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Congenital solitary functioning kidney and other associated congenital malformations

Tânia Carvalho1, Telma Francisco2, Raquel Santos2, Margarida Abranches2

1 – Pediatric Department, Hospital Vila Franca de Xira, Portugal
2 – Pediatric Nephrology Unit, Hospital Dona Estefânia, CHLC-EPE, Lisbon, Portugal

- 51th Annual Scientific Meeting of the European Society for Paediatric Nephrology, Antalya (Turkey), 3-6/October/2018 (E-poster with oral session)

Introduction: Congenital solitary functioning kidney (CSFK) is associated to other congenital anomalies. Most of them involve urogenital tract, cardiac, skeletal and central nervous system. There are also some syndromes associated to renal malformations.
Objective: To determine the prevalence of associated malformations in children with CSFK.
Material and methods: We reviewed 134 electronic processes of children with CFSK followed in a terciary department of paediatric nephrology between 2012 and 2016.
Results: Ages ranged from neonatal period to 18 years old (6.6±4.4 years). Prenatal diagnosis of solitary kidney was made in 89 cases (66.4%), and agenesia was the etiology in 33.5%. We found 41 children with at least one more malformation (30.6 %). One quarter of children with postnatal diagnosis of CSFK (7/28) had other anomalies. From all children with diagnosis of agenesia, 80% had at least one more malformation, whereas only 30% of other CSFK’s etiology had other anomaly indentified. Urological anomalies were the most frequent (34.1%); however, 27/41 children (65.8%) had at least one malformation of other system, without an urological anomaly. Gastrointestinal anomalies were identified 21.9%, cardiovascular anomalies 19.5%, genital malformations in 19.5%, neurological anomalies in 14.6% and other organ anomalies in 17%. The non-urological congenital malformation more frequent was single umbilical artery (12.2%). Fourteen children (34.1%) had their anomalies included in a syndrome, all them associated with renal anomalies in previous studies. Chronic kidney disease was seen in five of 134 children, three of which with another malformation.
Conclusions: This study intends to recall that CSFK may not be the only congenital malformation in a child. It also highlights that there are other anomalies besides urological tract that may be present and must be investigated, especially if there is a diagnosis of true renal agenesia. A good prenatal care and careful follow-up of children with CSFK are essential.

Keywords: Congenital solitary functioning kidney, congenital malformations