Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038379 (strabismus)
9,317 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A brief account of craniofacial synostosis is presented. The cranium is acrocephalic owing to the early closure of the coronal and lambdoid sutures. The crown is typically rudimentary where the anterior fontanel is still open. Cranial radiography shows digitate markings. Skeletal changes lead to hypertelorism, exophthalmos and strabismus. The nose is of the parrot's beak type and the hypoplasic upper jaw is in contrast with the lower jaw, resulting in pseudo-prognathism. These basic malformations are accompanied by several other deformities. The disease may appear as a hereditary or as a sporadic occurrence. The cases described in the present paper confirm the hereditary interpretation of the syndrome, since both were accompanied by positive familial histories. In the first case, the signs of the disease were less evident, whereas in the second the picture was complete and both mother and daughter presented impressive examples of the frog face sign.
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PMID:[Crouzon's craniofacial dysostosis]. 1734 89

A 3-month-old infant presented with unilateral proptosis. Ophthalmic and physical examinations, computed tomography, and histopathologic examination of a biopsy specimen were performed. Histopathologic examination confirmed fungal orbital cellulitis. The patient responded to 6 weeks of antifungal therapy. Fungal orbital cellulitis should be included in the differential diagnosis for infants with proptosis.
J Pediatr Ophthalmol Strabismus
PMID:Rare presentation of fungal orbital cellulitis in an immunocompetent infant. 1741 Sep 66

A 30-year-old male suffered an orbital trauma due to a traffic accident. At the Emergency Unit, the patient presented with avulsion of the upper left eyelid in the medial canthus, wounds in the lower eyelid and the inferior canaliculus, conjunctival laceration, proptosis and palpebral hematomas. The patient reported persistent diplopia. During the examination, exotropia and total absence of adduction were observed. Computerized tomography (CT) revealed a discontinuity at the left medial rectus. No orbital fractures were identifiable. The medial rectus was still attached to its anatomic insertion at the globe. The discontinuity was suggestive of laceration or rupture of this muscle at approximately 10-12 mm from its insertion. Surgical exploration revealed total rupture of the medial rectus at approximately 12 mm from its insertion. The posterior edge of the damaged muscle was found and sutured to its anterior edge with 6-0 polyglactin. The following day, the eyes were completely straight and the patient did not mention any signs of diplopia. Botulinum toxin injection into the ipsilateral lateral rectus was not necessary. After six months of follow-up, the patient still reported no diplopia. When muscular laceration is suspected after an orbital trauma, early CT is recommended. The only procedures that assure a significant recovery of the normal function of the eye are early muscle repair and avoidance, if possible, of transposition surgery.
Strabismus
PMID:Isolated medial rectus muscle rupture after a traffic accident. 1830 21

The authors describe a rare case of orbital rhabdomyoma in a 3-year-old girl who presented with progressive proptosis of the left eye. An axial computed tomographic scan of the left orbit demonstrated an irregular retrobulbar mass. The tumor was resected locally from the lateral wall of the orbit and the resected specimens were diagnosed as orbital rhabdomyoma. The authors review the literature and discuss the diagnostic implications and treatment strategies.
J Pediatr Ophthalmol Strabismus
PMID:Rhabdomyoma of the orbit. 1840 60

Conservative therapy in cases of severe thyroid ophthalmopathy has not given satisfactory results. Because of this, surgical treatment, that is, orbital decompression, is now being used with increasing frequency. Since the end of 1986 until the end of 2006, 103 patients with severe thyroid ophthalmopathy have been treated by personal 3-wall orbital decompression combined with removal of the periorbital, intraorbital, and retrobulbar fat, as well as with correction of eyelid retraction and deformities. After operation, all patients showed a significant reduction of exophthalmos (5-11 mm; 7.2 mm on average), reduction of intraocular pressure, marked improvement in ocular muscle function, and considerable reduction or disappearance of subjective symptoms. There was an improvement in vision in 68% patients who had impaired vision before the operation. There were no cases of subsequent impairment of ocular motility. Mild relapse of exophthalmos was recorded in 3 cases only, and only 1 patient required unilateral reoperation. Strabismus surgery had to be performed in 6 patients due to unsatisfactory correction of double vision. It can be concluded that this method of orbital decompression gives constantly very good functional and aesthetic long-term results and allows rapid recovery.
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PMID:Correction of exophthalmos and eyelid deformities in patients with severe thyroid ophthalmopathy. 1852 Mar 75

