Diabetic retinopathy in Mauriac's syndrome. Paradoxical deterioration with improved metabolic control.

We report four children with marked short stature, hepatomegaly, and delayed adolescence. Initial funduscopy demonstrated only occasional microaneurysms in two children and a single intraretinal hemorrhage in another. Improved control required large increases in insulin dosage. Growth rate improved significantly and hepatomegaly regressed. Puberty progressed rapidly in two older patients with poor final height. Paradoxically, with improved control, retinopathy progressed rapidly. One child with proliferative retinopathy in both eyes developed vitreous hemorrhage and blindness in one eye. Two required pan retinal photocoagulation with no further progression of their retinopathy. These rapidly progressive retinal changes remain unexplained. We advise caution when correcting metabolic derangements of diabetic patients who have been poorly controlled for a prolonged period.