Jean-Jacques De Laey

Vasoproliferative retinal tumors associated with peripheral chorioretinal scars in presumed congenital toxoplasmosis

Abstract · Background: The purpose of this retrospective clinical study was to demonstrate vascularization of peripheral hyperplastic chorioretinal scars in presumed congenital toxoplasmosis by choroidal vessels and secondly to report vasoproliferative tumors of the sensory retina seen in association with such lesions. · Methods: Retrospective study of a cohort of 13 patients with peripheral hypertrophic chorioretinal scars, including fluorescein angiography (eight eyes) and indocyanine green angiography (seven eyes). Congenital toxoplasmosis was serologically confirmed in seven cases and suspected on clinical grounds in six cases. · Results: Sixteen eyes exhibited a peripheral complex scar with a posterior atrophic and an anterior hypertrophic part. These scars were vascularized from the choroid. In eight of these eyes an additional vasoproliferative tumor was seen within or adjacent to the scar and in one fellow eye a vasoproliferative retinal tumor was found as well. These eyes experience visual loss, resulting from vitritis and exudative/traction retinal detachment. Regression of exudation was seen in five of seven cryocoagulated or endophotocoagulated tumors. · Conclusion: Peripheral hypertrophic scars in presumed congenital toxoplasmosis can be vascularized from the choroid. A vasoproliferative tumor of the sensory retina, secondary to presumed congenital toxoplasmosis scars, can cause vitritis and exudative/traction retinal detachment. Early coagulation of this lesion may prevent a poor outcome.

Bilateral Corneal Melting in a Patient with Paraneoplastic Pemphigus

An 80-year-old man, with a solid abdominal tumor and multiple skin lesions, was admitted to the hospital because of a perforated right cornea and an impending perforation of the left. The clinical, histological, immunohistological and immunoprecipitation findings of the skin lesions were consistent with Anhalt’s criteria for paraneoplastic pemphigus (PNP). The underlying malignancy proved to be an incurable peripheral neuronal shaft tumor. Both conjunctivae appeared normal. The right eye revealed a flat anterior chamber, due to a spontaneous, central corneal perforation. The central part of the left cornea had severely thinned, resulting in a descemetocele, which eventually perforated. Multiple surgical interventions were needed to restore the anterior chamber in both eyes. Although a causative association between PNP and corneal perforation could not be demonstrated, we think that corneal melting should be added to the list of ocular complications in patients with PNP.

Indocyanine green angiography and age-related serous pigment epithelial detachment

Abstract• Background: Fundus fluorescein angiography has shown that pigment epithelial detachment in age-related macular degeneration is often associated with choroidal neovascularisation (CNV). Indocyanine green angiography (ICG-A) provides a better visualisation of choroidal circulation and of CNV than fluorescein angiography (FA).• Methods: We studied the ICG angiograms of 58 eyes presenting age-related pigment epithelial detachment, either with signs of occult CNV (48 eyes) or without signs of CNV (10 eyes) on FA. In selected cases the neovascular complex defined on the ICG angiogram was photocoagulated.• Results: ICGA-revealed hyperfluorescence interpreted as CNV in 46 of 48 eyes with fluorescein angiographic signs of occult choroidal neovascularisation. The neovascular complex seen on the ICG angiogram was well delineated in 29 eyes and ill defined in 17 eyes. ICG-A revealed CNV in 2 of 10 eyes without signs of CNV on FA. In these two cases the neovascular complex was ill defined. Photocoagulation in selected cases resulted in stabilisation or even improvement of visual acuity and flattening of the pigment epithelial detachment in 9 of 18 cases.• Conclusion: ICG-A may offer a better definition of the neovascular complex associated with pigment epithelial detachment in age-related macular disease and be helpful in guiding laser treatment. In some cases FA still outlines more clearly the lesions to be treated. FA and ICG-A should thus be used concurrently to determine treatment strategy.

Paramacular Choriocapillaris Atrophy

This disease was probably first described under the name of sclerosis choroidea circinata by Knapp in 1907 [1]. Sorsby mentions this case in his classification of retinal and choroidal abiotrophies under the name ‘paracentral choroidal sclerosis’ [5, 6]. Krill and Archer, in 1971, mention again this disease and add a new clinical case [2, 3]. Schocket and Ballin in 1970 describe yet another case which they call circinate choroidal sclerosis [4].

Retinitis Pigmentosa and Allied Disorders

There are more than 250 entries with the term retinitis pigmentosa in OMIM. More than half of them describe a systemic disease associated with retinal dystrophy, whether central, peripheral or mixed.

Indocyanine Green Angiography

The first attempt to use indocyanine green (ICG) as a dye for the study of the chorioretina dates from 1969. Kogure and Choromokos used ICG to study the brain vasculature and in 1969 recorded the first ICG absorption angiography of the ocular fundus with infrared colour film. As the dye had to be injected in the carotid artery, its use was limited. Hochheimer demonstrated that it was possible to record ICG passage after intravenous injection with high-speed infrared black-and-white film and a regular fundus camera. The images obtained where however difficult to analyse.

Autosomal Dominant Stargardt-Like Macular Dystrophy (ELOVL4)

The autosomal dominant Stargardt-like macular dystrophy is caused by a mutation in the ELOVL4 gene and associates a foveo-macular atrophy with flavimaculatus flecks around the atrophic area.

Spastic Paraplegia and Retinal Degeneration: Kjellin Syndrome

Kjellin syndrome is a hereditary neuro-ophthalmologic syndrome characterized by spastic paraplegia, dementia, dysarthria, corpus callosum atrophy and dystrophy of the posterior pole of the ocular fundus. This disease was first described in Sweden by Kjellin in 1959 [1]. However, its features had already been very well detailed earlier in particular by Franceschetti and Bar in two large families [2, 3].

Extensive Macular Atrophy with Pseudodrusen-Like Appearance

EMAP is a recently described late-onset and rapidly progressive geographic macular atrophy starting in the fifth decade, associated with pseudodrusen or reticular drusen in the posterior pole and paving stone degeneration in the retinal periphery.

Congenital Hypotrichosis with Juvenile Macular Dystrophy

Congenital hypotrichosis with juvenile macular dystrophy (HJMD) is characterised by early loss of the first hair growth in the first months of life followed by an incomplete regrowth resulting in congenital localised hypotrichosis associated with juvenile-onset maculopathy. It was first described in 1935 [22].