Accommodative esotropia: the state of the art.

PURPOSE: To review the state of the art of Accommodative Esotropia (AE) through careful study of what has been reported up to the point in literature. METHODS: A literature search was done on PubMed using key words including "Accommodative esotropia", "Infantile esotropia", "Strabismus" and "Accommodation". We systematically reviewed and critically appraised what has been written about AE and we tried to analyze that according to the current management of AE. RESULTS: Accommodative Esotropia (AE) is a form of strabismus characterized by convergent misalignment of the visual axes that can be associated with hyperopia and abnormal fusional divergence. Also abnormal accommodative convergence/accommodation ratio could be found. In lots of cases, AE initially presents as an intermittent esodeviation at age 1.5 to 4 years. The prevalence of AE has been estimated near 1-2% in the United States. The only treatment with an optical correction usually is successful in re-establishing alignment, but surgical correction is necessary in approximately 30% of cases.

Determination of vertebral heart score in three species of Spider monkey (Ateles fusciceps, A. hybridus and A. paniscus).

BACKGROUND: The vertebral heart score (VHS) is a method of evaluation of cardiac size well documented in domestic mammals and in other primate species, and the aim of this study was to determine the VHS in three species of Spider monkey. METHODS: In this retrospective study, right lateral radiographs of thirty clinically well animals were reviewed and VHS determined. The species included were Ateles fusciceps (n=17), Ateles hybridus (n=8) and Ateles paniscus (n=5). RESULTS: The VHS was found to vary between species and was 9.73±0.81 for A. fusciceps, 10.53±0.37 for A. hybridus and 10.45±0.27 for A. paniscus. CONCLUSIONS: The observed values appear consistent with values determined for other primate species. There was statistically significant variation noted between species, and so VHS should be considered species-specific in this genus. The values determined may be of benefit in objectively evaluating cardiac size in the species investigated.

Diagnosis and treatment of concurrent dermal malignant melanoma and melanocytomas in a pygmy hippopotamus (Choeropsis liberiensis).

BACKGROUND: Dermal melanocytic neoplasms are common in some even-toed ungulates (Artiodactyla), yet this entity has not been reported in the pygmy hippopotamus to date. Concurrent occurrence of multiple benign and malignant melanocytic neoplasms is unusual. Malignant transformation occurs in a small percentage of benign melanocytic tumours in people but this phenomenon has not been well documented in animals. OBJECTIVES: To report the diagnosis and treatment of concurrent dermal melanocytomas and malignant melanomas in a pygmy hippopotamus. ANIMALS: A 36-year-old intact male pygmy hippopotamus, part of a zoological collection, housed with a 10-year-old female of the same species, presented with multiple raised and pigmented skin masses. METHODS: Initial impression smears of one ulcerated lesion were consistent with inflammation; subsequent histopathological findings from a skin biopsy revealed an underlying malignant melanoma. The animal was anaesthetised, ultrasonographic imaging of the local lymph nodes indicated no local involvement and all skin lesions were removed. Recovery from anaesthesia was unremarkable, skin healing was within normal limits for the species. There was no sign of recurrence 34 months post-surgery. RESULTS: A diagnosis of malignant melanomas and concurrent melanocytomas was made on histopathological evaluation. CONCLUSION AND CLINICAL IMPORTANCE: To the best of the authors' knowledge, this is the first reported case of melanocytic neoplasia in the pygmy hippopotamus. The occurrence of both benign and malignant melanocytic skin tumours should be considered in this species.

Determination of vertebral heart score in Goeldi's monkeys (Callimico goeldii).

BACKGROUND: Goeldi's monkeys (Callimico goeldii) are callitrichid primates commonly kept in zoological collections, and to date, no cardiac parameters have been recorded. The vertebral heart score (VHS) is an objective method of evaluation of cardiac size well documented in domestic mammals, and the aim of this study was to determine the VHS in Goeldi's monkeys. METHODS: In this retrospective study, right lateral radiographs of thirteen clinically well animals were reviewed and vertebral heart score determined. RESULTS: The vertebral heart score was found to be 9.35 ± 0.31. CONCLUSIONS: The observed value appears consistent within the study population and with values for other primate species. The value determined may be of benefit in objectively evaluating cardiac size in this species.

Torsional diplopia after orbital decompression and strabismus surgery.

