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Query: UMLS:C0038379 (strabismus)
 9,317 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The orbit in Graves disease undergoes expansion in soft tissue content as a result of the infiltration of orbital fat, extraocular muscles, and the lacrimal gland. Compression of the intraorbital contents leads to disorders of the lid-corneal interface, keratopathy, motility disturbances, exophthalmos, and optic neuropathy. Orbital decompression has traditionally been reserved for those patients with unremitting optic neuropathy. This article provides a historical review of orbital decompression, as well as a review of the evolution of surgical approaches toward both soft tissue and bony decompression. Recent trends in surgical management include fat decompression, more extensive posterior sculpting of the lateral wall, and direct approaches to the medial wall. Preoperative predictors of diplopia and hypoglobus are addressed, as are the various techniques that are employed to limit new postoperative strabismus. The roles of endoscopy and combined surgical techniques are also reviewed. Expanded indications for decompression and its effect on increased intraocular pressure are discussed as well.
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PMID:Orbital decompression: current concepts. 1221 62

Thyroid Orbitopathy (T.O) is the most common orbital disease caused by a systemic illness. The clinical manifestations of T.O are a result of inflammation, edema and scarring of orbital tissue, and include exophthalmos, soft tissue edema, lid retraction, chemosis, exposure keratitis that can lead to corneal ulceration and perforation, ocular motility disorders and strabismus, and compressive optic neuropathy. T.O is an autoimmune disease but the exact etiology is yet to be revealed. Most cases resolve spontaneously without the need for medical or surgical intervention, but more severe cases may result in devastating visual and cosmetic results. In recent years, there has been a significant progress in the understanding of the disease and the medical and surgical treatment of T.O. This work reviews new aspects of T.O and it's surgical treatment, and include clinical photographs of patients operated on in our department.
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PMID:[Thyroid orbitopathy]. 1280 64

Many phenotypic manifestations have been reported in cardiofaciocutaneous (CFC) syndrome, but none, to date, are pathognomonic or obligatory. Previous histopathological studies reported findings in skin and hair; no autopsy studies have been published. We report the clinical and autopsy findings of a 7-year-old boy with severe CFC syndrome and malnutrition of psychosocial origin. Manifestations of CFC, reported previously, included macrocephaly and macrosomia at birth; short stature; hypotonia; global developmental delays; dry, sparse thin curly hair; sparse eyebrows and eyelashes; dilated cerebral ventricles; high cranial vault; bitemporal constriction; supraorbital ridge hypoplasia; hypertelorism; ptosis; exophthalmos; depressed nasal bridge; anteverted nostrils; low-set, posteriorly-rotated, large, thick ears; decayed, dysplastic teeth; strabismus; hyperelastic skin; wrinkled palms; keratosis pilaris atrophicans faciei; ulerythema ophryogenes; hyperkeratosis; gastroesophageal reflux; and tracheobronchomalacia. Additional findings, not previously reported, include islet cell hyperplasia, lymphoid depletion, thymic atrophy and congenital hypertrophy of peripheral nerves with onion bulb formations. Although the islet cell hyperplasia, lymphoid depletion, and thymic atrophy are nonspecific findings that may be associated with either CFC or malnutrition, the onion bulb hypertrophy is specific for a demyelinating-remyelinating neuropathy. These findings implicate congenital peripheral neuropathy in the pathogenesis of the developmental delays, feeding difficulties, respiratory difficulties, ptosis and short stature in this case. Additional studies of other cases of CFC are needed.
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PMID:Cardiofaciocutaneous syndrome (CFC) with congenital peripheral neuropathy and nonorganic malnutrition: an autopsy study. 1600 34

Isolated abducens nerve palsy is a rare complication of treatment with various drugs. Here, the authors report the case of a 23-year-old female with isolated left abducens nerve palsy after long-term retinoic acid therapy. The association is based on the temporal relationship and the exclusion of other possible etiologic factors following extensive laboratory and imaging diagnostics. The authors suggest that isolated abducens nerve palsy may be a presenting sign of a toxic neuropathy associated with retinoic acid therapy. After the exclusion of other organic lesions, especially idiopathic intracranial hypertension, and an assessment of the risk-benefit ratio, discontinuation of treatment must be considered in such cases.
Strabismus 2005 Sep
PMID:Isolated abducens nerve palsy associated with retinoic acid therapy: a case report. 1625 Nov 42

