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 Table of Contents  
CASE REPORT
Year : 2016  |  Volume : 3  |  Issue : 2  |  Page : 134-136

Primary obstructive megaureter with ureteric calculi in a child


Department of Pediatric Surgery, Christian Medical College, Ludhiana, Punjab, India

Date of Web Publication29-Feb-2016

Correspondence Address:
Dhruva Nath Ghosh
Department of Pediatric Surgery, Christian Medical College and Hospital, Brown Road, Ludhiana - 141 008, Punjab
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2348-3334.177647

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  Abstract 

Congenital megaureter is an important differential diagnosis among the known causes of hydroureteronephrosis in children. Obstructive megaureter is usually due to a narrowed and aperistaltic prevesical portion of the ureter. The congenital megaureter is usually asymptomatic in children. Association of an obstructive megaureter with ureteric calculi, however, is a rare phenomenon in the pediatric age group. Calculi if present are usually secondary to stasis and asymptomatic, presenting usually at adulthood or adolescence. A differential diagnosis of obstructive megaureter should be kept in mind in a child who presents with symptoms such as hematuria, pain, and obstructive nephropathy, especially when there are an associated calculi in the ureter. We present a case of a 4-year-old child whose chief complaint was hematuria and on further investigation was found to have unilateral obstructive megaureter with secondary ureteric calculi on an intravenous urogram.

Keywords: Obstructive megaureter, pediatric, ureteric calculi


How to cite this article:
Ghosh DN, Bhatti W. Primary obstructive megaureter with ureteric calculi in a child. CHRISMED J Health Res 2016;3:134-6

How to cite this URL:
Ghosh DN, Bhatti W. Primary obstructive megaureter with ureteric calculi in a child. CHRISMED J Health Res [serial online] 2016 [cited 2019 Oct 21];3:134-6. Available from: http://www.cjhr.org/text.asp?2016/3/2/134/177647


  Introduction Top


Initially described by Caulk, congenital megaureter is a condition usually diagnosed in neonates and children.[1] Spontaneous resolution occurs in over half of all affected children, and this is hypothesized to occur as a result of the ureterovesical junction (UVJ) maturation and growth.[2],[3] Mega ureters are primary or secondary. The cause of primary obstructed megaureter is the aperistaltic, narrowed prevesical portion of the ureter. Primary nonrefluxing megaureters represent 23% of all prenatally diagnosed hydronephrosis. The association of megaureter along with a ureteric calculus is quite uncommon.[4],[5] We present a rare case report of a patient with unilateral megaureter with urolithiasis.


  Case Report Top


A 4-year-old boy presented with complaints of recent onset hematuria. There was no history suggestive of renal calculi, urinary tract infection or previous urological intervention. Urine microscopy was normal, and culture did not show any growth. Blood counts and serum creatinine were within normal limits. Renal ultrasound revealed left hydroureteronephrosis along with multiple calculi in the lower ureter [Figure 1]. An intravenous urogram showed a dilated left ureter and renal pelvis along with calculi in the lower ureter [Figure 2], with both kidneys showing normal uptake of the contrast media. Voiding cystourethrogram was normal. Urethrocystoscopy revealed a normally placed but narrow left ureteric orifice, which did not admit a 4 Fr ureteric stent.
Figure 1: Postvoid intravenous urogram image. White arrow showing lower ureteric calculi

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Figure 2: Ultrasound image showing calculi in the lower ureter (black arrow)

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Intraoperatively, the distal ureter was found to be dilated immediately above the intramural segment containing a 7 mm × 5 mm × 3 mm calculus not obstructing the UVJ. The patient underwent a left ureteric reimplantation with tapering of the dilated distal ureter. The excised portion of the intramural ureter showed features of acute on chronic ureteritis on histopathology.


