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Multicystic Dysplastic Kidney (MCDK)
Definition of Multicystic Dysplastic Kidney (MCDK)
Multicystic dysplastic kidney (MCDK) is a severe form of congenital malformation of the kidney characterized by renal noncommunicating cysts and the absence of a normal pyeloureteral system (Hains et al, 2009).
Incidence 1 of 4300 live birth. Multicystic renal dysplasia is the most common abdominal tumor in the neonatal period and the most common cystic lesion of the kidney in childhood.
The etiology is unclear, there are several theories leading to a multicystic dysplastic kidney:
- Fetal ureteral atresia
- Abnormal induction of the metanephritic blastema by the ureteral bud.
- Viral infections
Most cases of multicystic renal dysplasia are subject to spontaneous involution.
Signs and Symptoms of Multicystic Renal Dysplasia
- Abdominal tumors in Newborns and children.
- Later, spontaneous involution is typical and may lead to the misdiagnosis of renal agenesia.
- Associated malformations of the genitourinary tract: contralateral UPJ obstruction in 3–12%, contralateral vesicoureteral reflux in 5–20%, benign cystic dysplasia of the testis
- Fatal Potter's syndrome in bilateral disease possible
Diagnostic Work-Up in MCDK
- Ultrasound of the kidneys: cystic tumor in the renal fossa. Regular examinations are necessary due to the slightly increased risk of Wilms tumor.
- Voiding Cystourethrogram: for differential diagnosis and for the exclusion of contralateral vesicoureteral reflux.
- Renal scintigraphy: absent renal function on affected side.
Treatment of Multicystic Renal Dysplasia
Perform ultrasound imaging, blood pressure test and creatinine every 6–12 month until the age of 8 years or until involution of the multicystic renal dysplasia. Perform nephrectomy if the mass is causing symptoms (flank pain, infections, hypertension) or if the mass is suspicious for malignancy.
|Conggenital nephrotic syndr.||Index||Benign multilocular cysts|
Index: 1–9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
- Hains, D. S.; Bates, C. M.; Ingraham, S. & Schwaderer, A. L.
- Management and etiology of the unilateral multicystic dysplastic
kidney: a review.
Pediatr Nephrol, 2009, 24, 233-241.
Deutsche Version: Multizystische Nierendysplasie