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Saudi Journal of Kidney Diseases and Transplantation
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ORIGINAL ARTICLE Table of Contents   
Year : 2001  |  Volume : 12  |  Issue : 1  |  Page : 9-13
Congenital Midureteric Stricture


1 Department of Pediatric Urology, City Hospital NHS Trust, Hucknall Road, Nottingham, NG5 1PB, United Kingdom
2 Department of Nephrology, City Hospital NHS Trust, Hucknall Road, Nottingham, NG5 1PB, United Kingdom
3 Department of Radiology, City Hospital NHS Trust, Hucknall Road, Nottingham, NG5 1PB, United Kingdom

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   Abstract 

Congenital midureteric obstruction is a rare entity which can be caused by either ureteric valves or strictures. We report our experience with four patients with midureteric obstruction due to stricture over a six-year period. The condition needs to be differentiated from obstruction of the pelviureteric and vesicoureteric junctions. Obstruction can be initially screened by ultrasound and confirmed by a radionuclide scan with furosemide challenge. Retrograde ureteropyelography and/or intravenous urography may be required to define the level of the stricture.

Keywords: Ureteric obstruction, Midureter, Ureteric stricture.

How to cite this article:
Singh SJ, Watson AR, Somers J, Broderick N, Rance CH. Congenital Midureteric Stricture. Saudi J Kidney Dis Transpl 2001;12:9-13

How to cite this URL:
Singh SJ, Watson AR, Somers J, Broderick N, Rance CH. Congenital Midureteric Stricture. Saudi J Kidney Dis Transpl [serial online] 2001 [cited 2019 Nov 15];12:9-13. Available from: http://www.sjkdt.org/text.asp?2001/12/1/9/33879

   Introduction Top


Congenital midureteric obstruction is a rare entity and can be caused by either valve or stricture. [1] Patients are often mis­diagnosed pre-operatively as having pelvi­ureteric junction (PUJ) obstruction or vesicoureteric junction (VUJ) obstruction. [1] Awareness of this entity and accurate pre­operative diagnosis helps to plan the surgical approach.

We report here our experience of the clinical findings, pre-operative investigations, operative treatment and follow-up of four children with midureteric stricture.


   Patients and Methods Top


There were four cases of congenital mid­ureteric obstruction between 1991 and 1996. Three were on the left side and one on the right. Antenatal sonographic scanning iden­tified upper urinary tract dilatation in three of the four patients.


   Case Reports Top


Patient 1

A female infant was antenatally diagnosed to have left sided hydronephrosis. A postnatal ultrasound was not performed, but a radio­nuclide (DTPA) scan with furosemide challenge at three months of age showed an obstructive curve. Micturating cystourethro­gram (MCUG) revealed grade II right vesicoureteric reflux. At eight months of age, the patient was referred for pyelo­plasty. There was kinking and narrowing of the ureter 2.5 cm below the pelviureteric junction (PUJ) with increased peristalsis in the proximal ureter. Whitaker pressure test was performed on the operating table, which showed no evidence of obstruction. Therefore, no definitive procedure was carried out.

One year later, the ultrasound continued to show persistent but, diminished dilatation of the pelvicalyceal system. A subsequent retrograde ureteropyelogram (RUPG) showed narrowing in the upper half of the ureter but free flow of contrast through the narrowed area of the ureter [Figure - 1]. An intravenous pyelogram (IVU) performed at six years of age showed good function and drainage of the left kidney.

Patient 2

A two-month-old girl was diagnosed ante­natally as having left hydronephrosis. A left midureteric stricture was suggested on post­natal ultrasound. This was further defined by the IVU [Figure - 2]a and RUPG confirmed a left midureteric stricture [Figure - 2]b. At 20 months of age a uretero-ureterostomy was performed. The histology of the stenosed segment showed areas of fibrosis with chronic inflammatory cells in the submucosa. Post-operative radionuclide scan revealed slight delay in drainage of the left kidney with immediate washout with furosemide.

Patient 3

A 28-month-old male infant presented with urinary tract infection and the initial ultrasound showed a left-sided hydro­nephrosis and hydroureter down to the midureteric region [Figure - 3]a. The MCUG showed no reflux and a radionuclide scan did not reveal any evidence of scarring. The radionuclide scan repeated with furosemide challenge supported the diagnosis of a mid­ureteric obstruction [Figure - 3]b, which was further confirmed on an IVU [Figure - 3]c.