  Discussion Top


The term megaureter describes a dilated and tortuous ureter, which can be the result of obstruction, reflux, atony, or mal-development. The primary megaureter is a subcategory of obstructed megaureter and results from a normal-caliber aperistaltic distal (juxtavesical) ureteral segment, which causes a functional obstruction with tortuosity and dilatation of the intrinsically normal ureter above it.[4]

The exact underlying cause of primary congenital megaureter is not known. Hypotheses of ureteral wall muscle-fiber abnormalities and abnormal collagen composition/deposition have been explored.[6],[7]

Diagnostic criteria include dilated ureter: The absence of vesicoureteral reflux, absence of infravesical obstruction, and absence of distal ureteral obstruction.[5] When this condition does present in adults, it typically does so during the third or fourth decade, and unilateral disease (most often left-sided) is more common than bilateral disease.[1]

The diagnostic work-up included an ultrasound, a micturating cystourethrogram, and an intravenous or magnetic resonance urogram. The current belief is that primary obstructive megaureter presents primarily in adults when the congenital abnormality does not cause symptoms or illness and is not seen via an imaging study performed as children. Spontaneous regression fails to occur, yet patients remain asymptomatic through childhood and into their adult years. Eventual symptoms that may occur include urinary tract infections, renal parenchymal damage, and recurrent stone formation. In the largest reported series of 55 adults and adolescents with symptomatic primary obstructive megaureter, Hemal et al. identified 20 patients (36%) as having urinary tract calculi. Most of the calculi were located in the ureter. Stones can develop in the dilated portion of the ureter due to stasis of urine.[8] Delakas et al. described an adult patient with primary megaureter who developed a 12 cm urinary calculus within the dilated portion of the ureter.[9]

In our child, there was evident difference between the grossly dilated distal ureter and the relatively normal-caliber proximal ureteric segment. Furthermore, the calculus was found to be lying freely within the ureter; it was not impacted. Hence, very likely the stone development in this patient occurred secondary to urinary stasis. The association of ureteric calculi with megaureter in children is a rare phenomenon. In a study by Meyer et al., which included a work up on the associated anomalies with megaureter in children, no association with calculi was reported in a series of 75 cases while Turnock and Rangecroft reported the association of calculi with megaureter in 8 cases, which were secondary to infection.[4],[5]

Most primary megaureters regress spontaneously or remain stable without compromising renal function, but 10–25% require surgery because of a progressive reduction in renal function or increasing dilatation, or because they become symptomatic. The basic principles of surgical repair include resection of the obstructing segment, reduction in size of the dilated ureter, and reimplantation into the bladder using an anti-reflux technique.[10]


  Conclusion Top


In children presenting with distal ureteric calculi, investigations should be done keeping a differential diagnosis of the congenital megaureter in mind as calculi formation is secondary to the urinary stasis.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Caulk JR. Megaloureter: The importance of the ureterovesical valve. J Urol 1923;9:315-9.  Back to cited text no. 1
    
2.
Pitts WR Jr, Muecke EC. Congenital megaloureter: A review of 80 patients. J Urol 1974;111:468-73.  Back to cited text no. 2
    
3.
Dòmini M, Aquino A, Pappalepore N, Tursini S, Marino N, Strocchi F, et al. Conservative treatment of neonatal primary megaureter. Eur J Pediatr Surg 1999;9:396-9.  Back to cited text no. 3
    
4.
Meyer JS, Lebowitz RL. Primary megaureter in infants and children: A review. Urol Radiol 1992;14:296-305.  Back to cited text no. 4
    
5.
Turnock RR, Rangecroft L. Ureteral calculi in children: Review of 50 consecutive cases. Urology 1989;33:211-4.  Back to cited text no. 5
    
6.
Mackinnon KJ, Foote JW, Wiglesworth FW, Blennerhassett JB. The pathology of the adynamic distal ureteral segment. J Urol 1970;103:134-7.  Back to cited text no. 6
    
7.
Hanna MK, Jeffs RD, Sturgess JM, Barkin M. Ureteral structure and ultrastructure. Part II. Congenital ureteropelvic junction obstruction and primary obstructive megaureter. J Urol 1976;116:725-30.  Back to cited text no. 7
    
8.
Hemal AK, Ansari MS, Doddamani D, Gupta NP. Symptomatic and complicated adult and adolescent primary obstructive megaureter – Indications for surgery: Analysis, outcome, and follow-up. Urology 2003;61:703-7.  Back to cited text no. 8
    
9.
Delakas D, Daskalopoulos G, Karyotis I, Metaxari M, Cranidis A. Giant ureteral stone in association with primary megaureter presenting as an acute abdomen. Eur J Radiol 2002;41:170-2.  Back to cited text no. 9
    
10.
Merlini E, Spina P. Primary non-refluxing megaureters. J Pediatr Urol 2005;1:409-17.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2]



 

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