Ureterocele: A Clinical Study and A Report of Thirty-Seven Cases

URETEROCELE: A CLINICAL STUDY AND A REPORT OF THIRTY-SEVEN CASES GERSHOM J. THOMPSON Section on Urology, Mayo Clinic AND

LAURENCE F. GREENE Fellow in Urology, Mayo Foundation, Rochester, Minnesota

The cystoscopic observation of a ureterocele frequently comes as an agreeable surprise to the urologist. Until cystoscopy has been performed the diagnosis usually remains obscure. There are no pathognomonic symptoms. Excretory urography in cases of ureterocele will often reveal changes such as ureterectasis or pyelectasis, but only rarely will such a study clearly demonstrate a ureterocele as the underlying cause of the urinary obstruction. The discovery of a ureterocele is a source of pleasure to the urologist because it usually enables him to explain the cause of the patient's symptoms. Of even more importance, the urologist knows that by the use of comparatively simple surgical procedures he can eliminate obstruction to the outflow of urine, hence eliminate infection from the urinary tract and halt slow, but inexorable, destruction of renal tissue. Because of the gratifying results which may be expected from the treatment of ureterocele, once the diagnosis is made, we consider it appropriate to report a series of 37 cases which were observed at the Mayo Clinic. The cases varied from those in which the condition was entirely asymptomatic and required no treatment, to those in which the condition had resulted in severe renal injury necessitating nephro-ureterectomy. ETIOLOGY

Both a congenital and an acquired origin of ureterocele have been advanced, but the consensus is in favor of the former. Gottlieb stated that the formation of ureterocele depends upon the simultaneous occurrence of two factors, namely, an abnormally narrow ureteral orifice and a weakness of W aldeyer's sheath. It was his opinion that an abnormally narrow orifice, alone, produces retrograde dilatation while a weakness of W aldeyer's sheath, alone, produces herniation of the ureter. The views of Gottlieb are most widely accepted and have recently been upheld by O'Conor and Johnson. Other authors suggested an agglutination of the ureteral orifice during fetal life, a hyperplasia of the vesical and ureteral musculature in the orificial part of the ureter and an abnormally oblique course of the ureter through the vesical wall. The proponents of the theory of congenital origin point to the frequent coexistence of ureterocele and manifestly congenital malformations as additional evidence that the condition is not acquired. Spooner and Lindsey reported a case of ureterocele and malformations of the genito-urinary system in which the patient was an infant 4 days old. Patch and Lavandera estimated that asd 800

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sociated congenital anomalies of the genito-urinary system were present in 50 per cent of the cases. Congenital anomalies were present in only 24 per cent of our series of 37 cases. These consisted of bilateral duplication of the pelves and ureters in one case, complete unilateral duplication in 5 cases, congenital stricture of the external urethral meatus, elevation of the posterior vesical lip and abnormal insertion of the ureter into the pelvis. The early age of some of the patients would lend support to the congenital origin of ureterocele. Caille reported a case in which the patient was an infant, 2 weeks old. In 23 of a group of 32 cases reported by Campbell, the patients were less than 14 years of age. Riba recently reported 2 interesting cases in which the patients were identical twins; symptoms resulting from the ureterocele were present in 1 case and absent in the other. Other writers, notably Petillo, have completely discarded the theory of congenital origin of ureterocele and have stressed the acquired nature of the condition. Petillo pointed out that local infection of the genito-urinary tract (seminal vesiculitis, inflammation of the broad ligaments) may interfere with the innervation of the vesical end of the ureter, causing nerve block, paralysis and atony and consequent dilatation of the intramural part of the ureter. On the other hand, O'Conor and Johnson in a study of 19 cases, did not observe a definite or permanent atony of the ureter. That ureterocele may be of acquired origin is clearly shown in one of our cases. The patient, a woman, aged 34 years, first came to the clinic in 1922, complaining of urinary frequency and dysuria. Cystoscopy was performed and a granular urethritis noted; the ureteral orifices were considered to be normal. In 1935 the patient returned to the clinic because of recurrent attacks of urinary frequency, urgency, chills, fever and terminal hematuria. Cystoscopic examination revealed a ureterocele, 1 by 2 cm. in diameter, on the right, as well as pyelectasis, caliectasis and ureterectasis involving the right kidney and ureter. It is our belief that most ureteroceles are of congenital origin but it cannot be denied that in an occasional case the ureterocele is acquired. Sex and age. Most authors found that the incidence of ureterocele is greater among females than among males. O'Conor and Johnson, on the other hand, stated that the condition is found equally in both sexes in spite of the fact that in 12 of the 19 cases which they reported the patients were females. Martius said: "The condition occurs almost twice as frequently in women as in men." Our findings are in accord with this statement. In our series of 37 cases, 25 patients were females and 12 were males. Although most of the earlier clinical observations concerned young females in whom the ureterocele presented at the external urethral meatus, with the widespread use of cystoscopy it became evident that the condition occurs at all ages. It is known that ureterocele may remain entirely asymptomatic and only the advent of infection or obstruction calls attention to its presence. Thusthe youngest patient in our series who had symptoms referable to the ureterocele was nine years of age; the oldest, 64 years. Twenty-four patients, approxi-

