Re-recession of the medial rectus muscles in patients with recurrent esotropia.

PURPOSE: Retrospective evaluation of changes in ocular motility after surgical re-recession of the medial rectus (MR) muscles as treatment of recurrent esotropia (ET). METHODS: We describe 115 patients (age, 11 months-77 years; median, 11.1 years; 83 children and 32 adults) with an average amount of non-accommodative ET before surgery of 18.7 PD (SD = 8.8 PD). Preoperative alignment, amount of re-recession, distance from insertion to the limbus, and postoperative alignment and versions were collected. RESULTS: In most cases, MR muscles were re-recessed to a fixed distance of 12 mm from the limbus, with unilateral re-recessions in cases with relatively small ET (typically < 20 PD) and bilateral re-recessions in cases with larger amounts of ET (typically > 20 PD). No clear relation was found between the amount of re-recession and the change in alignment in prism diopters. The success rate (esotropia [ET] < or = 10 PD or exotropia [XT] < or = 8 PD) 4 weeks to 8 months after surgery was 85%, with 4 patients still showing ET and 13 patients showing XT. Incidence of XT was higher for bilateral than for unilateral re- recessions. Significant underaction of the MR muscles was noted in 7% of the patients. None of the undercorrected patients and only 1 of the overcorrected patients were adults. Among adults, incidence of MR underaction was 4%. Long-term follow-up (8-120 months; median, 25 months) data from 59 patients indicated that good stability in alignment can be expected. CONCLUSION: The results support the notion that MR re-recession to 12 mm from the limbus successfully corrects recurrent ET up to 35 PD and that it is particularly effective in adults.

Anterior and nasal transposition of the inferior oblique muscle: a preliminary case report on a new procedure.

PURPOSE: To describe this new extraocular muscle surgery. METHOD: Case Report of a child in need of treatment of a weak superior oblique muscle which, at surgery, was in fact found to be absent. After a prior only partially successful recession of the antagonist inferior oblique (IO), the IO muscle was detached and transposed to a new insertion in the inferior nasal quadrant converting the muscle, as a result of its functional origin being the neurovascular bundle ["Ligament of Stager"-ed], from an extorter and elevator of the globe to an intorter and depressor. RESULT: Satisfactory binocular alignment was achieved. CONCLUSION: This new surgical procedure provides significant advantages, and should be added to our surgical armamentarium.

Screening for amblyopia in preverbal children with photoscreening photographs: II. Sensitivity and specificity of the MTI photoscreener.

OBJECTIVE: To determine the sensitivity and specificity of vision screening using the Medical Technology and Innovations (MTI), Inc., PhotoScreener. DESIGN: Cross-sectional study. PARTICIPANTS AND TESTING: Three hundred ninety-two children less than 4 years of age received a complete ophthalmologic examination and were photographed using the MTI PhotoScreener. One hundred three children had normal examinations, and the remaining 284 children had conditions of interest for pediatric screening: ptosis, media opacity, refractive error, or strabismus. Five children were excluded. MAIN OUTCOME MEASURES: The grading of the photographs by the manufacturer's representative was compared with the results of the ophthalmologic examinations. Sensitivity and specificity of vision screening were determined. RESULTS: The analysis of all informative photographs resulted in a sensitivity of 65% and a specificity of 87%. The sensitivity of detection for children with some forms of strabismus was high, up to 95% for esotropia of 10Delta or more. Sensitivities for the detection of ptosis, media opacity, and refractive error were poor in patients where strabismus was not also present. CONCLUSIONS: The MTI PhotoScreener may play a role in preverbal vision screening; identification of two of three children with amblyopiogenic factors before age 4 would be an exciting advance in public health. However, improvement in the ability to identify children with media opacity and refractive error is necessary. Improvements may be possible with modifications of the examination failure and photograph grading criteria.

Screening for amblyopia in preverbal children with photoscreening photographs. III. improved grading criteria for hyperopia.

