitude, alignment, and versions outcomes were measured. Results: The highest percentage of patients experiencing two or more lines of improvement in distance and near acuity was in the SAEP group, who were younger than 10 years and had no ocular comorbidity (100%). Distance and near acuity improved at least one line in 71% and 86% of patients in the SAEP group and 72% and 61% in the MWR group, respectively. In both groups, patients younger than 10 years had the best acuity gains. Average nystagmus amplitude was reduced 76.3% (SAEP) and 68% (MWR). Horizontal versions were more compromised in the SAEP group and one patient experienced intraoperative orbital bleeding. Patients requiring reoperation for strabismus numbered 5 of 20 (20%) in the SAEP and 3 of 19 (16%) in the MWR groups. Conclusions: Similar to SAEP, the MWR procedure reduces nystagmus amplitudes and improves visual acuity but with less limitation of versions, risk of intraoperative bleeding, and risk of postoperative strabismus. Data suggest that early surgery (for patients younger than 10 years) can have a greater beneficial effect on postoperative visual acuity. [J Pediatr Ophthalmol Strabismus. 2016;53(3):156-166.].
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Clinical Orthoptics has become established as a basic reference text providing fundamental information on anatomy, innervation and orthoptic investigation, plus diagnosis and management of strabismus, ocular motility and related disturbances. It is aimed at trainee ophthalmologists and orthoptic undergraduate students. Qualified orthoptists, general ophthalmologists and optometrists will also find valuable guidance in these pages. In this edition, the author has maintained the goal of producing a user-friendly, clinically relevant and succinct book, while revising it to reflect a variety of developments in the field.
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Purpose To test the hypothesis that the topical carbonic anhydrase inhibitor brinzolamide (Azopt) has beneficial effects versus placebo on measures of nystagmus and visual acuity in adult subjects with infantile nystagmus syndrome (INS). Design Prospective, cross-over, double masked clinical trial. Methods Setting: Single centre. Study population: Five subjects ≥18 years old with typical INS and best-binocular visual acuity in their primary position null zone ETDRS 55 letters to 85 letters (20/200 to 20/50) and had no previous treatment for nystagmus. Intervention: In a randomised order, each subject received one drop of Azopt or placebo in both eyes three times a day separated by a washout period of at least a week followed by Azopt or placebo in both eyes three times a day; thus each subject got the drug and placebo, each acting as his or her own control. Outcome measures: The nystagmus acuity function and INS waveforms obtained from eye movement recordings, binocular optotype visual acuity, using the ETDRS protocol analysed individually and as a group before and after Azopt and placebo. Results Versus placebo and baseline measures, topical Azopt significantly improved; INS waveform characteristics in the primary position null zone, group mean values of the nystagmus acuity function across gaze (p<0.01) and group mean ETDRS binocular letter visual acuity (p<0.05). There was a predictable decrease in intraocular pressure (IOP) without any systemic or ocular adverse events. Conclusions Although a prospective large-scale clinical trial is needed to prove effectiveness, an eye-drop-based therapy for INS may emerge as a viable addition to optical, surgical, behavioural and systemic drug therapies for INS. Trial registration number NCT01312402.
