A case report on atypically presented Congenital Solitary pelvic kidney with neglected Ureteropelvic junction obstruction (UPJO) successfully managed with open Anderson-Hynes pyeloplasty. Case report

摘要

INTRODUCTION AND IMPORTANCE: Kidney birth defects rank second to cardiac/skeletal anomalies. Horseshoe kidney is the most common renal fusion anomaly; pancake/lump kidney is the rarest. Congenital anomalies of the kidney and urinary tract (CAKUT), a spectrum varying in severity, include pelvic kidney and UPJO, major contributors to pediatric ESRD. To our knowledge, congenital solitary pelvic kidney with UPJO has not been reported. Here, we present a 13-year-old with congenital solitary pelvic kidney and neglected UPJO, atypical presentation, successfully managed with open Anderson-Hynes (A-H) pyeloplasty. This report follows updated Surgical CAse REport (SCARE) guidelines [14]. CLINICAL PRESENTATION: A 13-year-old from the Somali region of Ethiopia presented with 7-month history of vague lower abdominal pain, worsening over 2 months, associated with low grade intermittent fever, weight loss, anorexia, uneasiness, and fatigue. CLINICAL DISCUSSION: Pelvic kidney, a birth defect where kidneys fail to ascend, often presents asymptomatically but increases risk of trauma, UTIs, stones, and urological issues due to location/drainage. UPJO, obstructing urine flow from renal pelvis to ureter (intrinsic: adynamic segments/valves; extrinsic: crossing vessels), causes hydronephrosis/renal damage. Prenatal ultrasound detects UPJO; adults are often diagnosed incidentally. Evaluation involves history, examination, and imaging (US, CT urography, renal scintigraphy) to plan intervention. Symptomatic UPJO with obstruction/declining function warrants pyeloplasty (open/laparoscopic/robotic). Endoscopic approaches (endopyelotomy) may also considered. Congenital solitary pelvic kidney complicated by UPJO is a rare, challenging scenario with substantial risk of progressive renal parenchymal loss and declining renal function, emphasizing the need for evaluation. CONCLUSION: Congenital solitary pelvic kidney is a rare condition that can be complicated by UPJO, potentially leading to loss of renal function. In pelvic kidney with UPJO, imaging may not be straightforward, particularly with late presentation. UPJO should be considered as a cause of renal function loss in patients with pelvic kidney. Early diagnosis and management are crucial for preserving renal parenchyma.