Congenital Bilateral Mid Ureteral Stenosis: A Rare Finding

Abstract

We report a case of an 11-year-old male child who presented with left sided lump in abdomen since one year. Patient had a history of intermittent constipation but no history of any urinary tract complaints. He had no history of renal stone disease or lithuria in past. He had no significant past medical history or any urological intervention. Renal function test and hemogram was within normal limits. Urine culture for Tuberculosis (TB) and urine for TB polymerase chain reaction (PCR) were negative. On physical examination, left sided lump was palpable in lumbar region extending up to iliac region and crossing midline [Table/ Fig-1]. Lump was cystic on palpation and non tender. The ultrasonography (USG) of abdomen revealed bilateral hydronephrosis with proximal hydroureters, massively enlarged left kidney with gross hydronephrosis & thinned out parenchyma and right kidney was moderately hydronephrotic with parenchymal thickness of 12 mm and proximal hydroureter. Bladder on USG was normal. Of note no ureteric dilatation was seen at the level of the bladder. On Intravenous pyelography the left kidney was non-functioning with no uptake of contrast, while the right kidney showed moderate hydronephrosis with dilatation of proximal ureter with delayed uptake and retention of contrast, which were suggestive of stricture in right sided mid ureter [ Fig-4]. Tc99m Diethylene Triamine Pentacaetic Acid (DTPA) scan showed non-functioning left kidney. Right side kidney showed decreased function with a glomerular filtration rate (GFR) of 40.44ml/min with a100% retention at two hours with retention in proximal ureter also. On bilateral retrograde uretropyelography (RGP), bilateral mid ureteric stenosis at the level of pelvic brim was documented. The length of stenosed segment on right side was approximately of 2 to 3 cm and that off left side was 4 to 5 cm. Left sided nephroureterctomy was done through flank incision, approaching extraperitoneally. Intraoperative findings were suggestive of a tight stenotic segment of left midureter with normal calibre ureter distal to it. On right side, extra peritoneal ureteric exploration was performed using a modified Gibson incision. On right side also a similar appearance of the mid ureter was seen but of a shorter length as compared to left side. Bilateral congenital mid ureteric stenosis is a very rare entity causing hydroureteronephrosis. The pathophysiology and aetiology of this condition is uncertain. Congenital functional obstruction of the mid ureter has only 17 reported cases, that too of unilateral pathology. We report a case of an 11-year-old male child who was presented with left sided lump in abdomen since one year. On bilateral retrograde ureteropyelography (RGP), bilateral mid ureteric stenosis was documented at the level of pelvic brim. Tc99m Diethylene Triamine Pentacaetic Acid (DTPA) scan showed non functioning left kidney and decreased function and retention on right side. We did a left nephroureterectomy and uretero-ureterostomy without tapering over a Double-J stent (DJ...

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