疑似自然消退型视网膜母细胞瘤伴牵引型视网膜脱离和晶状体脱位1例

　　作者：Visvaraja,Subrayan　　作者单位：马来西亚吉隆坡，马来亚大学医学院眼科

　　【摘要】 我们报告了1例疑似盲眼中自然消退型视网膜母细胞瘤病例。肿瘤稳定，无眼外扩散长达20a。其他症状包括：带状角膜病变，白瞳，晶状体脱位，视网膜肿块钙化和牵引型视网膜脱离。

　　【关键词】 视网膜母细胞瘤;钙化;牵引型视网膜脱离

　　Abstract

　　　We illustrated a case of presumed spontaneously regressed retinoblastoma in a blind eye. The tumour remained stable, with no extraocular spreading, for 20 years. Associating signs included band keratopathy, leukocoria, dislocated lens, calcified retinal masses and tractional retinal detachment.

　　　KEYWORDS: retinoblastoma; calcification; tractional retinal detachment

　　INTRODUCTION

　　Retinoblastoma is a malignant tumour of the developing retina. It is the commonest primary malignant intraocular tumour in children[1]. Spontaneous regression of retinoblastoma is well documented in the literature[2]. We illustrated the clinical features of a presumed spontaneously regressed retinoblastoma in this report. We believes this was the first report of tractional retinal detachment as a feature of spontaneously regressed retinoblastoma in the literature.

　　CASE REPORT

　　A 20yearold man presented to us with no vision in the right eye since childhood. He was seeking cosmotic treatment for leukocoria in this eye, which was first seen by an ophthalmologist at the age of 4 months. CT scan at that time revealed an intraocular mass with calcification. Retinoblastoma was suspected and enucleation advised. However, this was refused by the parents, and they defaulted further followup.

　　At presentation, ocular examination of the right eye showed a noperceptionoflight vision with band keratopathy (Figure 1A), absent red reflex and aphakia. The calcified lens was seen in the vitreous inferiorly just anterior to a densely calcified retinal mass at 6 Oclock (Figure 1B). Another calcified mass was seen along the superotemporal arcade with adjacent pigmentation (Figure 1C) and tractional retinal detachment resulted from extensive preretinal fibrosis involving the macula (Figure 1D). The left eye was completely normal (Figure 2).

　　Figure 1A: the right eye showing band keratopathy and poor dilatation of the pupil after instillation of phenylephrine and tropicamide eye drops; B: fundus view of the inferior retina showing a large calcified retinal mass short arrow and the calcified dislocated lens anterior to it in the vitreous long arrow; C: choroidal mass along the superotemporal arcade with adjacent pigmentation and fibrous tissue; D:extensive fibrosis of the macula with resultant tractional detachment of the retina

　　CT scan of orbits and brain showed extensive intraocular calcification in the right eyes with no extraocular extension and no intracranial abnormalities noted (Figure 3). Fluorescein Fundus Angiography (FFA) showed rich vascularization of both masses with double circulation pattern .

　　Figure 2Fundus photo of the left eye showing a normal fundus

　　Figure 3Axial CT scan of the orbits showing extensive chorioretinal calcification in the right eye (short arrow), and the calcified dislocated lens (long arrow)

　　Figure 4Venous phase of the Fluorescein Fundus Angiography (FFA) of the right eye showing double circulation within both the superotemporal mass (A) and the inferior mass (B)

　　Based on history, clinical appearance, CT scan and FFA findings, a diagnosis of presumed spontaneously regressed retinoblastoma was made. Enucleation was advised to confirm the diagnosis and for prosthetic replacement of the right eye, but the patients refused it, and defaulted further followup.

　　COMMENT

　　Retinoblastoma can undergo spontaneous regression albeit rare[3]. In this case, spontaneous regression is illustrated by calcification and retinal pigment epithelial changes. The lesion shown in Figure 1 resembles type III regression pattern of a treated retinoblastoma[4]. This entails extensive calcification in a greyish avascular mass.

　　Lens subluxation/dislocation in cases of retinoblastoma has been reported twice in the literature[5,6]. The mechanism thought to be responsible for lens subluxation or dislocation in this condition is disruption of the lens zonular fibres secondary to necrosis of the ciliary epithelium[5].

　　To the best of our knowledge, this is the first reported case of tractional retinal detachment in spontaneously regressed retinoblastoma. This could be attributed to stimulation of an immune reaction to the tumour antigen resulting in panuveitis and neovascularization that resolved into fibrous bands causing the tractional retinal detachment. The presence of band keratopathy supported this postulation. Another possible explanation was the regressed retinal neovascularization, which is a known feature of retinoblastoma.

　　【参考文献】

　　1 Mastrangelo D, De Francesco S, Di Leonardo A, Lentini L, Hadjistilianou T. The retinoblastoma paradigm revisited. Med Sci Monit 2008;14(12):RA231240

　　2 Kao LY, Yang ML. Spontaneous regression of retinoblastoma in a Taiwan series. J Pediatr Ophthalmol Strabismus2005;42(4):228232

　　3 Singh AD, Santos CM, Shields CL, Shields JA, Eagle RC Jr. Observations on 17 patients with retinocytoma. Arch Ophthalmol 2000;118(2):199205

　　4 Singh AD, GarwayHeath D, Love S, Plowman PN, Kingston JE, Hungerford JL. Relationship of regression pattern to recurrence in retinoblastoma. Br J Ophthalmol1993;77(1):1216

　　5 Kilby AE,Ip MS,Smith ME. Subluxated/dislocated lens and hyphema as features of retinoblastoma. Retina2003;23(6):872874

　　6 Byrnes GA, Shields CL, Shields JA, De Potter P, Eagle RC Jr. Retinoblastoma presenting with spontaneous hyphema and dislocated lens. J Pediatr Ophthalmol Strabismus1993;30(5):334336