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Congenital Hypoplasia of the Proximal Ureter
Vol. 113, February Printed in U.S.A.
THE JOURNAL OF UROLOGY
Copyright© 1975 by The Williams & Wilkins Co.
CONGENITAL HYPOPLASIA OF THE PROXIMAL URETER WILLIAM C. DEWOLF, ELWIN E. FRALEY
AND
COLIN MARKLAND
From the University of Minnesota Health Sciences Center, Minneapolis, Minnesota
tecture. Occasionaliy there is evidence of inflammation or fibrosis. 3 Diagnosis is made by radiographic examination and can be confirmed by the inability to pass a ureteral catheter easily into the renal pelvis. For example the ureter in a neonate should accept a 3F catheter and the ureter of a 1-year-old child should accept a 4F catheter. A variety of treatments have been recommended for segmental hypoplasia of the proximal ureter. Campbell and Harrison believed that the success
Congenital hypoplasia of the proximal ureter in infants and children poses an interesting and difficult problem. Previous reports have recommended many forms of treatment, including dilatation, plastic repair and nephrectomy. We herein report on 2 children who were treated with operative and non-operative therapy. These cases demonstrate some difficulties and principles of management. CASE REPORTS
Case 1. A 3-year-old white boy was admitted to the hospital for evaluation of a urinary tract infection. An excretory urogram (IVP) demonstrated a normal right kidney and hypoplasia of the proximal left ureter which was confirmed with a left retrograde pyelogram (fig. 1). The patient was taken to the operating room where a CulpScardino pyeloplasty was performed. Despite the use of a splinting ureteral catheter and nephrostomy tube, the pyeloplasty was unsuccessful and a nephrectomy was done. Case 2. An 11-month-old white girl was admitted to the hospital with chronic urinary tract infection. A voiding cystourethrogram showed total right vesicoureteral reflux. An IVP revealed mild right calicectasis and moderate left calicectasis with no visualization of the ureter. A left retrograde pyelogram confirmed the presence of a hypoplastic proximal left ureter which would not accept a 4F ureteral catheter (fig. 2). The patient was treated conservatively with antibiotics. At followup examination 6 months later the patient was free of infection with a stable IVP and normal serum creatinine. DISCUSSION
Congenital ureteral hypoplasia has been referred to as congenital ureteral stricture. It is usually segmental and found at those points associated with physiologic narrowing, specifically the ureteropelvic junction, the ureterovesical junction and the area where the ureter crosses the pelvic brim. Although the exact etiology of this condition is unknown, it is thought to be associated with vascular compression during embryonic development.'· 2 Microscopic section of the ureters usually shows simple narrowing with normal archi-
FIG. 1. Case 1-left retrograde pyelogram shows hypoplasia of proximal ureter.
Accepted for publication July 5, 1974. Read at annual meeting of Society of Pediatric Urology, American Urological Association, St. Louis, Missouri, May 18-19, 1974. 1 Allen, T. D.: Congenital ureteral strictures. J. Urol., 104: 196, 1970. • Kosto, B.: Congenital mid-ureteral stricture in a solitary kidney. J. Urol., 106: 529, 1971.
of catheter dilatation was proportional to the proximity of the stricture to the bladder; dilatation of hypoplastic segments near the ureteropelvic junction was ineffective and an operation was recommended.' In general, dilatation has not been satisfactory because it has to be repeated frequently and is associated with infection and • Bergman, H.: The Ureter. New York: Harper & Row, Publishers, Inc., p. 83, 1967. • Campbell, M. F. and Harrison, J. H.: Urology, 3rd ed. Philadelphia: W. B. Saunders Co., p. 1523, 1970.