The midureteric stricture was confirmed at operation and a left uretero-ureterostomy was performed. The histological examination of the stricture showed a thickened wall with increased smooth muscle and fibrous tissue.

Patient 4

A female infant was antenatally noted to have bilateral hydronephrosis. The post­natal ultrasound showed a left multicystic dysplastic kidney with right hydronephrosis. The radionuclide scan confirmed a non­functioning left kidney with a poorly functioning right kidney. The child had severe chronic renal failure with a serum creatinine of 220 µmol/l. When she was referred to us at three months of age, a new ultrasound examination suggested persistent right hydronephrosis with dilatation down to the midureteric level [Figure - 4]a. MCUG showed no reflux and radionuclide scan with furosemide also suggested midureteric obstruction.

The RUPG at operation confirmed the right midureteric stricture [Figure - 4]b. Pre­operatively the serum creatinine was 140 µmol/l and this fell to 80 µmol/l following a successful right uretero-ureterostomy. Histological examination showed congenital stricture with no inflammation or fibrosis.


   Discussion Top


Congenital midureteric obstruction is a rare entity, which may result from valves or stricture. [1] Differentiation between these two causes can only be made by gross macroscopical examination at the time of operation and microscopic histological examination [1] that shows smooth muscle fibers within the valve. Furthermore, there will be changes of obstructive uropathy proximal to the valve with normal distal tract and no evidence of other mechanical or functional obstruction. [2]

Congenital midureteric stricture is a definitive entity and has been attributed to various factors such as abnormal ureteric embryogenesis, [3] intrauterine ureteritis, [4] extrinsic compression by blood vessels [5] and some derangement during recannulization of the ureter during fetal life. [6] A series of seven cases of midureteric stricture collected over 17 years was reported from Boston in 1989. [1]

Percutaneous antegrade pyelography and RUPG are advocated to confirm the diagnosis of midureteric stricture. However, there is high-enough resolution with the currently available ultrasound technology that makes this modality more favorable. In three of our cases, the diagnosis was strongly suggested pre-operatively by ultrasonography. This was confirmed by IVU in two cases, RUGP in two cases and both in one case. RUGP was performed at the time of surgery in order to localize the stricture and to insert a stent to prevent recurrence of the stricture.

An MCUG, to exclude vesicoureteric reflux, and a diuretic-renogram, to demonstrate the degree of obstruction, are part of the usual postnatal investigation protocol for ante­natally detected urinary tract dilatation.

We conclude that midureteric stricture is a rare but important differential diagnosis of hydronephrosis. Ultrasound can be useful in the pre-operative evaluation. IVU and/or RUGP still have indispensable roles for the accurate diagnosis of the stenosis.


   Acknowledgment Top


We thank Ann Johnson and Judith Hayes for typing the manuscript.

 
   References Top

1.Docimo SG, Lebowitz RL, Retik AB, Colodny AH, Bauer SB, Mandell J. Congenital midureteral obstruction. Urol Radiol 1989;11:156-60.  Back to cited text no. 1  [PUBMED]  
2.Wall B, Wacher HE. Congenital ureteral valve: its role as a primary obstruction lesion: classification of the literature and report on an authentic case. J Urol 1952;68:684.  Back to cited text no. 2    
3.Sant GR, Barbalias GA, Klauber GT. Congenital ureteral valves-an abnormality of ureteral embryogenesis. J Urol 1985; 133: 427-31.  Back to cited text no. 3  [PUBMED]  
4.Campbell MF. The dilated ureter in children. A brief consideration of its causes, diagnosis and treatment. Am J Surg 1938;39:438.  Back to cited text no. 4    
5.Allen TD. Congenital ureteral strictures. Birth Defects Orig Artic Ser 1977;13:17-8.  Back to cited text no. 5  [PUBMED]  
6.Ruano-Gil D, Coca Payeras A, Tejedo-Mateu A. Obstruction and normal recana-lization of the ureter in the human embryo. Its relation to congenital ureteral obstruction. Eur Urol 1975;1:287-93.  Back to cited text no. 6    

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Correspondence Address:
Alan R Watson
Consultant Pediatric Nephrologist, City Hospital NHS Trust, Hucknall Road, Nottingham NG5 1PB
United Kingdom
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PMID: 18209354

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  [Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]



 

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    Abstract
    Introduction
    Patients and Methods
    Case Reports
    Discussion
    Acknowledgment
    References
    Article Figures
 

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