renal–hepatic–pancreatic dysplasia renal–hepatic–pancreatic dysplasia renal–hepatic–pancreatic dysplasia is an autosomal recessive congenital disorder characterized by pancreatic fibrosis, renal dysplasia and hepatic dysgenesis. it is usually fatal soon after birth.an association with nphp3 has been described. it was characterized in 1959. renal–hepatic–pancreatic dysplasiaother namesivemark ii syndrome, renohepaticopancreatic dysplasiagross photo of liver and pancreas showing multiple cysts in the latter in a patient with renal–hepatic–pancreatic dysplasia