Congenital esotropia.

The prognosis for congenital esotropia remains guarded. Monofixation syndrome currently is the best widely reported result following surgery. The reoperation rate in congenital esotropia (including reoperations for horizontal misalignment, dissociated vertical deulation, and inferior oblique overaction) also is high, approaching 50%. The role of very early surgical intervention needs to be examined further and could result in dramatic improvement in the long-term outcomes associated with congenital esotropia.

Orbital extension of a frontal sinus osteoma in a thirteen-year-old girl.

Osteomas are uncommon, slow-growing, benign osteogenic neoplasms that arise most frequently in the craniofacial skeleton. (1,2) Osteoma is the most common benign tumor of the nose and paranasal sinuses and the most common neoplasm of the frontal sinus. (3-5) Paranasal sinus osteomas originate in the sinus wall, fill the lumen with well-defined mature osseous tissue, and occasionally extend into the orbit where they give rise to orbital signs and symptoms. Osteomas most commonly become symptomatic in the second to fifth decade in life, but orbital involvement has rarely been reported in patients aged 18 years and younger. (2,6-10) We report a case of a frontal sinus osteoma with orbital extension in a 13-year-old girl.

Results of the superior oblique tendon elongation procedure for severe Brown's syndrome.

PURPOSE: To evaluate the outcomes of the silicone tendon expander for the treatment of Brown's syndrome. METHODS: This paper consists of 2 parts: a chart review of patients who have under gone the silicone tendon elongation procedure for Brown's syndrome and a survey of the surgical experience of the members of the American Association of Pediatric Ophthalmology and Strabismus. Charts of 15 consecutive Brown's syndrome patients who underwent the superior oblique tendon expander were reviewed by the author from 1987 to 1997. An additional patient referred to the author for management of complications of the silicone tendon expander procedure after surgery elsewhere for Brown's syndrome is reported. The second aspect of the study is a fax survey sent to all 450 members of the American Association of Pediatric Ophthalmology and Strabismus listed in the 1997-1998 directory. Pediatric ophthalmologists were asked to answer questions regarding their experience using the silicone tendon expander for Brown's syndrome. RESULTS: Of 15 patients operated on by the author, preoperative limitation of elevation in adduction measured -4 in 14 patients and -3 in 1 patient preoperatively. Postoperatively, 14 of the 15 patients showed improved motility, with essentially normal versions in 10 patients (no more than +/- 1 oblique dysfunction); 3 patients were undercorrected (2 mild and 1 severely); and 2 had a consecutive superior oblique paresis (1 mild and 1 requiring an inferior oblique weakening procedure). The 1 patient with a severe undercorrection that did not improve after the silicone tendon elongation procedure showed continued restriction by intraoperative forced ductions after superior oblique tenotomy. Superior oblique tendon was not the cause of Brown's syndrome in this patient. The average final result graded on a scale of 1 to 10 (10 being best) was 8.3. Thirteen of 15 patients (87%) achieved a final result score of 7 or better with a single surgery, and an additional patient was corrected with a second surgery providing an overall success rate of 93%. Ten of the 15 patients had at least 11 months' follow-up, and 6 of the 10 patients showed a delayed improvement over a 4- to 6-month period. Five patients had more than 5 years of follow-up, and 4 (80%) had an excellent long-term outcome (final result, 9 to 10) with a single operations; all 5 had a good final outcome (final result, 7 to 10, mean 9.2) with 1 patient requiring a second surgery. There were no long-term complications, including no extrusions, no restriction of ocular rotations, and no infections. The patient who underwent the procedure elsewhere demonstrated limited movement of the eye to adduction elevation, and there was underaction of the ipsilateral superior oblique muscle. A reoperation performed by the author revealed positive forced ductions up and nasal ward. There were scars and adhesions in the area of the silicone implant, including scar to superior nasal sclera and superior rectus muscle. The silicone implant was removed and scar excised until forced ductions improved. This resulted in improved motility. Thirty-nine AAPOS members reported on 140 patients who had undergone the silicone tendon elongation procedure for Brown's syndrome. Judged by the surgeon on a scale of 1 to 10 (10 being best), 26 surgeons rated 91 patients (65%) as good with a score of 8 to 10, and 14 surgeons rated 18 patients (13%) poor with scores of 1 to 3. Complications were recorded for 9 patients and included scarring and restriction with removal of silicone implant in 4 and spontaneous extrusion in 5. Three of the 5 spontaneous extrusions came from the same surgeon, and in another case a surgeon used a 10 mm length of silicone. CONCLUSIONS: A novel procedure, the silicone tendon expander, is an effective option for correcting Brown's syndrome caused by a stiff or inelastic superior oblique tendon; long-term outcomes are excellent. Proper technique with maintenance of the tendon capsule is critical to the successful outcome of the procedure.

