多囊性肾脏病变.ppt

Cystic disease of the kidneys in childhood is a confusing and complicated subject. Also the complex and often contradictory nomenclature used to describe cystic kidneys is not an aid to understanding the subject. This section aims to provide the sonographer with a simple approach to cystic kidneys in children. ● Multicystic kidney or multicystic dysplastic kidney—this refers to many cysts in one kidney often containing some dysplastic elements. Not all multicystic kidneys are dysplastic. The condition is still considered to be non-hereditary. If the multicystic kidney is unilateral, the other kidney may be normal, hydronephrotic or dysplastic. If bilateral, it is incompatible with life, and infants die soon after birth with hypoplastic lungs and/or renal failure. —Autosomal recessive polycystic kidneys were previously known as infantile polycystic kidneys. Confusingly these kidneys appear highly echogenic on ultrasound. There is generalized dilation of the collecting tubules. —Autosomal dominant renal disease was previously known as adult polycystic kidney disease. Cysts develop anywhere along the nephron. The ultimate diagnosis of the cystic renal disorder is not dependent on any one imaging modality and will depend on many factors. Sources of information when trying to come to the diagnosis should be collated from many areas, such as: ● obstetric history of the mother ● prenatal history and fetal ultrasonography ● family history information ● clinical examination of the child ● radiology of patient and parents ● laboratory data, for example DNA ● pathology if a biopsy is taken or from any other family members that may have had a biopsy or nephrectomy in the past. Ultrasound is still the imaging modality of choice in children, and the findings on ultrasound will direct further imaging as required. The ultrasound approach to any cystic renal disease in children must include observations about the following, which should be carefully stated in the ultr