Bilateral Multicystic Dysplastic Kidney Disease

Vladimir Lemaire, Robert D. Stewart
UT Southwestern Medical Center, Plano, Texas, United States of America.

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A 28-year-old patient, G10 P1081, was referred to our maternal fetal medicine unit at 23 weeks, 5 days for kidney cysts and anhydramnios. The fetus was female with negative genetic testing.

We present a case of bilateral multicystic dysplastic kidney disease (MCDK).

Multicystic dysplastic kidney disease is unilateral in approximately 75 to 80% of cases and due to abnormal development often associated with early obstruction of the collecting system (atretic ureters).

Ultrasound findings include enlarged kidneys with non-communicating macrocysts of variable size within the hyperechoic parenchyma. Severe olygohydramnios is usually present and the bladder cannot be visualized.

According to Paladini et al., the risk of chromosomal anomalies reaches 15  to 18% in MCDK's bilateral form, when isolated, and 25 to 28% when associated with other abnormalities. Associated non chromosomal syndromes include: brachio-otorenal syndrome, cerebro-reno-digital syndrome, VACTERL (vertebral anomalies, anal atresia, congenital heart defect, tracheo-esophageal fistula, limb anomalies), short-rib polydactyly syndrome, and Meckel-Gruber syndrome.

Differential diagnosis for bilateral MCDK includes Meckel-Gruber syndrome and bilateral obstructive cystic dysplasia. In bilateral obstructive cystic dysplasia, the bladder is dilated with thick walls. In Meckel-Gruber syndrome, extrarenal anomalies, such as cephalocele, microcephaly, and polydactyly, are always present.

Conservative treatment after 24 weeks of gestation is recommended due its unfavorable prognosis. Neonatal death occurs in the majority of cases due to pulmonary hypoplasia, which is a direct consequence of the severe olygohydramnios. It is crucial that an accurate and early diagnosis is reached, as it would allow a timely, safer, and less traumatic management of the pregnancies affected by this condition. wrgweg

Reference:

Paladini, D et al. "Multicystic Dysplastic Kidney Disease". Ultrasound of Congenital Fetal Anomalies: Differential Diagnosis and Prognostic Indicators. Boca Raton, FL: CRC Press; 2014. pgs 322-323.

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