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)

Of 500 consecutive patients referred to the Ocular Oncology Service at Wills Eye Hospital with the diagnosis of possible retinoblastoma, 288 (58%) were found on clinical evaluation to have retinoblastoma and 212 (42%) had lesions that simulated retinoblastoma. A total of 23 different conditions accounted for the 212 pseudoretinoblastomas. Three most common pseudoretinoblastomas were: persistent hyperplastic primary vitreous (28%), Coats' disease (16%), and presumed ocular toxocariasis (16%). Congenital cataract and retinopathy of prematurity accounted for a much lower percent of pseudoretinoblastomas in this series as compared to a previously reported series. The pertinent clinical features that serve to differentiate these simulating lesions from retinoblastoma are reviewed.
J Pediatr Ophthalmol Strabismus
PMID:Lesions simulating retinoblastoma. 175 60

A suspected diagnosis of retinoblastoma can almost always be supported by intraocular calcification detected by computerized tomography. We report two patients with diffuse unilateral opacification of the ocular media who failed to demonstrate calcification on a CT scan.
J Pediatr Ophthalmol Strabismus
PMID:Computerized tomography fails to show calcification in diffuse retinoblastoma. 201 51

A review of 400 consecutive patients with retinoblastoma who presented to the Ocular Oncology Service at Wills Eye Hospital showed that 34 (8.5%) patients were older than 5 years of age at the time of initial diagnosis. The tumor was active in 26 (76%) cases and inactive (retinoma) in 8 (24%). An evaluation of the 26 patients with active retinoblastoma showed several unique features. At the time of diagnosis, their median age was 6 years and the oldest was 18 years. In 20 (77%) cases, the patient volunteered symptoms that prompted the eye examination; the presenting symptoms included leukocoria (9 cases), decreased vision (9 cases), strabismus (4 cases), pain (1 case), floaters (1 case), and no symptoms (2 cases). All of the 26 patients (100%) had unilateral sporadic retinoblastoma. Misdiagnosis before referral was common in these older children with active retinoblastoma. Five patients (19%) had prior vitrectomy for presumed vitreous hemorrhage or endophthalmitis while the retinoblastoma was unsuspected clinically, one patient (4%) had cryotherapy for presumed Coats disease, and one (4%) was observed for 7 months for presumed vitreous hemorrhage. The clinician should seriously consider the possibility of retinoblastoma in children who present with signs of unexplained vitreous hemorrhage or endophthalmitis, even if they are older than 5 years of age.
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PMID:Retinoblastoma in older children. 202 62

A retrospective study of retinoblastoma was done during 1978-1987, analysing the clinics aspects of the entity. We found nineteen children during this period, from them eight were girls (42%) and eleven were boys (58%). Ages of diagnostic ranged between three months and four years, with and average of 17.53 months. Nine children (47%) were bilaterally affected. Leocokoria was the most common initial sign, followed by strabismus. Ophthalmoscopy was the diagnostic procedure used, and surgery the recommended therapeutic, using radiotherapy as a palliative method in the cases which surgery has no indications.
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PMID:[Retinoblastoma: review of 19 cases]. 204 2

A retrospective review of cases on file at the Ophthalmic Oncology Center of The New York Hospital-Cornell Medical Center, New York was performed in order to examine the appearance of radiation regression patterns 7 or more years after successful treatment of retinoblastoma with external beam radiotherapy. Forty-eight patients were found to have 89 tumors in 57 eyes which were treated solely with external beam radiation; they were followed for a minimum of 7 years and had sufficient information available for analysis. All but five of the patients had bilateral retinoblastoma. Seventy-four of the 89 tumors continued to be ophthalmoscopically visible after 7 or more years. Taking into account those that did change between the time of first evaluation (usually at the completion of treatment) and final evaluation (7 or more years after treatment), the number of Type I regressions increased by 10.1%, Type IIs decreased by 19.1%, Type IIIs fell by 7.8%, Type IVs rose by 10.1%, and the number of tumors that disappeared increased by 6.8%. Type II remained the most common regression throughout the follow-up. The regression with the greatest potential for change was the Type II regression. The pretreatment volume of the tumor correlated with long-term radiation regression patterns. The smallest tumors (mean size 1.1 dd [disc diameter] or less in size) completely disappeared, while the largest (mean 9.9 dd) became Type I regressions.
J Pediatr Ophthalmol Strabismus
PMID:Radiation regression patterns in treated retinoblastoma: 7 to 21 years later. 205 Dec 87