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mately two-thirds of the entire series, were between the ages of 30 and 50 years when the ureterocele was first detected. SYMPTOMS

There is nothing characteristic about the symptoms of ureterocele. The most frequent symptom noted was pain. Of 23 patients complaining of pain, 14 stated that the pain was most severe in the renal region, whereas 8 patients experienced pain in the lower part of the abdomen or in the groin; in 1 instance the pain was localized to the perinea! region. In several cases the patients described a similar type of pain; in most of these cases the ureterocele contained calculi. The pain was felt in the bladder at the termination of micturition and was severe and lancinating in character; extension to the renal region was observed in some cases. Sargent reported a case in which ureterocele interfered with micturition. We agree with him that it is, of course, mechanically possible for a large ureterocele to fall into the vesical outlet, particularly near the end of micturition. It also seems logical that a distended ureterocele might, as the bladder empties, cause almost the same effect as a stone or foreign body and, particularly if inflammation is present, lead to vesical spasm and hence cause pain. Such vesical symptoms as urinary frequency, dysuria and nocturia were present in seventeen of the 37 cases. Thirteen patients noted gross hematuria and the passage of urinary calculi was observed by 2 patients. Five patients without symptoms referable to the urinary tract were studied urologically in an attempt to find the cause of obscure abdominal symptoms; in each instance a ureterocele was discovered. CYSTOSCOPIC APPEARANCE

· Lazarus stated that ureterocele most frequently involves· the left ureteral orifice. Likewise, in a series of 19 cases, O'Conor and Johnson noted the presence of a left ureterocele in ten cases, a right ureterocele in 4, and bilateral ureteroceles in 5 cases. On the other hand, Campbell found the incidence of right and left ureteroceles to be equal; in 15 per cent of the cases a ureterocele was present bilaterally. In our series the incidence of right, left and bilateral ureteroceles was equal; in 13 cases the ureterocele was located on the right, in 12 on the left and in 12, bilaterally. As viewed cystoscopially, the appearance of the ureterocele will vary with its size. During the resting stage of a small ureterocele one usually observes a pinpoint ureteral orifice situated on what appea;s to be a papilla. Immediately before urine spurts from the meatus, however, the purported papilla suddenly balloons into the form of a rounded cystlike tumor which projects into the cavity of the bladder; the uretral orifice is carried forward by the tumor and is situated on its summit. A thin, forceful jet of urine then escapes from the ureteral orifice, the cystlike tumor collapses and the ureterocele again assumes the appearance noted during the resting stage. A moderate or large ureterocele appears as a permanent, broad-based, cystlike