OBJECTIVE: To examine the ability of the Medical Technology and Innovations (MTI), Inc., Photoscreener (Cedar Falls, IA) to detect hyperopia and to improve the photograph grading criteria to screen for amblyopiogenic levels of hyperopia. DESIGN: Cross-sectional study and reanalysis. PARTICIPANTS AND TESTING: In previous work, 392 participants received a complete ophthalmologic examination and were photographed using the MTI Photoscreener. For this study, all 209 participants with normal examination findings (65 children) or hyperopia without anisometropia (144 children) were selected. The data were reanalyzed using modified photograph grading and ophthalmologic examination failure criteria. Potential reasons for why many children with hyperopia passed photoscreening were explored. MAIN OUTCOME MEASURES: We determined whether a study participant would pass or fail screening with a given photograph grading and ophthalmologic examination failure criteria. RESULTS: Most children with hyperopia of +2.00 to +3.50 diopters (D) passed screening with the MTI instrument, in most cases because their photographs lacked bright crescents. When bright crescents in at least two of the four possible meridians were the grading guideline for screening failure and the pediatric ophthalmologists' consensus hyperopia failure criteria (> +3.50 D) were adopted, the sensitivity for hyperopia detection was 100% and the specificity was 88%. Identical results were obtained using the American Academy of Ophthalmology Preferred Practice Pattern hyperopia failure criteria (>/= +4.50 D). CONCLUSIONS: The MTI photograph grading guidelines can be simplified, and the ophthalmologic examination failure criteria for hyperopia can be improved. The presence of a bright crescent in the lower or the left pupillary margin indicate hyperopia in an amblyopiogenic range (> +3.50 D).

The five "S" levels of enterprise health.

Whether pride, necessity, or inattention is at the root, some "slowly boiling" physicians find themselves working harder for fewer compensations of all sorts, and may not be fully cognizant of their circumstances. This article helps to diagnose and manage the health of physicians' practices and/or related enterprises. There are five levels of enterprise health, ranging from success (S-1) to shutdown (S-5), that serve as weather vanes about how the enterprise is adapting to changes in its environment. How should physicians respond to chaos and the threats of deteriorating enterprise health? A five-step approach is offered: (1) Discern what is important; (2) place and keep your program in alignment with those patient interests that will enhance your enterprise viability; (3) keep score with an internal balanced scorecard; (4) manage and shepherd your resources in a manner that demonstrably adds value to patient care; and (5) know the score and use it.

A value analysis model applied to the management of amblyopia.

PURPOSE: To assess the value of amblyopia-related services by utilizing a health value model (HVM). Cost and quality criteria are evaluated in accordance with the interests of patients, physicians, and purchasers. METHODS: We applied an HVM to a hypothetical statistical ("median") child with amblyopia whose visual acuity is 20/80 and to a group of children with amblyopia who are managed by our practice. We applied the model to calculate the value of these services by evaluating the responses of patients and physicians and relating these responses to clinical outcomes. RESULTS: The consensus value of care for the hypothetical median child was calculated to be 0.406 (of 1.000). For those children managed in our practice, the calculated value is 0.682. Clinically, 79% achieved 20/40 or better visual acuity, and the mean final visual acuity was 0.2 logMAR (20/32). Value appraisals revealed significant concerns about the financial aspects of amblyopia-related services, particularly among physicians. Patients rated services more positively than did physicians. CONCLUSIONS: Amblyopia care is difficult, sustained, and important work that requires substantial sensitivity to and support of children and families. Compliance and early detection are essential to success. The value of amblyopia services is rated significantly higher by patients than by physicians. Relative to the measured value, amblyopia care is undercompensated. The HVM is useful to appraise clinical service delivery and its variation. The costs of failure and the benefits of success are high; high-value amblyopia care yields substantial dividends and should be commensurately compensated in the marketplace.

Screening for amblyopia in preverbal children with photoscreening photographs. National Children's Eye Care Foundation Vision Screening Study Group.

