Abstract
Background
We report the long-term follow-up of children with antenatally detected unilateral multicystic dysplastic kidney (MCDK) who were followed between 1985 and 2009.
Methods
Involution rates were documented over time based on the initial size of the MCDK, as documented on postnatal ultrasound (USS), as well as long-term complications and renal function.
Results
In 323 patients (182 male), 10 % of MCDK had involuted, as evidenced on the first postnatal USS, with survival function analysis showing the probability of complete involution to be 35 % in 249 patients by 2 years of age, 47 % in 180 patients by 5 years of age and 62 % in 94 patients by 10 years of age. There was a significant difference in the involution rates of MCKD at the 10-year follow-up between MCDK with an initial size of >5 cm versus MCDK with an initial size of ≤5 cm (p < 0.0001). No patients in the whole cohort developed sustained hypertension or malignancy during a median follow-up of 10.1 years (range 0.3–15.4 years). Median estimated glomerular filtration rate (eGFR) in 76 patients (7 at 5 years, 69 at 10 years) was 93 ml/min/1.73 m2 (range 46–175 ml/min/1.73 m2), with 40 (53%) having an eGFR of between 90 and 140 ml/min/1.73 m2. Twenty-three (30 %) of the 76 patients at 10 years had normal eGFR (90–140 ml/min/1.73 m2) as well as complete involution of the MCDK, compensatory hypertrophy of the contralateral kidney, no proteinuria and no hypertension.
Conclusions
Larger MCDK at birth are less likely to involute during the first decade of life. However, conservative management remains justified due to the lack of complications. A minority of patients fulfil current criteria for discharge from specialty follow-up at 10 years.
Similar content being viewed by others
References
Mallik M, Watson AR (2008) Antenatally detected urinary tract abnormalities: more detection but less action. Pediatr Nephrol 23:897–904
Schreuder MF, Westland R, van Wijk JAE (2009) Unilateral multicystic dysplastic kidney: a meta-analysis of observational studies on the incidence, associated urinary tract malformations and the contralateral kidney. Nephrol Dial Transpl 24:1810–1818
Aslam M, Watson AR, Trent & Anglia MCDK study group (2006) Unilateral multicystic dysplastic kidney: long term outcomes. Arch Dis Child 91:820–823
John U, Rudnik-Schoneborn S, Zerres K, Misselwitz J (1998) Kidney growth and renal function in unilateral multicystic dysplastic kidney disease. Pediatr Nephrol 12:567–571
Hains DS, Bates CM, Ingraham S, Schwaderer AL (2009) Management and etiology of the unilateral multicystic dysplastic kidney: a review. Pediatr Nephrol 24:233–241
Narchi H (2005) Risk of hypertension with multicystic dysplastic kidney disease: a systematic review. Arch Dis Child 90:921–924
Narchi H (2005) Risk of Wilms’ tumour with multicystic kidney disease: a systematic review. Arch Dis Child 90:147–149
de Man SA, Andrea JL, Bachman H, Grobbee DE, Isben KK, Laaser U, Lippert P, Hofman A (1991) Blood pressure in children: pooled findings in six European studies. J Hypertension 114:109–114
National High Blood Pressure Education Program Working Group on High Blood Pressure in Children and Adolescents (2004) The fourth report on the diagnosis, evaluation and treatment of high blood pressure in children and adolescents. Pediatr 114(2 Suppl 4th Report):555–576
Konus OL, Ozdemir A, Akkaya A, Erbas G, Celik H, Sl I (1978) Normal liver, spleen and kidney dimensions in neonates, infants and children. Evaluation with sonography. Am J Roentgenol 171:1693–1698
Schwartz GJ, Munoz A, Schneider M, Mak RH, Kasdel F, Warady BA, Furth S (2009) New equations to estimate GFR in children with CKD. J Am Soc Nephrol 20:629–637
