Follow-up of unilateral multicystic kidney dysplasia after prenatal diagnosis

J Matern Fetal Neonatal Med. 2003 Sep;14(3):177-86. doi: 10.1080/jmf.14.3.177.186.

Abstract

Objectives: Since the introduction of antenatal diagnostic screening, multicystic kidney dysplasia (MCKD) has been diagnosed in 1 of 4300 live births. We analyze our own experience and demonstrate a management regime based on these results and existing studies.

Methods: Retrospective data analysis was carried out in 110 patients, prenatally diagnosed with MCDK. A total of 93 patients with confirmed diagnosis of unilateral MCDK were born alive and followed up in our institutions.

Results: A total of 110 unilateral cystic kidneys were prenatally diagnosed; 93 were confirmed postnatally. These children were retrospectively allocated to two treatment groups: 51 were operated upon; 42 were treated conservatively. A micturition cystourethrogram was performed 88 times, yielding 20 pathological findings. Vesicoureteral reflux was identified 11 times (12.5%). Eleven children (12%) had associated non-urological abnormalities. Cardiac and musculoskeletal malformations predominated. After 1995, nephrectomy was performed only when clinically indicated. Thirty-seven children were treated conservatively for between 4 months and 6 years (mean 33 months); 28 dysplastic kidneys exhibited size reduction. Complete involution was noted in 16 of 28 children between 7 and 29 months (mean 16.2 months).

Conclusion: This study provides evidence that neonatal nephrectomy of unilateral MCDK is rarely required. It also shows that the discussed malformation is not an isolated developmental abnormality. In a high proportion, associated urogenital abnormalities were present and therapeutically relevant, and determined the overall prognosis.

MeSH terms

  • Abnormalities, Multiple
  • Blood Pressure / physiology
  • Child
  • Child, Preschool
  • Female
  • Fetal Diseases / diagnosis*
  • Follow-Up Studies
  • Humans
  • Hydronephrosis / complications
  • Infant
  • Infant, Newborn
  • Kidney Function Tests
  • Multicystic Dysplastic Kidney / complications
  • Multicystic Dysplastic Kidney / diagnosis*
  • Multicystic Dysplastic Kidney / physiopathology
  • Multicystic Dysplastic Kidney / therapy
  • Pregnancy
  • Prenatal Diagnosis*
  • Retrospective Studies
  • Ureteral Diseases / complications
  • Urinary Tract Infections / complications
  • Vesico-Ureteral Reflux / complications