The authors present the case of a child, 8 years old, victim of a car accident, suffering a cranio-cerebral trauma. The posttraumatic injuries were: bitemporal cranial fractures, LE VI nerve palsy with secondary convergent strabismus, left posttraumatic optic neuropathy with poor vision, right deafness, nasal bone fracture, left paramedian mandibular fracture. Four months later the child developed proptosis due to a high-flow carotid-cavernous fistula, on the site of the good eye, RE, which was clinically diagnosed and confirmed by the results of MRI, MRA and cerebral angiography. Considering the ophthalmologic and neurologic risks of this pathologic condition, the surgery was performed, using an external device (Silverstone clamp) with the purpose of progressive and adjustable control of the fistula thrombosis. The postoperative result was fistula thrombosis, preserving the visual acuity and visual field, with a residual hypertonia on the right eye. The peculiarity of the case consist in the small age of the patient and the severity of neuro-ophthalmologic lesions. The child will live for the rest of his life with a single well-seeing eye, whose function could be at risk at any moment, related to the posttraumatic vascular changes at the level of the right cavernous sinus.
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PMID:[Neuro-ophthalmologic multiple lesions in craniocerebral trauma]. 1677 44

Since its introduction into clinical medicine in 1980, botulinum toxin has become a major therapeutic drug with applications valuable to many medical sub-specialties. Its use was spearheaded in ophthalmology where its potential applications have expanded to cover a broad range of visually related disorders. These include dystonic movement disorders, strabismus, nystagmus, headache syndromes such as migraine, lacrimal hypersecretion syndromes, eyelid retraction, spastic entropion, compressive optic neuropathy, and, more recently, periorbital aesthetic uses. Botulinum toxin is a potent neurotoxin that blocks the release of acetylcholine at the neuromuscular junction of cholinergic nerves. When used appropriately it will weaken the force of muscular contraction, or inhibit glandular secretion. Recovery occurs over 3 to 4 months from nerve terminal sprouting and regeneration of inactivated proteins necessary for degranualtion of acetylcholine vesicles. Complications are related to chemodenervation of adjacent muscle groups, injection technique, and immunological mechanisms.
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PMID:Botulinum toxin in ophthalmology. 1721 88

Optic neuropathy affects a small proportion of patients with Graves' eye disease. It is due to optic nerve compression by enlarged extraocular muscles and can be treated by corticosteroids, irradiation or surgical orbital decompression. The current report evaluates the effectiveness of extracranial orbital decompression performed by one surgeon for optic neuropathy in Graves' eye disease. The records of 21 patients (33 orbits) undergoing extracranial orbital decompression for Graves' optic neuropathy were analysed for changes in visual acuity and colour vision and reduction in proptosis. Visual acuity and colour vision improved in all 33 eyes in the short-term postoperatively (4 weeks), but later deteriorated in 5 eyes (6.6%) of 4 patients (19%). The remainder maintained improved vision for the duration of the follow-up period (mean 22, range 3-54 months). Proptosis decreased by a mean 5.0 mm (range 1-8). Extraocular muscle imbalance and diplopia worsened in 9 21 patients (43%) and improved in 2 21 (9.5%). The patients having medial wall and floor decompressed all had worse diplopia (5 5 ), those with medial and lateral wall worsened in 4 10 cases and improved in 1 10 , and those having all three walls decompressed had no cases of worsening diplopia and 1 6 improved. All patients with symptomatic diplopia achieved binocular single vision in a useful range after one and sometimes two squint procedures. No patient lost vision as a result of the extracranial orbital decompression, but one lost vision in one eye after transfrontal decompression following failed extracranial decompression. Extracranial orbital decompression is effective in improving vision and reducing proptosis in most patients with optic neuropathy in Graves' eye disease, but induces or worsens diplopia in a high proportion of patients. The diplopia is readily correctable, and the change to decompressions that include the lateral wall may reduce the risk of postoperative diplopia.
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PMID:Extracranial orbital decompression for optic neuropathy in Graves' eye disease. 1863 10

Congenital iris ectropion is an uncommon malformation and no reports exist about the use of modern technologies in this pathology. The authors describe a case of unilateral and isolated congenital iris ectropion associated with juvenile glaucoma in a healthy and completely asymptomatic 6-year-old girl with an unusual form of anisocoria. Optical coherence tomography and confocal scanning laser tomography showed a progressive glaucomatous neuropathy. A trabeculectomy without antimetabolites was performed and intraocular pressure normalized without other medications during a follow-up of 2 years. The authors assert the utility of various diagnostic technologies to recognize congenital iris ectropion early to prevent blindness in young patients and improve their prognosis.
J Pediatr Ophthalmol Strabismus
PMID:Congenital iris ectropion associated with juvenile glaucoma. 1921 75