Article
To demonstrate the surgical choices for patients with complicated head posture associated with nystagmus. It was a retrospective clinical study. Thirty-eight cases of congenital nystagmus with abnormal head posture in all three axes without strabismus were retrospectively analyzed. Twenty-nine(76.32%) cases whose dominant head posture were with face turn, 3 cases (7.89%) with chin up or down , respectively, were performed horizontal null zone shift as well as vertical null zone transposition; 2 cases (5.26%) with head tilt as the dominant position were underwent one tendon width transposition of all four vertical muscles;4 cases (10.53%)basically with the same degree for face turn and chip up or down, 2 cases were profermed with recess a group of horizontal yoke muscles and a group of vertical yoke muscles, the other 2 cases were combined with weaken both synergistic oblique muscles. SPSS 13.0 was used to analyse the difference of them. In 29 patients with horizontal head posture dominanted, 15 cases (68.18%) with 25 °-30 ° in horizontal head posture were corrected completely, 5°-15° was the residue for 7 cases (31.82%) with 35 °- 40 °degree in horizontal before surgery. 15 °-20° was residue for 3 cases larger than 40 ° before operation after modified Parks procedure. Anderson procedure can correct the angle of 15°-20° in 4 cases. The horizontal, vertical and torsional components of 22 cases whose predominant head posture were in horizontal with 25°-40° (3.18° ± 1.01°, 4.32° ± 1.14°, 4.55° ± 1.95°) were significantly reduced (t = 63.13, 3.57, 3.95;P < 0.01) after Parks procedure. Recession a group of vertical muscles 5mm or combined with oblique muscles in 3 patients could correct the 20° of vertical head posture, but the improvement of the other two axes was about 5°-10°.One tendon width transposition of all four vertical muscles in 2 cases could correct the 10° of head tilt and 10°-15°of chip up or down. Recession a group of horizontal and vertical muscles can correct 20°-25° of face turn and 20° of vertical head posture. When head turn with 25°-40°predominates over the vertical and torsional components, recess the horizontal muscles could be effective way in diminishing the abnormal head position on all three axes.When vertical or torsional head posture predominates for the complicated nystagmus, individual designs should be considered.When necessary, reoperations should be needed.
Article
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Article
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Null zones and head postures were studied in two patients with idiopathic congenital nystagmus who had been carefully selected from a larger group of patients who had nystagmus with compensatory head postures. Oculomotor and sensory features of the two patients indicated that they would have a reasonable chance of profiting from surgery. Neither patient had a periodic alternating or manifest latent nystagmus or any ocular disease of the fixating eye. Postoperative evaluation of the head posture and the nystagmus behavior of both patients was carried out over a period in excess of 6 years. Neither of the patients readopted a head turn, and visual acuity was found to be equivalent to that prior to surgery when using the null zone. In one of the patients the postoperative null zone was located in the primary position, while in the other the null zone was decreased from an eccentricity of 30 degrees to 15 degrees. These results suggest that the position of the postoperative null zone is not always an accurate predictor of head turn.
Article
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To present the results of a new technique that the authors have developed to weaken the extraocular muscles. The biomechanics of this technique, which is termed "Y-split recession," are given in the companion paper. A retrospective study, testing the effects of a new surgical technique on strabismus, nystagmus, and visual acuity. 228 patients (aged 6.8 (SD 6.0) years) with variable angle strabismus and nystagmus were treated by splitting and detaching two rectus muscles, and re-attaching the two halves at an angle of about 65 degrees to each other. Subjects were examined with the usual orthoptic tests immediately after the operation, and up to 96 months later. The operation reduced the strabismus, eliminated or weakened the nystagmus in primary position, and improved binocular vision and the development of visual acuity. Only minor side effects were observed. The Y-split recession can be used to reduce the torque for extraocular rectus muscles, and provides an alternative to "Cuppers Fadenoperation," recession, and similar procedures.
Forty-three patients with congenital nystagmus who underwent surgery in the Ocular Motility Clinic at The Royal Victorian Eye and Ear Hospitar Melbourne, are analysed to assess the effect of different surgical approaches on the abnormal head posture and binocular visual performance. There were 38 patients with a horizontal head turn, and five with a vertical abnormal head posture. In those undergoing horizontal nystagmus surgery the different surgical procedures have been classified as (4 Anderson procedure, which involves large bilateral augmented recessions only; (ii) Kestenbaum and augmented Kestenbaum bilateral recess/resect surgery in which the recess:resect ratio is less than 1; and (iii) a modified Anderson-Kestenbaum procedure with the emphasis on large recessions. so that the bilateral recess/resect ratio is greater than 1. In this series the augmented recessions of the Anderson and Anderson-Kestenbaum procedures appeared to produce a more favourable result of improved binocular vision. The five patients with vertical abnormal head posture with nystagmus had various surgical procedures with very encouraging results, both in terms of reduction of the abnormal head posture and an even greater improvement of binocular vision than with the horizontal cases. The surgical techniques in these cases are presented and discussed.