PURPOSE: To investigate diplopia after orbital decompression in patients with thyroid-related orbitopathy. METHODS: We retrospectively analyzed medical records of 45 consecutive patients who had undergone orbital decompression surgery (DS) and a subgroup of 10/45 patients who underwent disinsertion of the inferior oblique (IO) muscle during DS. Diplopia was assessed before DS and 1 and 3 months after DS. Residual strabismus defects were treated surgically approximately 3 months after DS with follow-up visits 1 week, 3 months, and 6 months postoperatively. Patients with horizontal or vertical diplopia were evaluated using the alternate prism-and-cover test and torsional diplopia (TD) using a double Maddox rod test to quantitate their strabismus. RESULTS: Strabismus surgery was effective in treating the horizontal and vertical diplopia (13/40 cases with residual deviations required prism glasses), but did not resolve the TD. None of the patients with IO disinsertion during DS complained of TD before strabismus surgery. However, after strabismus surgery, TD occurred in all 10 patients with IO disinsertion vs 8/30 patients without IO disinsertion (p<0.001, Fisher exact test). Overall, TD was induced in 18/45 (40%) of the DS cohort. CONCLUSIONS: Patients with IO disinsertion during DS may be at increased risk of developing TD postoperatively. We recommend sparing or reattaching the IO muscle during DS when possible to reduce the risk of developing untreatable TD.

When and how to strengthen the superior oblique muscle.

PURPOSE: To review the history of procedures used to strengthen the action of the superior oblique (SO) muscle and methods of quantifying surgical dosage and to determine the relationship between congenital onset and tendon laxity measured at the time of surgery. METHODS: We reviewed medical records over a 10-year period of 30 patients who had undergone SO tendon tuck for SO muscle palsy using intraoperative assessment of forced ductions to determine surgical dosage. We also designed and tested a modified Bishop tucker, which can simultaneously measure tendon shortening and the force required. This allowed development of length tension curves between 0 and 200 g for individual SO tendons in patients with and without evidence of muscle palsy. RESULTS: In distinction to most other procedures on the extraocular muscles, intraoperative forced ductions are used to determine appropriate surgical dosage. Patients undergoing SO tendon tuck using a uniform and repeatable forced duction method receive a greater amount of tuck (mean, 3 mm) when there is known congenital onset. Patients with congenital SO muscle palsy received a mean tuck of 10.8 mm (range, 8-16 mm), whereas patients with equivocal or known adult-onset SO palsy received a mean of 7.8 mm (range, 4-12 mm; p = 0.002). CONCLUSIONS: Patients with congenital SO muscle palsy have increased tendon laxity when measured directly during SO tendon tuck. The excursion of presumed normal SO tendons through the trochlea is variable and may be less than previously thought. Modifying the Bishop tucker to provide length and tension data provides information that may be useful in determining surgical dosage.

Retinopathy of prematurity in extremely premature infants.

INTRODUCTION: The incidence and severity of retinopathy of prematurity (ROP) in extremely premature infants have not been reported since publication of the Early Treatment of ROP study results. The survival rate of these infants continues to increase. We sought to determine the characteristics of ROP in a group of surviving infants <25 weeks estimated gestational age (EGA) at birth compared to a group 25 to 27 weeks EGA at birth. METHODS: Retrospective review of infants born prior to 27 weeks EGA between January 2003 and July 2007 at a level-3 nursery at a regional academic medical center. RESULTS: A total of 231 medical records were reviewed and found to have analyzable data. Of 79 infants <25 weeks EGA, 69 (87%) developed ROP, compared to 95 of 152 (62%) infants 25 to 27 weeks EGA. Type 1 ROP developed in 23% of infants in the <25 weeks EGA group, compared to 9% of infants in the 25 to 27 weeks EGA group. There was no difference in mean postmenstrual age when type 1 ROP was diagnosed. Lower birth weight predicted increased risk of type 1 ROP in the 25 to 27 weeks EGA group, but not in the <25 weeks EGA group. CONCLUSIONS: Extremely premature infants are more likely to develop ROP and type 1 ROP, but the incidence may be lower than previously reported. Birth weight may not influence the incidence of type 1 ROP in this group of infants. Type 1 ROP does not develop at an earlier postmenstrual age in the extremely premature infant.

Sensory results after lateral rectus muscle recession for intermittent exotropia operated before two years of age.

