Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0086543 (cataract)
 29,165 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The survival rate of children with localized orbital rhabdomyosarcoma is now greater than 90% 3 years after diagnosis as a result of advances in radiation and chemotherapy. Ninety percent of these children develop cataracts within 1 to 4 years after the completion of radiotherapy. The correction of aphakia in these children is complicated by the concurrent keratoconjunctivitis and dryness associated with radiotherapy. Three patients with a diagnosis of orbital rhabdomyosarcoma underwent treatment for uniocular cataract. Two of the patients were unable to use extended wear contact lenses. Both underwent epikeratophakia with poor results. One patient who had clinically significant ocular drying prior to cataract extraction underwent successful implantation of an intraocular lens as a primary procedure, with excellent visual results. Clinicians should be aware of the difficulties associated with contact lens wear and epikeratophakia tissue lenses in children who have had high doses of radiation for orbital rhabdomyosarcoma. Such patients are probably best served by primary intraocular lens implantation or by preservation of the posterior capsule at the time of cataract extraction to allow secondary lens implantation if contact lens wear is unsuccessful.
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PMID:The correction of unilateral aphakia in children treated for orbital rhabdomyosarcoma. 234 12

Late effects of therapy were evaluated in 50 children surviving orbital rhabdomyosarcoma following treatment on Intergroup Rhabdomyosarcoma Study I. All patients had microscopic or gross tumor present following surgery and subsequently received radiotherapy and various combinations of chemotherapeutic agents. Problems in the eye included infections and functional and structural changes. Decreased vision in the treated eye was the most common functional problem and in most patients related to cataract formation, which occurred in 90% of eyes. Changes in the cornea and retina were also seen. Bony hypoplasia of the orbit and facial asymmetry were present in one half of the children. Gonadal development was normal. Statural growth was retarded in 61% of the patients. All children were in school, with five having learning or behavioral problems. One child developed acute myeloblastic leukemia. The excellent survival in patients with orbital rhabdomyosarcoma provides support for treatment programs that use multiple-agent chemotherapy and radiotherapy and do not include orbital exenteration initially.
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PMID:Late effects of therapy in orbital rhabdomyosarcoma in children. A report from the Intergroup Rhabdomyosarcoma Study. 395 18

Orbital rhabdomyosarcoma (RMS) accounts for 10% of childhood RMS and has a relatively good prognosis of up to 85% 5-year survival. Improved survival has led to increased interest in late effects of treatment. The objective of this study was to review the results of treating orbital RMS with multidisciplinary treatment at Women's and Children's Hospital and Royal Adelaide Hospital with emphasis on late effects of treatment. A retrospective review was carried out of all patients with orbital RMS treated with multidisciplinary treatment including radiation therapy and chemotherapy in the two institutions between 1982 and 2002. A total of five patients (age range 5.5-12 years) satisfied the eligibility requirements. Late effects were significant and included facial bone hypoplasia, cataract formation and growth hormone deficiency. Overall survival was 80% (4/5) with mean follow up of 8 years (range 3-13 years). Given the high cure rates achieved, future treatments must aim to maintain the good results but to reduce the high incidence of late effects of treatment. Standardised rating of late toxicity, long-term follow-up clinics, and implementation of modern radiation techniques (3-D conformal radiotherapy, intensity modulated radiotherapy, proton therapy) for patients with orbital RMS are important to improving outcome.
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PMID:Orbital rhabdomyosarcoma: multidisciplinary treatment experience. 1649 26

Rhabdomyosarcoma (RMS) is the most common soft tissue tumour in children, with the head and neck region accounting for 35-40% of cases. Nasopharyngeal RMSs tend to grow rapidly and invade adjacent structures. Both the Intergroup Rhabdomyosarcoma Studies and the European Studies have established that the ideal management of this disease is multimodal, using a combination of surgery, chemotherapy and radiotherapy. This case series examines the role of radiotherapy in the management of paediatric nasopharyngeal RMSs, with particular reference to long-term morbidity and disease-free survival. The cases of five children with nasopharyngeal RMS were reviewed and a systematic review of the literature contained in the PubMed databases was conducted to establish 24 individually detailed cases. Management in all patients was multimodal, using a combination of chemotherapy, radiotherapy as well as surgery. External beam radiotherapy is an integral component of treatment for nasopharyngeal RMSs. With more patients surviving for longer periods, more long-term sequelae of radiotherapy have been reported. Complications include sensorineural deafness, endocrine manifestations following radiation of the pituitary gland, cranial nerve palsies, second malignancies within the radiation field, cataract formation, retinopathy and growth disturbance. Morbidity from radiotherapy may be considerable and depends on the field and dose of radiation. Current advances in radiotherapy are aimed at improving the rate of tumour control and reducing such complications. Recent improvements in imaging and conformal techniques have the potential to reduce the morbidity associated with radiotherapy in this cohort.
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PMID:Paediatric nasopharyngeal rhabdomyosarcoma: A case series and literature review. 2071 21