Article
We report our results in nystagmus without null point surgery, utilizing a new technique consisting in the placement of a silicone encircling band around the globe over the four recti muscles, in the retroequatorial position. This method was tried only in nystagmus without null point and with both horizontal and vertical movements. We have not observed any ischemic complication of the anterior segment nor any important variation of intraocular pressure probably because the silicone band was not tightened. Other advantages of this method are its reversibility (cutting the silicone band) and the possibility of combining it with recession-resection of recti muscles at the same or at a later date.
Article
Ten patients had large recession of four horizontal recti at one procedure for treatment of nystagmus. Six patients had congenital motor nystagmus, two had oculocutaneous albinism, and two had optic nerve hypoplasia. Anomalous head posture with null point was also present in five patients. The rectus muscles were placed at or behind the equator in all but one case. Three patients with both esotropia and nystagmus had the medial recti placed 1 mm behind the equator and the lateral recti or at 1 mm anterior to the equator. Visual acuity improved an average of 1 line at distance and/or near in 8 patients who cooperated for testing. Although nystagmus was not eliminated in any patient, its its amplitude decreased in eight of ten patients, and anomalous head posture improved in three of five patients. In all patients, near vision was better than distance vision both preoperatively and postoperatively. Ductions were diminished minimally after the large recession and there were no other complications from surgery.
Article
Retroequatorial recessions of the horizontal rectus muscles 10 to 12 mm behind their insertions reduced the amplitude of manifest congenital nystagmus in three patients. Modest improvement of visual acuity occurred in two patients. In a third patient with periodic alternating nystagmus, a compensatory head turn was eliminated by shifting the neutral zone of the nystagmus to the primary position of gaze. In spite of large recessions of the muscle insertions, none of the patients had a functionally significant postoperative limitation of ocular motility.
Article
The records of 48 patients with congenital nystagmus who required surgery for a significant head turn were reviewed to compare the effectiveness of the surgical techniques. Patients were omitted from the study if the last postoperative visit was less than five months following surgery, or if the surgery was for a vertical head posture. The average preoperative head turn of the 38 patients with horizontal nystagmus was 41.3 degrees with an average postoperative head turn of 11.5 degrees. The net change in head turn was 33.4 degrees. The average duration of time from surgery to final examination was four years and six months. The study was divided into six groups of patients. The first group received the Parks modification of the standard Kestenbaum procedure, the 5-6-7-8 millimeter procedure. The second group received the above procedure plus 1 mm additional surgery. The third group received another modification by Parks of 6.5-8-9-10 millimeters. The fourth group received the Calhoun and Harley modification of 40% augmentation of the original Parks recommendation, and the fifth group received 60% modification. The sixth group required more than one procedure on the horizontal recti for the abnormal head posture. The pre- and postoperative measurements are compared for each of these six groups. In some patients followed for many years, there is a tendency for a gradual return toward the preoperative head posture. In four patients in the study, the gaze preference was shifted to the opposite direction postoperatively.
Article
Forty-three patients with congenital nystagmus who underwent surgery in the Ocular Motility Clinic at The Royal Victorian Eye and Ear Hospital, Melbourne, are analysed to assess the effect of different surgical approaches on the abnormal head posture and binocular visual performance. There were 38 patients with a horizontal head turn, and five with a vertical abnormal head posture. In those undergoing horizontal nystagmus surgery the different surgical procedures have been classified as: Anderson procedure, which involves large bilateral augmented recessions only; Kestenbaum and augmented Kestenbaum bilateral recess/resect surgery in which the recess:resect ratio is less than 1; and a modified Anderson-Kestenbaum procedure with the emphasis on large recessions, so that the bilateral recess/resect ratio is greater than 1. In this series the augmented recessions of the Anderson and Anderson-Kestenbaum procedures appeared to produce a more favourable result of improved binocular vision. The five patients with vertical abnormal head posture with nystagmus had various surgical procedures with very encouraging results, both in terms of reduction of the abnormal head posture and an even greater improvement of binocular vision than with the horizontal cases. The surgical techniques in these cases are presented and discussed.
