Ureterocele, its etiology, pathogenesis and diagnosis

SURGICAL

REVIEW

Ureterocele,

Its Etiology, Diagnosis

Pathogenesis

LEONARD PAUL WERSHUB, M.D. AND THOMAS J. KIRWIN, M.D., Diplomates, Urology, New York, New York From the Department

Board

of

infants. EIeven years Iater Freyere found two stones in a ureterocele and it was he who first suggested that calcuIi might be a primary cause. The first case of ureteroceIe to be observed cystoscopicaIIy was reported by LipmannWulP in 1899. At cystoscopic examination he beIieved that he was viewing a tumor of the bIadder; only at operation did he recognize that what he thought was a tumor was actually cystic diIation of the Iower end of the ureter. AIthough GrosgIik* is credited with the first endoscopic recognition of ureteroceIe in 1901, it was not untiI three years later that Cohn9 became the first surgeon to operate on one which he had correctly diagnosed through the cytoscope. The first extensive report on this subject was made by Marmier’O in 1913. This author studied forty-two cases that he had collected from AIbarran, Pasteau, Bazy and others of the period, and found that of these forty-two collected cases thirty-eight showed evidence of stenosis of the uretera orifice. It is of historical interest to note that the first case of a ureteroceIe reported in the American literature was that of CaiIIe,” published in 1888. AIthough the cases cited heretofore present the morphoIogic picture of ureterocele, the term first suggested by Stoeckel*2 did not come into use unti1 1912, when Leshnew13 empIoyed it. Prior to this date the pathoIogic entity was referred to as either “second bIadder” or “doubIe bIadder.” EngIischel4 in 1898 introduced the expression “cystic diIatation of the vesicaI end of the ureter,” an expression which is stiI1 prevaIent today. Fenwick16 and BIum16 objected to this terminoIogy, pointing out

of Urology, New York Medical College, Flower and Fijtb Avenue Hospitals. Tbis paper was delivered at tbe Annual Meeting of tbe American Urological Society, May 13, 1953, St. Louis, MO.

contributions made to the literature on ureteroceIe, the subject is stir1 vague in many respects. Therefore repeated emphasis of the cIinica1 picture, radiographic evidence and descriptions of the cystoscopic appearance of ureteroceIe are stiIl justified. We have reviewed the Iiterature and considered the theories of etioIogy, the pathogenesis and the radiographic evidence of this not too rare condition in the hope of defining its characteristics more cIearIy. The presence of a ureteroceIe does not necessariIy impIy a pathologic Iesion with definite clinical symptoms. In fact, as wiII be discussed Iater, there are no pathognomonic symptoms associated with ureterocele per se. Yet ureteroceIe is potentially a cIinicopathoIogic entity which sooner or Iater may become a surgical probtem. Prior to the beginning of the twentieth century ureteroceIes were found onIy on the operating tabIe or at postmortem examination. LechIerl in 1834 gave the first clear description of what is today termed “ureterocele,” although he mistook the condition for a double bladder. Again, in 1856 a case was described by LiIienfeId2 which he calIed a “second bIadder which projected into the Iumen of the urethra.” StrubeI3 in 1858 ligated a proIapsed ureterocele which appeared at the externa1 meatus in a girI of fourteen. Wrany’s4 case, described in 1870, was that of a Iittle girI who had three ureters, one of which bulged into the bIadder as a sac about the size of a hazelnut. Weigert6 in 1886 described two additional cases in stillborn

D

American

and

ESPITE many

317

UreteroceIe

2

I

FIG. 1. Ureterocele, or dilation of that part of the ureter which has no muscular support, i.e., the intravesical termination. On endoscopic examination this dilation presents a semi-transIucent appearance, and the superficial bIood vessels may give the impression that its waII consists onIy of mucous membrane. The ureteroceIe may vary in size from a single centimeter in diameter to a size HIing most of the bIadder cavity. FIG. 2. It is possibIe to observe the ureteroceIe HI, distend and suddenIy coIlapse foIIowing ejacuIation of urine. With collapse, the vesica1 waI1 and ureteral orifice may appear essentiaIIy normal. The cystoscopist may note after coIlapse of the ureterocele a wrinkled stalk, the size of which is in direct proportion to the size of the distended ureteroceIe. As demonstrated in this figure, the deflated cyst may appear as a conica projection with a smaI1 uretera orifice on its top.

