Exomphalos with macroglossia: A study of eleven cases

Summary In 11 cases of exomphalos with macroglossia other features of the syndrome, which is sometimes familial, included high birth weight, accelerated postnatal growth and skeletal development, facial nevus flammeus, abnormal pinnae and other cranio-facial characteristics, a diaphragmatic anomaly, renal enlargement with abnormal histological features, adrenal cytomegaly and possibly pancreatic islet-cell hyperplasia. The reported occurrence of neo-natal hypoglycaemia in this syndrome is stressed.