Idiopathic isolated abscess in an extraocular muscle in a child.

male predominance (76%), the majority with unilateral disease (95%). Aggressive management for early cases, including laser photocoagulation, cryotherapy, and drainage of subretinal exudates using pars plana vitrectomy techniques, may prevent neovascular glaucoma and a painful blind eye. Because the definitive genetic defects for both VATER association and Coats disease in humans are unknown, we can only speculate about the possible common genetic link. Recently, an adriamycin-induced rat model of the VATER association, with defects in the hedgehog gene pathway, has been developed. The secreted glycoprotein, sonic hedgehog (SHH), acts as an endodermal signal that controls gut and lung patterning. Interestingly, the SHH protein is also secreted by retinal ganglion cells to help optic disc astrocyte precursor cells to guide retinal axon growth, and to convert optic stalk neuroepithelial cells into pigmented cells. Both VATER and retinal defects can be produced by targeted SHH mutations. Ultrastructural retinal cell abnormalities are not infrequently found in pathologically examined eyes of patients with Coats disease. Nevertheless, it remains to be elucidated whether any aberrations in these pathways may explain the unusual clustering of specific defects in our case.