In the US, leukocoria is the most common presenting sign of retinoblastoma, and strabismus is the second most common finding. Retinoblastoma less frequently presents as an inflammatory process involving the eye or orbit, which often masks the symptoms of retinoblastoma. Children who present with leukocoria strabismus, hypopyon, heterochromia, rubeosis, hyphema, or an orbital cellulitis should be referred to an ophthalmologist for a dilated fundus examination of both eyes, under general anesthesia if necessary, to rule out the possibility of a retinoblastoma in the differential diagnosis.
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PMID:Unusual presenting signs of retinoblastoma: a case study. 206 38

A retrospective review of 297 cases of enucleated and histologically proven unilateral retinoblastoma with a minimum of 5-year follow-up was analyzed to see if there were any clinical or prognostic differences between endophytic and exophytic type retinoblastoma. Endophytic retinoblastoma (181 cases) was found more often than exophytic (116 cases). The following features were not associated with the clinical appearance of either endophytic or exophytic tumors: sex of patient, right vs left eye, propensity for bilateral development, initial sign or symptom, presence of rubeosis, preoperative metastasis, optic nerve invasion, orbital recurrence, survival of the patient, length of follow-up, or age at diagnosis. Three features were found that correlated with type: a disproportionately higher percentage of patients with endophytic retinoblastoma had a positive family history; a disproportionately high percentage of patients with exophytic retinoblastoma developed glaucoma; and choroidal invasion occurred significantly more often in patients who had exophytic retinoblastoma than in those who had endophytic retinoblastoma.
J Pediatr Ophthalmol Strabismus
PMID:Endophytic vs exophytic unilateral retinoblastoma: is there any real difference? 191 75

From 49 eyes enucleated for retinoblastoma, two new cell lines, WERI-Rb24 (W-24) and WERI-Rb27 (W-27), were established in long-term culture (greater than 5 years). The W-24 cell line was derived from a 22-month-old boy with sporadic retinoblastoma; the W-27 cell line was derived from a 24-month-old boy with bilateral retinoblastoma. Both cell lines show abnormal mRNA transcripts corresponding to the retinoblastoma gene. At the DNA level, one retinoblastoma allele was not present in the W-24 cell line. In the W-27 cell line a mutation was identified in one allele, while the other appears grossly normal. Since no normal retinoblastoma mRNA can be detected in either cell line, a subtle mutation must occur on the grossly normal allele. Comparison of DNA in W-27 lymphocytes and tumor cells with DNA probe p6NR-0.5 indicates that the observed mutation occurred somatically. The application of these two new retinoblastoma cell lines to the characterization of defects in the retinoblastoma gene and to gene replacement therapy in retinoblastoma is discussed.
J Pediatr Ophthalmol Strabismus
PMID:Characteristics of two new retinoblastoma cell lines: WERI-Rb24 and WERI-Rb27. 239 23

An 8-month-old girl underwent enucleation of her right eye because of a calcified retrolental white mass filling the entire vitreous cavity. The conventional histological examination revealed a totally necrotic unidentifiable tumor, with scattered foci of calcification. In addition, we found two islands of viable small round cells, suspected to be retinoblastoma cells, which were located in the optic nerve behind the lamina cribrosa. These cells stained positively for neuron-specific enolase (NSE), but not for glial-fibrillary-acidic-protein (GFAP), supporting the diagnosis of retinoblastoma. Because one of the latter foci of viable tumor cells was located at the cut end of the optic nerve, the baby was treated by external beam irradiation.
J Pediatr Ophthalmol Strabismus
PMID:Immunohistochemical diagnosis of a totally necrotic retinoblastoma: a clinicopathologic case. 245 19

Magnetic resonance imaging (MRI) is playing a growing role in the evaluation of ocular and orbital tumors. We report the case of a 26-month-old child presenting an undifferentiated retinoblastoma with a differentiated retinocytoma component in his left eye. After the eye was enucleated, we could correlate the clinicopathologic findings with the MRI scans. Both the undifferentiated retinoblastoma and the differentiated retinocytoma components could be determined on magnetic resonance imaging. Because different levels of metabolic activity within a tumor mass may influence largely T1 and T2 parameters, accurate determination of these values may be useful in determining the differentiation of a retinoblastoma, enabling a better therapeutic approach and assessment of the response to treatment.
J Pediatr Ophthalmol Strabismus
PMID:Magnetic resonance imaging in retinoblastoma and retinocytoma: a case report. 262 46


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