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Bilateral maculopathy in a thalassemia patient on iron chelation therapy: a case report / Maculopatia bilateral em paciente com talassemia em terapia quelante de ferro: relato de caso
Tang, Chin Shi; Hanapi, Maya Sapira; Lee, Soon Yap; Ngoo, Qi Zhe.
  • Tang, Chin Shi; Hospital Raja Perempuan Zainab II. Jalan Hospital. Department of Ophthalmology. Kota Bharu. MY
  • Hanapi, Maya Sapira; Hospital Raja Perempuan Zainab II. Jalan Hospital. Department of Ophthalmology. Kota Bharu. MY
  • Lee, Soon Yap; Hospital Raja Perempuan Zainab II. Jalan Hospital. Department of Pediatrics. Kota Bharu. MY
  • Ngoo, Qi Zhe; Universiti Sains Malaysia. School of Medical Sciences. Department of Ophthalmology. Kota Bharu. MY
Rev. bras. oftalmol ; 80(4): e0026, 2021. graf
Article in English | LILACS | ID: biblio-1288636
ABSTRACT
ABSTRACT A 10-year-old Malay girl with underlying HbE/beta-thalassemia, on regular blood transfusion and deferoxamine iron chelation therapy, presented with two-month history of bilateral blurring of vision. On examination, her vision was 6/36 both eyes. Other optic nerve functions were normal. Anterior segment examination of both eyes was unremarkable. Fundus examination of both eyes revealed dull foveal reflex. Optical coherence tomography of both maculae showed increased central subfield thickness. Fundus fluorescence angiography showed patchy hypofluorescence over macular region for both eyes and late staining, indicating retinal pigment epithelium anomalies. A diagnosis of iron-chelation-therapy-related bilateral maculopathy was made. Patient was co-managed with pediatric hematology team to adjust the dose of deferoxamine, and was given three monthly appointments to monitor the progression of maculopathy at the ophthalmology clinic. However patient defaulted ophthalmology follow-up after the first visit.
RESUMO
RESUMO Uma menina malaia de 10 anos de idade com doença de base- B/beta-talassemia, em transfusão de sangue regular e terapia quelante de ferro deferoxamina, apresentou história de dois meses de visão turva bilateral. Ao exame, sua visão era de 6/36 em ambos os olhos. Outras funções do nervo óptico estavam normais. O exame do segmento anterior de ambos os olhos foi normal. Exame do fundo de ambos os olhos revelou reflexo foveal opaco. A tomografia de coerência óptica de ambas as máculas mostrou aumento da espessura do subcampo central. A angiografia de fluorescência do fundo mostrou hipofluorescência irregular sobre a região macular de ambos os olhos e coloração tardia, indicando anomalias de epitélio pigmentar da retina. Um diagnóstico de maculopatia bilateral relacionada à terapia quelante de ferro foi feito. A paciente foi avaliada em conjunto com a equipe de hematologia pediátrica para ajustar a dose de deferoxamina, e foram oferecidas três consultas mensais na clínica oftalmológica, para monitorar a progressão da maculopatia. No entanto, ela não compareceu para acompanhamento oftalmológico após a primeira visita.
Subject(s)


Full text: Available Index: LILACS (Americas) Main subject: Siderophores / Beta-Thalassemia / Deferoxamine / Transfusion Reaction / Macular Degeneration Type of study: Diagnostic study Limits: Child / Female / Humans Language: English Journal: Rev. bras. oftalmol Journal subject: Ophthalmology Year: 2021 Type: Article Affiliation country: Malaysia Institution/Affiliation country: Hospital Raja Perempuan Zainab II/MY / Universiti Sains Malaysia/MY

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Full text: Available Index: LILACS (Americas) Main subject: Siderophores / Beta-Thalassemia / Deferoxamine / Transfusion Reaction / Macular Degeneration Type of study: Diagnostic study Limits: Child / Female / Humans Language: English Journal: Rev. bras. oftalmol Journal subject: Ophthalmology Year: 2021 Type: Article Affiliation country: Malaysia Institution/Affiliation country: Hospital Raja Perempuan Zainab II/MY / Universiti Sains Malaysia/MY