INTRODUCTION: Early surgical treatment of early-onset intermittent exotropia is controversial. The condition does not always progress, and postoperative esotropia can produce adverse consequences, such as suppression, amblyopia, and loss of binocular vision, particularly stereopsis. It is not known whether surgery before age two years affects the sensory outcome. METHODS: Using a noncomparative (nonrandomized, uncontrolled) interventional case series, we reviewed the medical records during a 14-year period of 24 patients with intermittent exotropia with onset before age 1 year who also received bilateral lateral rectus muscle recessions before two years of age. Twelve patients were identified who received follow-up examinations at ages > or =4 years, when testing with Worth 4-Dot and Titmus stereo circles would likely be reliable. RESULTS: Mean age of the patients at last follow-up examination was 87 months. Stereoacuity was measured at 40 arcsec in 2 patients, 100 arcsec in 3 patients, 140-400 arcsec in 2 patients, and none in 5 patients. Among the 5 patients without demonstrated stereopsis, 1 had a history and clinical course consistent with congenital exotropia, and 1 had a nonstrabismic explanation for poor stereopsis. Overall, 7 patients (58%) ultimately obtained favorable motor alignment, defined as a phoria or intermittent tropia <10(Delta) at distance and near. No patient required treatment for amblyopia. CONCLUSIONS: Early-onset intermittent exotropia usually responds well to surgical treatment, and high-grade stereopsis can be achieved in some cases. More than one clinical entity presenting as intermittent exotropia in infancy probably exists.

A 10-year overview of double elevator muscle weakening procedures.

OBJECTIVE: To report the effect of weakening the superior rectus and inferior oblique (IO) muscles on ocular rotations. DESIGN: Observational case series. We reviewed a 10-year period of medical records of consecutive patients who underwent bilateral 5- to 11-mm (mean, 8.0 mm; SD, 1.1 mm) superior rectus muscle recessions combined with an IO muscle recession, myectomy, or anterior transposition. The effects on ocular rotations and eyelid position were recorded for the 37 patients (69 eyes) who were followed up for at least 6 months postoperatively. Nonparametric 1-way analysis of variance was used to compare results across the 3 procedures. The setting was a subspecialty practice at an academic institution. RESULTS: Supraduction deficiency was significantly associated with transposition of the IO muscle anterior to the inferior rectus muscle insertion compared with the standard IO muscle recession (P = .001), and IO muscle myectomy (P = .009). Y-pattern exotropia occurred more frequently after transposition of the IO muscle anterior to the inferior rectus muscle insertion than other weakening procedures (P<.001). CONCLUSION: Transposition of the IO muscle anterior to the inferior rectus muscle insertion, combined with ipsilateral superior rectus muscle recession, results in more supraduction deficiency and more frequent Y-pattern exotropia compared with standard IO muscle recession.

One-muscle surgery in small-angle residual esotropia.

PURPOSE: To report the outcome of unilateral lateral rectus resection for treatment of small-angle residual esotropia following bilateral medial rectus muscle recession. METHODS: A retrospective medical record review was performed for all patients who had undergone bilateral medial rectus muscle recession for congenital esotropia prior to 6 years of age that required further surgical treatment of residual esotropia. We compared two different dosing strategies for resection of a single lateral rectus muscle in the nondominant eye. In group 1, the amount of resection was calculated by doubling the angle of strabismus and applying the recommended surgical dosage to one lateral rectus muscle. In group 2, the amount of unilateral resection was the same as the bilateral dosage for the measured angle, but augmented by 1.5 mm. Postoperative evaluation was performed 1 and 6 months after surgery. RESULTS: Data from 35 patients were analyzed, 17 in group 1 and 18 in group 2. No significant intergroup difference was noted in terms of age at first surgery (p = 0.266), initial surgical dosage (p = 0.693), residual angle of esotropia (p = 0.881), or age at reoperation (p = 0.679). Postoperative alignment was better in group 1 patients at 6 months than at 1 month (residual deviation 3.5(Delta) versus 6.7(Delta), p = 0.022). CONCLUSIONS: Resection of a single lateral rectus muscle with the surgical dosage calculated by doubling the angle of strabismus and applying the recommended surgical dosage to one lateral rectus muscle is a treatment option for patients with small-angle residual esotropia following bilateral medial rectus muscle recession.

The management of strabismus in adults--III. The effects on disability.