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tumor which occupies the site of the ureteral orifice. The ureteral orifice is most commonly situated eccentrically and frequently on the posterior aspect of the ureterocele. The detection of the ureteral orifice may be aided by the intravenous administration of indigo carmine but frequently it is impossible, and as will be brought out.later, unnecessary, to find the ureteral orifice. The vesical mucosa covering the ureterocele may be smooth and shiny or inflamed; in the former instance the appearance of a benign vesical neoplasm may be closely simulated. With each ballooning, the walls of the ureterocele become thin and transparent and fine tracery of the blood vessels becomes strikingly prominent. If there are considerable inflammation and coincident edema of the mucosa, the cystoscopist may mistakenly consider the lesion to be a sessile broad-based neoplasm. A very large and inflamed ureterocele may confuse even a highly skilled urologist; repetition of the examination, sometimes under anesthesia, to insure satisfactory relaxation, may be necessary before a ;diagnosis can be ~~i The size of the ureteral orifices is of importance because of its theoretic relationship to the formation of ureterocele. In 16 cases the orifice was pinpoint in size while in 8 cases the orifice was not visible. In 4 cases the orifice was of sufficient size to admit a 4 or 5 F. catheter; in the remaining 9 instances no mention was made of the size of the orifices. DIAGNOSTIC VALUE OF EXCRETORY UROGRAPHY

As observed in an excretory cystogram, a ureterocele appears as a spherical or sausage-shaped filling defect or sometimes accentuation of media situated near the trigonal region and in line with the terminal portion of the ureter (fig. la). A fine, distinct halo is occasionally noted immediately about the ureterocele (fig. lb). These roentgenographic findings may be considered pathognomonic of ureterocele. When the ureterocele contains stones, opaque shadows will be noted within the filling defect (fig. 2a, b, c, d, 3a, band c). Most writers state that excretory urography is of great aid in the diagnosis of this condition and should be employed as a routine method of examination. Examination of the excretory urograms made in our cases fails to support this view wholeheartedly. Twenty-one cases were studied by means of excretory urography and a diagnosis of ureterocele was established without the necessity of cystoscopy in only 4 instances; in one other case the excretory urogram was merely suggestive of ureterocele. Large ureteroceles,' as well as small ones, were not visualized by excretory urography, which indicates that size alone was not an important factor. The roentgenographic deformity noted most frequently was an irregular dilatation of the lower third of the ureter; as the dilated ureter approached the bladder it gradually tapered down to a point. Such a deformity we believe is not characteristic of ureterocele but may be observed in cases of ureterovesical obstruction due to several other causes. It is not our intent to depreciate the value of excretory urography in the study of a patient who has a ureterocele. By means of excretory urography it is

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GEHSHOM J, THO,lPSO:\ .\ND LAURENCE F . GRl£ENJ
pos8ible to determine the pre,;ence of other types of congetnial anomalies of the urinary tract, to estimate the function of both kidneys, to note secondary in the urinary tract which result from the ureterocele, and to identify 8hadows of obscure nature present over the vesical area, such as stones in a ureterocele. Finally, in a small percentage of cases it is possible to make a diagnosis of urP· terocele by means of excretory urography alone. \Ve wish to point out, however,

Fm. la. l;rogram showing filling defect on one side and accentuation of media on h, halo noted about ureterocole on ea.ch side. Bes(, visualized on the right.

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that excretory urography umially j,, not sufficient to establish the diagnosis aud it is pradically always necessary to resort to cystoscopy. 'l'HEATMEJ\T

A ureterocele may be discovered in a case in which the patient has no complaints and whose urinary tract, in all other respects, is normal. In ,meh cases tlw ureterocele is discovered during urologic examination carried out for the purpose of explaining bizarre abdominal complaints. If the urologist is eonfidcnt

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Fm. 2a. Excretory cystogram showing marked filling defect caused by ureterocclc; calculi are clearly vislrnlizcd; I,, postoperative excretory cystogrnm showing filling defect practically gone; the meter has returned to normal siie; c, rocntgenogram sliowing wlculi which were situated within the ureterocele; d, calculi evacun,tcd from the ureterocle after trans urethral incision.

that the ureterocele is not responsible for these: symptoms, treatment is not necessary. It i:;; sufficient to inform the patient of the presence of the ureteroeele and suggest that he undergo an examination of the urinary tract at some future