that the name “cyst” is misIeading since the diIation does not have the structure of a true cyst. Fenwick16 proposed in Igo3 that the term “baIIooning of the ureter” be utiIized. In Igzo BIumle reviewed the Iiterature coIIecting IOO cases and recommended the term “intravesica1 proIapse of the ureter.” Only six years before, Kotzenburg” spoke of the condition as ureteral “cysts” and in 1917 PIeschne+ advocated the term “phimosis of the ureter.” The convenience of having a singIe word to describe this entity offsets the etymoIogic inadequacy of the derivation of the word. The word ureteroceIe is compounded of two Greek words meaning ureter and hernia. Etiology and Pathogenesis. Having accepted the name “ureterocele,” it now becomes necessary to define cIearIy the morphoIogic picture presented by this condition. Gutierrezlg pointed out that the different types of ureteroceIe described in the Iiterature “are in reaIity the expressions of a singIe disease entity, the difference being that in some the pathoIogica1 condition is more advanced than in others.” A ureteroceIe is a diIation of that part of the 318

ureter which has no muscuIar support, that is, the intravesica1 termination. It is composed of two Iayers with a quantity of areoIar tissue and occasionaIIy some intervening muscIe tissue. These Iayers Iie back to back; one derived from the vesica1 mucosa, the other from the ureter. Viewed cystoscopicaIIy, it has a very smaI1 ureteric orifice which may be situated in the center of a reddish, nippIeshaped prominence. OccasionaIIy the orifice may not be Iocated in the center but on the side, depending upon the contour of the diIation. The diIation on endoscopic examination presents a semi-transIucent appearance, and the superficia1 bIood vesseIs may give the impression that its waI1 consists only of mucous membrane. (Fig. I.) When biIatera1, the only variation from this description may be in the size of the two ureteroceIes. A ureteroceIe20 may vary from I cm. in diameter to a size fiIIing most of the bIadder cavity. The extreme size of the Iesion is demonstrated in those cases which may actuaIIy protrude from the externa1 urinary meatus into the vagina. There are two schools of thought as to the

UreteroceIe etiology of this condition, one believing it to be congenital, the other holding that it is an acquired lesion. Gottlieb2’ studied IOO cases collected from the Iiterature and five of his own. He was able to show that stenosis or stricture aIone could not be the sole cause of a ureterocele, because if this were so, the condition would be more common than it is. He believed that there must be some congenital weakness or inferiority of the connective tissue of the ureter, especially that of Waldeyer’s sheath. He further stated that if this weakness alone existed, the result would be a herniation of the ureter; but if there were only a stricture, the resutt would be retrograde dilation. Neither factor aIone can cause the lesion; a combination of both factors is needed. UnfortunateIy, Gottlieb confuses the issue here by stating that while weakness of the connective tissue fiber might be congenital, the stenosis might be either congenital or acquired through inflammation, stone or tumor. ChwaIIa22 observed in embryos 12 to 30 mm. in Iength an epitheIia1 downgrowth which separates the ureter from the primary excretory duct. A defect might occur in the development of the embryo some time between the appearance of the ureteric bud at 12 mm. Iength and the separation of the ureter from the primary excretory duct and cloaca at about 28 mm. length. In the normal course of embryonic development this epitheIia1 spur wouId be reabsorbed when bIadder and rectum became separate and distinct organs. Persistence of this spur or primitive valve has been cited as an embryologic expIanation of stenosis of the ureteral orifice, thus accounting for one of the congenital factors in the production of ureterocele. The other embryologic factor wouId be as mentioned heretofore, the congenitaI weakness or inferiority of Waldeyer’s sheath. PiersoI23 describes Waldeyer’s sheath as folseveral centimeters above Iows : “Beginning the bIadder the adventitia of the ureter is strengthened and thickened by robust Iongitudinal bundIes of invomntary muscle that foIIow the duct to its vesical orifice and, in conjunction with the fibrous tissue in which they are imbedded, form the ureteral sheath.” When this sheath is poorly developed,*4 it is not difficult to visualize how a narrowed vesical orifice of the ureter would result in 319