Article
Sixty-four patients with nystagmus blockage syndrome (NBS) underwent surgery consisting of unilateral recession-resection, bimedial recession with and without posterior fixation suture, and bimedial recession with resection of one or both lateral recti. The results of the unilateral recession-resection and bimedial recession with or without posterior fixation were comparable. Over- and undercorrections occurred more frequently and the number of reoperations was higher than in a control group of essential infantile esotropia without nystagmus. These unpredictable surgical results in NBS present yet another feature that distinguish this condition from essential infantile esotropia without nystagmus. While restoration of normal binocular vision was not achieved in a single case, a functionally desirable result of subnormal binocular vision or microtropia occurred in 26%. An unusual complication consisting of postoperative asymmetric gaze nystagmus with an anomalous head posture was observed in five patients and required a Kestenbaum-Anderson operation in four to eliminate the head turn.
Article
In five patients with congenital nystagmus and compensatory head turn we performed a modified Kestenbaum operation intended to correct the compensatory head turn and to improve visual acuity. All patients had nystagmus without strabismus and fully developed binocular function. The nystagmus decreased but did not cease completely during the lateral gaze, and the patients adopted compensatory head turn positions to improve their visual acuity. The near acuity was better than the distance and the head turn decreased on near fixation. In such cases the original Kestenbaum procedure transfers the neutral zone to the primary position and corrects the head turn, but does not improve the visual acuity because a considerable amount of nystagmus is still present. Our aim was to perform an asymmetric Kestenbaum operation with a greater amount of surgery to the eye that turns out and a lesser amount to the eye that turns in, thus creating a postoperative divergence of the eyes. The result of compensating for this divergence (through stimulation of the fusional reserve) blocks the residual nystagmus of the neutral zone and, consequently, improves visual acuity. The results obtained from performing this modified Kestenbaum operation in five patients were very satisfactory. The compensatory head turn was corrected in all patients and remained so during the follow-up period. In four cases, the postoperative visual acuity in the neutral zone was better than before. There was no improvement in one case.
Article
A modified Kestenbaum procedure is advised for managing the horizontal face turn maintained to keep the eyes in the most quiet zone in nonstrabismic children with congenital nystagmus. The eyes are relocated toward the side the face is turned with bilateral recess resect procedures, recessing the medial rectus 5 mm, resecting the opposite medial rectus 6 mm, recessing the lateral rectus 7 mm, and resecting the opposite lateral rectus 8 mm. The improvement in the torticollis makes this a justifiable procedure. Strabismus has not been produced by this surgery. Three congenital nystagmus patients whose sagittal plane torticollis was improved by vertical rectus muscle surgery are described. One of the three patients had combined horizontal and sagittal plane torticollis and the modified Kestenbaum procedure was combined with the vertical rectus muscle surgery. A similar patient with combined horizontal and sagittal plane torticollis received only the modified Kestenbaum surgery and the sagittal plane torticollis persisted postoperatively. This suggests that there is a place for combined horizontal and vertical surgery in a patient with combined horizontal and sagittal plane torticollis and the combination of six recti muscles simultaneously operated on can be of benefit to such a patient.
Article
Operative treatment of congenital jerk-type nystagmus with ocular torticollis is discussed. The mechanism causing the head turn, methods used in measurement of the head and eye deviation, as well as the selection of the surgical procedures indicated are analyzed. A review of the ophthalmic literature on the subject is included with particular emphasis on the original work and theories of Kestenbaum, Anderson, and Goto. The results of seven cases operated on for this condition utilizing our techniques are presented.
Article
Patients with nystagmus and an eccentric null point in lateral gaze may assume an abnormal head position to maximise visual acuity. Surgical procedures for this condition can result in significant undercorrection of the head turn. A follow-up of 15 patients for an average of 33 months revealed a sustained improvement in head position with the use of the augmented modified Kestenbaum procedure.