OBJECTIVE: This study aimed to determine the ability of healthcare professionals and lay volunteers to grade photoscreening photographs. DESIGN: The study design was a cross-sectional study. PARTICIPANTS AND INTERVENTION: One hundred children 3 years of age or younger received a complete ophthalmologic examination and were photographed using the Medical Technology Innovations (MTI) photoscreener. Twenty-six children had normal examination results, and the remaining 74 children had conditions that are of interest for pediatric screening, including strabismus, refractive error, media opacities, and ptosis. Eighteen volunteers, including pediatric ophthalmologists, pediatricians, ophthalmic technicians, health department nurses, Prevention of Blindness Society personnel, and Lions Club volunteers, graded each of the 100 photoscreening photographs. MAIN OUTCOME MEASURES: Sensitivity and specificity of vision screening and of photograph grading were measured. RESULTS: Results from various graders yielded sensitivities ranging from 37% to 88% and specificities ranging from 40% to 88%. No single grader achieved sensitivity and specificity both greater than 70%. The grading of the manufacturer's representative had a sensitivity of 43% and a specificity of 85%. Sensitivity decreased to 31% for strabismus and 18% for refractive error when the correct type of strabismus or refractive error was required to be considered true-positives. Results were not positively correlated with the ophthalmologic knowledge of the participant. CONCLUSIONS: The wide variability in sensitivities and specificities among graders indicates inconsistent photograph interpretation skills or deficient screening guidelines or both. For off-axis photoscreening as implemented by the MTI system to become a useful vision-screening method, additional photograph interpretation skill transfer may be beneficial, although not necessarily sufficient.

Neurofibromatosis type 1 in children.

OBJECTIVE: To document ophthalmic and general characteristics of Neurofibromatosis Type 1 (NF1) in children; and to characterize the iris changes of NF1 including their variability and reliability. DESIGN AND PATIENTS: One hundred and ninety-six patients with NF1 were evaluated for general characteristics; 156 patients underwent prospective eye evaluations; and 151 NF1 patients and controls had iris photography in accordance with a protocol. Masked evaluation of photographs compared to a known diagnosis, and interobserver reliability tests were performed. MAIN OUTCOME MEASURES: Incidence of iris changes consistent with NF1 (Lisch nodules, plus) by age group; spectrum of iris changes found; correlation of iris findings to known diagnosis and Kappa coefficients for interobserver reliability. RESULTS: Iris changes were common in children over age 5, and increased with age; masked evaluation of photographs compared to a known diagnosis yielded fair to poor correlation (Kappa = -.02 to .50); interobserver reliability was poor (Kappa = -.02 to .24, overall .174); iris changes found in NF1 are more diverse than classic descriptions of Lisch nodules, and including a broader spectrum probably increases the sensitivity and decreases the specificity of their diagnostic value. CONCLUSION: Iris changes as a diagnostic marker for NF1 may need rethinking; this study calls to question their reliability and validity.

Ocular findings associated with neurofibromatosis type II.

BACKGROUND: Neurofibromatosis has been recently acknowledged as consisting of a number of different diseases. Neurofibromatosis (NF) type I and NF type II are the most clearly defined. Type II is characterized by bilateral acoustic neuromas and is rare (its incidence is 1/50,000). The previously reported ocular associations of NF type II are posterior subcapsular cataracts, Lisch nodules, and combined hamartomata of the retinal pigment epithelium and retina. In this study, the authors attempt to define further the ocular manifestations of NF type II. METHODS: The authors prospectively examined 9 patients who met the diagnostic criteria for NF type II (age, 18 to 38 years; mean, 25 years). RESULTS: Seven of nine patients had epiretinal membranes in the posterior pole. None of these epiretinal membranes were visually significant. In addition, five patients had central posterior cortical cataracts and five had peripheral wedge-shaped cortical cataracts. CONCLUSION: The presence of epiretinal membranes in young patients may represent another clinical finding associated with NF type II. Epiretinal membranes, central posterior cataracts, peripheral cortical cataracts, or combined hamartoma of the retinal epithelium and retina in young patients should alert the ophthalmologist to include NF type II in the differential diagnosis in patients with stigmata of NF type II.

