Home | About Us | Current Issue | Ahead of print | Archives | Search | Instructions | Subscription | Feedback | Editorial Board | e-Alerts | Login 
Journal of Indian Association of Pediatric Surgeons
     Journal of Indian Association of Pediatric Surgeons
Official journal of the Indian Association of Pediatric Surgeons         
 Users Online:866 
  Print this page Email this page   Small font sizeDefault font sizeIncrease font size


 
Table of Contents   
CASE REPORT
Year : 2014  |  Volume : 19  |  Issue : 1  |  Page : 41-43
 

Segmental dilatation of ureter: Report of two cases


Department of Pediatric Surgery, Assam Medical College and Hospital, Dibrugarh, Assam, India

Date of Web Publication28-Jan-2014

Correspondence Address:
Hemonta Kr. Dutta
Department of Pediatric Surgery, Assam Medical College and Hospital, Dibrugarh, Assam - 786 002
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-9261.125965

Rights and Permissions

 

   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

How to cite this URL:
Dutta HK. Segmental dilatation of ureter: Report of two cases. J Indian Assoc Pediatr Surg [serial online] 2014 [cited 2019 Dec 14];19:41-3. Available from: http://www.jiaps.com/text.asp?2014/19/1/41/125965



   Introduction Top


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. [1] 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 Top


Case 1

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)

Click here to view


Case 2

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)

Click here to view



   Discussion Top


Segmental dilatation of ureter is a rare entity, and only 12 case reports are found in the English literature. [1] 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. [2] 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. [3] Pinter speculated abnormal recanalization of the solid ureteral duct during development as a possible cause. [4] 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. [5]

 
   References Top

1.Karaman A, Demircan M, Uguralp S, Ozcan CC, Sigirci A. Segmental Cystic Dilatation of Ureter in Children. Inonu Univ Tip Fakultesi Dergisi 2010;17:37-40.  Back to cited text no. 1
    
2.Ramaswamy S, Bhatnagar V, Mitra DK, Gupta AK. Congenital segmental giant megaureter. J Pediatr Surg 1995;30:123-4.  Back to cited text no. 2
[PUBMED]    
3.Perera Soler R, Ruiz Gonzalez AP, Molini Menchon N, Garcia Nieto VM. Unilateral multicystic dysplastic kidney and contralateral megacalycosis. An unusual association An Pediatr (Barc) 2004;60:473-6.  Back to cited text no. 3
    
4.Pinter AB, Szabo L, Szever ZS, Magyarlaki T. Bilateral congenital segmental megaureter. J Urol 1997;158:570-1.  Back to cited text no. 4
    
5.Lauzurica R, Felip A, Serra A, Saladie JM, Montserrat E, Encabo B, et al. Xanthogranulomatous pyelonephritis and systemic amyloidosis: A report of 2 new cases and the natural history of this association. J Urol 1991;146:1603-6.  Back to cited text no. 5
[PUBMED]    


    Figures

  [Figure 1], [Figure 2]



 

Top
Print this article  Email this article

    

 
  Search
 
  
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Article in PDF (926 KB)
    Citation Manager
    Access Statistics
    Reader Comments
    Email Alert *
    Add to My List *
* Registration required (free)  


    Abstract
   Introduction
   Case Reports
   Discussion
    References
    Article Figures

 Article Access Statistics
    Viewed1420    
    Printed86    
    Emailed0    
    PDF Downloaded86    
    Comments [Add]    

Recommend this journal


Contact us | Sitemap | Advertise | What's New | Copyright and Disclaimer 

  2005 - Journal of Indian Association of Pediatric Surgeons | Published by Wolters Kluwer - Medknow 

Online since 1st May '05