Prevalence and clinical characteristics of age-related distance esotropia.

Age-related distance esotropia (ARDET) is characterized by an esodeviation greater at distance than near in older aged patients and generally managed with prism spectacles or surgery. The purpose of this study is to describe the prevalence, clinical characteristics, and natural history of age-related distance esotropia in a defined population. The medical records of all adult (≥19 years of age) residents of Olmsted County, Minnesota, diagnosed with an esodeviation at least 2 prism diopters (PD) greater at distance than near, from 1 January 1985, through 31 December 2004, were retrospectively reviewed. Seventy-three (9.7%) of 751 new cases of adult-onset strabismus were diagnosed with age-related distance esotropia during the 20-year period. The mean age of onset was 70 years (range, 19 to 93 years) and 48 (65.8%) were female (p = .007). The mean angle of esodeviation was 7.6 (range, 2 to 20) prism diopters (PD) at distance and 0.4 (range, 10 PD of XT to 12 PD of ET) PD at near. The Kaplan-Meier rate of progression, as defined by a ≥ 6 prism diopter (PD) increase in esotropia, was 50% by 15 years after diagnosis. Half of the patients had hypertension, while one-third had coronary artery disease or other cardiac comorbidities. Age-related distance esotropia comprised 1 in 10 adults with new-onset strabismus in this population and was significantly more common among women. Hypertension and cardiovascular disease may be risk factors for this form of strabismus, and approximately half of the patients worsened over a 15-year period.

Congenital ocular motor apraxia with wheel-rolling ocular torsion-a neurodiagnostic phenotype of Joubert syndrome.

Joubert syndrome is a multisystem disorder that is associated with a constellation of cyclic ocular motor disturbances. We describe 2 children with congenital ocular motor apraxia who displayed wheel-rolling torsional eye movements and tonic alternating cyclodeviations of the eyes on retinal examination as a neurodiagnostic phenotype of Joubert syndrome.

Successful Müller muscle-conjunctiva resection in patients with Boston keratoprosthesis type I.

PURPOSE: To present a case series of patients with Boston keratoprosthesis type I (KPro) who have had successful ptosis repair with Müller muscle-conjunctiva resection (MMCR). METHODS: Patients with KPros managed with MMCR for ptosis were identified. Records were examined to obtain preoperative and postoperative visual acuity, KPro viability, and eyelid measurements including margin-to-reflex distance 1 (MRD1), levator function, and lagophthalmos. RESULTS: Seven eyes of 6 patients with KPros and ptosis treated with MMCR were identified. Four eyes underwent a lower lid tarsal strip procedure concurrently with the MMCR to minimize the risk of exposure to the graft postoperatively. The average time between KPro surgery and ptosis surgery was 29.1 months (range, 4-79 months). Preoperatively, the average MRD1 was -2.4 mm and the average levator function was 11.3 mm. Postoperatively, all eyelids were elevated and the average MRD1 was 0.9 mm. One eye had 0.5 mm of lagophthalmos noted whereas all other eyes had no lagophthalmos. The average follow-up time was 25.9 months (range, 13-41 months). All 7 eyes maintained a viable KPro without any compromise to the conjunctiva or donor corneal tissue. CONCLUSIONS: This retrospective case series describes 7 eyes with KPros that underwent successful MMCR without compromise to the integrity of the KPro. Because these patients are at high risk for potential complications, we recommend careful patient selection, a goal of undercorrection, and consideration of supplemental procedures to the lower eyelids to minimize complications to the KPro.

"Ocular moyamoya" syndrome in a patient with features of microcephalic osteodysplastic primordial dwarfism type II.

Primordial dwarfism refers to severely impaired growth beginning early in fetal life. There are many genetic causes of primordial dwarfism, including disorders classified as microcephalic osteodysplastic primordial dwarfism. Microcephalic osteodysplastic primordial dwarfism type II is an autosomal-recessive disease characterized by small stature, bone and dental anomalies, and characteristic facies. Affected patients have a high risk of stroke secondary to progressive cerebral vascular anomalies, which often are classified as moyamoya disease. We present the case of a boy with features suggestive of MOPD II with unilateral moyamoya cerebrovascular changes and correlative moyamoya collaterals involving the iris of the ipsilateral eye.

Utility of large spot binocular indirect laser delivery for peripheral photocoagulation therapy in children.

The purpose of this article is to demonstrate the utility of the large spot size (LSS) setting using a binocular laser indirect delivery system for peripheral ablation in children. One patient with bilateral retinopathy of prematurity received photocoagulation with standard spot size burns placed adjacently to LSS burns. Using a pixel analysis program called Image J on the Retcam picture, the areas of each retinal spot size were determined in units of pixels, giving a standard spot range of 805 to 1294 pixels and LSS range of 1699 to 2311 pixels. Additionally, fluence was calculated using theoretical retinal areas produced by each spot size: the standard spot setting was 462 mJ/mm2 and the LSS setting was 104 mJ/mm2. For eyes with retinopathy of prematurity, our study shows that LSS laser indirect delivery halves the number of spots required for treatment and reduces fluence by almost one-quarter, producing more uniform spots.

Periocular capillary hemangiomas: indications and options for treatment.

Capillary hemangiomas are the most common periocular and orbital tumors of childhood that typically arise in infancy. Though the diagnosis is frequently made on clinical examination, various diagnostic modalities may be helpful in initial evaluation and follow-up. Tests may be necessary in diagnosing suspect cases or aid in the differentiation of potential malignant tumors. In the vast majority of cases these tumors undergo spontaneous involution without sequelae. However, some periocular and orbital capillary hemangiomas require intervention to prevent serious complications. Other tumors require treatment to lessen the surgical burden for cosmetic repair. When treatment is necessary, there are a number of therapeutic options available. As there is no standard, potential risks and benefits must be discussed with the family and treatment should be specific in each case. A complete understanding of the natural history of the tumor, indications for treatment, and response to different therapies is imperative in managing this common lesion.