|Year : 2014 | Volume
| Issue : 1 | Page : 41-43
Segmental dilatation of ureter: Report of two cases
Hemonta Kr. Dutta
Department of Pediatric Surgery, Assam Medical College and Hospital, Dibrugarh, Assam, India
|Date of Web Publication||28-Jan-2014|
Hemonta Kr. Dutta
Department of Pediatric Surgery, Assam Medical College and Hospital, Dibrugarh, Assam - 786 002
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Segmental dilatation of ureter is a giant, focal segmental ureteral dilatation producing an elongated and distorted ureter. Two children presented with this condition, one had ipsilateral megacalycosis and contralateral vesicoureteric reflux. The other had duplication of the kidney. The non-functioning lower moiety showed structure of xanthogranulomatous pyelonephritis.
Keywords: Segmental dilatation of ureter, segmental giant ureter, xanthogranulomatous pyelonephritis.
|How to cite this article:|
Dutta HK. Segmental dilatation of ureter: Report of two cases. J Indian Assoc Pediatr Surg 2014;19:41-3
| Introduction|| |
Congenital segmental dilatation of ureter is a rare entity in children and characterized by a giant, focal segmental dilatation of mid ureter. Distal ureter may be normal, stenotic, or atretic. Cases are reported in the literature who have duplex system on the affected side.  We report two children with segmental dilatation of ureters; one child had ipsilateral megacalycosis and contralateral vesico-ureteric reflux (VUR) presenting with acute renal failure and sepsis. The other child had unilateral duplex system with segmental dilatation of both upper and lower moiety ureters with evidence of xanthogranulomatous pyelonephritis (XGP) in the lower moiety. We report these cases because of their unusual presentation and rare association with XGP.
| Case Reports|| |
Master BT, 7 years male presented with high fever and scanty, high-colored urine, and lethargy. A large lump was palpated on left renal area extending upto the hypogastrium. His blood urea and serum creatinine values at admission were 229 mg% and 5.4 mg%, respectively. Ultrasonography (USG) showed bilateral gross hydronephrosis and a large cyst on the left side, possibly a bladder diverticulum. An intravenous pyelography (IVP) done elsewhere 2 months earlier had shown right-sided gross hydro-ureteronephrosis on right side and non-functioning left kidney. Blood urea and serum creatinine values decreased to 90 mg% and 1.7 mg%, respectively, following bilateral percutaneous nephrostomies. Micturating cystourethrogram (MCUG) showed right-sided grade V VUR. Cystoscopy showed right golf hole orifice and absent left ureteric orifice with hemitrigone. Patient underwent right ureteric reimplantation two weeks later. Exploration revealed a dilated left upper ureter (12 cm long) opening into a large cyst (10 cm × 6 cm), which was thick-walled and adherent to the bladder wall [Figure 1]. A 2 cm long lower ureter was seen from the cyst to the bladder neck. The dilated segment along with the lower ureter was excised, and end to side ureteroureteral anastomosis was done to the contralateral ureter. Histology of dilated segment showed damaged epithelium with infiltration of sub-epithelial layers with inflammatory cells. The patient was put on chemoprophylaxis for 6 months (Co-trimoxazole and Nalidixic acid alternately every 6 weeks) and has been asymptomatic for last 4 years. His blood urea and serum creatinine values on last visit were 48 mg% and 1.1 mg%, respectively.
|Figure 1: Normal-looking upper ureter opening into the dilated segment (case 1)|
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A 14-month-old male child presented with abdominal distension and pain of 2 months duration. USG revealed moderate hydronephrosis on right side; ureters were not seen. A thick-walled cystic structure with thick fluid inside (4 cm × 3 cm) was noted on right side just above the bladder. IVP showed right-sided hydronephrosis, as the radiologist missed the non-functioning lower moiety. Left kidney was normal. MCUG showed normal urinary bladder and no reflux. Renal biochemical parameters were within normal limits. At operation, right-sided duplex kidney was noted. Normal size upper moiety ureter (8 cm long) drains into the dilated segment (3 cm × 2 cm). The small lower moiety and dilated upper ureter (4 cm long) drains into a markedly dilated, thickened ureteral segment (6 cm × 4 cm). The dilated segments were densely adherent to the bladder wall and extended up to the recto-vesical pouch. Normal size lower ureters of both moieties opened into the bladder separately. Partial nephroureterectomy (lower moiety) was done with excision of the dilated segment of both upper and lower moiety ureters. Normal upper moiety ureter was re-implanted into the bladder by Leadbetter-Politano technique. Histology of the lower moiety showed features of XGP [Figure 2]. The patient had uneventful recovery and was doing well till last follow up at 18 months.
|Figure 2: Photomicrograph (H and E stain x200) showing groups of large round to oval cells with eosinophilic cytoplasm and vesicular nuclei mixed with interlacing fibrocollagenous tissue, infiltrating into the sub-epithelial stroma s/o juvenile xanthogranulomata (case 2)|
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| Discussion|| |
Segmental dilatation of ureter is a rare entity, and only 12 case reports are found in the English literature.  This condition is characterized by giant, focal segmental ureteral dilatation producing an elongated and distorted ureter. It may be associated with megacalycosis, duplication of collecting system and hypoplastic, dysplastic or non-functioning kidney. Distal ureter may be normal, stenotic, or atretic. They usually have a dysplastic or disorganized muscle coat, lined with columnar epithelial mucosa, rather than the usual transitional epithelium. The associated kidney or the moiety may be hypoplastic or the site of comparatively mild hydronephrosis associated with atrophic renal parenchyma. Ramaswamy et al. reported a case of congenital segmental megaureter with sparing of the proximal and distal ureter.  The authors suggested that attenuated nexuses and thin myofilaments might be responsible for this entity. Soler reported a case of an unilateral multicystic dysplastic kidney and contralateral megacalycosis associated with ipsilateral distal segmental megaureter.  Pinter speculated abnormal recanalization of the solid ureteral duct during development as a possible cause.  XGP is an atypical form of chronic pyelonephritis characterized by diffuse, focal or segmental suppurative destruction of renal parenchyma. Urinary tract obstruction, urosepsis, renal ischemia, lymphatic obstruction, and altered immune response may be responsible for XGP. In the rare localized form, the lesion can be confined to one or other pole, as was seen in our second case. Its association with duplex kidney is known, but its association with segmental dilatation of ureter has not been reported earlier.
The treatment of segmental dilatation of ureter is excision of the diseased segment with end-to-end ureteroureterostomy. A better option is to anastomose the upper ureter end-to-side to the contralateral ureter, as was done in our first case. Nephroureterectomy may be necessary for poorly functioning renal unit. Treatment for XGP is excision of the diseased tissue. Localized form of the disease, as in our 2 nd case, is rare, and a partial nephrectomy is ideal. These patients need long-term follow up as secondary amyloidosis has been reported in association with XGP. 
| References|| |
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[Figure 1], [Figure 2]