236
CONGENITAL HYPOPLASIA OF PROXIMAL URETER
In such cases an intubated ureterostomy be the alternate procedure of choice, although the likelihood of scarring with urinary extravasation does not make this an ideal operation. Nephrectomy is sometimes the only answer This choice is based-on the degree of renal the severity of infection and the presence of bilateral disease. Non-operative therapy may also be treatment, especially if the patient is less than 2 years old with a long hypoplastic segment. Success depends on the maintenance of sterile urine and the preservation of renal substance. Although patients with bilateral disease may he treated by conventional therapy, advanced struction of renal tissue and associated phrosis, high urinary diversion or procedures volving ureteral substitution are necessary. 5 Autotransplantation may also be a consideration in treatment. However, this would seem inadvisa ble because of the attendant difficulties in urete ropyelostomy and vascular anastomoses in small children, especially in the presence of infection. CONCLUSION
FIG. 2. Case 2-left retrograde pyelogram shows hypoplasia of proximal ureter.
trauma. It is easy to convert a congenital segmental ureteral hypoplasia to a traumatic stricture. Surgical repair may be possible. Dismembered ureteral procedures are not recommended for several reasons: 1) the length of the diseased ureter is usually too long to allow approximation of normal ureter to the pelvis, 2) the blood supply of these small ureters is easily compromised during mobilization and 3) there is an increased risk of occlusive scarring when dealing with a circumferential suture line in small ureters. Flap procedures, using a nephrostomy and ureteral splint, offer the best hope for a satisfactory result. The repair in case 1 failed probably because the defect was too extensive to bridge with a flap.
Two cases of congenital hypoplasia of the ma! ureter are presented. One patient was treated successfully with non-operative management ar,d the other was treated unsuccessfully by an opera· tion. Principles of management include: 1) nonoperative management should be considered children less than 2 years old with a long hypoplas. tic segment, 2) dilatation may be hazardous, ::l) nephrectomy is indicated for severe chronic infection with hydronephrosis, 4) if pyeloplasty is ble, a flap procedure, usually combined with a ureteral splint and nephrostomy, is probably the procedure of choice, 5) bilateral disease may re quire high urinary diversion or ureteral substitution and 6) autotransplantation, although theoretically possible, is inadvisable. 5 Markland, C.: Drainage of the massively dilated kidney. Surg., Gynec. & Obst., 129: 775, 1969.
THE JOURNAL OF UROLOGY
Copyright© 1975 by The Williams & Wilkins Co.
CONGENITAL HYPOPLASIA OF THE PROXIMAL URETER WILLIAM C. DEWOLF, ELWIN E. FRALEY
AND
COLIN MARKLAND
From the University of Minnesota Health Sciences Center, Minneapolis, Minnesota
tecture. Occasionaliy there is evidence of inflammation or fibrosis. 3 Diagnosis is made by radiographic examination and can be confirmed by the inability to pass a ureteral catheter easily into the renal pelvis. For example the ureter in a neonate should accept a 3F catheter and the ureter of a 1-year-old child should accept a 4F catheter. A variety of treatments have been recommended for segmental hypoplasia of the proximal ureter. Campbell and Harrison believed that the success
Congenital hypoplasia of the proximal ureter in infants and children poses an interesting and difficult problem. Previous reports have recommended many forms of treatment, including dilatation, plastic repair and nephrectomy. We herein report on 2 children who were treated with operative and non-operative therapy. These cases demonstrate some difficulties and principles of management. CASE REPORTS
Case 1. A 3-year-old white boy was admitted to the hospital for evaluation of a urinary tract infection. An excretory urogram (IVP) demonstrated a normal right kidney and hypoplasia of the proximal left ureter which was confirmed with a left retrograde pyelogram (fig. 1). The patient was taken to the operating room where a CulpScardino pyeloplasty was performed. Despite the use of a splinting ureteral catheter and nephrostomy tube, the pyeloplasty was unsuccessful and a nephrectomy was done. Case 2. An 11-month-old white girl was admitted to the hospital with chronic urinary tract infection. A voiding cystourethrogram showed total right vesicoureteral reflux. An IVP revealed mild right calicectasis and moderate left calicectasis with no visualization of the ureter. A left retrograde pyelogram confirmed the presence of a hypoplastic proximal left ureter which would not accept a 4F ureteral catheter (fig. 2). The patient was treated conservatively with antibiotics. At followup examination 6 months later the patient was free of infection with a stable IVP and normal serum creatinine. DISCUSSION
Congenital ureteral hypoplasia has been referred to as congenital ureteral stricture. It is usually segmental and found at those points associated with physiologic narrowing, specifically the ureteropelvic junction, the ureterovesical junction and the area where the ureter crosses the pelvic brim. Although the exact etiology of this condition is unknown, it is thought to be associated with vascular compression during embryonic development.'· 2 Microscopic section of the ureters usually shows simple narrowing with normal archi-
FIG. 1. Case 1-left retrograde pyelogram shows hypoplasia of proximal ureter.