such a degree of pressure from the reno-ureteral side, as to herniate a Ioosely attached Iower portion of the ureter. Although Campbell26 accepts the postulate of weakness of Waldeyer’s sheath, he also emphasizes that no convincing proof of this is present. In fact he states, “nor is such a condition essential to the genesis of ureterocele; rather it well may be the result of ensuing back pressure. As the urine coming down from the ureter meets the impeding meatal stenosis, a back-pressure factor is buiIt up, the total of which is the renal excretory pressure and the hydrostatic pressure of the urine Iess the reIative drainage coefficient of the abnormahy smaIl meata opening. As this force strikes the meatal obstruction, the contiguous uretero-vesica1 mucosa is dilated and bahooned outward, the process proceeding to a clinica ureterocele.” The proponents of the theory of acquired origin ignore the possibility of congenital factors in the production of ureterocele, stressing only the acquired factors in its production. CauIkz6 is of the opinion that many ureteroceIes represent a condition secondary to an inffammatory process which may have resuhed from a IocaI ulcer, ureteritis fohowing a prior renaI Iesion or even from the passage of stones. PetiIIoz7 beheves that ureteroceIes are the result of a paralysis of the lower end of the ureter with failure of its contractile power. He considers the paraIysis to be a seque1 of infection, that is, postinfection adhesions between the ureter and the broad ligaments in the female, or the seminal vesicIes in the maIe. The underlying factor would be salpingitis or seminar vesiculitis, and the ureterocele would be the fina end product of the infectious process. This theory is opposed by the findings of O’Conor and Johnson28 who were unable to demonstrate permanent or definite atony of the ureter in any of the nineteen cases of ureterocele they studied. We are in compIete accord with Gutierrezr9 who affirms that stenosis and muscuIar weakness of the ureter taken together are the basic factors in the production of ureterocele. Gutierrez states, “The normal physiological action of the uretera musculature is impaired by this anatomical weakness. Pyeloscopic studies of the upper urinary tract have revealed the continuous peristahic waves that are present in the normal ureter. Any weakness or malformation in the intramural portion of

UreteroceIe the uretera muscuIature wouId interfere with this physioIogica1 dynamism and invite formation of a ureterocele.” Most authors writing on this subject tend to favor congenita1 origin mainIy because this theory is strengthened by the high incidence of the condition associated with other congenita1 anomaIies. It is our beIief that most ureteroceles are of congenita1 origin, but we cannot deny that in an occasional case the condition may be acquired. Distribution. Riba quotes Young as stating in Igo3 that only about seventeen cases of ureteroceIe had then been reported. With the improvement of the uroIogist’s endoscopic and radioIogic armamentarium, the number of cases reported since that time has progressiveIy increased. CampbeIIZ5 gives the figure as I to 2 per cent of a11 patients subjected to cystoscopic examination and notes further that it is doubtIess missed in many examinations. Thompson and Greenezg found the incidence to be 24 per cent in a series of thirty-seven cases. Its association with congenita1 anomaIies of the urinary tract appears to be higher. Patch30 and Pavendera estimated it at 50 per cent of cases. Most authors emphasize that the incidence of ureteroceIe is higher among females than in maIes. Martius31 states, “The condition occurs aImost twice as frequently in women as in men.” In contrast to this beIief, O’Conor and Johnson2s were of the opinion that ureterocele existed equaIIy in both sexes, yet in their series of nineteen cases tweIve of the patients were femaIes. The condition has been found in the newborn and has aIso been reported in patients who have reached the seventh decade. Gross and coIIected twenty-one cases of CIatworthy32 ureteroceIe over a period of twenty years at the ChiIdren’s HospitaI in Boston. These authors beIieve that even though ureteroceIes are reIativeIy few in number, pediatricians shouId be reminded of the fact that congenita1 abnormaIities of the urinary tract are common and that they are often folIowed by pyuria and a wide variety of compIaints. These authors aIso stress the high degree of renaI damage caused by ureteroceIes, the ease in diagnosis of the disease and the reIativeIy simpIe means of cure. CampbeII33 does not beIieve ureteroceIe is as rare in chiIdren as reported and believes that when uroIogic examination is carried out in chiIdren more often, a vastIy greater number of ureteroceIes wiI1 be observed. CampbeII fur320

