Congenital Valve in the Upper Ureter

Congenital Valve in the Upper Ureter

CONGENITAL VALVE IN THE UPPER URETER JOHN T. MACLEAN, SURG. LT. COMDR., R.C.N.V.R. From the Urological Special Treatment Centre at Montreal Military H...

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CONGENITAL VALVE IN THE UPPER URETER JOHN T. MACLEAN, SURG. LT. COMDR., R.C.N.V.R. From the Urological Special Treatment Centre at Montreal Military Hospital, Montreal

A congenital valve in the upper ureter is extremely rare, although valves occurring at the normal narrowings of the ureters are said to be present in twenty per cent of all individuals3•5 • Clinically they are almost always located at the ureteral orifices or in the bladder. An embryological explanation of their presence in this region is offered by Vermooten. 9 Eisendrath 4 in 1912 showed in his figure 19 a ureter containing spiral twists, constrictions, and a valve in the upper third, although the case is not described in the text of the paper. Hunner 8 in 1927 reported a case of a double left kidney with double ureter in a girl of 12 years. The ureter that drained the upper kidney opened into the vestibule just beneath the external urethral orifice. The accessory ureter showed a congenital valve formation with dilatation above it. A histological section of the valve is shown. Gottlieb 7 is quoted by Eisendrath and Rolnick 6 as having reported in 1929 a clinical case which caused obstruction. "In this case a large hydronephrosis was found at operation due to a valve in the uppermost portion of the ureter. Unfortunately no drawing of the specimen accompanied the report, so that it is uncertain whether the valve was primary or secondary to a kinking of the ureter by a hydronephrosis." 6 CampbelP in his figure 194 shows a case of multiple ureteral valves which were diagnosed clinically, and confirmed at autopsy 2 • The detailed report of a case of a congenital valve at the junction of the middle and upper thirds of the right ureter, with surgical excision of the valve, is presented. CASE REPORT

R. vV., V-43296, a 26 year old male patient, was admitted to the Royal Canadian Naval Hospital, Esquimalt, B. C., March 6, 1943, complaining of frequency, nocturia, and dysuria of 1 month's duration. In addition he complained of pain in the right costo-vertebral angle of 4 days' duration. There was no history of haematuria, trauma, or of having passed sand or gravel. His previous history was non-contributory, with the exception of his having been a bed wetter up until the age of 10 years. 1 Campbell, M. F.: Pediatric Urology. New York: The Macmillan Company, 1937, vol. 1, p. 285. 2 Campbell, M. F.: Personal communication. 3 Caulk, J. R.: Megaloureter: The importance of uretero-vesical valve. J. Urol., 9: 315, 1923. 4 Eisendrath, D. N.: Congenital malformations of ureters. Ann. Surg., 55: 571, 1912. 6 Eisendrath, D. N.: Congenital strictures of the ureter. Surg. Clin. N. America, 4: 565, 1924. 6 Eisendrath, D. N., and Rolnick, H. C.: Urology. Philadelphia: J. B. Lippincott Co., 1943, p. 537. 7 Gottlieb: Ztschr. Urol. Chir., 26: 301, 1929. 8 Runner, G. L.: Ureteral stricture and chronic pyelitis in children. Am. J. Dis. Child., 34: 603, 1927. 9 Vermooten: A new etiology for certain types of the dilated ureters in children. J. Urol., 41: 455, 1939.

