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Background The pathophysiology of pigment epithelial detachment (PED) secondary to age-related macular degeneration (AMD) is as yet incompletely understood and treatment remains challenging. Spectral domain optical coherence tomography (SD-OCT) allows for improved morphological characterisation of the space underneath the retinal pigment epithelium (RPE). Objective To investigate eyes with PED for structures underneath the detached RPE cell layer. Methods In a retrospective observational case study, SD-OCT scans of AMD-related PEDs were assessed for the presence of distinctive morphological features in the space between the detached RPE and inner Bruch9s membrane. Results Structures present in the space between the detached RPE and Bruch9s membrane were found in 14 of 90 eyes with AMD-related PED. Each of these eyes shows hyper-reflective material underneath the PED, presenting as highly reflective, multilayered, laminar structures, usually orientated parallel to Bruch9s membrane. Conclusions The findings indicate that SD-OCT may be useful for a more refined phenotypic stratification of AMD-associated PED. Further studies are warranted to explore the correlates on other imaging modalities, to investigate the composition of this material and to assess the potential prognostic relevance of this new finding.

Aim/background—The most common choice of treatment for choroidal haemangiomas (CH) in the past has been the employment of scatter photocoagulation of the surface. This management often requires repetitive treatment or additional invasive management due to massive exudative detachment of the retina. The aim of this retrospective study was to investigate the outcome of the alternative application of low dose external beam irradiation with high energetic photons on these tumours. Methods—A total absorbed dose of 20 Gy was applied to a total of 51 symptomatic eyes: 36 with a circumscribed CH of the posterior pole and 15 with diVuse CH as part of the Sturge‐Weber syndrome. The indication for treatment was an exudative retinal detachment including or threatening the fovea. The mean follow up times in each group were 4.5 and 5.3 years, respectively. Out of a group of 33 patients from whom reliable data could be derived, 17 had symptoms lasting longer than 6 months. Results—In 23 cases (63.8%) with circumscribed CH complete resolution of the subretinalfluid was achieved; the remaining 13 cases (36.2%) showed residual serous detachment distant to the fovea. The visual acuity improved by two or more lines in 14 cases (38.9%), remained stable in 14 cases (38.9%), and decreased in eight cases (22.2%). The functional success was dependent on the lag duration between onset of first subjective symptoms and treatment. The morphological results with diVuse CH were similar to those of the group of circumscribed CH. The visual acuity (VA) at last examination was improved in seven cases (46.6%);in the remaining eight cases, VA was unchanged or had deteriorated. The poor functional outcome in the latter was mainly attributable to secondary glaucoma. Conclusion—External beam irradiation is a useful and a low invasive therapeutic option for CH. A successful functional outcome is dependent on the time delay between first onset of symptoms and the beginning of therapy, the formation of subretinal fibrosis, and also on secondary glaucoma in the case of Sturge‐Weber syndrome. (Br J Ophthalmol 1997;81:267‐273) Haemangiomas of the choroid are rare vascular hamartomas that can occur in two forms: circumscribed choroidal haemangiomas (CH), which are solitary and predominantly located at the posterior pole, and diVuse CH, which are usually part of the Sturge‐Weber syndrome. Histological examination of these tumours shows a vascular pattern, dominated by cavernous portions, but capillary tumours and mixed patterns are also observed. 12

Pseudoxanthoma elasticum is a multi-organ disease which usually develops in midlife—that is, at a time when the patient is still of working age. It is an hereditary disease involving the skin, gastrointestinal tract, cardiovascular system and eyes. A mutation in the ATP-binding cassette subtype C number 6 gene (ABCC6) on chromosome 16p13.1 is the cause, and encoding of this protein has an important role in extracellular calcium and calcium phosphate metabolism.1 Reports of pathological changes in the eyes associated with pseudoxanthoma elasticum by Deschweinitz date back to 1896.2 Angioid streaks, reticular macular dystrophy, optic nerve drusen and a “peau d’orange” appearance in the fundus can develop.3 The prognosis for visual acuity is closely related to whether chorioidal neovascularisation (CNV) ensues. Here, destructive calcification in Bruch’s membrane causes angioid streaks which, as seen by ophthalmoscopy, often spread around the optic disc like a star. Frequency rates of 72–86% for developing CNV have been reported; presentation is also bilateral in a large number of patients.3 4 If left untreated, CNV progresses considerably. Clinically and angiographically, numerous isolated chorioidal membranes can be observed, ending with the development of a disciform scar, which ultimately dramatically worsens …