Effects of retinal image stabilization in acquired nystagmus due to neurologic disease.

We studied the effects of variable amounts of artificial retinal image stabilization (RIS) upon oscillopsia and visual acuity in eight patients with acquired nystagmus due to neurologic disease. We measured horizontal and vertical eye movements with the magnetic search coil technique and used these electronic signals to control the position of a visual stimulus on a screen in front of the patient. We also used an optical device to stabilize images of the real world upon the retina. During electronic stabilization, RIS was progressively increased until oscillopsia was abolished; this was achieved in all eight patients and corresponded to retinal image drift of 5 degrees/sec or less. In five patients with downbeat nystagmus, further increases in RIS caused the oscillopsia to reappear, but in the opposite direction. Electronic stabilization also improved visual acuity in four of five patients; the limitation of improvement could be related to coexistent visual system defects. Using electronic feedback, we could measure the range of RIS that any individual required to abolish oscillopsia; from this measurement, the components of the optical device that were best suited to provide a stable field of vision could be calculated.

Visual acuities in infants with congenital cataracts operated on prior to 6 months of age.

Seven infants with complete bilateral cataracts were operated on and fitted with extended-wear silicone contact lenses. The infants were matched to 16 normal control subjects. A modified preferential looking technique was used to assess visual acuity; the Bayley Scale of infant Development was administered to measure psychological development. Of the seven infants with cataracts, those operated on prior to 8 weeks of age seemed to have normally developing vision. All others showed a substantial visual lag when compared with the control group. Bayley scores for infants operated on early (before 8 weeks) fell within the range of normal variability. The infants operated on later demonstrated a statistically significant lag in development. Also, a pendular nystagmus was noted in those infants with cataracts who were operated on later.

Hypopyon uveitis in patients with acquired immunodeficiency syndrome treated for systemic Mycobacterium avium complex infection with rifabutin.

OBJECTIVE: Iridocyclitis has been identified as a dosage-dependent side effect in patients with the acquired immunodeficiency syndrome (AIDS) who are treated for Mycobacterium avium complex (MAC) infection with systemic rifabutin. We reviewed cases of acute hypopyon uveitis occurring in patients with AIDS to establish whether there was an association. DESIGN: Retrospective case series. SETTING: Outpatient clinic and inpatient hospital-based ophthalmology referral practice and infectious disease specialty service. PATIENTS: Seven patients with AIDS, aged 10 to 40 years, presenting with acute unilateral hypopyon mimicking infectious endophthalmitis. MAIN OUTCOME MEASURES: Findings from complete ophthalmological evaluation and ancillary laboratory testing. RESULTS: At the time of presentation, all seven patients were receiving treatment for MAC infection with rifabutin (dosage range, 300 to 600 mg/d) and clarithromycin. Results of microbiological investigations in five patients were negative. Iridocyclitis became bilateral in all seven patients, and hypopyon developed in the contralateral eye in five of seven patients. Hypopyon resolved rapidly with intensive topical corticosteroid therapy. Residual inflammation responded to topical corticosteroids with or without reduction of the rifabutin dosage. CONCLUSIONS: Concomitant use of rifabutin, clarithromycin, and fluconazole may precipitate hypopyon uveitis in patients with AIDS being treated for MAC infection.

The amblyopia treatment study visual acuity testing protocol.

OBJECTIVE: To evaluate the reliability of a new visual acuity testing protocol for children using isolated surrounded HOTV optotypes. METHODS: After initial pilot testing and modification, the protocol was evaluated using the Baylor-Video Acuity Tester (BVAT) to present isolated surrounded HOTV optotypes. At 6 sites, the protocol was evaluated for testability in 178 children aged 2 to 7 years and for reliability in a subset of 88 children. Twenty-eight percent of the 178 children were classified as having amblyopia. RESULTS: Using the modified protocol, testability ranged from 24% in 2-year-olds to 96% in 5- to 7-year-olds. Test-retest reliability was high (r = 0.82), with 93% of retest scores within 0.1 logMAR unit of the initial test score. The 95% confidence interval for an acuity score was calculated to be the score +/-0.125 logMAR unit. For a change between 2 acuity scores, the 95% confidence interval was the difference +/-0.18 logMAR unit. CONCLUSIONS: The visual acuity protocol had a high level of testability in 3- to 7-year-olds and excellent test-retest reliability. The protocol has been incorporated into the multicenter Amblyopia Treatment Study and has wide potential application for standardizing visual acuity testing in children.