Ataxia with oculomotor apraxia type 2 (AOA2) is an autosomal recessive disease due to mutations in the senataxin gene, causing progressive cerebellar ataxia with peripheral neuropathy, cerebellar atrophy, occasional oculomotor apraxia and elevated alpha-feto-protein (AFP) serum level. We compiled a series of 67 previously reported and 58 novel ataxic patients who underwent senataxin gene sequencing because of suspected AOA2. An AOA2 diagnosis was established for 90 patients, originating from 15 countries worldwide, and 25 new senataxin gene mutations were found. In patients with AOA2, median AFP serum level was 31.0 microg/l at diagnosis, which was higher than the median AFP level of AOA2 negative patients: 13.8 microg/l, P = 0.0004; itself higher than the normal level (3.4 microg/l, range from 0.5 to 17.2 microg/l) because elevated AFP was one of the possible selection criteria. Polyneuropathy was found in 97.5% of AOA2 patients, cerebellar atrophy in 96%, occasional oculomotor apraxia in 51%, pyramidal signs in 20.5%, head tremor in 14%, dystonia in 13.5%, strabismus in 12.3% and chorea in 9.5%. No patient was lacking both peripheral neuropathy and cerebellar atrophy. The age at onset and presence of occasional oculomotor apraxia were negatively correlated to the progression rate of the disease (P = 0.03 and P = 0.009, respectively), whereas strabismus was positively correlated to the progression rate (P = 0.03). An increased AFP level as well as cerebellar atrophy seem to be stable in the course of the disease and to occur mostly at or before the onset of the disease. One of the two patients with a normal AFP level at diagnosis had high AFP levels 4 years later, while the other had borderline levels. The probability of missing AOA2 diagnosis, in case of sequencing senataxin gene only in non-Friedreich ataxia non-ataxia-telangiectasia ataxic patients with AFP level > or =7 microg/l, is 0.23% and the probability for a non-Friedreich ataxia non-ataxia-telangiectasia ataxic patient to be affected with AOA2 with AFP levels > or =7 microg/l is 46%. Therefore, selection of patients with an AFP level above 7 microg/l for senataxin gene sequencing is a good strategy for AOA2 diagnosis. Pyramidal signs and dystonia were more frequent and disease was less severe with missense mutations in the helicase domain of senataxin gene than with missense mutations out of helicase domain and deletion and nonsense mutations (P = 0.001, P = 0.008 and P = 0.01, respectively). The lack of pyramidal signs in most patients may be explained by masking due to severe motor neuropathy.
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PMID:Ataxia with oculomotor apraxia type 2: clinical, biological and genotype/phenotype correlation study of a cohort of 90 patients. 1969 32

Dysthyroid orbitopathy is a disease that is edematous and inflammatory, generally chronic, sometimes subacute or acute, and is characterized by exophthalmos in the majority of cases, retraction of the lid, restrictive strabismus with diplopia, corneal ulceration, ocular hypertension, and compressive optic neuropathy. Proptosis is the cardinal sign. Exophthalmos is frequently axial and is bilateral in 85%-90% of cases. Displacement of the globe in the vertical and horizontal axes is not rare, particularly when intense enlargement of an extraocular muscle produces a mass effect. Proptosis stems from a conflict for the space in the orbital cavity. The infiltration of the fat, muscles, and lacrimal gland by lymphocytes, plasma cells, and mucopolysaccharides, which are very hydrophilic, all contribute to the orbitopathy. The majority of patients with minimal exophthalmos do not require special treatment since they tend to improve spontaneously. In severe forms, it is important to evaluate the activity for steroid use to eliminate the inflammation in the soft tissues and to make rehabilitative surgery possible under better conditions. When a good response is not obtained, radiotherapy is evaluated. The surgical treatment by bone orbital decompression and sometimes lipectomy is indicated in inactive forms, disfiguring exophthalmos, certain particular cases such as dysthyroid optic neuropathy, ocular hypertension, corneal exposition.
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PMID:[Basedow exophthalmos]. 1985 65


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