Article
Records of 32 patients with congenital nystagmus who underwent surgery for a significant head turn were reviewed to assess the effectiveness of current surgical approaches. Eighteen patients (Group I) had single binocular vision and had bilateral recess/resect procedures either according to Parks' 5-6-7-8 scheme (nine patients) or "augmented" from 10% to 40% for large head turns (nine patients). Fourteen (78%) had a reduction of the turn to 15 degrees or less, six (33%) being 5 degrees or less. One was overcorrected . Fourteen patients had a heterotropia in addition to head turn. Seven (Group II) had bilateral surgery to attempt to correct the head turn and reduce the tropia to less than 10 prism diopters. In three cases (43%) there was both successful treatment of the tropia and reduction of the turn to 15 degrees or less. The tropia was successfully treated in six patients (86%). In seven patients (Group III) monocular recess/resect surgery was done to attempt to correct both abnormalities. In four (57%) the turn was reduced to 15 degrees or less with successful treatment of the tropia at the same time. Five (71%) had a tropia of less than or equal to 10 diopters postoperatively. Overall, the head turns were reduced to 15 degrees or less in 23 (72%) of the 32 patients including 11 (35%) with 5 degrees or less residual turn. Three (9%) were overcorrected at least 10 degrees. The frequency of undercorrections indicates that larger amounts of surgery have to be done, especially for large turns, although ductions will be limited in some gaze positions, at times significantly, to achieve this goal.
Article
The authors compared the preoperative and postoperative binocular visual acuities and eye movement recordings of patients who underwent eye muscle surgery consisting of the Anderson-Kestenbaum procedure or the artificial divergence procedure modeled after Cüppers, or a combination of both procedures, for the treatment of infantile nystagmus with head turn. Binocular visual acuities and eye movement recordings by electro-oculography (EOG) were compared preoperatively with those done within 3 weeks postoperatively. Shifting and broadening of the minimal intensity zone and foveation time and changing of the waveform were measured. The treatment of 6 of 18 patients was based on the artificial divergence principle; for 7 patients, treatment was in accordance with the Anderson-Kestenbaum principle; and 5 patients had combined procedures. The improvement in binocular visual acuities was two Snellen lines or more in one of six patients in the artificial divergence group and four of five patients in the combined treatment group. The EOG recordings showed shifting of the minimal intensity zone toward the primary position in all three groups. A broadening of the minimal intensity zone occurred mostly in the artificial divergence and combined groups. Increases in foveation time and changes in waveforms from jerk to jerk with foveation were found in three of six patients in the artificial divergence group and in two of five patients in the combined group. With less muscle surgery, the artificial divergence and combined operations gave better vision improvement than the Anderson-Kestenbaum operation.
Article
To evaluate the effect of large muscle recessions on visual acuity in albinos with nystagmus. This is a retrospective, unmasked, chart review of 12 patients with ocular or oculocutaneous albinism demonstrating nystagmus who underwent four horizontal rectus muscle retroequatorial recessions. Pre- and postoperative visual acuity were measured. Best corrected visual acuity improved postoperatively by two or more Snellen lines in 7 of 12 patients (58%). Subjective improvement of vision and nystagmus was noted by patients and examiners. Follow up was performed between 3 and 41 months; 25% of patients required reoperation for ocular alignment. Large four-muscle horizontal rectus recessions may improve uniocular and binocular visual acuities in albinos with nystagmus as measured by Snellen acuity. Patients may note a qualitative decrease in the severity of their nystagmus.
In patients with congenital nystagmus and an eccentric rest point for maximum visual acuity, surgery on the ocular muscles is an effective and safe method of reducing or eliminating the head turning. There are no adverse effects on binocular function as a result. In a series of 19 patients, 6 were considered as having a cure with no apparent head turn postoperatively. Four of these 6 patients had more than the classic maximum surgery performed. Two patients had no apparent effect from the classic maximum surgery.
