- Email: [email protected]
Congenital Ureteral Valve
TUE JOURNAL OF UROLOGY
Vol, 90, No. 1 July 1963 Copyright © 1963 hy The Williams & Wilkins Co.
Printed in U.S.A.
CONGENITAL URETERAL VALVE FREDERICK M. BUSCH,* DAVID C. WEIBEL, WILLIAM E. MORRIS C. EDWARD POHL
AND
From the Department of Urology, United States Naval Hospital, Philadelphia, Pa.
A congenital ureteral valve is, indeed, rare. In fact, only 10 cases in the literature meet the rigid criteria of Wall and Wachter. 1 These authors in 1952 made an extensive review of the literature and clarified the subject by listing the following definite criteria for these valves: 1) anatomically demonstrable transverse folds of ureteral mucosa which contain bands of smooth muscle, 2) changes of obstructive disease above the valve with normal urinary tract below, 3) no other evidence of mechanical or functional obstruction. Therefore, the commonly noted mucosal folds seen in tortuous, dilated ureters cannot be included as they are manifestations of obstructions lower down in the urinary tract. These folds or kinks are acquired, while true valves are congenital in origin and contain all structural layers of the ureter except for the adventitia. It is also important not to confuse a congenital valve with a congenital ureteral stricture. The valve is a transverse fold of mucosa with underlying muscle while a congenital stricture has a narrowed lumen with no structural changes of the mucosa. The following case report describes such a valve.
but an excretory urogram showed a poorly functioning, markedly dilated, left collecting system and ureter with a small, irregular calculus in the lower calyx. The right kidney was normal. A retrograde pyelogram confirmed the findings of the excretory urogram and dern.onstrated an obstruction just above the left ureterovesical junction (fig. 1, A). Cystoscopy was normal except for diminished activity of the left ureteral orifice. On September 15, under general anesthesia, the left ureter was explored and a cusp-like valve was noted approximately 2.0 cm. above the ureterovesical junction. A probe could not be passed down this structure although retrograde passage was easy. The portion of the ureter containing the valve was excised and an end-to-end anastomosis was performed. Microscopic sections revealed the transverse fold of mucosa with the underlying muscle bands (fig. 1, B). The postoperative course was complicated inflammation and stricture of the anastomotic site secondary to the migration of the renal calculus to this area. Conservative therapy was of no benefit. The strictured area was excised, the calculus removed and a ureteroneocystostomy, using the Boari bladder flap technique, was performed. This procedure was again followed by a stricture at the anastomotic site due to inflammation secondary to Pseudomonas which was resistant to all antibiotics. Conservative therapy was again unsuccessful; consequently, a left nephro-ureterectomy was necessary. H6 was discharged shortly afterward and has been well since.
CASE REPORT
A 22-year-old Caucasian male sailor was admitted to the Philadelphia Naval Hospital on August 15, 1960 with the chief complaint of left flank pain 3 weeks in duration. He gave no history of hematuria but while at sea 18 months previously he had had an episode of "kidney infection" which responded to antibiotics. No radiographic studies were done at that time. Physical examination was normal except for minimal left flank tenderness. All of the admission laboratory studies were normal except the urinalysis which showed 10 mg. per cent albumin. X-ray studies revealed a normal cystogram,
DISCUSSION
Chwalle's membrane is usually considered to be the embryological explanation for congenital valves in the distal ureter. No satisfactory explanation has been given for upper uretera1 valves. In 1927 Chwalle 2 described physio 2 Chwalle, R.: Process of formation of cystic dilation of the vesical end of the ureter and of diverticula at the ureteral ostium. UroL & Cutan. Rev., 31: 499-504, 1927.
Accepted for publication January 30, 1963. * Current address: Department of Urology, U. S. Na val Hospital, San Diego, Calif. 1 Wall, B. and Wachter, H. E.: Congenital ureteral valve; its role as a primary obstructive lesion: classification of the literature and report of authentic case. J. Urol., 68: 684-690, 1952. 43
: I
44
BUSCH, WEIBEL, MORRIS AND POHL
FIG. 1 TABLE
Case Report
Hunner and Wharton: J. Urol., 15: 57, 1926 Cabot: Boston Med. & Surg. J., 196: 276, 1927 Gottlieb: Ztschr. Urol. Chir., 26: 301, 1929 MacLean: .T. Urol., 54: 374, 1945 Wall and Wachter' Simon, Culp and Parkhill: J. Urol., 74: 336, 1955 Ibid. Foroughi and Turner: J. Urol., 81: 272, 1959 Passaro and Smith: J. Urol., 84: 290, 1960 Samellas: J. Urol., 88: 363, 1962 Authors, 1963
Age
1 Type
Location
Method of Diagnosis
Junction of middle and lower third 6 cm. from bladder
Surgery
Sex
Side
12 yrs.
