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Segmental dilatation of ureter in children

Hemonta Kr. Dutta, Shree Harsh
Assam Medical College & Hospital
Dibrugarh, Assam, India

 

Correspondence

Dr. Hemonta Kr. Dutta
Assistant Professor of Pediatric Surgery
Deptartment of Pediatric Surgery
Assam Medical College & Hospital
Dibrugarh, Assam-786002, India
E-mail: This email address is being protected from spambots. You need JavaScript enabled to view it.

 

Abstract

Segmental dilatation of ureter is characterized by a giant, focal segmental dilatation of mid ureter. It may be associated with megacalycosis, duplication of collecting system and hypoplastic, dysplastic or nonfunctioning kidney. We describe a case of segmental dilatation of ureter with ipsilateral megacalycosis and contralateral vesico-ureteric reflux in a child, who presented with acute renal failure and sepsis. Cystoscopy revealed absence of ipsilateral ureteric meatus in the bladder with hemitrigone and golf-hole opening of the refluxing ureter. The ureter distal to the segmental dilatation was normal caliber, but opened in the bladder neck. These associations were not reported earlier.

Key words: segmental dilatation of ureter, megaureter, segmental giant ureter

 

Introduction

Congenital segmental megaureter is a rare condition. It is characterized by giant, focal segmental ureteral dilatation producing an elongated and distorted ureter. Distal ureter may be normal, stenotic or atretic. Cases are reported in the literature who have duplex system on the affected side [1]. Although diagnosis is now possible in antenatal period, there has been no therapeutic standardization [2]. We report a child with segmental giant megaureter and contralateral vesico-ureteric reflux presenting with acute renal failure and sepsis.

Case Report

A case of congenital segmental megaureter with normal proximal and distal ureter is presented. 7 year old male child presented with high fever, scanty and high colored urine and lethargy. A large lump palpated on left renal area extending up to left iliac fossa. His blood urea level was 229mg% and serum creatinin 5.4mg%. Ultrasonography showed grossly dilated pelvicalyceal system (PCS) with thin parenchyma in left kidney and moderate dilatation of right PCS. Ureters could not be identified because of bowel gas. An intravenous pyelography (IVP) done elsewhere 2 months earlier showed right hydroureteronephrosis. No excretion of dye noted on the left side. Urine microscopy showed plenty of pus cells.

The bladder was catheterized and intravenous antibiotics started based on urine culture sensitivity report. The patient had slow recovery. A repeat ultrasonography 7 days later showed dilated and tortuous right ureter. The cyst on the left side did not decrease in size. So, bilateral nephrostomy was done. Good urine output was recorded in both the kidneys. Blood urea and serum creatinin levels on 5th post-operative day were 90 mg% and 1.7 mg% respectively. Voiding cystourethrography (VCUG) showed right sided grade V vesicoureteric reflux. Antegrade pyelography on left kidney showed grossly dilated pelvicalyceal system, left ureter was not seen, but a dilated cyst filled up with contrast was visualised near the bladder. There was no evidence of bladder outlet obstruction and post void residue was insignificant. However, significant contrast was noted in the right dilated ureter. On cystoscopy, golf hole right ureteric orifice was noted in normal location, with hemitrigone and absence of left ureteric orifice in bladder.

On exploration, dilated left upper ureter (12 mm wide and 5 cm long) was seen draining into a cyst (size-10 cm x 6 cm), which was compressing the left side of the urinary bladder. About 2 cm long normal calibre ureter distal to the dilated segment was seen opening in the bladder neck area. The segmental megaureter was excised, a Leadbetter- Politano ureteric reimplantation was done on right side and the left upper ureter was anastomosed end-to-side to the right ureter. The patient had an uneventful recovery. His renal parameters were within normal limits (blood urea - 42 mg%, serum creatinin - 1.3 mg%) 15 days after surgery. Serial ultrasonography was done every 3 months for one year. An IVP done at 3 months following surgery showed poor function in left kidney with no outflow obstruction and good function and drainage on right side. The patient was put on chemoprophylaxis for 6 months and has been asymptomatic for last 20 months. A renal nuclear scan done at 18 months showed differential function of 75:25 (RK:LK) with GFR of 96 ml and prompt drainage of both systems.

7.1.12

Figure 1: Normal looking upper ureter opening into the dilated segment.

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Figure 2: Antegrade pyelography (left) showing the dilated segment of ureter.

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Figure 3: Histology of dilated part of ureter shows damaged

uroepithelium with infiltration of submucosa by lymphocytes and plasma cells

Figure 4: Lower end of left ureter being dissected out from bladder neck

Discussion

Segmental dilatation of ureter or segmental cystic dilatation of ureter is a rare entity and only a few case reports have been found in the literature. This condition is characterized by giant, focal segmental ureteral dilatation producing an elongated and distorted ureter. 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. Mandell et al. reported four cases of congenital megacalycosis associated with ipsilateral segmental megaureter [2]. However, in all of them the distal ureteric segments were dilated, more likely to be secondary megaureters than segmental megaureter.

Kawaguchi et al. described two types of segmental dilatation: 1) those arising from a double pelvis with a colon like ureter (Megaloureter type) and 2) those arising from aplastic, hypoplastic or supernumery kidneys [4].

In 1995, a case of congenital segmental megaureter with sparing of the proximal and distal ureter, as in our case, was reported by Ramaswamy at al [5]. A similar case but with bilateral segmental megaureter was presented by Pinter et al. in 1997 [6]. In 2004, Soler reported a case of an unilateral multicystic dysplastic kidney and contralateral megacalycosis associated with ipsilateral distal segmental megaureter [7]. Recently, Prieto et al. reported a case of a congenital severe midureteral dilatation associated with mild proximal ureteral dilatation and a distal ureter with a normal diameter [8]. Gregory et al. presented two unusual cases of urolithiasis associated with unilateral isolated distal segmental megaureter [9].

Several theories have been speculated regarding the pathophysiology of segmental megaureter. Ramaswamy suggested that segmental megaureter was a variant of nonrefluxing megaureter and attenuated nexuses and thin myofilaments might be responsible for this entity [5, 10]. Although, in another study, it was suggested that aganglionosis might be responsible for the dilatation. However, the absence of ganglion cells has not subsequently been confirmed [3]. Pinter speculated that recanalization of the solid ureteral duct, if abnormal, might produce segmental ureteral dilatation [6].

The treatment of segmental cystic dilatation of ureter should be planned according to the ipsilateral kidney function and length of the normal ureteric segment. If the remaining parts of distal and proximal ureter have enough length, end-to-end ureteroureterostomy after excision of the segmental megaureter should be performed. Tailoring and trimming of the dilated segment is a difficult choice. A better option is to anastomose the upper segment endto- side to the contralateral ureter, as was done in our case. If the ipsilateral ureterorenal unit is poorly functioning, hypoplastic or dysplastic, a nephroureterectomy should be planed.

 

 

 

References

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10.Tokunaka S, Koyanagi T. Morphologic study of primary nonreflux megaureters with particular emphasis on the role of ureteral sheath and ureteral dysplasia. J Urol 1982;128:399-402.