Characteristics of Okihiro syndrome.

PURPOSE: To describe Okihiro syndrome, a syndrome consisting of Duane's syndrome, radial ray anomaly, and sensorineural deafness. METHODS: The incidence of anomalies in 41 individuals from 9 affected families was assessed by literature review and patient examination, and the frequency of the characteristics occurring together was determined. RESULTS: Of all the individuals with at least one of the above characteristics, only 12% had all three characteristics. However, 44% had both Duane's syndrome and radial ray anomaly, whereas sensorineural deafness alone occurred in only 17% of patients. CONCLUSION: Okihiro syndrome is consistent with autosomal dominance with variable penetrance, manifesting primarily with Duane's syndrome and radial ray anomaly, and less frequently, sensorineural deafness.

Chorioretinitis sclopetaria from BB ex memoria.

Chorioretinitis sclopetaria presents a characteristic pattern of choroidal and retinal changes caused by a high velocity projectile passing into the orbit, in close proximity to the globe. While it is unlikely that a patient should completely forget the trauma causing such damage, preserved or compensated visual function may blur the patient's memory of these events over time. Characteristic physical findings help to clarify the antecedent history. Despite the lack of an acknowledged history of ocular trauma or surgery, in our case, the characteristic ocular findings discovered at presentation allowed for recognition of the underlying etiology. Because of good visual function, the patient had completely forgotten about the trauma that occurred 12 years earlier. Strabismus surgery was performed for treatment of the presenting symptomatic diplopia. The pathognomonic findings in chorioretinitis sclopetaria are invaluable in correctly diagnosing this condition, especially when a history of ocular trauma is unavailable.

Monocanalicular intubation for dacryostenosis in oculo-auriculo-vertebral dysplasia (hemifacial microsomia) with congenital corneal anesthesia.

PURPOSE: To present a case of oculo-auriculo-vertebral dysplasia associated with corneal anesthesia and ipsilateral dacryostenosis that was successfully treated with monocanalicular lacrimal intubation. METHODS: Case report. RESULTS: Previous neurotrophic corneal ulcers in a child with hemifacial microsomia had become secondarily infected from a stagnant tear lake, resulting in significant corneal scarring and visual loss. A single monocanalicular stent maintained nasolacrimal patency without causing further corneal trauma, despite constant medial gaze because of wide lateral tarsorrhaphy and contralateral occlusion therapy for amblyopia. CONCLUSIONS: Monocanalicular stents may aid the treatment of dacryostenosis in the face of compromised corneal sensation.

Eye injuries from merchandise display hooks.

PURPOSE/METHODS: Four patients had ocular or ocular adnexal injuries, which resulted from store merchandise display hooks. RESULTS/CONCLUSIONS: Ocular and periocular injuries from display hooks include conjunctival, eyelid, and canalicular lacerations, as well as penetrating brain injury. Display hooks are commonly used in retail establishments and pose a high risk when placed below eye level.

Transection of the superior rectus muscle during intended superior oblique tenotomy: a report of three cases.

Reported complications of superior oblique surgery include postoperative vertical or torsional deviation, Brown's syndrome, head tilt, blepharoptosis, and conversion of an A-pattern to a V-pattern. McNeer reported three cases of postoperative vertical deviation, one of which was attributed to severing of the superior rectus tendon. We report three additional cases of inadvertent unrecognized transection of the superior rectus during intended superior oblique tenotomy to emphasize the importance of direct visualization of these tendons during surgery.

Prevalence of primary monofixation syndrome in parents of children with congenital esotropia.

The prevalence of primary monofixation syndrome (MFS) in the general population is approximately 1%. This study was performed to determine the prevalence of primary monofixation in biological parents of children with congenital esotropia. Ninety children with congenital esotropia were seen between November 1991 and June 1992 by one ophthalmologist (M.M.P.). One hundred and twenty-nine biological parents of these children were screened for sensorimotor abnormalities. Twelve parents were found to have secondary MFS and were removed from the analysis. This left 78 apparently non-strabismic families consisting of a total of 117 parents. Seven parents were identified as having primary MFS. The prevalence of primary MFS in this population is 9% of families and 6% of parents. Congenital esotropia is believed to be inherited in a multifactorial fashion. We believe that this increase in the prevalence of primary MFS compared to the general population lends support to the hypothesis that primary MFS may be a mild (subthreshold) effect of the "gene(s)" that cause congenital esotropia.

Bread crumb-flecked retinopathy.

The case of a young girl with bilateral retinal flecks, normal fluorescein angiography results, and normal electrophysiologic findings is reported. The fundus appeared to consist of multiple, scattered, pale, bread crumb-like lesions that seemed to lie deep to the retinal vessels and anterior to the retinal pigment epithelium. The lesions were located outside the posterior pole in the midperiphery, and spared the macula and perimacular areas. The disorder may be associated with anomalies of the central nervous system and extraocular motility. No reports of similarly described fundus lesions have been found in the literature.

Magnetic resonance imaging evaluation of uveal tumors.

We detected seven tumors with magnetic resonance imaging, which demonstrate some of the uses and limitations of this diagnostic technique. In one patient, the correct diagnosis of extraocular extension was demonstrated with magnetic resonance imaging when both clinical and ultrasonographic data were not diagnostic. In another case, an extremely small area of extraocular extension was detected with ultrasound evaluation but was not noted on magnetic resonance imaging. Magnetic resonance imaging is highly reliable in the detection of intraocular tumors. In pigmented uveal melanomas it is both sensitive and highly specific. In amelanotic processes magnetic resonance imaging can identify a mass but cannot always adequately characterize it. Further experience will be necessary to determine the proper role of magnetic resonance imaging in the evaluation of uveal tumors.