F
L
43 yrs.
M
R
23 yrs.
F
26 yrs.
M
6 mos. 18 yrs.
M F
5 yrs. 68 yrs.
F M
L R
Annular Cusp-like
F
L
94 yrs.
M
L
22 yrs.
M
L
2 cm. below U-P Autopsy junction Valve-like mem- Junction of middle Autopsy and lower third brane Cusp-like 2 cm. above bladder Surgery
5 yrs.
Cusp-shape
Exactly like valves of veins R Like aortic valves R Annular with pin-point opening L&R Annular L Annular
Iris-type
Just below U-P junction Junction of upper and middle third Lower third 6cm. belowU-P junction Upper ureter Distal ureter
Autopsy Autopsy Surgery
Surgery Surgery Surgery Autopsy
45
CONGENITAL URETERAL VALVE
logical atresia of the mouth of the ureterical bud where it branches from the wolffian duct in a 12 to 28 mm. embryo. The occluding membrane is composed of epithelium on the underside identical to the embryonic bladder mucosa while the upper side is identical to the ureteral epithelium. In embryos over 20 mm. in length, the membrane becomes progressively thinner and at approximately the 30 mm. stage, when metanephric urinary secretion begins, the membrane is ruptured by pressure necrosis and ureteral patency is re-established. Chwalle used the persistence of this membrane to explain the etiology of ureteroceles. The implication that Chwalle's membrane caused congenital valves came later. The physiological atresia of the embryonic ureter has been confirmed by Brown3 who noted 3 Brown, A.: An analysis of the developing metanephros in mouse embryos with abnormal kidneys. Amer. J. Anat., 47: 117-172, 1931.
that the metanephros in mice embryos starts to function about the fifteenth or sixteenth day of fetal life. At this same time there was a valvelike obstruction in the lower end of the ureter creating temporary hydronephrosis and hydroureter. Four or five days later the obstruction either disappeared spontaneously or the hydroureter persisted into adult life. SUMMARY
A case report of a congenital ureteral valve is described and the literature is reviewed. Only IO cases of this uncommon cause of ureteral obstruction have been noted in the literature (table 1). Chwalle's membrane, as seen normally in a 12 to 28 mm. embryo, is still the only embryological explanation for distal ureteral valves. The etiology of upper ureteral valves remains in doubt.
Vol, 90, No. 1 July 1963 Copyright © 1963 hy The Williams & Wilkins Co.
Printed in U.S.A.
CONGENITAL URETERAL VALVE FREDERICK M. BUSCH,* DAVID C. WEIBEL, WILLIAM E. MORRIS C. EDWARD POHL
AND
From the Department of Urology, United States Naval Hospital, Philadelphia, Pa.
A congenital ureteral valve is, indeed, rare. In fact, only 10 cases in the literature meet the rigid criteria of Wall and Wachter. 1 These authors in 1952 made an extensive review of the literature and clarified the subject by listing the following definite criteria for these valves: 1) anatomically demonstrable transverse folds of ureteral mucosa which contain bands of smooth muscle, 2) changes of obstructive disease above the valve with normal urinary tract below, 3) no other evidence of mechanical or functional obstruction. Therefore, the commonly noted mucosal folds seen in tortuous, dilated ureters cannot be included as they are manifestations of obstructions lower down in the urinary tract. These folds or kinks are acquired, while true valves are congenital in origin and contain all structural layers of the ureter except for the adventitia. It is also important not to confuse a congenital valve with a congenital ureteral stricture. The valve is a transverse fold of mucosa with underlying muscle while a congenital stricture has a narrowed lumen with no structural changes of the mucosa. The following case report describes such a valve.
but an excretory urogram showed a poorly functioning, markedly dilated, left collecting system and ureter with a small, irregular calculus in the lower calyx. The right kidney was normal. A retrograde pyelogram confirmed the findings of the excretory urogram and dern.onstrated an obstruction just above the left ureterovesical junction (fig. 1, A). Cystoscopy was normal except for diminished activity of the left ureteral orifice. On September 15, under general anesthesia, the left ureter was explored and a cusp-like valve was noted approximately 2.0 cm. above the ureterovesical junction. A probe could not be passed down this structure although retrograde passage was easy. The portion of the ureter containing the valve was excised and an end-to-end anastomosis was performed. Microscopic sections revealed the transverse fold of mucosa with the underlying muscle bands (fig. 1, B). The postoperative course was complicated inflammation and stricture of the anastomotic site secondary to the migration of the renal calculus to this area. Conservative therapy was of no benefit. The strictured area was excised, the calculus removed and a ureteroneocystostomy, using the Boari bladder flap technique, was performed. This procedure was again followed by a stricture at the anastomotic site due to inflammation secondary to Pseudomonas which was resistant to all antibiotics. Conservative therapy was again unsuccessful; consequently, a left nephro-ureterectomy was necessary. H6 was discharged shortly afterward and has been well since.
