Cases reported "Amblyopia"

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1/11. Refractive laser surgery in children with coexisting medical and ocular pathology.

    PURPOSE: To report the visual, refractive, and functional outcomes of photorefractive keratectomy (PRK) and of laser-assisted subepithelial keratectomy in a group of children with significant refractive error and underlying medical conditions or ocular pathology who were noncompliant with traditional management. SETTING: Nonhospital surgical facility and a hospital clinic. methods: This case series comprised 5 individual cases of anisometropic amblyopia and/or high myopia. Underlying medical and ocular conditions were as follows: upper eyelid hemangioma with oblique myopic astigmatism, Pelizaeus-Merzbacher leukodystrophy with nystagmus, klippel-trenaunay-weber syndrome with glaucoma, incontinentia pigmenti with unilateral optic nerve atrophy, and goldenhar syndrome with unilateral optic nerve hypoplasia. photorefractive keratectomy or LASEK was performed in 6 eyes of 5 patients. Age range at the time of surgery was 1.0 to 7.0 years. All procedures were performed under general anesthesia. RESULTS: Best corrected visual acuity improved by 2 lines in 2 patients and 1 line in 2 patients by 6 months after surgery. Stereopsis and/or fusional status improved in 3 patients. amblyopia treatment compliance improved in 1 patient. Alignment improved without strabismus surgery in 2 cases. A functional vision survey demonstrated a positive effect on the ability of all 5 children to function in their environment. CONCLUSION: During the period of visual cortical plasticity, refractive surgery, by eliminating the refractive component of amblyopia and by promoting fusional ability, provides considerable improvement in children, even those with underlying medical conditions associated with ocular pathology.
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ranking = 1
keywords = nystagmus
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2/11. Ocular and systemic findings in the Aarskog (facial-digital-genital) syndrome.

    The Aarskog (facial-digital-genital) syndrome is an X-linked disorder in which short stature is accompanied by hypertelorism, digital anomalies, and shawl scrotum. Except for hypertelorism and blepharoptosis, ophthalmic abnormalities have been rarely noted in this condition. We examined four patients who had Aarskog syndrome and unilaterally or bilaterally decreased vision on initial examination. Three family members had V-pattern esotropia, latent nystagmus, inferior oblique overaction, and amblyopia. A fourth patient had bilateral blepharoptosis and severe astigmatism. Other ocular features included hyperopia, anisometropia, deficient ocular elevation, blue sclerae, and posterior embryotoxon. These findings underscore the need for ophthalmic examination in asymptomatic patients with Aarskog syndrome to rule out treatable causes of visual loss.
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keywords = nystagmus
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3/11. The effect of corneal grafting on vision in bilateral amblyopia.

    Two children with bilateral congenital corneal opacities (sclerocornea) received unilateral corneal transplants at the ages of 4 1/2 and 16 years, respectively. Both developed reading vision and reduced nystagmus excursions.
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4/11. The ETHAN syndrome.

    Two mechanisms by which patients compensate for congenital nystagmus are assumption of a head position that places the eyes in a "null zone" of least nystagmus, known as nystagmus compensation syndrome (NCS), and convergence to dampen nystagmus, known as nystagmus blockage syndrome (NBS). We followed five patients in two groups who combine these mechanisms. The first group presented with spontaneous alternation between nystagmus and a head turn with orthotropia (NCS), and a large-angle esotropia with a variable head position that persisted even when patched (NBS). The second group of patients presented with a constant large-angle esotropia that lessened their nystagmus and variable head posture (NBS). Following recession of both medial rectus muscles with or without placement of posterior fixation sutures, they developed an abnormal head posture with orthotropia (NCS). The presence of mild amblyopia may contribute to the alternation between the two compensatory mechanisms.
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ranking = 7
keywords = nystagmus
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5/11. Congenital nystagmus surgery. A quantitative evaluation of the effects.