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GERSHONl J. THOMPSON AND LAURENCE F. GREENE

date if other Hymptoms develop. In our series of 37 cases, no surgical or urologic measures were considered necessary in 9 instances. Cystm,copic dilatation of the mouth of the ureterocele by means of ureteral bougies and catheters and irrigation of the renal pelvis and ureter with antiseptic

Fm. 3a. Excretory urogram showing large ureterocele on right side; halo delineating its edge is clearly visible; b, roentgenogram showing calculi in ureterocele and dilated lower ureter; c, calculi evacuated from the ureter after transurethral incision of the uretcrocele.

solutions were employed before the introduction of high frequency currents and before the advent of cystoscopic knives and scissors. Such methods of therapy are acceptable by virtue of their antiquity and were carried out in only three cases in our series. In a small percentage of cases, dilatation may be expected to

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improve drainagP, n'dllC(' infcction ancl ameliorate syrnptoms. l\lorc frcqnently, however, dilatation of the ureteral orifice will he of temporary and partial benefit only and repeated dilatation will be required. lVIore often than not, dilatation produced temporary edema vYhich increased the pain and discomfort. The more radical methods of treatment should be substituted for simple dilatation. Simple trnnsurethrnl fulguration of the ureteral orifice or, better than that, nreteromeatotomy can be expected to yield most satiRfaetory result;;:. The former procedure consists of the introduction of a fulgurnting electrode into the nreteral orifice and the destruction of the uretcrocele by mearrn of the electrocoagulation current. Simple fulguration alone was suecesRfully employed in 3 caRes. Temporarily fulgnration may increase the degree of obstruction which the ureteroecle causes, and unlel:ls there is some technical reaRon for it:-s nRe, incision with a cutting current or scissors is preferred. Any of a variety of acceptable cystoscopie scissors and knife elcctrodeR ma)' be employed in performing ureteromeatotomy. Lacking these, the urologist can fatihion a very efficient cutting instrument by removing the lead tip and a few millimeters of insulation from an ordinary wire electrode; if the end of thic: wire is bent slightly to make it comma shaped, excellent cutting can be accomplished 'With a cutting current by using any type of cystoseope. Incision of a ureterocck in an infant is easily done by using such a wire electrode through a Nitzc, Butterfield or infant cystoscope. \Vhen possible, all supcrfluons tis;;:uc should he removed. However, as a general rule, the redundant tissue quickly retract,; into the surrounding tisRncs from which it originally sprung. It i;, for thi;; reason that transurethral procedures in cases of ureterocele are practically always as good if not better than open i'inrgical procedures involving excision of this cxecss tissue. ~VIeatotomy was performed in 17 car,es in this sericc:: and thn average period of postoperative hospitalization in this group was only 2 days. In some eac:es the c:urgcon may fail to find the nrcteral orifice. This offcri-3 no obstacle, however, and the most acces:c;ible point in the ureterueele if' selected for folgurntion or incif-!ion, Thf' incision c:hould be large, certainly equal to the diameter of the dilated meter. In several of our ea:::es an inci,-;ion 2 or :3 cm. in length was made. Calculi in a ureterocde, of :mch :c,izc as to require litbolopax:r, aft.er they have been pulled into the bladder, may he easily and Rafdy rernovc~d. Small storn:s which remain in the ureterocclc following incision may be f'vacnatcd by prn,sing a eystrn,cope which has no heak, ::mch as a small l\IcCarthy panendmwope, into the ureterocelc and washing them out A urethral catheter should be im,c1ted to drain the bladder for the first 24 hours or until all bleeding has stopped. Late bleeding oceurring several weeks after rneatotomy is observed infr('quent.ly hut it will not frighten the patient if hr) has been told that it might occur. It will stop spontancom:ly almost ,vithout exception. Open surgical proePdure.c.: were required in .5 cases. This group included tho:-;e eases in which renal function had been destro?ed or in ,vhich urinary calculi ,vhich could not be removed by transurethral methods were present. The operations performed consisted of nephro-ureterectomy with excision of the

