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)

We report a 60-year-old woman who presented with orbital cellulitis, restricted ocular motility, proptosis, and visual acuity of counting fingers in her left eye 3 days after strabismus surgery. Although she initially responded well to antibiotic and anti-inflammatory therapy, visual acuity in the left eye again decreased on postoperative day 5. Radiographic imaging revealed an intraconal orbital abscess, and she underwent left lateral orbitotomy with abscess drainage, with continued antibiotics and a tapering dose of steroids. To our knowledge, this is the first case of orbital cellulitis and intraconal abscess after strabismus surgery in an adult.
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PMID:Orbital cellulitis and intraconal abscess formation after strabismus surgery in an adult patient. 2456 92

Graves' orbitopathy (GO) is the most frequent and invalidating extrathyroidal expression of Graves' disease. Its incidence and prevalence are, however, low. About three quarters of Graves' patients have no GO at diagnosis, and moderate-to-severe and severe forms represent no more that 5-6% of cases. Progression to severe forms occurs rarely, but it may be caused by risk factors, the most important being smoking and poor control of thyroid dysfunction. Lot of progress has been recently achieved in the understanding of GO pathogenesis, while the disease remains a therapeutic challenge and dilemma. Common treatments for moderate-to-severe and active forms of GO (glucocorticoids and orbital radiotherapy) frequently provide incomplete responses and may be followed by relapse or progression of GO. After the disease has been inactivated by medical treatment, many patients need rehabilitative surgery for residual manifestations (orbital decompression for exophthalmos, squint surgery for extraocular muscle dysfunction, eyelid surgery for eyelid malposition). Novel pharmacological treatments are on the horizon and might target pathogenetic mechanisms of the disease better than glucocorticoids. Clinical evidence concerning their efficacy and safety is presently lacking.
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PMID:Graves' orbitopathy: imperfect treatments for a rare disease. 2478 57

A case of optociliary veins, disc pallor, and long-standing visual loss in an eye of a patient followed from age two to age 25 is presented. A review of the literature indicates the above triad to be usually suggestive of sphenoidal ridge meningioma. Our case, the youngest thus far reported in the literature, also developed ocular hypertension in this eye and exophthalmos. The patient has refused further diagnostic workup.
J Pediatr Ophthalmol Strabismus
PMID:Optociliary veins, visual loss, and disc pallor in a child with a 22 year follow-up. 2487 46

Crouzon's syndrome (CS) is a rare autosomal dominant condition with multiple mutations of the fibroblast growth factor receptor (FGFR2) gene, which accounts for 4.8% of all cases of craniosynostosis. It is characterized by premature closure of cranial sutures, cranial deformities, midface hypoplasia, relative mandibular prognathism, hypertelorism, proptosis, strabismus and short upper lip, crowding of teeth, pseudocleft or sometimes cleft palate and other associated abnormalities. The CS can vary in severity from mild presentation to severe forms involving multiple cranial sutures. We report a case of CS in 11-year-old boy. How to cite this article: Kumar GR, Jyothsna M, Ahmed SB, Lakshmi KS, Crouzon's Syndrome: A Case Report. Int J Clin Pediatr Dent 2013;6(1):33-37.
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PMID:Crouzon's Syndrome: A Case Report. 2520 85

We present a case of a 3-year-old girl with a positive family history of neurofibromatosis type-1 (NF1) presented with best corrected visual acuity of 20/40 in the right eye and <20/400 in the left eye. External ocular examination revealed left eye proptosis of 3 mm, grade II left relative afferent pupillary defect and full range of ocular motility with no strabismus. Slit lamp examination revealed iris lisch nodules bilaterally. Dilated fundus examination of the right eye was normal. Left eye disclosed a large mass extending from the optic nerve head, with associated subretinal fluid. There was neovascularization at the optic disc as well as a superior retinal hemorrhage. Computed tomography of brain/orbits showed an enlarged left optic nerve with a large mass at the optic nerve head, with no evidence of calcification. In addition, a large left optic pathway glioma (OPG), multiple hamartomas within the brain and a smaller low-grade right OPG was also reported. The remarkable feature of our case is the rare intraocular optic nerve involvement of the OPG. Early and regular ophthalmological assessment of all NF1 suspect/confirmed cases is of paramount importance in order to detect OPG early, resulting in timely intervention and salvage of vision.
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PMID:Rare case of optic pathway glioma with extensive intra-ocular involvement in a child with neurofibromatosis type 1. 2562 86

