Left Renal Agenesis and Right Ureterohydronephrosis in a Newborn

Authors

Keywords:

renal agenesis; ureterohydronephrosis; newborn; chronic kidney failure; renal tubular acidosis.

Abstract

Introduction: Unilateral renal agenesis is a rare congenital malformation that may be associated with anomalies of the contralateral kidney and can lead to chronic kidney disease from the neonatal period.

Objective: To describe a case of left renal agenesis with right ureterohydronephrosis in a newborn who presented with advanced kidney failure during the neonatal period.

Case presentation: A male newborn, son of a 17-year-old mother, with no perinatal risk factors. Prenatal diagnosis of left renal agenesis was confirmed at birth by ultrasound, which also showed right ureterohydronephrosis (ureter 8 mm and renal pelvis 12 mm). Serum creatinine progressively rose to 252 µmol/l in the fourth week of life, with an estimated glomerular filtration rate of 5 ml/min/1.73m² (Schwartz formula). The patient presented with metabolic acidosis (HCO3- 19.5 mmol/l, BE -4.3) and hyperkalemia (K+ 6.1 mmol/l). Urine pH was elevated at 6.1. Treatment with oral sodium bicarbonate and ceftriaxone was indicated. The patient was discharged with a diagnosis of left renal agenesis, neonatal stage V chronic kidney disease, and renal tubular acidosis.

Conclusions: The association of unilateral renal agenesis and ureterohydronephrosis of the solitary kidney constitutes a high-risk condition for the development of advanced chronic kidney disease in the neonate, requiring close nephrological follow-up and complementary imaging studies (cystourethrography and renal scintigraphy) to guide prognosis.

Downloads

Download data is not yet available.

References

1. Plutecki D, Kozioł T, Bonczar M, Ostrowski P, Skorupa A, Matejuk S, et al. Renal agenesis: A meta-analysis of its prevalence and clinical characteristics based on 15 641 184 patients. Nephrology (Carlton). 2023;28(10):525-33. DOI: https://doi.org/10.1111/nep.14190

2. Ashley DJB, Mostofi FK. Renal agenesis and dysgenesis. J Urol. 1960;83(3):211-30. DOI: https://doi.org/10.1016/S0022-5347(17)65695-7

3. Martínez L, Fernández A. Incidencia de agenesia renal en América: estudio multicéntrico. Nefrol Latinoam. 2019;16(2):88-94. DOI: https://doi.org/10.24875/NEFRO.19000015

4. García Pérez M, Rodríguez L, Hernández R. Registro Cubano de Malformaciones Congénitas: incidencia y características de la agenesia renal 2018-2023. Rev Cubana Pediatr. 2024 [acceso 01/04/2026];96(1):e2458. Disponible en: http://www.revpediatria.sld.cu/index.php/ped/article/view/2458

5. Schreuder MF, Westland R, van Wijk JAE. Unilateral renal agenesis: a systematic review of clinical features and long-term outcomes. Eur J Pediatr. 2019;178(8):1155-63. DOI: https://doi.org/10.1007/s00431-019-03412-4

6. Akyol Onder EN, Yilmaz O, Taneli C, Ertan P. Follow up of renal outcomes in children with solitary kidney. Pediatr Int. 2023;65(1):e15488. DOI: https://doi.org/10.1111/ped.15488

7. Sanna-Cherchi S, Ravani P, Corbani V, Parodi S, Haupt R, Piaggio G, et al. Renal outcome in patients with congenital solitary functioning kidney: a 40-year follow-up study. J Am Soc Nephrol. 2018;29(1):214-24. DOI: https://doi.org/10.1681/ASN.2017020189

8. Kiblawi R, Pohl M, Dittrich K, Hölttä T, Zurowska A, Dursun I, et al. Unilateral renal agenesis and its association with contralateral kidney anomalies: a multicenter study. Pediatr Nephrol. 2022;37(8):1857-65. DOI: https://doi.org/10.1007/s00467-021-05394-y

9. Groen in 't Woud S, Westland R, Feitz WFJ, Roeleveld N, van Wijk JAE, van der Zanden LFM, et al. Clinical Management of Children with a Congenital Solitary Functioning Kidney: Overview and Recommendations. Eur Urol Open Sci. 2021;25:11-20. DOI: https://doi.org/10.1016/j.euros.2021.01.003

10. Madariaga Domínguez L, Ordóñez Álvarez FÁ. Manejo de las anomalías renales y del tracto urinario detectadas por ecografía prenatal. Uropatías obstructivas. Protoc Diagn Ter Pediatr. 2022 [acceso 18/04/2026];1:253-70. Disponible en: https://www.aeped.es/protocolos/

11. Capone VP, Morello W, Guarino S, La Scola C, Mencarelli F, Pasini A, et al. Congenital anomalies of the kidney and urinary tract: a clinical review. Ital J Pediatr. 2021;47(1):100. DOI: https://doi.org/10.1186/s13052-021-01053-0

12. Schwartz GJ, Muñoz A, Schneider MF, Mak RH, Kaskel F, Warady BA, et al. New equations to estimate GFR in children with CKD. J Am Soc Nephrol. 2009;20(3):629-37. DOI: https://doi.org/10.1681/ASN.2008030287

13. National Kidney Foundation. KDIGO 2024 Clinical Practice Guideline for the Evaluation and Management of Chronic Kidney Disease. Kidney Int. 2024;105(4S):S1-S150. DOI: https://doi.org/10.1016/j.kint.2023.10.018

14. La Scola C, Ammenti A, Bertulli C, Bodria M, Brugnara M, Camilla R, et al. Management of the congenital solitary kidney: consensus recommendations of the Italian Society of Pediatric Nephrology. Pediatr Nephrol. 2022;37(9):2185-207. DOI: https://doi.org/10.1007/s00467-022-05528-y

15. Rodríguez Ramos JF, Herrera Miranda GL. Guía de práctica clínica para la atención a niños y adolescentes con enfermedad renal crónica. MEDISAN. 2024 [acceso 18/04/2026];28(2):e4568. Disponible en: http://scielo.sld.cu/scielo.php?script=sci_arttext&pid=S1029-30192024000200007

Published

2026-05-12

How to Cite

1.
Crespo Campos A, Yasell Aroche RE, Sarmiento Portal Y, Isaac Ferrer OL. Left Renal Agenesis and Right Ureterohydronephrosis in a Newborn. Rev Cubana Pediatría [Internet]. 2026 May 12 [cited 2026 May 13];98. Available from: https://revpediatria.sld.cu/index.php/ped/article/view/8432

Issue

Section

NEONATOLOGÍA