Article
Abnormal, nystagmus related head postures can be treated by Kestenbaum's procedure, if the concept of artificial divergence (Cüppers procedure) is not expected to work. In this retrospective study, we evaluated the effects of Kestenbaum surgery in order to establish dosage recommendations. Solely patients who received Kestenbaum surgery (maximum 3 mm dosage difference between both eyes, i.e., without additional artificial divergence) for a horizontal head turn (HT) due to nystagmus were included in this study. Exclusion criteria were previous eye muscle surgery, strabismus, lacking binocular vision, and cerebral disease. The millimetres of surgery and the pre- and 3-6 months-postoperative findings (HT and visual acuity at 5 m distance, stereopsis) were evaluated. Of the 34 patients, 21 had a HT to the left side and 20 were male. The age at surgery (median, 10%- and 90%-quantile) was 7 years (4-32), the total amount of surgery 32 mm (20-40), and the preoperative HT 30 degrees (20-40). Postoperatively (n = 31), the HT amounted to 7 degrees (0-20). The reduction of HT was 67% (25-100), the efficacy of surgery 0.8 degree (0.3-1.0) per millimetre total amount of surgery on both eyes together. Four patients needed further surgery due to residual HT. Postoperatively, the maximum visual acuity was available without HT or with significantly less HT than preoperatively. Stereopsis showed a trend of improvement. The Kestenbaum procedure has a dose/effect ratio similar to that of recess/resect surgery for strabismus. Due to a long term efficacy of 1.5 degrees/mm, a dosage (in millimetres) on each eye of two thirds of the HT (in degrees) can be recommended. Physiological and methodical factors (false measurements) have to be discussed as an explanation for apparently low efficacy of surgery.
Article
Congenital nystagmus (CN) is an aperiodic oscillatory eye movement disorder of unknown etiology. We examined the effect of horizontal rectus tenotomy with simple re-attachment on the dimensionality of the dynamical mechanism underlying CN. The correlation dimensions (CDs) were calculated from eight patients who had tenotomy surgery. We found no significant differences in the CDs that could be associated with the surgery. The change in dimensionality was less than 5% on average. The results suggest that the tenotomy has no effect, or only a quite small effect, on the underlying mechanism of the CN beats.
Article
Congenital nystagmus (CN) is an aperiodic oscillatory eye movement disorder. Horizontal rectus tenotomy with simple re-attachment has been proposed as a therapy for CN. This therapy might affect vision and/or eye movements. Another paper deals with improvements in visual acuity. This and the companion paper examine changes in eye movements. In this study, we examined the effect of tenotomy on nystagmus waveforms using wavelet spectral analysis. No common effect was found across the patients on the wavelet spectra of the CN beat, suggesting that tenotomy surgery has no effect, or only a quite small effect, on the waveform structure of CN.
Article
We wished to determine the effectiveness of horizontal rectus tenotomy in changing the nystagmus of patients with congenital nystagmus and, secondarily, how their visual function changed. This was a prospective, noncomparative, interventional case series. Ten adult patients with varied associated sensory defects and oculographic subtypes of congenital nystagmus (including asymmetric periodic or aperiodic alternating nystagmus) and no nystagmus treatment options. By using standard surgical techniques, simple tenotomy of all four horizontal recti with reattachment at the original insertion was accomplished. Search-coil eye movement recordings and clinical examinations were performed before and 1, 6, 24, and 52 weeks after surgery. The primary outcome measure was the expanded nystagmus acuity function, obtained in "masked" fashion directly from ocular motility recordings. Secondary outcomes included breadth of null zones, preoperative and postoperative masked measures of visual acuity (Early Treatment Diabetic Retinopathy Study [ETDRS] chart), and the National Eye Institute Visual Function Questionnaire (NEI-VFQ-25). At 1 year after tenotomy and under binocular conditions, 9 of 10 patients had persistent, significant postoperative increases in the expanded nystagmus acuity function of their fixing (preferred) eye; 1 remained high, and 1 was not tested under the same conditions. Average foveation times increased in all 9 fixing (preferred) eyes. Binocular visual acuity measured with the ETDRS chart increased in 5 patients and was unaffected in five, whereas the NEI-VFQ-25 showed an improvement in vision-specific mental health in 9 patients. There were no adverse events. Tenotomy also radically changed the periodicity of one patient's asymmetric periodic or aperiodic alternating nystagmus. In 9 of 10 adult patients with clinical and oculographic variations in their congenital nystagmus, tenotomy resulted in significant improvements in a nystagmus measure and subjective visual functions.
