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Multicystic dysplastic kidney with ipsilateral abnormalities of genitourinary tract: experience in children
Thierry Merrot1, David B Lumenta, Stéphane Tercier
1Department of Pediatric Surgery, Hôpital Nord, Marseille, France. tmerrot@ap-hm.fr
Insights
Multicystic dysplastic kidney (MCDK) in children is often associated with ipsilateral internal genitalia malformations. Extended follow-up through puberty is crucial for timely detection and management of these related genitourinary anomalies.
Area of Science:
- Pediatric Urology
- Congenital Malformations
- Renal Imaging
Background:
- Multicystic dysplastic kidney (MCDK) is a common congenital renal anomaly.
- Associated genitourinary malformations can impact patient outcomes.
- Early identification and management are essential.
Purpose of the Study:
- To determine the incidence and nature of ipsilateral internal genitourinary malformations in children diagnosed with MCDK.
- To evaluate the management strategies for these associated anomalies.
- To establish optimal follow-up protocols for patients with MCDK.
Main Methods:
- Retrospective analysis of medical records and imaging studies for 93 pediatric patients with MCDK.
- Utilized ultrasonography, voiding cystourethrography, intravenous urography, and radionuclide renal imaging.
- Follow-up included serial ultrasonography for a mean of 6.54 years.
Main Results:
- 15% of patients (14/93) with MCDK had associated ipsilateral internal genitourinary malformations.
- Identified malformations included Gartner duct persistence, cystic masses, and blind-ending hemivagina.
- Genital anomalies were detected even after MCDK involution, with diagnoses occurring from infancy to adolescence.
Conclusions:
- A significant proportion of children with MCDK present with concurrent ipsilateral internal genitourinary malformations.
- Persistence of seminal cysts and Gartner ducts can occur irrespective of MCDK involution.
- Extended follow-up until the end of puberty is recommended for comprehensive management of MCDK-associated genitourinary anomalies.
Objectives:
To investigate the incidence, nature, and management of associated ipsilateral genitourinary malformations in children with multicystic dysplastic kidney (MCDK).
Methods:
In this retrospective study, we analyzed the medical records and imaging studies of 93 patients with MCDK. Patients underwent ultrasonography, voiding cystourethrography, intravenous urography, and radionuclide renal imaging studies during their first month of age.
Results:
A diagnosis of MCDK associated with malformation of the ipsilateral internal genitalia was confirmed in 11 patients after birth investigations of prenatal MCDK. Three were diagnosed at 1, 12, and 14 years of age because of epididymitis, pelvic pain associated with amenorrhea, and accidentally during lumbar pain assessment, respectively. The male/female sex ratio was 10:4. The left side was involved in 9 patients. We had 3 cases of Gartner duct persistence, 6 of cystic retrovesical and laterovesical masses with vanishing MCDK, 4 of cystic retrovesical or laterovesical masses with compressive MCDK, and 1 of a blind-ending hemivagina. Nine patients were periodically observed, and four underwent nephroureterectomy. All patients underwent 6-month follow-up examinations with ultrasonography (mean follow-up 6.54 years, range 36 months to 14 years).
Conclusions:
Of the 93 patients with MCDK, 14 (15%) had malformations of the ipsilateral internal genitalia. Persistence of seminal cysts in boys and Gartner ducts were encountered even if the MCDK had involuted. These results suggest that follow-up of patients with MCDK should be performed until the end of puberty to detect genitourinary malformations.
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