Examination and refractive management of patients with nystagmus.

Patients with nystagmus present unique challenges to the ophthalmologist. These patients can be difficult to examine and refract. Treatment options to improve vision or reduce disturbing visual symptoms are limited, which is disappointing to the patient and frustrating to the clinician. This paper will provide the clinician with one method of clinically organizing nystagmus, describe the patients who may benefit from optical treatments, and discuss the methodology used in their implementation. Techniques that will be discussed include patient examination and objective and subjective refraction. Optical treatments discussed include spectacles, prisms, contact lenses, and retinal image stabilization.

Ocular motor signs in an infant with carbohydrate-deficient glycoprotein syndrome type Ia.

PURPOSE: To document the evolution of ocular motor abnormalities in an infant with carbohydrate-deficient glycoprotein syndrome. METHODS: Case report. An infant with carbohydrate-deficient glycoprotein syndrome type 1a underwent magnetic resonance imaging and infrared eye movement recording. RESULTS: A 10-month-old male with carbohydrate-deficient glycoprotein syndrome type Ia had rapid horizontal oscillations of the eyes when startled or awakened from sleep. Clinical examination confirmed this finding and disclosed congenital ocular motor apraxia with a reduced vestibulo-ocular reflex. Infrared eye movement recording showed ocular flutter and square wave jerks superimposed on a horizontal pendular nystagmus. Magnetic resonance imaging showed diffuse cerebellar hypoplasia. CONCLUSION: Carbohydrate-deficient glycoprotein syndrome type Ia can be associated with multiple cerebellar eye signs including ocular flutter, square-wave jerks, and congenital ocular motor apraxia.

The visual-evoked response in infants with central visual impairment.

We studied ten children with central visual impairment with a known neurologic defect and an abnormal visual-evoked response who had results of repeat electrophysiologic testing evaluated at the Children's Hospital of Philadelphia from December 1989 through July 1991. Central visual impairment is defined as poor visual function with a normal anterior visual pathway. Age at first examination ranged from 5 to 48 months with a followup of two to 31 months. Repeat visual-evoked response testing showed improvement in seven patients and no change in three. Grating acuity as measured by the Teller acuity card procedure, performed in nine of ten patients, improved in seven, showed no change in one, and declined in one. We found a potentially favorable prognosis for those infants with central visual impairment, despite an initially abnormal visual-evoked response. We used a clinical approach to this disorder to improve diagnostic categorization and prognostic capabilities in central visual impairment.

Computerized method of visual acuity testing: adaptation of the amblyopia treatment study visual acuity testing protocol.

PURPOSE: To report a computerized method for determining visual acuity in children using the Amblyopia Treatment Study visual acuity testing protocol. METHODS: A computerized visual acuity tester was developed that uses a programmed handheld device that uses the Palm operating system (Palm, Inc, Santa Clara, California). The handheld device communicates with a personal computer running a Linux operating system and 17-inch monitor. At a test distance of 3 m, single letters can be displayed from 20/800 to 20/12. A C program on the handheld device runs the Amblyopia Treatment Study visual acuity testing protocol. Using this method, visual acuity was tested in both the right and left eyes, and then the testing was repeated in 156 children age 3 to 7 years at four clinical sites. RESULTS: Test-retest reliability was high (r =.92 and 0.95 for and right and left eyes, respectively), with 88% of right eye retests and 94% of left eye retests within 0.1 logarithm of minimal angle of resolution (logMAR) units of the initial test. The 95% confidence interval for an acuity score was calculated to be the score +/- 0.13 logMAR units. For a change between two acuity scores, the 95% confidence interval was the difference +/- 0.19 logMAR units. CONCLUSIONS: We have developed a computerized method for measurement of visual acuity. Automation of the Amblyopia Treatment Study visual acuity testing protocol is an effective method of testing visual acuity in children 3 to 7 years of age.