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Physical examination was essentially normal throughout, except for tenderness in the right costo-vertebral angle, and a temperature of 98.6°F. The right kidney could not be palpated. The blood pressure was 100/60 mm. Hg. Urinalysis showed albumin 1 plus, pus cells 50-100 per high power field. A stained smear showed a few cocci present. Kahn, negative. Blood count: hemoglobin 98 per cent, red blood cells 5,100,000, white blood cells 6,550. An intravenous pyelogram series showed a normal sized kidney in normal position on each side, with no evidence of calculus within the urinary tract. The pyelogram on the left side was normal. On the right side there was a moderate hydronephrosis with apparent obstruction at the ureteropelvic junction, and dilatation of the upper end of the right ureter. The patient was given sulphathiazole therapy for 10 days, followed by man-delic acid therapy. His urine became free of infection, and cystoscopic examination was carried out. At cystoscopy a No. 24 F. cystoscope was passed with ease. The trigone was normal in appearance. The ureteral orifices were normal in size, shape, and position. Clear urine was seen to come from the left orifice, none from the right. The bladder wall elsewhere was normal in appearance. There was no evidence of tumor, ulceration, diverticulum, or stone. AN o. 5 F. ureteral catheter was passed up the left ureter to the renal pelvis, and a No. 5 F. up the ureter on the right to a point where it met obstruction at the junction of the middle and upper thirds of the ureter. Repeated injections of diodrast were made until the moderate hydronephrosis above the obstruction, and the ureter below the obstruction were satisfactorily demonstrated, the obstrnction itself being clearly defined (figs. 1, 2 and 3). A diagnosis of stricture in the upper third of the right ureter was made. At a subsequent cystoscopic examination, repeated attempts to pass bougies beyond the point of obstruction in the upper ureter were unsuccessful. One cubic centimeter of phenolsulphonphthalein dye was given intravenously. At the end of 1 hour, 40 per cent was recovered from the left side, none from the right side, and 8 per cent from the bladder. Operation on the right kidney was recommended, having in mind some type of plastic procedure, depending upon the actual conditions found at operation. Operation had to be postponed almost 5 months because of recurrent attacks of an acute upper respiratory infection. Operation wa,.:, finally carried out on July 29, 1943. A right curved loin-kidney incision was made. The skin and subcutaneous tissues were divided, the bleeding points clamped and tied. The external oblique, internal oblique, transversus abdominis, and a portion of the latissimus dorsi, were divided. Bleeding points were clamped and tied with No. 1 plain catgut. The lumbar fascia was incised, and the incision enlarged by blunt dissection. The twelfth nerve was seen in the posterior angle of the wound. The perirenal fascia was opened with a straight haemostat. The opening was enlarged by blunt dissection. The kidney was completely freed up by blunt dissection. The pelvis and upper ureter, which were dilated, were carefully examined without discovering the cause of the dilatation. The ureter was freed down to near the pelvic brim. Its calibre remained

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the same in cross section (approximately 1 cm.). The pelvis of the kidney was then opened and a catheter passed down the ureter to the junction of the middle and upper thirds where it met obstruction which could not be passed. Various

Fm. 1. Intravenous pyelogram, demonstrating hydronephrotic kidney on right side, and normal kidney on left,

Fm. 2. Ureterogram showing abrupt cessation of ascent of dye up right ureter. Obstruction is at level of third lumbar vertebra. Note very thin thread-like streak of dye in ureter above this point.

sized ureteral catheters were then tried, but would not pass the point of obstruction. A longitudinal incision was therefore made over the point of obstruction, revealing a valve, the thickness of which was equal to the thickness of the wall of the ureter. This valve had a minute opening the size of a pin point. The valve

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was cut away from its attachments to the circumference of the wall of the ureter with scissors. AN o. 14 F. catheter was inserted through the pyelotomy incision above and threaded through the ureter to a point 3 inches below the incision in the ureter. Several eyes were made in the catheter in the area that would lie within the renal pelvis. The opening in the ureter was closed transversely over the catheter, and a layer of fat applied. A nephrostomy was then done through a thin scarred area of kidney tissue, bordering on the inferior major calyx. A No. 36 F. tube was used. It was desired to thread the catheter in the ureter through the nephrostomy tube, but this could not be done, so the catheter was brought out through the flank incision

Frn. 3. Pyelogram and ureterogram showing ureteral valve opposite transverse process of third lumbar vertebra on right side. At operation valve was found to be thickness of ureteral wall.

separately. The two tubes were then brought out through the posterior angle of the wound, along with two Penrose drains and the incision closed in layers. The lumbar fascia and transversus abdominis were closed with a continuous running suture of No. 1 plain catgut; the internal oblique, external oblique, and the fascia covering the external oblique, each as a separate layer, with a continuous running suture of No. 1 plain catgut. The subcutaneous tissue was closed with interrupted sutures of No. 1 plain catgut, and the skin with silk. The patient withstood the operation well, and was returned to the ward in good condition. On the second post operative day the patient was doing very well, and had a normal temperature. The ureteral catheter (No. 14 F.) was draining well; the

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nephrostomy tube was hardly draining at all. The patient was coughing up a moderate amount of thick, blood streaked phlegm. That evening his temperature went up to 101 °. Examination of the chest did not reveal any abnormal physical signs. On the fourth postoperative day the patient was still bringing up a considerable amount of thick mucoid-purulent sputum with rusty blood streaking. Temperature was 101 °. The chest was dull to percussion over the whole left side. BlmYing breathing was present. He had consolidation of the greater portion of the left lung. Roentgenogram of the chest showed complete atelectasis of the left lung (fig. 4). The wound was clean and healing well. One of the Penrose drains was removed, and the other shortened. The nephrostomy tube was irrigated gently with saline. It was felt that the patient's condition