Photophobia, epiphora, and torticollis: a masquerade syndrome.

Three young children presented with photophobia, epiphora, and torticollis as the initial manifestation of a posterior fossa tumor. In each case there was a delay in treatment due to the presumptive diagnosis of a local ocular inflammatory condition. We recommend that children with unexplained photophobia, epiphora, and torticollis undergo an imaging technique to evaluate the posterior fossa.

Clinicopathological findings in a growing optic nerve melanocytoma.

We present an unusual case of a melanocytoma of the optic disc that showed documented progressive growth over a period of six years. It reached the largest size of any reported optic nerve melanocytoma and the eye was enucleated because of the possibility of malignant degeneration. Optic nerve melanocytomas are locally invasive but are not known to undergo transformation into malignant melanoma. Follow-up should remain the primary approach in the management of patients with optic nerve melanocytomas, as previously advocated by Zimmerman.

Recession and anterior transposition of the inferior oblique for treatment of superior oblique palsy.

We evaluated the effectiveness of inferior oblique recession with anterior transposition in treating 12 patients with superior oblique palsy. Mean decreases of hypertropia measured 17 prism diopters in the primary position, 24 prism diopters in adduction, and 21 prism diopters on ipsilateral head tilt. Head tilt and diplopia were uniformly eliminated. No surgical complications were encountered. Postoperative deviations were mild and infrequent. Only one patient demonstrated postoperative underaction of the recessed inferior oblique.

Ring stromal opacity in lens-corneal adhesion.

Severe congenital corneal clouding may be associated with abnormal anterior segment development (dysgenesis). Often, congenital corneal opacities preclude visualization of intraocular anatomy. In this report, five eyes of four patients with congenital corneal clouding and lens-corneal adhesion (LCA) are described. All had a ring- or doughnut-shaped haze within the stroma surrounding a central clear corneal zone delineating the adhesion. Recognition of LCA is pivotal in planning surgical therapy for eyes with congenital corneal clouding. As a clue to recognition of this anomaly, the ring-shaped stromal opacity may aid in diagnosis of this phenomenon and in avoidance of surgical complications.

Learning disabilities: update comment on the visual system.

The role of the eye in vision and its relationship to learning disabilities is discussed in this very timely article. A historical overview is included, as well as a consideration of the more recent neuroanatomic research in this area.

Collagen maturation defects in Ehlers-Danlos keratopathy.

Ehlers-Danlos syndrome is one of many inherited abnormalities of connective tissue, specifically collagen. A 16-year-old caucasian female with Type VI Ehlers-Danlos syndrome had five unusual corneal findings, four of which have not been reported in association with Ehlers-Danlos syndrome: micro-cornea (previously reported), cornea plana, keratoconus posticus, stromal haze at the level of Bowman's layer and a peripheral ring opacity suggestive of anterior embryotoxon. Type VI Ehlers-Danlos syndrome is associated with deficient activity of lysyl hydroxylase which ultimately is responsible for cross-linking collagen chains, the source of its tensile strength. Genetic regulation of procollagen formation is believed to be mediated by mRNA. Aggregation and maturation in the extracellular matrix is probably influenced and mediated by its evolving biochemical environment, particularly by complex carbohydrate moieties. An understanding of collagen maturation, and the pathophysiologic and biophysical consequences of abnormalities at this level of biochemical organization, permits more specific understanding of ocular dysmorphology and collagen diseases.

Accommodative esotropia: review of current practices and controversies.

The treatment of accommodative esotropia requires a thorough evaluation at first presentation to elucidate all factors involved. The degree of hypermetropia, character of the esodeviation (i.e., intermittent or constant), presence or absence of amblyopia, and the AC/A ratio are all important in the initial management of these patients. In addition, the binocular status can be of prognostic value. Close follow-up is needed to ensure maintenance of binocularity and appropriate early intervention to modify the course before bifixation is lost.