ther believes that the advanced upper urinary tract damage consequent to obstructive ureteroceIe and infection keeps many chiIdren from reaching aduIthood. UreteroceIe was found in one of twenty-five infants and chiIdren uroIogicaIIy examined by CampbeI126v34,36 because of persistent pyuria. However, it was found in onIy four of 15,919 of this age group examined postmortem. In an additiona survey of 36,005 autopsies over fifteen years no ureteroceIes were found. This is not surprising since the ureterocele coIIapses after death and may easiIy be overIooked. In fact, smaI1 ureteroceIes examined in vivo may be overIooked, particuIarIy if examined during the phase of coIIapse. (Fig. 2.) In Smith and Orkin’s statistica study of anomaIies of the genitourinary system based on 18,400 admissions to the Royal Victoria HospitaI, MontreaI, onIy one case of ureteroceIe was identified. When one considers that in this Iarge statistica study the most frequent congenita1 anomaly was that of doubIe renaI peIvis and ureter which occurred ninety-seven times for a cIinica1 incidence of I : Igo (20.7 per cent), it is difFicuIt to accept the finding of one ureteroceIe in this series since a11 authors agree that ureteroceIes are reported in from 24 to 50 per cent of a11 cases in which there are other coincidenta anomaIies. Some authors28,2s,33,36 have been impressed by the finding of ureteroceIe more frequentIy on the Ieft side as compared to its presence on the right side. Others, particuIarIy CampbeII,33 have found the incidence of right and Ieft ureteroceIe to be equa1. Thompson and Greenezs found in their series of thirty-seven cases that in thirteen the ureteroceIe was Iocated on the right, in tweIve on the Ieft and in tweIve biIateraIIy. Others estimate that the disorder is uniIatera1 in approximately 85 per cent of cases and biIatera1 in 15 per cent. Symptomatology and Diagnosis. As stated previousIy, the presence of a ureteroceIe does not necessariIy impIy a pathoIogic lesion with definite cIinica1 symptoms. ObviousIy the size of the ureteroceIe is in direct proportion to the cIinica1 manifestations as produced by obstructive uropathy. If the ureteroceIe is smaI1, there may be no symptoms at aI1; and if infection is concomitantIy present, the symptoms may be mereIy those of the infection. There is no doubt that many cases of ureteroceIe have existed for a period of years without symptoms

UreteroceIe

FIG. 3. The pathologic ureter. If biIatera1, the as here demonstrated. uropathies (see Figures

effect of ureteroceIe may be that of obstruction with diIatation of the involved kidney and obstructive factors may produce dilation of the ureter and hydronephrosis on both sides However, even if bilateral but smaI1 in size, the ureteroceIes wiI1 produce no obstructive 5 and 6). Note characteristic halo, more marked on right side.

FIG. 4. This is same case as Figure 3. No halo is demonstrable, for there is a lack of dye in the ureterocele and in the bladder at the time of the exposure, whereas in Figure 3 the exposure was taken at the time that contrast media was in ureter and bladder. FIG. 5. This is a case of bilateral ureterocele, diagnosed cystoscopically. No ha10 is demonstrated despite repeated exposures. Compare with Figure 3, biIatera1 ureteroceIes, Iarger in size. Note displacement downward of bladder by tumor mass (fibroid) and demonstration of retrovesical portions of right and Ieft ureter. FIG. 6. Same case as Figure 5. Retrograde pyeIography aIso failed to demonstrate any radioIogic evidence of biIateraI ureteroceles. The diIation of Ieft pelvis and ureter is undoubtedly due to overdistention with contrast medium.