Successful visual rehabilitation after neonatal penetrating keratoplasty.

BACKGROUND: Penetrating keratoplasty in infancy and childhood has traditionally met with limited visual success due to a combination of unique physiology and technical problems in this patient population. With the advances in microsurgical instrumentation, corneal preservation, and visual developmental physiology ophthalmologists are finding increasing indications for penetrating keratoplasty in the childhood population. The long term results of neonatal penetrating keratoplasty in two patients with unilateral congenital corneal opacification are reported. METHODS: Penetrating keratoplasty was performed on one eye in each of two infants within the first 3 weeks of life. Amblyopia treatment and optical therapy have been continued since surgery. RESULTS: After 6 years both grafts have remained clear. One patient developed the infantile esotropia syndrome. Visual development using Snellen optotypes is age normal for both transplanted eyes. CONCLUSIONS: Penetrating keratoplasty when combined with optical correction and amblyopia therapy may restore and preserve vision in selected patients with congenital corneal opacification if performed in the neonatal period.

Ophthalmic features and visual prognosis in the Treacher-Collins syndrome.

The ocular findings and visual prognosis were reviewed in 24 patients with the Treacher-Collins syndrome who were evaluated in the craniofacial clinic in the Division of Pediatric Ophthalmology at Children's Hospital of Philadelphia between 1980 and 1991. All patients had some abnormality. Vision loss was present in 37% of patients. Amblyopia was present in 33%, significant refractive errors were present in 58%, and anisometropia was documented in 17%. Strabismus was present in 37% and significant lid and adnexal abnormalities were seen in 96%. The prognosis for normal vision in at least one eye is good but vision loss secondary to amblyopia is more resistant to treatment owing to other medical problems and social concerns.

Clinical and ocular motor analysis of the infantile nystagmus syndrome in the first 6 months of life.

BACKGROUND/AIMS: The infantile nystagmus syndrome (INS) usually begins in infancy and may or may not be associated with visual sensory system abnormalities. Little is known about its specific waveforms in the first 6 months of life or their relation to the developing visual system. This study identifies the clinical and ocular motility characteristics of the INS and establishes the range of waveforms present in the first 6 months of life. METHODS: 27 infants with involuntary ocular oscillations typical of INS are included in this analysis. They were evaluated both clinically and with motility recordings. Eye movement analysis was performed off line from computer analysis of digitised data. Variables analysed included age, sex, vision, ocular abnormalities, head position, and null zone, neutral zone characteristics, symmetry, conjugacy, waveforms, frequencies, and foveation times. RESULTS: Ages ranged from 3 to 6.5 months (average 4.9 months). 15 patients (56%) had abnormal vision for age, nine (33%) had strabismus, five (19%) had an anomalous head posture, 13 (48%) had oculographic null and neutral positions, nine (33%) had binocular asymmetry, and only two showed consistent dysconjugacy. Average binocular frequency was 3.3 Hz, monocular frequency 6.6 Hz. Average foveation periods were longer and more "jerk" wave forms were observed in those patients with normal vision. CONCLUSIONS: Common clinical characteristics and eye movement waveforms of INS begin in the first few months of infancy and waveform analysis at this time may help with both diagnosis and visual status.

The role of ultrasound biomicroscopy in surgical planning for limbal dermoids.

