Author(s): Mennel S, Meyer CH, Peter S, Schmidt JC, Kroll P
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Abstract BACKGROUND: Retinal hamartoma is a common finding in tuberous sclerosis, but the symptomatic changes of this lesion have rarely been described. This evidence-based review evaluated the incidence of symptomatic retinal hamartoma and compared possible treatment modalities. METHODS: We carried out a review of the literature using MEDLINE. Older publications not listed in MEDLINE were obtained from the reference list of currently published papers. RESULTS: Three observational case series with a follow-up of up to 34 years included 93 patients and reported progression from a flat to a more elevated lesion without visual symptoms in nine patients (9.7\%). Additional symptomatic changes were described in 11 case reports published over a period of three decades. The symptomatic alterations were caused by an enlarged tumour with leakage, macular oedema, accumulating lipoid exudates, serous retinal detachment (n = 8/11) and vitreous haemorrhage (n = 4/11). Most symptomatic cases involved a retinal hamartoma type 1 (n = 6/8). Spontaneous resolution of symptomatic exudative hamartomas occurred in three patients within 4 weeks, although a delayed resorption of subretinal fluid caused permanent visual impairment in one patient. Investigational reports described a slow resorption of subretinal fluid after argon laser photocoagulation (n = 2), although recurrent laser applications induced choroidal neovascularization and destruction of the neurosensory retina (n = 1). A vitrectomy was used to remove a vitreous haemorrhage in another reported patient. In one case, complete resorption of subretinal fluid and an increase in visual acuity was observed within 2 weeks after a single treatment with photodynamic therapy (PDT). No complications were noted during a follow-up of 4 years. CONCLUSIONS: Symptomatic changes are very rare in retinal hamartomas secondary to tuberous sclerosis. Spontaneous resolution of subretinal fluid may occur within 4 weeks. If a macular oedema with increasing lipoid exudates persists over a period of 6 weeks, treatment should be considered. Although previous reports demonstrated possible visual stabilization after argon laser photocoagulation, vision-threatening complications can occur. Current treatment strategies may include PDT based on favourable anatomical and functional results.
This article was published in Acta Ophthalmol Scand
and referenced in Journal of Analytical & Bioanalytical Techniques