Accepted for publication July 5, 1974. Read at annual meeting of Society of Pediatric Urology, American Urological Association, St. Louis, Missouri, May 18-19, 1974. 1 Allen, T. D.: Congenital ureteral strictures. J. Urol., 104: 196, 1970. • Kosto, B.: Congenital mid-ureteral stricture in a solitary kidney. J. Urol., 106: 529, 1971.
of catheter dilatation was proportional to the proximity of the stricture to the bladder; dilatation of hypoplastic segments near the ureteropelvic junction was ineffective and an operation was recommended.' In general, dilatation has not been satisfactory because it has to be repeated frequently and is associated with infection and • Bergman, H.: The Ureter. New York: Harper & Row, Publishers, Inc., p. 83, 1967. • Campbell, M. F. and Harrison, J. H.: Urology, 3rd ed. Philadelphia: W. B. Saunders Co., p. 1523, 1970.
236
CONGENITAL HYPOPLASIA OF PROXIMAL URETER
In such cases an intubated ureterostomy be the alternate procedure of choice, although the likelihood of scarring with urinary extravasation does not make this an ideal operation. Nephrectomy is sometimes the only answer This choice is based-on the degree of renal the severity of infection and the presence of bilateral disease. Non-operative therapy may also be treatment, especially if the patient is less than 2 years old with a long hypoplastic segment. Success depends on the maintenance of sterile urine and the preservation of renal substance. Although patients with bilateral disease may he treated by conventional therapy, advanced struction of renal tissue and associated phrosis, high urinary diversion or procedures volving ureteral substitution are necessary. 5 Autotransplantation may also be a consideration in treatment. However, this would seem inadvisa ble because of the attendant difficulties in urete ropyelostomy and vascular anastomoses in small children, especially in the presence of infection. CONCLUSION
FIG. 2. Case 2-left retrograde pyelogram shows hypoplasia of proximal ureter.
trauma. It is easy to convert a congenital segmental ureteral hypoplasia to a traumatic stricture. Surgical repair may be possible. Dismembered ureteral procedures are not recommended for several reasons: 1) the length of the diseased ureter is usually too long to allow approximation of normal ureter to the pelvis, 2) the blood supply of these small ureters is easily compromised during mobilization and 3) there is an increased risk of occlusive scarring when dealing with a circumferential suture line in small ureters. Flap procedures, using a nephrostomy and ureteral splint, offer the best hope for a satisfactory result. The repair in case 1 failed probably because the defect was too extensive to bridge with a flap.
Two cases of congenital hypoplasia of the ma! ureter are presented. One patient was treated successfully with non-operative management ar,d the other was treated unsuccessfully by an opera· tion. Principles of management include: 1) nonoperative management should be considered children less than 2 years old with a long hypoplas. tic segment, 2) dilatation may be hazardous, ::l) nephrectomy is indicated for severe chronic infection with hydronephrosis, 4) if pyeloplasty is ble, a flap procedure, usually combined with a ureteral splint and nephrostomy, is probably the procedure of choice, 5) bilateral disease may re quire high urinary diversion or ureteral substitution and 6) autotransplantation, although theoretically possible, is inadvisable. 5 Markland, C.: Drainage of the massively dilated kidney. Surg., Gynec. & Obst., 129: 775, 1969.