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ureterocele, nephrectomy, nephrolithotomy, ureterolithotomy, and bilateral ureterolithotomy combined with pelviolithotomy. Postoperative treatment of the infection which is practically always associated with ureterocele is, of course, important. If the incision has been large enough and if free drainage of urine from the dilated ureter has been established, the infection will disappear rapidly. A small dose of a urinary antiseptic should be given and the patient observed periodically. Excretory urography, if one elects to use it, will reveal graphic evidence of the benefit which operation has provided. If symptoms persist, cystoscopic examination, in order to inspect the meatotomy to make certain that scarring has not caused stenosis of the artificial meatus, is advisable. Postoperative dilatation of the ureteral orifice is, as a rule, not necessary if the operation has been correctly done. Routine dilatation we regard as meddlesome. RESULTS OF

TREATMENT

The elimination of infection and of symptoms as previously stated is very striking. The disappearance of ureterectasis can be expected in all cases except those in which there is extreme dilatation of the ureters at the time of admission. We have not subjected all of our patients to routine urographic study a number of months after operation for the reason that, lacking symptoms, some of them refused to submit to the inconvenience and expense of such study. In a number of cases, however, urographic study was repeated with very gratifying results. In no instance was it necessary at a later date to perform nephrectomy because ureteral meatotomy had failed. Nor was it necessary to open the bladder to excise any redundant tissue. The large size of some of the ureteroceles in this series of cases is a very interesting point. To the present date stones have not recurred in any case in which stones were removed from the ureterocele. There were no deaths in this series of 37 cases. The operation seems to us one which can be regarded as rather safe. However, attention is called to the fact that this is a small series of cases. SUMMARY AND CONCLUSIONS

Although some ureteroceles are acquired, the majority are of congenital origin. Ureteroceles occurred twice as frequently among women as they do among men. The ages of the patients varied between 9 and 64 years; two-thirds of the patients were between the ages of thirty and fifty years. The symptoms noted by the patients in the order of their frequency were pain, urinary frequency, dysuria, gross hematuria and the passage of calculi. The diagnosis can always be established by cystoscopic examination. The diagnosis can be established by excretory urography alone in only a small percentage of cases. The most acceptable forms of treatment are simple fulguration of the small

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ureteral orifice or a combination of ureteromeatotomy and fulguration of the walls of the ureterocele. Open operation is necessary in the presence of destroyed renal function or urinary calculi which cannot be removed by transurethral methods. REFERENCES CAILLE, AuGusTus: Prolapse of the inverted lower portion of the right ureter through the urethra of a child two weeks old. Am. J.M. Sc., 96: 481-486, 1888. CAMPBELL, M. F.: Ureterocele. J. Urol. 46: 598-611, 1941. GOTTLIEB, J.: Zur frage iiber Pathogenese und Therapie der Ureterocele. Ztschr. f. urol. Chir., 19: 345-367, 1926. LAVANDERA, M.: Further observations on ureteroceles; review of the literature and a report of four cases. Surg., Gynec. & Obst. 32: 139-140, 1921. LAZARUS, J. A.: Cystic dilatation of the lower end of the ureter: special reference to the transurethral treatment with the high frequency cntting current; report of two cases. J. Urol., 36: 139-149, 1936. MARTIUS, H.: Zur Behandlung der Ureterocele vesicalis. Zentralbl f. Gyn:ik. 61: 327-333, 1927. O'CoNoR, V. J., AND JOHNSON, A. B.: Ureterocele; a clinical study of nineteen cases. J. U rol., 23: 33-42, 1930. PATCH, F. S.: Ureterocelc; with report of a case. J. Urol.16: 125-136, 1926. PETILLO, DIOMEDE: Ureterocele; clinical significance and process of formation; report of four cases. Surg., Gynec. & Obst., 40: 811-818, 1925. RrnA, L. W.: Ureterocele; with case reports of bilateral ureterocele in identical twins. Brit. J. Urol., 8:119-131, 1936. SARGENT, J.C.: Blind ureterocele: report of case with unusual clinical and pathologic aspects. J. Urol., 36: 499-502, 1936. SPOONER, C. JVI., AND LINDSEY, lVIAUDE L.: Intravesical ureteral cyst, associated with embryonic cartilage in the kidney of a newborn infant. J. Urol., 17: 453-459, 1927.