Unilateral or asymmetric congenital ptosis, head posturing, and amblyopia.

PURPOSE: To determine the prognostic implication of compensatory head posturing in patients with unilateral or asymmetric congenital ptosis. METHODS: A retrospective review of 80 consecutive patients with unilateral or asymmetric congenital ptosis was performed. The presence of documented compensatory head posturing, age of onset, age of presentation, visual acuity, refraction, and amblyopia were recorded, and binocularity was tested. RESULTS: Five of seven (71%), patients with unilateral or asymmetric congenital ptosis and compensatory head posturing had amblyopia. All of these patients had straight eyes and four of the five amblyopic patients had anisometropia of less than 2 diopters (D). CONCLUSION: The high incidence of amblyopia in this group can occur in the absence of significant anisometropia and strabismus. This unusually high incidence of amblyopia in this subgroup of patients with unilateral or asymmetric congenital ptosis and compensatory head posturing warrants compulsive examination and prophylactic part-time occlusion therapy of the nonptotic eye until reliable vision testing can be performed.

Binocular function and patient satisfaction after monovision induced by myopic photorefractive keratectomy.

PURPOSE: To measure binocular function and patient satisfaction with monovision induced by photorefractive keratectomy (PRK) in myopic presbyopic patients. SETTING: Refractive Department, Cleveland Clinic, Cleveland, Ohio, USA. METHODS: This study comprised 21 myopic presbyopic patients with monovision induced by PRK. Sixteen emmetropic patients who had PRK served as a control group. Monovision was induced by undercorrecting the nondominant eye by 1.25 diopters for near vision and correcting the dominant eye with emmetropia for distance vision. Monocular and binocular uncorrected Snellen visual acuities at 20 feet and 13 inches, manifest refraction, ocular dominance, stereopsis at 20 feet and 13 inches, monocular and binocular contrast sensitivities, Worth-4-Dot test at 20 feet and 1/3 of a meter, and fusional convergence amplitudes were examined in each patient. RESULTS: In the monovision group at near and distance, 20 patients (95.3%) had binocular visual acuity of 20/25 or better. No patient in the monovision group used reading glasses postoperatively; 4 of 16 patients (25.0%) in the control group used such glasses. All patients maintained binocular fusion and stereo acuity ranging from 40 to 800 seconds of arc. Mean patient satisfaction was 86% (range 40% to 100%). In the control group, 12 patients (75.0%) had binocular distance visual acuity of 20/25 or better and 11 (68.8%) had binocular near visual acuity of 20/25 or better. CONCLUSION: Monovision PRK patients had better near vision than control PRK patients, with minimal compromise in stereo acuity and overall high patient satisfaction.

Brown's syndrome: diagnosis and management.