CASE REPORT
A 22-year-old Caucasian male sailor was admitted to the Philadelphia Naval Hospital on August 15, 1960 with the chief complaint of left flank pain 3 weeks in duration. He gave no history of hematuria but while at sea 18 months previously he had had an episode of "kidney infection" which responded to antibiotics. No radiographic studies were done at that time. Physical examination was normal except for minimal left flank tenderness. All of the admission laboratory studies were normal except the urinalysis which showed 10 mg. per cent albumin. X-ray studies revealed a normal cystogram,
DISCUSSION
Chwalle's membrane is usually considered to be the embryological explanation for congenital valves in the distal ureter. No satisfactory explanation has been given for upper uretera1 valves. In 1927 Chwalle 2 described physio 2 Chwalle, R.: Process of formation of cystic dilation of the vesical end of the ureter and of diverticula at the ureteral ostium. UroL & Cutan. Rev., 31: 499-504, 1927.
Accepted for publication January 30, 1963. * Current address: Department of Urology, U. S. Na val Hospital, San Diego, Calif. 1 Wall, B. and Wachter, H. E.: Congenital ureteral valve; its role as a primary obstructive lesion: classification of the literature and report of authentic case. J. Urol., 68: 684-690, 1952. 43
: I
44
BUSCH, WEIBEL, MORRIS AND POHL
FIG. 1 TABLE
Case Report
Hunner and Wharton: J. Urol., 15: 57, 1926 Cabot: Boston Med. & Surg. J., 196: 276, 1927 Gottlieb: Ztschr. Urol. Chir., 26: 301, 1929 MacLean: .T. Urol., 54: 374, 1945 Wall and Wachter' Simon, Culp and Parkhill: J. Urol., 74: 336, 1955 Ibid. Foroughi and Turner: J. Urol., 81: 272, 1959 Passaro and Smith: J. Urol., 84: 290, 1960 Samellas: J. Urol., 88: 363, 1962 Authors, 1963
Age
1 Type
Location
Method of Diagnosis
Junction of middle and lower third 6 cm. from bladder
Surgery
Sex
Side
12 yrs.
F
L
43 yrs.
M
R
23 yrs.
F
26 yrs.
M
6 mos. 18 yrs.
M F
5 yrs. 68 yrs.
F M
L R
Annular Cusp-like
F
L
94 yrs.
M
L
22 yrs.
M
L
2 cm. below U-P Autopsy junction Valve-like mem- Junction of middle Autopsy and lower third brane Cusp-like 2 cm. above bladder Surgery
5 yrs.
Cusp-shape
Exactly like valves of veins R Like aortic valves R Annular with pin-point opening L&R Annular L Annular
Iris-type
Just below U-P junction Junction of upper and middle third Lower third 6cm. belowU-P junction Upper ureter Distal ureter
Autopsy Autopsy Surgery
Surgery Surgery Surgery Autopsy
45
CONGENITAL URETERAL VALVE
logical atresia of the mouth of the ureterical bud where it branches from the wolffian duct in a 12 to 28 mm. embryo. The occluding membrane is composed of epithelium on the underside identical to the embryonic bladder mucosa while the upper side is identical to the ureteral epithelium. In embryos over 20 mm. in length, the membrane becomes progressively thinner and at approximately the 30 mm. stage, when metanephric urinary secretion begins, the membrane is ruptured by pressure necrosis and ureteral patency is re-established. Chwalle used the persistence of this membrane to explain the etiology of ureteroceles. The implication that Chwalle's membrane caused congenital valves came later. The physiological atresia of the embryonic ureter has been confirmed by Brown3 who noted 3 Brown, A.: An analysis of the developing metanephros in mouse embryos with abnormal kidneys. Amer. J. Anat., 47: 117-172, 1931.
that the metanephros in mice embryos starts to function about the fifteenth or sixteenth day of fetal life. At this same time there was a valvelike obstruction in the lower end of the ureter creating temporary hydronephrosis and hydroureter. Four or five days later the obstruction either disappeared spontaneously or the hydroureter persisted into adult life. SUMMARY
A case report of a congenital ureteral valve is described and the literature is reviewed. Only IO cases of this uncommon cause of ureteral obstruction have been noted in the literature (table 1). Chwalle's membrane, as seen normally in a 12 to 28 mm. embryo, is still the only embryological explanation for distal ureteral valves. The etiology of upper ureteral valves remains in doubt.