INTRODUCTION: This is the third article in a series on the various facets of the management of strabismus in adults. Here, we give a broad overview of the types and severity of disability and provide initial validation of an instrument (questionnaire) to assess these disability aspects. METHODS: After undergoing strabismus surgery, 101 patients from 6 centers completed a 6-item questionnaire in which they rated both the before-surgery and after-surgery severity of problems associated with their strabismus, ranging from specific health, daily functioning, social interaction, concerns about the future, and self-image to job-related difficulties. RESULTS: The before-surgery outcomes showed significant variation across the 6 types of problems (P < 0.001), with "specific health" and "daily tasks" yielding the highest problem rating. Patients with diplopia reported more severe problems with "daily tasks" (P = 0.004) and "concerns about the future" (P = 0.026) than patients without diplopia. Overall, all problem ratings declined after surgery (P < 0.001), but patients who were not successfully aligned were left with higher problem ratings on "specific health" (P = 0.005), "daily tasks" (P = 0.003), and "social interaction" (P = 0.024). CONCLUSIONS: The results indicate a wide range of disability aspects in adult patients with strabismus, with moderate differences between patients with or without diplopia. Improvements in disability after surgery, as reflected by these ratings, should be taken into account when assessing the health value of adult strabismus management.

Iris hypoplasia and aorticopulmonary septal defect: a neurocristopathy.

BACKGROUND: Errors in neural crest development are responsible for a number of ocular disorders. In some cases, these ocular abnormalities may be associated with systemic disorders, some of which may be life threatening. METHODS: We reviewed the medical record of 3 children from 2 institutions with a similar constellation of ocular and cardiac abnormalities. RESULTS: All 3 children had iris hypoplasia with pupils 6-7 mm in diameter that had minimal or no response to light or pharmacological agents. The ocular examinations were otherwise normal. All 3 children also had large aorticopulmonary septal defects which were surgically repaired. CONCLUSIONS: We report an association between iris hypoplasia and aorticopulmonary septal defects. Like the iris stroma, the aorticopulmonary septum forms from neural crest cells during embryogenesis which divide the truncus arteriosus into the ascending aorta and pulmonary trunk. Children with iris hypoplasia should undergo an immediate cardiac evaluation. It is important that opthalmologists be aware of the association between these two disorders since an aorticopulmonary septal defect is a life-threatening disorder.

The management of strabismus in adults--II. Patient and provider perspectives on the severity of adult strabismus and on outcome contributors.

PURPOSE: As part of a larger study intended to evaluate the management of strabismus in adults, we documented and compared patient and provider perspectives on the various factors that may contribute to treatment outcome and value of strabismus care. METHODS: By completing a questionnaire, 170 patients with strabismus (ages 19 to 87 years) and 11 strabismus specialists who performed surgery on these patients each indicated the relative weight of several disease- and treatment-related contributors to the outcome and value of care. In addition, each respondent rated the severity of the strabismus before and after surgery. RESULTS: Overall severity ratings improved as a result of surgery. The improvement perceived by the physicians was 5.1 +/- 1.7 on a 10-point scale and was larger than the 2.6 +/- 3.6 points improvement perceived by the patients ( P < 0.001). Patients and physicians expressed different views on the relative contributions to outcome and value (overall P < 0.001). Both groups indicated "the condition," "the physician," and "the procedures" as the largest contributors, but "condition" was perceived as more important by the physicians than by the patients ( P < 0.001), and "physician" was more important to the patients than to the physicians ( P < 0.001). CONCLUSION: Although in approximate agreement on the main contributors to outcome and value, discrepancies exist between perspectives of patients and physicians on the relative weights of those contributors. Public information, education, and counseling may bring these perspectives better in line and ultimately improve both quality and patient satisfaction.

Cataracts and glaucoma in patients with oculocerebrorenal syndrome.

BACKGROUND: Oculocerebrorenal syndrome is an X-linked recessive hereditary oculocerebrorenal disorder characterized by congenital cataract, mental retardation, and Fanconi syndrome of the proximal renal tubules. Other ocular findings include glaucoma, corneal opacity (keloid), enophthalmos, and hypotonia. OBJECTIVE: To describe the treatment of 7 patients (14 eyes) with bilateral cataracts associated with oculocerebrorenal syndrome. Method Retrospective review. RESULTS: Seven patients with oculocerebrorenal syndrome had visually significant bilateral cataracts detected on their first full ophthalmic examination. All underwent bilateral cataract surgery. The mean age (of 14 eyes) at cataract extraction was 1.25 (median, 1.1) months. Glaucoma diagnosis and treatment were more variable. The mean age (of 11 eyes) at glaucoma diagnosis was 24.1 (range, 0.2-70.0) months: the mean age was 0.2 month (of 4 eyes) when glaucoma was diagnosed before cataract extraction, and the mean age was 37.7 months (of 7 eyes) when glaucoma was diagnosed after cataract extraction. All eyes were followed up for a mean of 100.8 (range, 38-190) months. Main Outcome Measure Treatment of 7 patients (14 eyes) with bilateral cataracts associated with oculocerebrorenal syndrome. CONCLUSIONS: Early identification and surgical removal of cataracts is recommended in patients with oculocerebrorenal syndrome. Despite this, visual acuity results will only rarely be better than 20/70, and nystagmus is likely. Patients should be monitored closely and regularly for changes in intraocular pressure, optic nerve cupping, and refractive error to rule out the development of glaucoma.