PURPOSE: To better understand the various etiologies of Brown's syndrome, define specific clinical characteristics of Brown's syndrome, describe the natural history of Brown's syndrome, and evaluate the longterm outcome of a novel surgical procedure: the silicone tendon expander. Also, to utilize a computer model to simulate the pattern of strabismus seen clinically with Brown's syndrome and manipulate the model to show potential surgical outcomes of the silicone tendon expander. METHODS: Charts were reviewed on patients with the diagnosis of Brown's syndrome seen at a children's hospital ophthalmology clinic from 1982 to 1997, or seen in the author's private practice. Objective fundus torsion was assessed in up gaze, down gaze, and primary position in 7 Brown's syndrome patients and in 4 patients with primary superior oblique overaction. A fax survey was taken of members of the American Association of Ophthalmology and Strabismus (AAPOS) listed in the 1997-1998 directory regarding their results using the silicone tendon expander procedure for the treatment of Brown's syndrome. A computer model of Brown's syndrome was created using the Orbit 1.8 program by simulating a shortened superior oblique tendon or by changing stretch sensitivity to create an inelastic muscle. RESULTS: A total of 96 patients were studied: 85 with Brown's syndrome (38 with congenital and 47 with acquired disease), 6 with masquerade syndromes, 1 with Brown's syndrome operated on elsewhere, and 4 with primary superior oblique overaction in the torsion study. Three original clinical observations were made: 1. Significant limitation of elevation in abduction occurs in 70% of Brown's syndrome cases surgically verified as caused by a tight superior oblique tendon. Contralateral pseudo-inferior oblique overaction is associated with limited elevation in abduction. 2. Traumatic Brown's syndrome cases have larger hypotropias than nontraumatic cases (P < .001). There was no significant hypotropia in primary position in 56 (76%) of 74 congenital and nontraumatic acquired cases despite severe limitation of elevation. 3. Of 7 patients with Brown's syndrome, 6 had no significant fundus torsion in primary position, but had significant (+2 to +3) intorsion in up gaze. Spontaneous resolution occurred in approximately 16% of acquired nontraumatic Brown's syndrome patients. The silicone tendon expander was used on 15 patients, 13 (87%) were corrected with 1 surgery and 14 (93%) with 2 surgeries. The only failure was a Brown's syndrome not caused by superior oblique pathology. Five of the silicone tendon expander patients had at least 5 years follow-up (range, 5 to 11 years). Four (80%) of the 5 patients had an excellent outcome with 1 surgery, final results graded between 9 and 10 (on a scale of 1-10, 10 is best). The fifth patient had a consecutive superior oblique paresis and a good outcome after a recession of the ipsilateral inferior oblique muscle. The AAPOS survey had a mean outcome score of 7.3, with 65% between 8 and 10. There were 9 (6%) complications reported: 4 related to scarring and 5 extrusions of the implant. Three of the 5 extrusions were reported from the same surgeon. The computer model of an inelastic superior oblique muscle-tendon complex best simulated the motility pattern of Brown's syndrome with severe limitation of elevation in adduction, mild limitation of elevation in abduction, minimal hypotropia in primary position, no superior oblique overaction, and intorsion in up gaze. CONCLUSIONS: The presence of mild to moderate limitation of elevation in abduction is common, and its presence does not eliminate the diagnosis of Brown's syndrome. The majority of Brown's syndrome patients have a pattern of strabismus consistent with an inelastic superior oblique muscle-tendon complex that does not extend, but can contract normally; not the presence of a short tendon. The presence of inelastic or tethered superior oblique muscle-tendon can be diagnosed without forced duction testing by observing the pattern of strabismus including torsion. Because of the chance for spontaneous resolution, conservative management, not surgery, should be the first line of treatment for acquired Brown's syndrome. If surgery is indicated, a novel procedure, the silicone tendon expander, is an effective option with excellent long-term outcomes.

Pseudotumor cerebri sine papilledema with unilateral sixth nerve palsy.

A 17-year-old woman presented with a history of 1-week of headache and 3 days of horizontal diplopia. Examination revealed 20/20 vision in both eyes, no papilledema, and an abduction deficit in her left eye. Lumbar puncture revealed an opening pressure of 440 mm H2O. After treatment with acetazolamide, the headache and abduction deficit resolved. Papilledema never developed. This is a unique case of pseudotumor cerebri sine papilledema with a unilateral abduction deficit. We suggest that young women with headache and unilateral abduction deficits may be unrecognized cases of pseudotumor cerebri.

Effect of graded anterior transposition of the inferior oblique muscle on versions and vertical deviation in primary position.

INTRODUCTION: There are various methods for weakening the inferior oblique muscle; here we describe the results of a graded anterior transposition. METHODS: Charts of 21 children (37 eyes) who underwent graded anterior transposition of the inferior oblique muscle were reviewed. Graded anterior transposition consisted of reinsertion of the inferior oblique muscle at various points along the temporal aspect of the inferior rectus muscle; the more severe the overaction, the more anterior the placement of the new insertion. In all cases the new inferior oblique insertion line was oriented parallel to the inferior rectus muscle axis. We analyzed the preoperative to postoperative change in inferior oblique overaction (versions) and vertical alignment in primary position. RESULTS: Postoperatively, 18 of 21 patients had normal versions, 2 patients had -1 underaction of 1 eye, and 1 patient had +1 overaction of both eyes. Eleven patients (15 eyes) had a preoperative vertical deviation in primary position of 4 PD or more. Three of these patients had unilateral congenital superior oblique palsy and a preoperative hypertropia of 20 PD. They underwent unilateral graded anterior transposition with a mean postoperative vertical change of 18 PD. Three patients had asymmetric primary inferior oblique overaction with true hypertropia, 1 patient had amblyopia and primary inferior oblique overaction, and 4 patients had dissociated vertical deviation associated with inferior oblique overaction. All patients had improvement after surgery, with no significant vertical deviation in primary position. CONCLUSIONS: Graded anterior transposition of the inferior oblique muscle is effective in normalizing versions and correcting vertical deviations in primary position.