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

The congenital retinocephalic facial vascular malformation syndrome is characterized by unilateral, nonhereditary retinal and cerebral arteriovenous malformations (AVMs) and is occasionally associated with orbital vascular changes. Typical signs are facial and oral mucosal vascular changes, rarely with changes of the maxilla or mandible. An AVM causes high blood flow because of direct connection (shunting) of major vessels without interposition of capillaries. Ocular complications include retinal and vitreous hemorrhages, edema, venous occlusion (risk of rubeosis iridis and secondary glaucoma). Neuroophthalmological changes comprise optic atrophy, papilledema, proptosis, pupillary changes, hemianopia, gaze paresis, nystagmus, cranial nerve palsies, strabismus, and amblyopia. Neurological complications include headache, subarachnoid hemorrhage, convulsions, cerebral hemorrhages, increased intracranial pressure, hydrocephalus, and stroke with hemiparesis. Threatening oral hemorrhages or epistaxis may rarely occur.
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PMID:[Congenital retinocephalic facial vascular malformation syndrome. Bonnet-Dechaume-Blanc syndrome or Wyburn-Mason syndrome]. 1915 63

Intravenous glucocorticoids and orbital radiotherapy are effective treatments for moderate-to-severe and active Graves' orbitopathy. We examined the ophthalmological outcome in daily life activities, such as driving competency. In a retrospective case series, 23 patients with moderate-to-severe and active Graves' disease (14 women and 7 men) were consecutively treated with combined orbital radiotherapy (13 Gy) and intravenous methylprednisolone (500 mg weekly for 6 consecutive weeks and 250 mg weekly for consecutive 6 weeks). The pretreatment Clinical Activity Score of 5.2 points (SD+/-0.9) decreased significantly to 2.7 points (SD+/-1.1) in the 12-month follow-up. Inflammatory eye signs, for example, pain and pressure sensation, caruncle inflammation, eyelid swelling, and exophthalmos decreased significantly. However, there was no significant improvement of eye motility. Despite anti-inflammatory treatment, 13 patients (61.9%) were not fit to drive a car under binocular conditions and additional stabismologic therapy including strabismus surgery was required. The combined therapy of intravenous glucocorticoids and orbital radiotherapy reduced inflammatory ophthalmological signs. In most cases strabismologic intervention including eye muscle surgery was necessary to restore driving competency.
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PMID:Effect of combined intravenous glucocorticoids and orbital radiotherapy in restoring driving competency in patients with Graves' orbitopathy. 1919 36

A 3-month-old infant with a digital hemangioma had relative proptosis of the left eye. Ultrasound, computed tomography, and magnetic resonance imaging showed a retro-orbital mass with features consistent with a capillary hemangioma. Despite the radiographic appearance, however, biopsy findings showed histologically benign tissues of a hamartomatous nature, inconsistent with a capillary hemangioma.
J Pediatr Ophthalmol Strabismus
PMID:Benign hamartoma masquerading as a deep orbital hemangioma. 1964 3

A 10-year-old boy with unilateral ophthalmoplegia, ptosis, and proptosis underwent diagnostic examination. Symptoms of headache, nausea, dyspnea, fatigue, weakness, and loss of appetite began 14 days after the onset of ocular manifestations and 7 days before he was examined. Complete blood count showed an increased white blood cell count with 64% blast cells, anemia, and thrombocytopenia. The patient was transferred to a pediatric hematology unit, where he underwent bone marrow aspiration biopsy. Bilateral ocular inflammatory findings and left-sided mild proptosis became evident. He was diagnosed as having B-cell non-Hodgkin lymphoma infiltrating the bilateral cavernous and sphenoid sinuses.
J Pediatr Ophthalmol Strabismus
PMID:Cavernous sinus syndrome as the initial presentation of childhood non-hodgkin lymphoma. 1964 4


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