Bilateral Spontaneous Hyphaema in Juvenile Xanthogranuloma: A case report and literature review

Abstract

Background: A four-month-old Nigerian male was presented with spontaneous hyphaema in both eyes initially thought to be due to trauma or possibly non accidental injury. There was associated secondary glaucoma and skin lesions typical of juvenile xanthogranuloma. This condition, to the best of our knowledge, has not yet been reported in Nigeria. Case report: There was redness in the right eye with no associated tearing or photophobia. On examination, there was visually directed reaching in both eyes, with central, steady and maintained fixation, corneal haze and 15% hyphaema. The left eye, which appeared normal, later also developed 10% hyphaema, upon which an impression of juvenile xanthogranuloma was entertained. Laboratory investigations, including a clotting profile, were all within normal limits. Examination under anaesthesia showed progressively increasing buphthalmos and vitreous haemorrhage in the left eye with high intraocular pressure and large cup disc ratios. Histology showed irregularly disposed spindle-shaped cells in the dermis that were intimately associated with small capillaries containing red blood cells. Occasional tuton-like giant cells were seen with hyperkeratosis and papillomatosis of the epidermis which is consistent with juvenile xanthogranuloma. Conclusion: Ocular complications provide the primary concern in this condition and could eventually lead to blindness. Therefore, a high index of suspicion is the key to the prevention of blindness. Keywords: juvenile xanthogranuloma, hyphaema, glaucomaNigerian Journal of Opthalmology Vol. 15 (2) 2007: pp. 61-63