Shaheen IS, Watson AR, Broderick N (2005) Multicystic dysplastic kidney and pelviureteric junction obstruction. Pediatr Surg Int 21:282–284
Cambio AJ, Evans CP, Kurzrock EA (2008) Non-surgical management of multicystic dysplastic kidney. BJU Int 101:1464–4096
Rabelo EA, Oliveira EA, Diniz JS, Silva JM, Filgueiras MT, Pezzuti IL, Tatsuo ES (2004) Natural history of multicystic kidney conservatively managed: a prospective study. Pediatr Nephrol 19:1102–1107
Luque-Mialdea R, Martin-Crespo R, Cebrian J, Moreno L, Carrero C, Fernande A (2007) Does the multicystic dysplastic kidney really involute? The role of the retroperitoneoscopic approach. J Pediatr Urol 3:48–52
La Salle HD, Stock JA, Hanna MK (1997) Insurability of children with congenital urological anomalies. J Urol 158[Suppl 3]:1312–1315
Elder JS, Hladky D, Selzmann AA (1995) Outpatient nephrectomy for non-functioning kidneys. J Urol 154[suppl 2]:712–714
Ismaili K, Avni FE, Alexander M, Schulman C, Collier F, Hall M (2005) Routine voiding cystourethography is of no value in neonates with unilateral multicystic dysplastic kidney. J Pediatr 146:759–763
Homsy YL, Anderson JH, Oudjhane K, Russo P (1997) Wilms tumour and multicystic dysplastic disease. J Urol 158:2256–2260
Beckwith BJ (1998) Editorial comment. J Urol 158:2258–2259, discussion 2259-2260
Psooy K (2010) Multicystic dysplastic kidney in the neonate: the role of the urologist. Can Urol Assoc 4:95–97
González Celedón C, Bitson M, Tullus K (2007) Progression of chronic renal failure in children with dysplastic kidneys. Pediatr Nephrol 22:1014–1020
Weinstein A, Goodman TR, Iragorri S (2008) Simple multicystic dysplastic kidney disease: End points for subspecialty follow up. Pediatr Nephrol 23:111–116
Mansoor O, Chandar J, Rodriguez MM, Abitbol CL, Seeherunvong W, Freundlich M, Zilleruelo G (2011) Long-term risk of chronic kidney disease in unilateral multicystic dysplastic kidney. Pediatr Nephrol 26:597–603
Vu KH, Van Dyck M, Daniels H, Proesmans W (2008) Renal outcome of children with one functioning kidney from birth. A study of 99 patients and a review of the literature. Eur J Pediatr 167:885–890
Abou Jaoude P, Dubourg L, Bacchetta J, Berthiller J, Ranchin B, Cochat P (2011) Congenital versus acquired solitary kidney: is the difference relevant? Nephrol Dial Transplant 26:2188–2194
Westland R, Schreuder MF, Bokenkamp A, Spreeuwenberg MD, van Wijk JA (2011) Renal injury in children with a solitary functioning kidney: the KMONO study. Nephrol Dial Transpl 26:1533–1541
Sanna-Cherchi S, Ravani P, Corbani V, Parodi S, Haupt R, Piaggio G, Innocenti ML, Somenzi D, Trivelli A, Caridi G, Izzi C, Scolari F, Mattioli G, Allegri L, Ghiggeri GM (2009) Renal outcome in patients with congenital anomalies of the kidney and urinary tract. Kidney Int 76:528–533
Acknowledgements
We thank Judith Hayes for maintaining the database and Dr. Nigel Broderick, Dr. John Somers and Dr. Kath Halliday for radiology advice.
Members of the Trent & Anglia MCDK study group include: Dr. Jag Ahluwalia, Addenbrooke’s Hospital, Cambridge, UK; Dr. Peter Houtman, Leicester Royal Infirmary, UK; Dr. Simon Rhodes, Kings Mill Hospital, UK; Dr. Alastair Scammell, Lincoln County Hospital, UK; Dr. Richard Bowker, Derby Children’s Hospital, UK; Dr. Gail Moss, Sheffield Children’s Hospital, UK; Dr. Margaret Crawford, Pilgrim Hospital, Boston, UK; Dr. Henry Mulenga, Bassetlaw District Hospital, Worksop, UK.
Author information
Authors and Affiliations
Consortia
Corresponding author
Rights and permissions
About this article
Cite this article
Hayes, W.N., Watson, A.R. & Trent & Anglia MCDK Study Group. Unilateral multicystic dysplastic kidney: does initial size matter?. Pediatr Nephrol 27, 1335–1340 (2012). https://doi.org/10.1007/s00467-012-2141-9
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00467-012-2141-9