Article
We wished to determine the effectiveness of horizontal rectus tenotomy in changing the nystagmus of children with infantile nystagmus syndrome. This was a prospective, noncomparative, interventional case series in five children with varied sensory and oculographic subtypes of congenital nystagmus (including asymmetric (a)periodic alternating nystagmus) and no nystagmus treatment options. Simple tenotomy of all four horizontal recti with reattachment at the original insertion was accomplished. Search-coil and infrared eye movement recordings and clinical examinations were performed before and 1, 6, 26, and 52 weeks after surgery. Outcome measures included masked pre- and postoperative expanded nystagmus acuity function (NAFX), foveation time obtained directly from ocular motility recordings, and masked measures of visual acuity. At 1 year after tenotomy and under binocular conditions, two of the three patients for whom the NAFX could be measured had persistent, significant postoperative increases in the NAFX of their fixating eye. Average foveation times increased in these patients' fixating eyes. Measured binocular visual acuity increased in four patients; the remaining patient had retinal dystrophy. There were no adverse events due to surgery. In the two children who could fixate the targets for several seconds and for whom we could obtain accurate measurements of their infantile nystagmus, tenotomy resulted in significant improvements in nystagmus foveation measures. In those patients plus two others (four of five), measured visual function improved.
Article
The purpose of this report is to characterize the clinical and electrophysiological effects of extraocular muscle surgery in 15 patients with oculo-cutaneous albinism (OCA) and infantile nystagmus syndrome (INS). Our hypothesis is that surgery on the extraocular muscles of patients with OCA and INS changes their nystagmus and their visual function. Interventional, prospective, cohort, noncomparative case series. All 15 patients had surgery on all four virgin horizontal recti; three for strabismus alone, three for nystagmus alone, five for an eccentric gaze null zone alone, and four for an eccentric gaze null zone plus strabismus. All patients have been followed for at least six months. All 15 patients had the subjective outcome measure of pre- and postoperative binocular best optically corrected acuity (BBOCA). Objective outcome measures included anomalous head posture (AHP) in nine patients, eye movement recording measures of expanded nystagmus acuity function (NAFX) in 10 patients, null zone position (NUZP) and null zone width (NUZW) in 10 patients, and foveation time (FOV) in nine patients. The results are summarized as follows; BBOCA increased 0.1 LogMar or greater in 14 of 15 patients. In those operated on for an AHP with or without associated strabismus the AHP improved significantly (P < .01 for all). The NAFX, NUZP, NUZW, and FOV measured from eye movement recordings showed persistent, significant increases in all patients (P < .01 for all). This report adds to the evidence that surgery on the extraocular muscles in patients with INS has independent neurologic and visual results.
Article
This work was undertaken to evaluate the effect of bilateral horizontal rectus recession on visual acuity, ocular deviation and head posture in patients with nystagmus. Twenty patients underwent recession of 4 horizontal rectus muscles to dampen the nystagmus. At least 2 muscles were recessed posterior to the equator. Monocular and binocular visual acuity improved from 1 to 3 Snellen lines after the procedure in 13 patients (76.5%). Visual changes in log MAR notations were statistically significant with Wilcoxon analysis in each and both eyes. Preoperative binocular visual acuity was 0.73 +/- 0.26 log MAR that reached to 0.62 +/- 0.32 log MAR after surgery (P = 0.02). The greatest improvement was observed in patients with congenital motor nystagmus. Ten patients had horizontal strabismus in addition to nystagmus before the surgery. One-millimeter additional recession of both medial rectus muscles caused an average reduction of 6 PD in esotropia. Corresponding figures for similar additional recessions of the lateral rectus muscles was 11 PD for correction of exotropia. Abnormal head posture decreased in all the cases and improved completely in most of them. Large horizontal rectus recession can improve visual acuity and decrease nystagmus in sensory and motor types. By revision in surgical planning, strabismus and abnormal head posture can also be corrected.
Nystagmus in childhood
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Most of the studies of surgical treatment for nys-tagmus have been designed to evaluate the improve-ment of an abnormal head posture. Such surgery is successful in 76% to 100% of patients without induc-ing significant limitation in eye movements
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Nystagmus: clinical evaluation and surgical management
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Nouvelle operatia du nystagmus
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