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Query: UMLS:C0029089 (ophthalmoplegia)
 3,338 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A case of mitochondrial encephalomyopathy (Kearns-Sayre syndrome) with corneal endothelial abnormality is reported. A 22-year-old woman had retinitis pigmentosa, external ophthalmoplegia, complete heart block, ataxia, muscle weakness, dementia, sensorineural hearing loss, and was of short stature. Renal dysfunction, diabetes mellitus, and amenorrhea were also observed. Biopsy revealed decreased cytochrome c oxidase (complex IV) activity in muscle mitochondria. The corneal endothelium examined by specular microscope showed decreased cell density, severe polymegathism, and pleomorphism in both eyes. To our knowledge, this is the first report concerning primary corneal endothelial abnormality in a case with mitochondrial encephalomyopathy. The corneal endothelium is one of the tissues that could be affected by the enzyme deficiency present in this disease.
Cornea 1989 Sep
PMID:Corneal endothelium in a case of mitochondrial encephalomyopathy (Kearns-Sayre syndrome). 274 82

Irreversible heterochromia-iridis, internal ophthalmoplegia, and punctate subcapsular lenticular opacities developed in a 9-year-old girl after she received a bee sting in her right cornea. These complications persisted even after an 11-month follow-up period. To the author's knowledge, this presentation is the first of its nature. The pathogenesis of these changes is discussed and the literature is reviewed.
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PMID:Bee sting of the cornea. 672 43

Malignant exophthalmos or the ophthalmic component of Graves' disease is a functionally impairing and cosmetically deforming disorder of orbital tissue, separate from the sympathetic effects of thyrotoxicosis. The deposition of immune complexes of thyroglobulin and its antibodies within orbital muscles results in progressive exophthalmos, ophthalmoplegia, chemosis, and lid fullness. Increasing intraorbital edema and optic nerve tension may produce irreversible retinal ischemia. If unresponsive to medical treatment, patients exhibiting increasing loss of visual acuity are therefore candidates for emergency surgery. Early attempts at surgical correction of the mechanical aspect of Graves' ophthalmopathy were unsuccessful. The current technique of transantral orbital decompression, when done before extreme advancement of the ocular disease, is successful and meets the following objectives: It restores visual loss and prevents further deterioration of visual acuity, protects the cornea, restores extraocular muscle movements, decreases intraorbital pressure, produces a satisfactory cosmetic result, and avoids serious complications. Patients with persistent extraocular muscle imbalance or lid lag may ultimately require oculoplastic surgery.
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PMID:Ophthalmic surgery in malignant exophthalmos. 689 48

Four tarsorrhaphy sutures are placed through the lower eyelid and brow following ptosis surgery in a system designed for patients with external ophthalmoplegia, third nerve paralysis, and myashenia gravis. Three of the sutures connect the lower eyelid to the forehead, and the fourth suture is passed through the lower eyelid and taped to the cheek. The three lid-brow sutures are released during the first two postoperative weeks, one by one, and topical ointment instillations are gradually tapered. The tarsorrhaphy system allows the cornea to adapt gradually to the lagophthalmos that follows ptosis surgery. It also keeps the eyelids partially closed during the first two postoperative weeks in patients with frontalis sling surgery who have marked difficulty lowering their eyebrow and closing their eyelids because of early postoperative forehead edema and pain. The tarsorrhaphy system has prevented serious keratopathy in six patients with ptosis associated with abnormal ocular motility and in one patient with lagophthalmos following trauma. It also allowed the six ptosis patients to have full, rather than partial, correction of their ptosis.
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PMID:Suture tarsorrhaphy system to control keratopathy after ptosis surgery. 699 12

The authors describe the clinical, molecular genetic, and pathologic findings of a patient with corneal decompensation associated with the mitochondrial ophthalmoplegia plus (Kearns-Sayre) syndrome. Ultrastructurally abnormal mitochondria were observed and possibly implicate this organelle in the pathogenesis of corneal edema.
Cornea 1994 May
PMID:Corneal decompensation in mitochondrial ophthalmoplegia plus (Kearns-Sayre) syndrome. A clinicopathologic case report. 803 80

Aarskog syndrome is characterised by a disproportionately short stature and facial, skeletal and urogenital anomalies ('shawl' scrotum and cryptorchidism). Ophthalmic findings include a slight downward slant to the palpebral fissures, hypertelorism, blepharoptosis, strabismus, ophthalmoplegia, hypermetropic astigmatism and a large cornea. Findings on the extremities include joint hyperextensibility, short and broad hands, interdigital webbing, a short fifth finger, clinodactyly and broad feet with bulbous toes. We report on a 7 1/2-year-old boy with typical findings of Aarskog syndrome, hypermetropia and bilateral proximal implantation of the fifth toes. These associated abnormalities have hitherto never been described, to our knowledge.
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PMID:Aarskog syndrome associated with hypermetropia and toe anomaly. 949 38

