Coats' disease in Tanzania: first case report and literature review
- PMID: 25352900
- PMCID: PMC4209640
- DOI: 10.4314/ahs.v14i3.37
Coats' disease in Tanzania: first case report and literature review
Abstract
Background: Coats' disease is an exudative retinal detachment with vascular telangiectasis occurring mostly in male children, the age group most affected by retinoblastoma.
Objectives: Compare the differential diagnoses of Coats' disease Establish recommendation to early disease detection.
Materials and methods: A 3-year-old female child was referred to Muhimbili National Hospital (MNH), Tanzania, in September 2011. She had presented at the peripheral hospital with gradual onset of left eye leukocoria for 1 year and pain for 2 months. B-scan showed a mass in the left eye. A clinical diagnosis of retinoblastoma was made. Left eye enucleation was performed; the patient was referred to MNH, with the enucleated specimen.
Results: Brain and orbits scan revealed no residual tumour. The globe measured 2 x 1.8 cm, the optic nerve stump measured 3 mm. A whitish mass filled the vitreous, with complete retinal detachment. Microscopy showed retinal gliosis, detachment with sub retinal PAS positive exudates, vacuolation and cholesterol clefts. Foreign body giant cells were present; telangiectatic thin-walled blood vessels were identified. Clinico-pathological findings were of stage 4 Coats' disease.
Conclusion: Coats' disease is an important differential diagnosis of retinoblastoma. Delay to detect Coats' disease leads to vision loss which necessitates eye enucleation as was in this child.
Keywords: Coats' disease; Tanzania.
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References
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- Coats G. Forms of retinal disease with massive exudation. R Lond Ophthal Hosp Rev. 1908;17:440–525.
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- Shields CL, Uysal Y, Benevides R, Eagle RC, Jr, Malloy B, Shields JA. Retinoblastoma in an Eye with Features of Coats' Disease. J Pediatr Ophthalmol Strabismus. 2006;43:313–315. - PubMed
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- Black GCM, Perveen R, Bonshek R, Cahill M, Clayton-Smith J, Lloyd, et al. Coats' Disease of the Retina (Unilateral Retinal Telangiectasis) Caused by Somatic Mutation in the NDP Gene: A role for Norrin in retinal Angiogenesis. Hum Mol Genet. 1999;8(11):2031–2035. - PubMed
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- Shields JA, Shields CL, Honavar SG, Demirci H. Clinical variations and complications of Coats disease in 150 cases: The 2000 Sanford Gifford Memorial Lecture. Am J Ophthalmol. 2001;131(5):561–571. - PubMed
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