DIDMOAD syndrome [Wolfram syndrome] with megacystis, megaureter and hydronephrosis

A case of DIDMOAD syndrome [diabetes insipidus, diabetes mellitus, optic atrophy, and nerve deafness] occurring as an autosomal recessive disorder is described. The patient presented with diabetes mellitus, diabetes insipidus, severe urinary tract dilatation, and asymptomatic involvement of optic and auditory nerves. The diabetes insipidus had both neurogenic and nephrogenic components. A marked reduction in urinary output was demonstrated following the administration of desmopressin. The possible factors that lead to functional obstruction with consequent urinary tract dilatation are discussed. Bladder neck resection was performed and an ileal conduit was constructed. Following the surgery there was an improvement in glomerular function