Leukocoria caused by occult penetrating trauma in a child.

PURPOSE: To report a child with leukocoria caused by occult penetrating trauma. METHODS: Case report. The clinical findings and surgical repair of acquired leukocoria of the right eye in a 2-year-old boy are presented. RESULTS: In the right eye, slit-lamp examination disclosed a retrolenticular cyclitic membrane and moderately severe (3+) cells and flare anterior chamber reaction. The eye was hypotonous, and B scan showed that the membrane was associated with a retinal detachment. Surgery was performed to repair the retinal detachment and to remove the retrolenticular membrane. Two months before initial examination, the patient had been attacked by a rooster. CONCLUSION: Occult penetrating trauma should be considered in the differential diagnosis of pediatric leukocoria.

Pediatric cataracts.

Posterior chamber intraocular lenses are a well-accepted treatment of aphakia in children 2 years of age and older, with many now considering them as the treatment of choice. Infants, however, are usually treated with contact lens, rather than intraocular lens implantation, as the infant eye undergoes significant axial elongation. The use of intraocular lenses in children with cataracts associated with juvenile rheumatoid arthritis remains controversial, but a recent article [9] describes good results in these patients, who historically have a poor prognosis. The management of amblyopia associated with unilateral congenital cataracts is evolving. In the 1970s and 1980s, full-time occlusion of the sound eye was advocated for infants with unilateral congenital cataracts. It was also taught that binocular fusion was impossible to obtain, and children with unilateral cataracts inevitably develop strabismus. Recent studies have shown that part-time occlusion may in fact yield better results, allowing the development of binocular vision and stereopsis and reducing the incidence of strabismus.

Late overcorrection after inferior rectus recession.

PURPOSE: Describe characteristics and possible etiology of late overcorrection after inferior rectus recession in patients without Graves disease. METHODS: Seven adult patients with initial good alignment (< 5 prism diopters) but an overcorrection 1 month after inferior rectus recession were studied. RESULTS: Two patients had congenital superior oblique paresis, one had traumatic superior oblique paresis, two had orbital fracture, one had strabismus after retinal detachment surgery, and one had hypotropia after cataract surgery. Overcorrection occurred 4 to 6 weeks after surgery, measuring 12 to 25 prism diopters. Six patients with late overcorrection after inferior rectus recession underwent repeat surgery of the inferior rectus muscle. All six patients had scarring of the Lockwood ligament but no muscle slippage. CONCLUSION: Late overcorrection can occur unrelated to Graves ophthalmopathy or a slipped muscle. Postoperative scarring around the Lockwood ligament was identified, which could result in reduced inferior rectus muscle force. It is hypothesized that late scar contracture in the vicinity of the Lockwood ligament could pull the inferior rectus muscle anteriorly, thus slackening the anterior aspect of the muscle. This slackening of the anterior aspect of the inferior rectus muscle would weaken the depression function, thus producing a late overcorrection.

Clinical optokinetic nystagmus asymmetry in treated esotropes.