Fm. 4. Roentgenogram of chest taken on fourth postoperative day showing complete atelectasis of left lung.

was rather desperate and that unless the obstruction of the left main bronchus ·was removed, he might be in very serious trouble. A bronchoscope was therefore passed by Surgeon Commander Paton, and the trachea aspirated. During this procedure the patient coughed up two very large plugs of thick, blood stained, mucoid material. Sulfathiazole therapy, 5 gm. a day, was continued. The following morning his temperature dropped to normal, and there was exchange of air going on in the left lung. He continued to run a temperature between 98° and 102.5° until the fifteenth postoperative day when it reached normal and stayed normal thereafter. The ·wound healed very nicely. On the eighth postoperative day one half of the skin sutures were removed. Mercurochrome in saline was injected into the soft

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rubber catheter acting as a ureteral splint. Half an hour later the patient was voiding red-colored urine, thus demonstrating the patency of the right ureter. On the eleventh postoperative day the remaining skin sutures were removed, the wound being completely healed, except for the site where the tubes em8rged from its posterior angle. The rubber catheter acting as a ureteral splint came out of its mrn accord on the eleventh postoperative day. Mercurochrome in saline was

Fm. 5. (Left), pyelogram and ureterogram 5 weeks after operation showing ureter of good calibre at site of former obstruction opposite third lumbar vertebra on right side. (Right), pyelogram and ureterogram 13 weeks after operation, showing considerable reduction in size of hydronephrosis. Ureter is of good calibre.

injected into the nephrostomy tube and the tube clamped off. The patient later voided mercurochrome-stained urine, again demonstrating the patency of the right ureter. The repeat roentgenogram of the chest done on this date showed almost normal appearance on the left side, with only a slight degree of atelectasis present. On the thirteenth postoperative day there ·was some separation between the superficial fatty layer of the wound and the underlying muscles. The nephrostomy tube which was then draining only ½ ounce in 24 hours, was re-

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moved, and the skin and subcutaneous tissues at the posterior angle of the wound were opened wider. A latex multiple-eyed drain was inserted as a through and through drain from the point at the anterior end of the incision to the posterior angle of the wound. Irrigation with saline was carried out, but healing was not satisfactory. Four days later the skin and superficial fat were opened by sharp dissection along the line of the former incision. The wound was then allowed to granulate in. On the thirty-first postoperative day the wound was healed except for a small opening at the anterior end, this appeared as though it would heal in a day or two, and it did. On the thirty-seventh postoperative day cystoscopy was carried out and a No. 7 catheter passed up the right ureter to the renal pelvis with ease. A pyelogram and ureterogram were made (fig. 5 left). The patient was discharged from the hospital on his forty-fifth postoperative day at which time the urine was not infected, he was voiding normally, the wound was well healed, and his chest was clear to physical examination. The patient was readmitted to the hospital 8 weeks later. A No. 24 F. cystoscope was passed with ease. The trigone was normal in appearance. The ureteral orifices were normal in size, shape, and position. Clear urine was seen to come from each orifice. Nos. 6 and 7 F. ureteral catheters were passed up the right ureter with difficulty. An attempt was made to pass a No. 9 Garceau catheter up the right ureter, but it could not be passed more than ¼cm. beyond the right orifice, where it was held in the folds of mucous membrane. AN o. 7 F. catheter was therefore reinserted to the renal pelvis. A plain roentgenogram was taken, and a pyelogram made by injecting 10 cc of diodrast. A combined pyelogram and ureterogram was made by pulling the catheter down into the lower ureter and injecting more diodrast. The films show a normal sized kidney on the right side in good position. The hydronephrosis on the right side is considerably reduced in size in comparison to the preoperative film. The right ureter is somewhat enlarged throughout, being almost 1 cm. in width. The calibre is the same throughout. There is no evidence of stricture at the site of the previous valve which was removed at operation (fig. 5, right). This patient returned to full duty at sea 2½ months after operation, and remained entirely well. CONCLUSIONS

Congenital valve in the upper ureter is believed to be rare. A detailed report of a case of a congenital valve occurring at the junction of the middle and upper thirds of the right ureter, with surgical removal of the valve, is presented. Three months after removal of the valve there was considerable reduction in the size of the hydronephrosis. It is surprising that the patient did not have urinary symptoms before the age of twenty-six. One would have anticipated recurring attacks of pyelonephritis. This patient returned to full duty at sea 2½ months after operation, and remained entirely well.

Ste. Anne's Hospital, Ste. Anne de Bellevue, Que.