We describe four members spanning three generations of a Caucasian family affected with distal arthrogryposis (DA). Based on Hall's original classification, we have placed our family under type IIB and present previously unreported ophthalmic features. All the members had different degrees of ophthalmoplegia, ptosis, astigmatism, and strabismus. Other findings in affected family members included keratoconus in the index patient, which was associated with abnormal electron microscopy of the affected cornea and increased thickness of the central cornea, small axial length of the globe and choroidal folds in the others.
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PMID:Distal arthrogryposis type IIB: unreported ophthalmic findings. 1627 94

During pregnancy, hormonal and hemodynamic modifications can generate a number of organic consequences, specifically ocular, more or less regressive in systemic disease. Refraction disorders are marked by myopization, often not very significant and always regressive within six weeks after the childbirth. Pregnancy could cause a thickening of the cornea and a modification of its curvature. Likewise, contact lens intolerance is common, secondary to the modification of lacrimal film, but does not systematically contraindicate lens wearing. Photorefractive surgeries are ill-advised during pregnancy. Hemeralopia is the most common visual complaint of the pregnant woman. Oculomotor disorders are exceptional. Convergence insufficiency or accommodation disorders are the most common anomalies described. These disorders usually regress in the postpartum period. Oculomotor palsies could be the first symptoms of pre-eclampsia or an associated intracranial pathology. They are specific palsies such as in the abducens nerve and the oculomotor nerve in very unusual cases. Nevertheless, oculomotor paralysis in the pregnant woman should motivate neuroradiological exploration. Intensive labor can be rhegmatogenous in women with severe myopia. Examination of the retinal periphery is systematic before and during pregnancy in these cases. Systematic cesarean section is not formally indicated. It is nevertheless preferable to facilitate delivery with epidural anesthesia.
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PMID:[Understanding refraction disorders and oculomotor problems during pregnancy]. 2045 Dec 89

Ophthalmologic manifestations of mitochondrial disorders are frequently neglected or overlooked because they are often not regarded as part of the phenotype. This review aims at summarizing and discussing the etiology, pathogenesis, diagnosis, and treatment of ophthalmologic manifestations of mitochondrial disorders. Review of publications about ophthalmologic involvement in mitochondrial disorders by search of Medline applying appropriate search terms. The eye is frequently affected by syndromic as well as nonsyndromic mitochondrial disorders. Primary and secondary ophthalmologic manifestations can be differentiated. The most frequent ophthalmologic manifestations of mitochondrial disorders include ptosis, progressive external ophthalmoplegia, optic atrophy, retinopathy, and cataract. More rarely occurring are nystagmus and abnormalities of the cornea, ciliary body, intraocular pressure, the choroidea, or the brain secondarily affecting the eyes. It is important to recognize and diagnose ophthalmologic manifestations of mitochondrial disorders as early as possible because most are accessible to symptomatic treatment with partial or complete short-term or long-term beneficial effect. Ophthalmologic manifestations of mitochondrial disorders need to be appropriately diagnosed to initiate the most effective management and guarantee optimal outcome.
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PMID:The Eye on Mitochondrial Disorders. 2627 73

This article evaluates the "Bobby Pin" procedure in the correction of myogenic ptosis accompanying extraocular muscle weakness. We retrospectively reviewed 26 eyelids of 13 patients who underwent "Bobby Pin" procedure for myogenic ptosis accompanying extraocular muscle weakness. We evaluated the patients' clinical features such as age, etiology of ptosis, symptoms, standard ptosis measurements, associated systemic diseases, additional ophthalmic conditions, complications, and recurrence. Etiology of myogenic ptosis and extraocular muscle weakness was oculopharyngeal dystrophy in 4 (31%) patients, chronic progressive external ophthalmoplegia in 4 (31%) patients, myotonic dystrophy in 2 (23%) patients, and idiopathic in 3 (15%) patients. The mean levator function was approximately 5 mm pre- and post-operatively (range 1 to 12 mm). The mean margin-to-reflex distance 1 increased from -1.1 mm (below the light reflex) pre-operatively to +0.4 mm (above the light reflex) post-operatively. After a mean follow-up of 40 months, only 1 (8%) patient experienced ptosis recurrence. Upper eyelids were symmetric in both contour and height in all patients. Mild superficial keratopathy involving less than 10% of cornea was observed in 4 (31%) patients. The "Bobby Pin" procedure is an effective and long-lasting treatment option for correcting acquired ptosis accompanying extraocular muscle weakness. The procedure is safe, simple, easily learned, time- and cost-effective, and does not require any expensive equipment.
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PMID:Correcting myogenic ptosis accompanying extraocular muscle weakness: The "Bobby Pin" procedure. 2754 41


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