    Nystagmus intensities at various gaze angles were studied both preoperatively and postoperatively, using accurate ocular motility recordings, in three cases of congenital nystagmus. In addition to shifting the nystagmus null, the surgery broadened the null region and resulted in an overall reduction in nystagmus intensity at all gaze angles. Surgical rotation also resulted in improved visual acuity in all cases. The postoperative acuity at 0 degrees was better than the preoperative acuity at both 0 degrees and the patient's preferred gaze angle (ie, the preoperative null angle). This was not only for the two patients who showed an improved preoperative acuity with their head turn but also for the patient whose preoperative acuity did not substantially improve with her preferred head turn. Eye movement recordings have made it possible to accurately determine the amount of surgery required and to predict acuity increases even when undetectable during the preoperative clinical examination.
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ranking = 7
keywords = nystagmus
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6/11. The effects of congenital nystagmus surgery.

    Anderson-Kestenbaum operations for congenital nystagmus with severe head turn have been employed for the past 25 years. They have been documented as being effective in correcting head turns associated with congenital nystagmus. We have studied a group of patients with congenital nystagmus and head turn, both preoperatively and postoperatively, by means of recordings of eye movements. We have documented the effect of surgery in shifting the null position, in broadening the null position, and in lowering the nystagmus intensity. In some cases, an improvement of the visual acuity has also occurred.
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ranking = 8
keywords = nystagmus
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7/11. Bilateral deprivation amblyopia.

    Two children had toxic bilateral cataracts at the ages of 36 months and 41 months and were deprived of treatment for the next 15 and eight years, respectively. After successful surgery the visual results were about 20/40 in both eyes. This visual recovery suggests that the susceptibility to bilateral stimulus deprivation at this age is slight but not absent, and furthermore that the longtime deprivation beyond the sensitive period does not effect greatly the ultimate outcome of the treatment. Absence of nystagmus supports the opinion that the visual motor system is mature by the age of 3 years.
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ranking = 1
keywords = nystagmus
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8/11. Manifest latent nystagmus of late onset: a case report.

    A case of manifest latent nystagmus of late onset in a 13-year-old girl is reported. The nystagmus became manifest during the development of a hypertropia of the left eye. The spontaneous nystagmus was successfully treated by surgical correction of the hypertropia. The observations are discussed with regard to theories on the origin of latent nystagmus.
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ranking = 8
keywords = nystagmus
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9/11. Binocular versus monocular acuity in a patient with latent nystagmus.

    This is a case of latent nystagmus with a significant improvement in acuity with both eyes open rather than with either eye alone. If this patient's visual acuity was tested only O.D. alone and O.S. alone, the best visual acuity obtainable would have been 20/60. This might have had serious implications for A.F.'s work since a minimum visual acuity of 20/40 is needed to keep a driver's license in new york State. Since binocular acuity was 20/20, I assured him that driving is safe.
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ranking = 5
keywords = nystagmus
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10/11. amblyopia in ptosis.

    amblyopia can result from strabismus, anisometropia, media opacities, and congenital disorders such as nystagmus. Complicated forms of ptosis (ie, associated with neurofibroma or hemangioma) are also known to cause amblyopia. A previously unconfirmed cause of amblyopia is uncomplicated ptosis. We carefully examined 123 consecutive surgical ptosis patients with uncomplicated congenital or early acquired ptosis. Twenty-five cases of amblyopia (20%) were seen. Four cases (3.2%) were thought to be caused by the ptosis. In two of these cases (1.6%), the amblyopia was directly attributed to the ptosis. In one of the remaining two cases, exotropia and amblyopia developed on the ptotic side while the patient was being observed for the ptosis. In another patient, a progressively increasing cylindrical refractive error and amblyopia developed, which were attributed to the ptosis. We recommend careful evaluation of cases of congenital ptosis for the detection and treatment of amblyopia.
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ranking = 1
keywords = nystagmus
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