PURPOSE: Limbal dermoids usually involve corneal stroma and more rarely might extend into the anterior chamber. Depending on the size and amount of penetration of the lesion, different therapeutic approaches are indicated. Depth information is difficult to obtain by slit-lamp examination because the structure is opaque. We investigated the use of ultrasound biomicroscopic (UBM) examination for surgical planning in limbal dermoids. METHODS: The UBM (Zeiss Humphrey) with its 50-MHz probe was used for preoperative evaluation of the depth of penetration in two cases of infantile limbal dermoid. Histology specimens of the lesion corresponding to the UBM images were obtained. RESULTS: UBM was able to distinguish normal cornea from the more sonolucent lesion. Presence or absence of Descemet's membrane could be identified. Depth of involvement of limbal dermoids could be assessed. CONCLUSION: UBM is able to assess depth involvement of opaque corneal lesions such as limbal dermoids. Because planning of the surgical approach in these cases is facilitated by preoperative knowledge about the depth of penetration of these opaque lesions, UBM can be regarded as a helpful tool in the clinical management.

Oculographic and clinical characterization of thirty-seven children with anomalous head postures, nystagmus, and strabismus: the basis of a clinical algorithm.

BACKGROUND AND PURPOSE: We studied children with nystagmus who also had anomalous head postures and strabismus to determine the etiology of the conditions and present a diagnostic clinical algorithm. METHODS: The patients for this study were among the 560 patients evaluated in the ocular motor neurophysiology laboratory between the years 1991 and 1997. Clinical characteristics, infrared oculography data, and medical and surgical treatments were entered into a database for analysis. Oculography was performed on all patients according to a standard protocol, and data were stored and analyzed off-line. Etiology of anomalous head posture was determined with both clinical and oculography information. RESULTS: Thirty-seven children are the subjects of this report. The etiology of anomalous head posture was a "gaze null" due to congenital nystagmus in 23 (62%) patients, an "adduction null" due to manifest latent nystagmus in 12 (32%) patients, spasmus nutans in 1 (3%) patient, and strabismus in 1 (3%) patient. The patients' ages ranged from 9 months to 12 years and averaged 4.4 years. Sixty-nine percent were male patients. Nineteen (63%) of 30 patients had abnormal recognition (linear optotype) acuity in at least 1 eye on monocular cover; the recognition remained abnormal in 5 (17%) of 30 patients under binocular conditions. Thirty percent of patients had amblyopia, 16% had some structural disease of the eyes, 22% had some systemic syndrome or abnormality, 57% had a significant refractive error, and 27% had some ability to fuse. CONCLUSIONS: The major etiology for anomalous head posture in these patients was to adopt a gaze null due to congenital nystagmus (62% of patients) regardless of the direction of their anomalous head posture or type of strabismus. Moving the fixing eye as the first step for the anomalous head posture, combined with moving the nonfixing eye for the resulting strabismus may help treat these patients.

Clinical and ocular motor analysis of congenital nystagmus in infancy.

PURPOSE: The purpose of this study was to identify the clinical and ocular motility characteristics of congenital nystagmus and to establish the range of waveforms present in infancy. BACKGROUND: The clinical condition of congenital nystagmus usually begins in infancy and may or may not be associated with visual sensory system abnormalities. Little is known about its specific waveforms in infancy or their relationship to the developing visual system. METHODS: Forty-three infants with involuntary ocular oscillations typical of congenital nystagmus were included in this analysis. They were evaluated both clinically and with motility recordings. Eye movement analysis was performed off line from both chart recordings and computer analysis of digitized data. Variables analyzed included age, sex, vision, ocular abnormalities, head position, null-zone or neutral-zone characteristics, symmetry, conjugacy, waveforms, frequencies, foveation times, and responses to convergence and to monocular cover. RESULTS: Patient ages ranged from 3 to 18 months (average, 9.2 months). Seventeen patients (40%) had abnormal vision, 3 had a positive family history of nystagmus, 11 had strabismus, 16 (37%) had a head posture, 26 (60%) had null and neutral positions, 14 (33%) had binocular asymmetry, and all were horizontally conjugate. Average binocular frequency was 2.8 Hz, and average monocular frequency was 4.6 Hz. The waveforms were both jerk and pendular; average foveation periods in patients with normal vision were more than twice as long as those in patients with abnormal vision. CONCLUSIONS: Common clinical characteristics and eye-movement waveforms of congenital nystagmus begin in infancy, and waveform analysis at this time helps with both diagnosis and visual status.

