Challenges of Management of Symptomatic Congenital Pelvic - Ureteric Junction Obstruction in Children: Case Series.

Abstract

Introduction: Congenital pelvi-ureteric junction obstruction usually presents later in adulthood. It may present early with severe symptoms such aspyonephrosis or renal impairment which may require intervention. There may be challenges in getting clear diagnosis from imaging, urinary diversion, definitive treatment and post-operative management. Objective: To present six cases of symptomatic congenital pelvi-ureteric junction obstruction in children. Case series: We present six cases of symptomatic pelvi-ureteric junction obstruction (PUJO) which was bilateral in two of the cases. Abdominopelvic ultrasound scan revealed hydronephrosis ± pyonephrosis. Abdominal ultrasound revealed complex cystic renal mass in a patient with ectopic kidney which was confirmed to be PUJO on CT uropathy. Nephrostomy and subsequent antegrade pyelogram were done in two patients after drainage of pyonephrosis. There was difficulty in performing percutaneous nephrostomy in one patient with pyonephrosis and partial PUJO. Intravenous urogram showed non-functioning kidney in patients with severe hydronephrosis. The patients had Anderson-Hynes pyeloplasty ± Boari flap uretero-neocystostomy, pyelo-vesicostomy in 3 patients with ectopic kidneys and short/atretic ureter. Uretero-neocystostomy using Boari flap was done after re-exploration for an atretic ureter with non- functioning kidney following Andreson-Hynes pyeloplasty despite patent ureter as confirmed by ureteroscopy. A patient developed persistent retroperitoneal urinary drainage which spontaneously stopped. The ureteric stents were removed at the appropriate time and the patients were a symptomatic presently. Conclusion: The challenges of Pelvi-ureteric junction management may be diagnostic and/or therapeutic. The imaging studies may not be definite or unremarkable. Urinary diversion and definitive surgery could be challenging due to anatomic factors.