A 4-month-old male infant with proptosis and episcleral injection initially thought to have orbital inflammation was found to have an indirect carotid-cavernous sinus fistula. He was treated conservatively without worsening of symptoms. The authors report the first case of indirect carotid-cavernous sinus fistula in an infant originating from the internal carotid artery.
J Pediatr Ophthalmol Strabismus 2015 Mar 13
PMID:Indirect internal carotid-cavernous fistula in infancy. 2575 Dec 39

Retinoblastoma is the most common primary intraocular malignancy of childhood. It typically presents with leukocoria or strabismus. In later stages of the disease, the child may exhibit proptosis, buphthalmos, or hypopyon. The pathognomonic molecular aberration is a loss of function mutation in the RB1 gene on chromosome 13q. The degree of tumor involvement within the eye is defined by its group. Grouping was historically done with Reese-Ellsworth System. Recent therapeutic advances have led to the development of a new grouping system, the International Classification of Retinoblastoma (ICRB). In cases of extraocular extension and metastatic disease, the degree of tumor involvement outside of the eye is defined by its stage. Retinoblastoma is staged using the International Retinoblastoma Staging System (IRSS). Children with intraocular retinoblastoma have an excellent overall and ocular survival. In order to avoid the morbidity of enucleation and external beam radiation, treatments for isolated intraocular retinoblastoma have progressively moved toward targeted local modalities. Patients with extraocular involvement, such as those with trilateral retinoblastoma, have a poorer prognosis. The majority of these higher stage patients are now able to be cured with combination chemotherapy.
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PMID:Retinoblastoma. 2602 80

We present two cases of infants with a similar constellation of clinical findings: retro-orbital infantile hemangioma (IH), internal carotid artery (ICA) arteriopathy, and intracranial IH. In both cases, intracranial vascular anomalies and hemangiomas were found incidentally during evaluation of unilateral proptosis. Neither infant had evidence of cutaneous segmental IH of the face or neck, which might have provided a clue to the diagnosis of PHACE syndrome or of intracranial hemangiomas. In one case, intracranial involvement was particularly extensive and function threatening, with mass effect on the brain parenchyma. These cases serve to highlight the fact that clinical findings of proptosis, globe deviation, and strabismus should prompt immediate imaging to confirm the presence of orbital IHs and to exclude other diagnoses. Moreover, based on our cases and the embryologic origin of the orbit as a unique developmental unit, patients with confirmed retro-orbital IHs should undergo evaluation for anomalies associated with PHACE syndrome. Patients with orbital IHs and an additional major criterion for PHACE syndrome should be considered to have definite, and not just possible, PHACE syndrome.
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PMID:Orbital Hemangioma with Intracranial Vascular Anomalies and Hemangiomas: A New Presentation of PHACE Syndrome? 2644 88

Orbital diseases are common in dogs and cats and can present on emergency due to the acute onset of many of these issues. The difficulty with diagnosis and therapy of orbital disease is that the location of the problem is not readily visible. The focus of this article is on recognizing classical clinical presentations of orbital disease, which are typically exophthalmos, strabismus, enophthalmos, proptosis, or intraconal swelling. After the orbital disease is confirmed, certain characteristics such as pain on opening the mouth, acute vs. chronic swelling, and involvement of nearby structures can be helpful in determining the underlying cause. Abscesses, cellulitis, sialoceles, neoplasia (primary or secondary), foreign bodies, and immune-mediated diseases can all lead to exophthalmos, but it can be difficult to determine the cause of disease without advanced diagnostic imaging, such as ultrasound, magnetic resonance imaging, or computed tomography scan. Fine-needle aspirates and biopsies of the retrobulbar space can also be performed.
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PMID:Management of Orbital Diseases. 2649 2

A 1-year-old male castrated Pug was referred for unilateral exophthalmos unresponsive to oral antibiotic and anti-inflammatory therapy. Clinical findings included exophthalmos of the left eye with lateral strabismus, resistance to retropulsion, and an elevated nictitans. Hematologic and biochemical analyses were within normal limits. Findings following computed tomography (CT) of the head included an expansile retrobulbar soft tissue mass with bony lysis extending into the left nasal cavity and nasopharynx. Ultrasound-guided fine-needle aspirates and biopsy samples obtained via rhinoscopy were nondiagnostic. Palliative exenteration was elected; the patient was euthanized 13 weeks following exenteration due to development of neurologic signs and perceived poor quality of life. The histopathologic diagnosis was a malignant pigmented peripheral nerve sheath tumor.
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PMID:Retrobulbar pigmented peripheral nerve sheath tumor in a dog. 2652


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