Multicystic dysplastic kidney
Encyclopedia
Multicystic dysplastic kidney (MCDK) is a condition that results from the malformation of the kidney
during fetal development. The kidney consists of irregular cysts of varying sizes and has no function.
Multicystic dysplastic kidney is the most common type of renal cystic disease, and it is one of the most common causes of an abdominal mass in infants
characteristic of Potter's syndrome. The bilateral condition is incompatible with survival, although one infant reportedly survived for 69 days.
The contralateral system frequently is abnormal as well. Contralateral ureteropelvic junction obstruction is found in 3% to 12% of infants with multicystic kidney and contralateral vesicoureteral reflux is seen even more often, in 18% to 43% of infants . Because the high incidence of reflux, voiding cystourethrography usually has been considered advisable in all newborns with a multicystic kidney.
Cystic dysplasia of the testes, a benign rare lesion of the rete testis, may be associated with an ipsilateral multicystic dysplastic kidney.
Hypertension can complicate the clinical course. Malignant transformation to Wilm's tumor has been reported.
examination before birth. Mean age at the time of antenatal diagnosis is about 28 weeks, with a range of 21 to 35 weeks.
DMSA scan shows no uptake in affected kidney.
of MCDK is estimated to be 1 in every 2400 live births.
Males are more likely to have unilateral multicystic dysplastic kidneys (2.4:1), whereas bilateral multicystic kidneys appear twice as often in females.
Kidney
The kidneys, organs with several functions, serve essential regulatory roles in most animals, including vertebrates and some invertebrates. They are essential in the urinary system and also serve homeostatic functions such as the regulation of electrolytes, maintenance of acid–base balance, and...
during fetal development. The kidney consists of irregular cysts of varying sizes and has no function.
Multicystic dysplastic kidney is the most common type of renal cystic disease, and it is one of the most common causes of an abdominal mass in infants
Signs and symptoms
When a diagnosis of multicystic kidney is made in utero by ultrasound, the disease is found to be bilateral in 19% to 34% of cases. Those with bilateral disease often have other severe deformities or polysystemic malformation syndromes. In bilateral cases, the newborn has the classic abnormal facies and oligohydramniosOligohydramnios
Oligohydramnios is a condition in pregnancy characterized by a deficiency of amniotic fluid. It is the opposite of polyhydramnios.-Diagnosis:Diagnosis is made by ultrasound measurement of the amniotic fluid index...
characteristic of Potter's syndrome. The bilateral condition is incompatible with survival, although one infant reportedly survived for 69 days.
The contralateral system frequently is abnormal as well. Contralateral ureteropelvic junction obstruction is found in 3% to 12% of infants with multicystic kidney and contralateral vesicoureteral reflux is seen even more often, in 18% to 43% of infants . Because the high incidence of reflux, voiding cystourethrography usually has been considered advisable in all newborns with a multicystic kidney.
Cystic dysplasia of the testes, a benign rare lesion of the rete testis, may be associated with an ipsilateral multicystic dysplastic kidney.
Hypertension can complicate the clinical course. Malignant transformation to Wilm's tumor has been reported.
Diagnosis
MCDK is usually diagnosed by ultrasoundUltrasound
Ultrasound is cyclic sound pressure with a frequency greater than the upper limit of human hearing. Ultrasound is thus not separated from "normal" sound based on differences in physical properties, only the fact that humans cannot hear it. Although this limit varies from person to person, it is...
examination before birth. Mean age at the time of antenatal diagnosis is about 28 weeks, with a range of 21 to 35 weeks.
DMSA scan shows no uptake in affected kidney.
Treatment
MCDK is not treatable. However, the patient is observed periodically for the first few years during which ultrasounds are generally taken to ensure the healthy kidney is functioning properly and that the unhealthy kidney is not causing adverse effects. In some cases like renal hypertension or malignant transformation, the unhealthy kidney is removed entirely.Epidemiology
In the United States, the incidenceIncidence (epidemiology)
Incidence is a measure of the risk of developing some new condition within a specified period of time. Although sometimes loosely expressed simply as the number of new cases during some time period, it is better expressed as a proportion or a rate with a denominator.Incidence proportion is the...
of MCDK is estimated to be 1 in every 2400 live births.
Males are more likely to have unilateral multicystic dysplastic kidneys (2.4:1), whereas bilateral multicystic kidneys appear twice as often in females.