Since its initial characterization,<unk>  has been defined into five distinct subclassifications. There are those in the medical and research fields that use the term<unk>  to specifically refer to only cases of BRA, while other groups use the term to loosely refer to all instances of oligohydramnios and anhydramnios regardless of the specific cause. The assignment of nomenclature to the various causes (types) was employed in order to help clarify these discrepancies, but these subclassifications and nomenclature system have not caught on in the medical and research communities. Type OMIM Description Classic form n/a This term is traditionally used when the infant has bilateral renal agenesis (BRA), meaning that kidneys do not develop (malformation of the ureteric bud). True BRA also presents with bilateral agenesis of the ureters. After the creation of the nomenclature system for this<unk> , BRA was recognized as possibly being an extreme variation of<unk>  II. However, some clinicians and researchers still use the term classic<unk>  so as to emphasize that they are specifically referring to cases of BRA and not another form. Type I Type I is due to autosomal recessive polycystic kidney disease (ARPKD), which occurs at a frequency of approximately one in 16,000 infants. The kidneys of the fetus/neonate will be enlarged, have many small cysts filled with fluid, and will fail to produce an adequate volume of fetal urine. The liver and pancreas of the fetus may also show fibrosis and/or a cystic change. Type II Type II is usually due to renal agenesis, which can also fall under the category known as hereditary urogenital adysplasia or hereditary renal adysplasia (HRA). This is characterized by the complete agenesis or absence of one kidney and the remaining solitary kidney being small and malformed. Bilateral renal agenesis is believed to be the most extreme phenotypic variation of HRA. However, BRA is often referred to as classic<unk> , as it was this particular phenotype of neonates and fetuses that<unk>  originally reported in her 1946 manuscripts when characterizing this birth defect. Type III Type III is due to Autosomal dominant polycystic kidney disease (ADPKD) linked to mutations in the genes PKD1 and PKD2. While ADPKD is considered to be an adult-onset polycystic kidney disease, it can also present in the fetus and neonate in rare cases. Like ARPKD, ADPKD can also present with hepatic cysts and an enlarged spleen. An increased prevalence of vascular disease is also observed in these cases of ADPKD. Type IV n/a Type IV occurs when a longstanding obstruction in either the kidney or ureter leads to cystic kidneys or hydronephrosis. This can be due to chance, environment, or genetics. While these types of obstructions occur frequently in fetuses, they rarely tend to lead to fetal demise. Others Often cystic kidneys that do not fall under the classification of being polycystic will be termed as being multicystic renal dysplasia (MRD). Recently many cases of MRD have been linked to the mutations in the gene PUJO, however, this new possible genetic cause has not been assigned a<unk>  nomenclature number.Another cause of<unk>  (oligohydramnios or anhydramnios—little or no amniotic fluid) can be the rupturing of the amniotic sacs that contain the amniotic fluid of the fetus. This can happen spontaneously, by chance, environment, maternal trauma and in rare cases - maternal genetics.
Potter sequence
Lethal congenital contracture syndrome
Stafne defect
Crista dividens
Jordans' anomaly
Philadelphia basement kidnapping
Renal dysplasia-limb defects syndrome
Crisscross heart