Comitant esodeviation resulting from neurologic insult in children.

PURPOSE: Our purpose was to establish whether comitance was a common or uncommon finding in children with esodeviation associated with a neurologic insult. METHODS: A retrospective chart review was performed of children with acquired esodeviation associated with an identifiable neurologic insult. RESULTS: Examinations of 30 children seen over a 2-year period were analyzed. Twenty-two (73%) had brain tumors. Twelve (40%) had comitant esodeviation, and the other 18 (60%) had incomitant measurements. Of the patients with comitant esodeviation, 6 had normal abduction OU (two after recovery from bilateral sixth nerve palsies), and 6 had mild or minimal abduction deficits. Moderate or severe abduction deficits were associated with incomitant measurements. CONCLUSIONS: Comitant esodeviation can be common in children with identifiable neurologic insults.

A new surgery for congenital nystagmus: effects of tenotomy on an achiasmatic canine and the role of extraocular proprioception.

PURPOSE: Human eye-movement recordings have documented that surgical treatment of congenital nystagmus (CN) also produces a broadening of the null zone and changes in foveation that allow increased acuity. We used the achiasmatic Belgian sheepdog, a spontaneously occurring animal model of human CN and see-saw nystagmus (SSN), to test the hypothesis that changes induced by surgical interruption of the extraocular muscle afference without a change in muscle-length tension could damp both oscillations. METHODS: An achiasmatic dog with CN and SSN underwent videotaping and infrared oculography in a sling apparatus and head restraints before and after all extraocular muscles (stage 1: 4 horizontal rectus muscles and stage 2 [4 months later]: 4 vertical rectus muscles and 4 oblique muscles) were surgically tenotomized and immediately reattached at their original insertions. RESULTS: The dog had immediate and persistent visible, behavioral, and oculographic changes after each stage of this new procedure. These included damped CN and SSN, increased ability to maintain fixation, and increased periods of maintaining the target image on the area centralis over a broad range of gaze angles. CONCLUSIONS: Severing and reattaching the tendons of the extraocular muscles affect some as-yet-unknown combination of central nervous system processes producing the above results. This new procedure may prove effective in patients with CN with either no null, a null at primary position, or a time-varying null (due to asymmetric, (a)periodic, alternating nystagmus). We infer from our results in an achiasmatic dog that tenotomy is the probable cause of the damping documented in human CN after Anderson-Kestenbaum procedures and should also damp CN and SSN in achiasma in humans. It may also prove useful in acquired nystagmus to reduce oscillopsia. The success of tenotomy in damping nystagmus in this animal suggests that the proprioceptive feedback loop has a more important role in ocular-motor control than has been appreciated. Finally, we propose a modified bimedial recession procedure, on the basis of the damping effects of tenotomy.

Clinical characteristics of surgically treated adult strabismus.

BACKGROUND: Strabismus is present in approximately 4% of the population with most forms becoming clinically evident in infancy and childhood. Adult strabismus, including that resulting from recurrent childhood deviations, varies in etiology, presentation, symptomatology, and response to treatment. This report is intended to prospectively characterize adults who required surgical correction of their strabismic deviation. METHODS: Beginning in 1991, a prospective computerized database was collected that contained all clinical, laboratory, and surgical information on the adult ocular motility service at the Scheie Eye Institute and The Children's Hospital of Philadelphia (Philadelphia, Pa). Analysis of those patients who underwent surgical correction of their strabismus is included in this report. Demography, clinical characteristics, and surgical procedures including results were analyzed separately in adults with their strabismus beginning before visual maturation (BVM) (9 years of age) and after visual maturation (AVM). RESULTS: A minimum of 6 months and average of 1.8 years follow up is reported on 262 procedures in 255 patients with an average age of 37 between January 1991 and January 1996. Sixty-two percent of patients had their strabismus onset BVM. Fifty-one percent of BVM patients and 80% of AVM patients had incomitant deviations. Adjustable suture surgery was performed on 97% of all patients, with 28% of BVM and 57% of AVM patients having multiplanar surgery. Successful motor alignment was achieved in 85% of all patients after one surgical procedure. Sensory success was achieved in 81% of all AVM patients and 25% of all BVM patients. Subjective success was 94% in both groups of patients. CONCLUSIONS: Numerous differences exist between childhood and adult strabismus characteristics and response to surgery. Although strabismus surgery is effective, differences in results reflect the varied physiology regarding etiology, pathophysiology, and symptomology. Classification into AVM and BVM groups will provide continued clarity for future studies.

