ABSTRACT
Familial exudative vitreoretinopathy is an inherited disorder characterized by retinal traction, peripheral vitreous opacities, and subretinal and intraretinal exudates. We observed a family in which four boys (the children of three sisters) were affected with this disorder and an X-linked recessive inheritance was apparent. The differential diagnosis includes retinopathy of prematurity, primary hyperplastic primary vitreous, Coats' disease, peripheral uveitis, retinoblastoma, and Norrie's disease, but this differentiation can usually be made on the basis of clinical findings alone. Knowledge of X-linked recessive transmission is important for correct diagnosis and for genetic counseling.
Subject(s)
Genetic Linkage/genetics , Retinal Diseases/genetics , Vitreous Body , X Chromosome , Child, Preschool , Chromosome Aberrations/diagnosis , Chromosome Disorders , Diagnosis, Differential , Exudates and Transudates , Eye Diseases/diagnosis , Eye Diseases/genetics , Humans , Infant , Male , Pedigree , Retinal Diseases/diagnosisABSTRACT
We surgically removed lenses and as much retina as accessible in one eye of six pigmented rabbits which were followed for up to four months postsurgically. Clinically, pre-RPE membranes were noted to form within the first weeks after surgery, and eyes remained hypotensive in the absence of notable inflammation. Morphologic studies demonstrated a variable response of RPE, ranging from atrophy to hypertrophy and hyperplasia. Pre-RPE membranes appeared to arise from both the RPE as well as from tags of peripheral retina and were of two morphologic types: spindle cell-collagenous membranes, and a highly cellular glial membrane. Bone formation within collagenous membranes was seen in four of six eyes studied.
Subject(s)
Retina/surgery , Animals , Eye/pathology , Membranes/pathology , Microscopy, Electron , Microscopy, Electron, Scanning , Pigment Epithelium of Eye/pathology , Pigment Epithelium of Eye/ultrastructure , Rabbits , Retina/pathologyABSTRACT
Fungal endophthalmitis is an uncommon complication of intraocular surgery. We report, to our knowledge, the first case of a postoperative Zygomycetes endophthalmitis that occurred after uncomplicated phacoemulsification and the insertion of a posterior chamber intraocular lens. Diagnosis, in the presence of negative aqueous and vitreous cultures, was confirmed by immunofluorescence staining of an anterior chamber inflammatory mass. Successful treatment of the eye with 20 micrograms of intraocular amphotericin B (Fungizone) may have been made possible in part by the fact that the posterior capsule remained intact, keeping the eye bicompartmental.
