Diffuse mesangial sclerosis: association with unreported congenital anomalies and placental enlargement

A case of diffuse mesangial sclerosis (DMS) associated with a number of und escribed congenital anomalies is reported. The occurrence of additional ano malies, especially ocular anomalies, is a common finding in DMS. However, n either megalocornea, Dandy-Walker malformation, postaxial hexadactyly, rock er-bottom feet, nor atrial septal defect, as observed in our patient, has b een reported previously in association with DMS. This case might be conside red an atypical manifestation of the Galloway-Mowat syndrome. In contrast t o most cases of DMS, the patient revealed intrauterine proteinuria as the p lacenta was enlarged to 31% of birth weight. This case demonstrates that th e large placenta, >25% of birth weight, is not only pathognomonic of the co ngenital nephrotic syndrome of the Finnish type but can also occur in DMS.