The management of strabismus in adults--I. Clinical characteristics and treatment.

INTRODUCTION: This is the first in a series of articles intended to evaluate the management of strabismus in adults, including clinical outcomes and the quality, cost, and value of treatment from the perspectives of patients and health care providers. Here we present clinical characteristics, complexity of surgery, treatment success, and resolved complaints in a group of adult patients who underwent strabismus surgery. METHODS: This is a multicenter retrospective study analyzing the type and amount of ocular misalignment before and after surgery in adult patients with strabismus onset before (BVM, or age < 9 years) or after (AVM, or age >/= 9 years) visual maturation. Success was evaluated in terms of alignment, motility, and the presence of diplopia; subjective success was measured in terms of resolved complaints. The complexity of surgery was determined using the Intensity/Complexity Index and compared with success rates. RESULTS: Data are reported on 299 patients (90 BVM and 145 AVM) whose eyes were successfully aligned in 63% of the BVM cases and 81% of the AVM cases. Subjective complaints resolved at similar rates in the BVM and AVM subgroups. Successful alignment was not correlated with complexity of surgery, but motility and sensory success rates were correlated with complexity of surgery. CONCLUSION: Within each of the BVM and AVM subgroups, this study of adult strabismus showed similar surgical success rates compared with published data. This qualifies these patient groups as clinically typical of adults undergoing strabismus surgery. Additional studies will expand on health value analyses.

Retinopathy of prematurity in infants with birth weights greater than 1250 grams.

PURPOSE: To determine whether severe retinopathy of prematurity (ROP) occurs in infants whose birth weights exceed 1250 g. METHODS: We retrospectively reviewed the medical records of 1118 premature infants with birth weights > 1250 g, who were referred for screening examinations for ROP, to determine the severity of ROP observed. We then cross-tabulated these data with each infant's estimated gestational age and birth weight. Finally, we applied currently recommended screening guidelines retrospectively to determine whether they would have identified infants who had developed severe disease and who were subsequently treated. RESULTS: Stage 3 ROP was detected in infants with estimated gestational ages up to and including 32 weeks and birth weights up to and including 1874 g. A total of 26 of the participants received laser treatment for threshold ROP. Seven of these infants would not have been referred for screening according to current guidelines. CONCLUSIONS: Current screenings guidelines may fail to detect severe ROP in larger, more mature infants.

Evidence-based screening criteria for retinopathy of prematurity: natural history data from the CRYO-ROP and LIGHT-ROP studies.

BACKGROUND: The Multicenter Trial of Cryotherapy for Retinopathy of Prematurity (CRYO-ROP) demonstrated the efficacy of treatment for threshold ROP and indicated the need for worldwide ROP screening. Previous guidelines for ROP screening have been largely based on clinical impression; we can now develop evidence-based screening recommendations. OBJECTIVE: To define the appropriate ages and retinal ophthalmoscopic signs that determine when to commence and conclude acute phase ROP screening. DESIGN: Analysis of data from 2 prospective randomized controlled trials: CRYO-ROP (January 1, 1986, to November 30, 1987) and Light Reduction in ROP (LIGHT-ROP) (July 1, 1995, to March 31, 1997). SETTING: Neonatal intensive care units in 23 study centers in the United States for CRYO-ROP and 3 centers for LIGHT-ROP. PATIENTS: Eyes were examined sequentially in 4099 infants with birth weight less than 1251 g (CRYO-ROP study) and in 361 infants with birth weight less than 1251 g and gestational age less than 31 weeks (LIGHT-ROP study). RESULTS: In 99% of infants, retinal conditions indicating a risk of poor outcome were not observed before 31 weeks' postmenstrual age or 4 weeks' chronologic age. Signs indicating that the risk of visual loss from ROP was minimal or had passed were the infant's attainment of 45 weeks' postmenstrual age without the development of prethreshold ROP or worse, progression of retinal vascularization into zone III without previous zone II ROP, and full vascularization. CONCLUSIONS: The initial eye examination should be conducted by 31 weeks' postmenstrual age or 4 weeks' chronologic age, whichever is later. Acute phase ROP screening can be discontinued when any of the 3 signs is present, indicating that the risk of visual loss from ROP is minimal or passed.