The use of hyaluronic acid during adjustable suture surgery.

We prospectively studied the force required to move an extraocular muscle after adjustable suture surgery with and without the perioperative use of hyaluronic acid (Healon). The 15 muscles that received hyaluronic acid averaged 15.0 g of force to move the muscle 2.0 mm which was significantly different than the average force of 36.15 g required for the 13 control muscles. The decreased force required to adjust the muscle along with potential antiinflammatory properties of hyaluronic acid make this a useful adjunct during adjustable suture surgery.

Insertion site dynamics and histology in a rabbit model after conventional or suspension rectus recession combined with ipsilateral antagonist resection.

We have used a rabbit model to study insertion movement, rotational forces, disinsertion forces, and histology of the surgically created insertion site after recession of the inferior rectus (IR) using a conventional (CONV) or suspension (SUS) technique combined with an ipsilateral superior rectus (SR) resection. During the 5-week observation period, the CONV and SUS recessed IR showed an initial posterior movement followed by an anterior movement while all resected SR had an initial posterior movement which remained stable. SR rotational forces increased and IR remained constant throughout the 5-week observation period. There were no rotation force differences between the SUS and CONV recession techniques. Disinsertion forces showed an initial large decrease in force followed by a gradual increase to preoperative levels by 3 postoperative weeks. Disinsertion force differences between the CONV and SUS recessions were observed for 5 weeks after surgery. Histologic analysis showed a delayed inflammatory response on the SUS recession compared to the CONV recession and at the middle of the insertion compared to the poles which was minimized by 3 weeks after surgery.

The intraoperative oculocardiac reflex as a predictor of postoperative vaso-vagal responses during adjustable suture surgery.

Adjustable suture surgery has become a popular method of strabismus correction in those cases in which results are less predictable, such as reoperations, thyroid ophthalmopathy, and blind eyes. Complications related to adjustable strabismus surgery are, in general, no different than those related to standard surgical techniques. We noticed a subgroup of patients who experienced significant vaso-vagal responses (V-VR) during postoperative adjustment and sought to discover a method of identifying these patients prior to adjustment. Sixty patients were studied prospectively from July 1991 to July 1992. All patients had surgery under general anesthesia and were adjusted 24 hours later. A positive oculocardiac reflex (OCR) occurred when a 10% or greater intraoperative change in heart rate was associated with traction on an extraocular muscle. A positive V-VR postoperatively consisted of one or more subjective findings (dizziness, light-headedness, nausea, or body temperature changes), and two or more objective findings (10% or greater change in heart rate, hypotension, pallor, diaphoresis, vomiting, disorientation, or loss of consciousness). Variables studied for predictive value included OCR, age, sex, strabismus type, previous surgery, muscle adjusted, and systemic disease. Twenty-five patients (41.6%) had a positive V-VR during adjustment. Twenty-seven patients (45%) had a positive OCR. Eighty-five percent of patients with a positive OCR and 9% of patients with a negative OCR had a positive V-VR. Younger patients were also more likely to have a positive V-VR. A positive intraoperative OCR under general anesthesia during strabismus surgery is highly predictive of a postoperative V-VR during planned adjustment.