Atypical segmental multicystic dysplastic kidney with severe reflux in a newborn
Segmental cases of multicystic dysplastic kidney (SMCDK) are rare and hard to diagnose, imaging characteristics are not evident. SMCDK can be presented with duplex collecting system, vesicoureteral reflux (VUR), pyeloureteric junction obstruction and orthotopic ureterocele. Typical cases are localiz...
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| Format: | Article |
| Language: | English |
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Elsevier
2022-11-01
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| Series: | Journal of Pediatric Surgery Case Reports |
| Subjects: | |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S221357662200269X |
| _version_ | 1811342186446323712 |
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| author | Dorottya Balogh Viktória Vass Bálint Sulya Ádám Fukász Bálint Szmodics András Kiss |
| author_facet | Dorottya Balogh Viktória Vass Bálint Sulya Ádám Fukász Bálint Szmodics András Kiss |
| author_sort | Dorottya Balogh |
| collection | DOAJ |
| description | Segmental cases of multicystic dysplastic kidney (SMCDK) are rare and hard to diagnose, imaging characteristics are not evident. SMCDK can be presented with duplex collecting system, vesicoureteral reflux (VUR), pyeloureteric junction obstruction and orthotopic ureterocele. Typical cases are localized to the upper pole of a duplex collecting system. Atypical cases are rarely reported. We present an unique atypical SMCDK case, with unconventional disease manifestation to bring attention to the variable presentation of this rare disorder and to draw attention to differential diagnostic challenges of cystic kidney malformations.In our patient newborn screening abdominal ultrasound detected severe hydronephrosis and hydroureter of the lower pole of the right kidney. Dynamic renal scintigraphy and sonocystography confirmed duplication of both collecting systems and severe VUR in the right lower pole. Heminephrectomy was performed. The suspected diagnosis of atypical SMCDK was confirmed by histopathology. The patient recovered uneventfully.This unusual case of atypical SMCDK highlights the importance of considering the possibility of SMCDK in patients with partial kidney malformation or unusual appearance of congenital kidney disease. |
| first_indexed | 2024-04-13T19:07:03Z |
| format | Article |
| id | doaj.art-8638cc86d1934e2db163840f0cd76eb2 |
| institution | Directory Open Access Journal |
| issn | 2213-5766 |
| language | English |
| last_indexed | 2024-04-13T19:07:03Z |
| publishDate | 2022-11-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Journal of Pediatric Surgery Case Reports |
| spelling | doaj.art-8638cc86d1934e2db163840f0cd76eb22022-12-22T02:33:57ZengElsevierJournal of Pediatric Surgery Case Reports2213-57662022-11-0186102442Atypical segmental multicystic dysplastic kidney with severe reflux in a newbornDorottya Balogh0Viktória Vass1Bálint Sulya2Ádám Fukász3Bálint Szmodics4András Kiss5Semmelweis University, 26. Üllői street, Budapest, 1085, Hungary; Department of Pediatric Urology, Heim Pal Children's Hospital, 22-24. Madarász Viktor street, Budapest, 1131, Hungary; Corresponding author. Department of Pediatric Urology, Heim Pal Children's Hospital, 22-24 Madarász Viktor street, Budapest, 1131, Hungary.Department of Pathology, Heim Pal Children's Hospital, 86. Üllői street 86, Budapest, 1089, HungaryDepartment of Pediatric Urology, Heim Pal Children's Hospital, 22-24. Madarász Viktor street, Budapest, 1131, HungaryDepartment of Pediatric Urology, Heim Pal Children's Hospital, 22-24. Madarász Viktor street, Budapest, 1131, HungaryDepartment of Pediatric Urology, Heim Pal Children's Hospital, 22-24. Madarász Viktor street, Budapest, 1131, HungaryDepartment of Pediatric Urology, Heim Pal Children's Hospital, 22-24. Madarász Viktor street, Budapest, 1131, HungarySegmental cases of multicystic dysplastic kidney (SMCDK) are rare and hard to diagnose, imaging characteristics are not evident. SMCDK can be presented with duplex collecting system, vesicoureteral reflux (VUR), pyeloureteric junction obstruction and orthotopic ureterocele. Typical cases are localized to the upper pole of a duplex collecting system. Atypical cases are rarely reported. We present an unique atypical SMCDK case, with unconventional disease manifestation to bring attention to the variable presentation of this rare disorder and to draw attention to differential diagnostic challenges of cystic kidney malformations.In our patient newborn screening abdominal ultrasound detected severe hydronephrosis and hydroureter of the lower pole of the right kidney. Dynamic renal scintigraphy and sonocystography confirmed duplication of both collecting systems and severe VUR in the right lower pole. Heminephrectomy was performed. The suspected diagnosis of atypical SMCDK was confirmed by histopathology. The patient recovered uneventfully.This unusual case of atypical SMCDK highlights the importance of considering the possibility of SMCDK in patients with partial kidney malformation or unusual appearance of congenital kidney disease.http://www.sciencedirect.com/science/article/pii/S221357662200269XSegmental multicystic dysplastic kidneyVesicoureteral refluxCase report |
| spellingShingle | Dorottya Balogh Viktória Vass Bálint Sulya Ádám Fukász Bálint Szmodics András Kiss Atypical segmental multicystic dysplastic kidney with severe reflux in a newborn Journal of Pediatric Surgery Case Reports Segmental multicystic dysplastic kidney Vesicoureteral reflux Case report |
| title | Atypical segmental multicystic dysplastic kidney with severe reflux in a newborn |
| title_full | Atypical segmental multicystic dysplastic kidney with severe reflux in a newborn |
| title_fullStr | Atypical segmental multicystic dysplastic kidney with severe reflux in a newborn |
| title_full_unstemmed | Atypical segmental multicystic dysplastic kidney with severe reflux in a newborn |
| title_short | Atypical segmental multicystic dysplastic kidney with severe reflux in a newborn |
| title_sort | atypical segmental multicystic dysplastic kidney with severe reflux in a newborn |
| topic | Segmental multicystic dysplastic kidney Vesicoureteral reflux Case report |
| url | http://www.sciencedirect.com/science/article/pii/S221357662200269X |
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