BACKGROUND: Monocular optokinetic nystagmus (OKN) asymmetry is associated with disruption of early binocular visual development. METHODS: Three groups of treated esotropic patients and a group of normal controls were evaluated for the presence of clinically detectable monocular OKN asymmetry. Clinical assessment of monocular OKN asymmetry was performed by observing eye movements in response to a hand-held rotating drum. Asymmetry was quantitated on a scale of 0 to +3 OKN asymmetry. Clinical OKN asymmetry was evaluated in the following groups of patients: those with congenital esotropia treated with very early surgery achieving high-grade stereo acuity (group 1); those with congenital esotropia treated with late surgery achieving no stereopsis (group 2); those with acquired esotropia achieving high-grade stereo acuity after treatment with spectacle correction (group 3); and normal controls (group 4). RESULTS: Two of the three patients in group 1 showed +3 OKN asymmetry despite having high-grade stereo acuity; the third one, who was surgically aligned earliest (13 weeks), demonstrated +1 OKN asymmetry. This patient achieved orthotropia, 40 seconds stereo acuity, perfect Randot stereo acuity, and had no dissociated vertical deviation or latent nystagmus. All 10 patients in group 2 (those with late alignment-after 1 year-and no stereo acuity) showed +3 OKN asymmetry. All four patients in group 3 (those with acquired hypermetropic esotropia and high-grade stereo acuity after treatment) and all 10 patients in the normal control group showed no OKN asymmetry. CONCLUSION: Clinically obvious monocular OKN asymmetry can occur in patients with congenital esotropia who are aligned early and develop high-grade stereo acuity. Even brief periods of strabismus during the early period of binocular motor development can result in persistent OKN asymmetry. This suggests that binocular motor processing may develop distinct from, and prior to, the development of high-grade stereo acuity. OKN asymmetry appears to be a clinical sign of an insult to early binocular motor development.

Comitant strabismus.

This review reports on articles written on comitant strabismus during the past year. Congenital esotropia was a recurring theme in these publications. Included in this review are articles that provide an insight into the basis for motor epiphenomena such as optokinetic nystagmus asymmetry and latent nystagmus. The optimum window of opportunity to achieve quality binocular vision by surgical alignment in congenital esotropia is discussed. We also report on articles that address various aspects of the management of comitant strabismus including the amount of medial rectus recession for esotropia, the target angle for best results in accommodative esotropia with high accommodation convergence/accommodation ratio, and issues related to comitant exotropia.

Combined study on the causes of strabismus after the retinal surgery.

Extraocular muscle imbalance and diplopia after retina surgery have been previously reported, but the etiology is still controversial. In order to better understand the cause of strabismus after retinal surgery, the authors retrospectively studied 30 patients with persistent strabismus following retinal surgery and combined the result about seven patients of strabismus after retinal surgery in the previous report. Results showed multiple etiologies for the strabismus. Causes of strabismus included fat adherence syndrome (14 patients), non-specific restrictive adhesion (11), displacement of superior oblique tendon (2), scleral explant interfering with ocular motility (1), lost or slipped muscle with adhesion (2), sensory strabismus (2), macular pucker causing ectopic fovea (2), and previous strabismus before the retinal surgery (3). Knowledge of the varieties of abnormalities that can cause strabismus and diplopia will help both the retina and strabismus surgeon prevent and treat strabismus after retinal surgery.

Independence of optokinetic nystagmus asymmetry and binocularity in infantile esotropia.

OBJECTIVE: Children with congenital esotropia, amblyopia, or early visual deprivation have persistent asymmetric monocular pursuit, as measured by optokinetic nystagmus (OKN), and favor targets moving in a temporal to nasal direction. Previous studies suggest that binocular visual development is necessary for the development of symmetric monocular OKN. We recently treated patients with congenital esotropia with unconventionally early surgery to establish good binocularity. We wished to determine if mature symmetric OKN responses could develop in patients with congenital esotropia following the acquisition of good stereopsis. METHODS: Electro-oculographic recordings documented horizontal eye movements in response to an electronic OKN stimulus. PATIENTS: The recordings of three patients with congenital esotropia that had been surgically aligned before age 20 weeks and who had achieved high-grade stereopsis with random dot and Titmus stereographic testing were studied. We compared this group of children with groups of age-matched controls, including the following: (1) three patients with congenital esotropia who underwent surgical alignment after age 6 months and who had no amblyopia but poor stereopsis; (2) three children with accommodative esotropia, good ocular alignment when wearing spectacles, and good stereopsis; and (3) three normal children. RESULTS: The normal children and those with accommodative esotropia demonstrated normal symmetrical OKN. The patients with congenital esotropia showed poor nasal to temporal OKN regardless of the degree of stereopsis or timing of surgery. CONCLUSIONS: Our results indicate that good binocularity, as measured by stereopsis, is not sufficient for the development of symmetric OKN in patients with congenital esotropia.