Caroli's disease is characterized by multifocal segmental dilatation of the intrahepatic bile ducts. It is a rare congenital condition, which appears to be autosomal recessively inherited in most cases. There are two forms of disease, one associated with congenital hepatic fibrosis and a simple form occurring alone. Recent reports suggest that the simple form may be as common as that with conge...

There are several congenital lung lesions that result in severe pulmonary hypoplasia and neonatal death. These malformations include congenital diaphragmatic hernia (CDH), oligohydramnios induced pulmonary hypoplasia, and congenital cystic adenomatoid malformation of the lung. Severely affected newborns are not salvageable by current surgical techniques or medical treatment, although one new ex...

Congenital cystic dilatation of the intrahepatic biliary ducts [Caroli’s disease (CD)] has been seldom seen in clinical practice. It is often associated with autosomal recessive polycystic kidney disease and congenital hepatic fibrosis. Here we report an image of a female asymptomatic patient with CD and polycystic kidney disease. A 51-year-old female patient with a one month history of pruritu...

Obstruction-induced fibrosis is a leading cause of end-stage renal failure in children. The pathophysiological mechanisms may involve apoptosis and the renin-angiotensin system. We studied apoptosis and fibrosis in a well-established neonatal pig model with unilateral partial ureteral obstruction (PUUO) induced during ongoing nephrogenesis in 2-day-old piglets. The role of angiotensin II (ANG I...

PURPOSE High-resolution magnetic resonance imaging (MRI) can now directly demonstrate innervation to extraocular muscles and quantify optic nerve size. A quantitative MRI technique was developed to study the oculomotor nerve (CN3) and applied to congenital fibrosis of extraocular muscles (CFEOM) and congenital oculomotor palsy. METHODS The subarachnoid portions of the CN3s were imaged with a ...