321

Ureteroceie of any kind. (Figs. 3 to 6.) The symptomatoIogy can be divided into manifestations either of obstruction or infection, but in neither instance are the symptoms su&ientIy characteristic to make an accurate clinical diagnosis possibIe. When the ureterocele is Iarge enough to reach the vesica1 neck, it may act as a vaIve or obstruction, as wouId a median Iobe of a prostate causing obstruction and retention of urine. (In the female it may even pioIapse beyond the externa1 urinary meatus.) With obstruction the clinica picture of renaI and uretera coIic may be produced, bringing attention to the possibiIity of a pathoIogic condition in the upper urinary tract. The pathoIogic effect of ureteroceIe may be that of obstruction with diIation of the invoIved kidney and ureter. (Figs. 3, 4, 7 and 8.) If biIatera1 the obstructive factors may produce diIation of the ureter and hydronephrosis on both sides. (Figs. 3 and 4.) A priori even if biIatera1, but smaI1 in size, the ureteroceIe will produce no obstructive uropathies. (Figs. 5 and 6.) Some authors38,3g claim that the diagnosis of ureteroceIe can be made only cystoscopicaIIy. Others beIieve it may be overIooked cystoscopicaIIy unless suspicion of the lesion is aroused by radiographic evidence. In our opinion, diagnosis rests entireIy upon the cystoscopic examination and rarely upon the radiographic interpretation. The Iatter is of heIp but too frequentIy confused. Some authors36 fai1 to reaIize that radioIogic signs are not aIways present. The cystoscopic picture of an IntravesicaI cyst undergoing periodic gradua1 urinary inff ation and deffation is classic, and it is unlikely that a competent cystoscopist wouId overIook it or confuse it with some other vesica1 disease. A ureterocele may be visualized as a gIobuIar or elongated, sausage-shaped mass at the site normaIIy occupied by the uretera orifice. This mass may vary in size from that of a pea to that of a waInut, and at times may occupy the entire haIf of the bIadder. In the femaIe it may actuaIIy protrude through the urethra. The mass wiI1 appear semitranslucent (Fig. I) and a fine network of smaI1 bIood vesseIs may be detected on its surface of vesica1 mucous membrane. The mucous membrane may appear inffamed and edematous in the presence of infection. The ureteral orifice can, in most instances, be seen on baIIooning 322

of the ureter. It is usuaIIy eccentrically situated and appears as a smaI1, circuIar invagination. The meatal opening is smaI1, onIy admitting a No. 4 or No. 5 uretera catheter. At times it is impossibIe to pass a catheter regardIess of the size used. Its detection may be facilitated by chromoscopy. It is easy to indent the mass with the beak of the cytoscope, differing in this respect from tumor, proIapse or stone with incrustation. On first visuaIizing a ureterocele the cystoscopist may easiIy confuse it with a tumor of the bIadder. However, further inspection and study, particuIarIy during the ejacuIation of urine, will reveal the true nature of the ureteroceIe. The distended cyst may actuaIIy coIIapse and it is frequentIy possibIe further to observe the cyst fiI1, distend and again suddenIy COIlapse. With coIIapse of the cyst the vesica1 waI1 and uretera orifice may appear essentiaIIy normaI. Following coIlapse of the ureterocele, the cystoscopist may visuaIize a wrinkled stalk, the size of which is in proportion to the size of the distended ureteroceIe. At times the deflated cyst may appear as a conica projection with a smaI1 uretera orifice on its top. (Fig. 2.) There may be no eIevation in this phase and the onIy cIue wiI1 be a smaI1 ureteral orifice. RandaII and CampbeII (Cabot4O) beIieve that during the study of these phases the rate of contractions of the ureter is no criteria to diagnosis since some may show hyperactivity and others may have normal or sIuggish contractions. Owing to stenosis of the uretera orifice, catheterization of the invoIved ureter may be diffIcuIt or impossibIe. This is further compIicated by the tendency of the waI1 to invaginate as it is being probed. When ureteroceIe is biIatera1, the opposite side may be similar; but as a ruIe the degree of invoIvement is usuaIIy more marked on one side than the other. In Figures 5 and 6 it can be seen that the ureteroceIe was Iarger on the Ieft side. In this case endoscopy was the onIy means of making the diagnosis. In Figures 3 and 4, it can be noted that the ureteroceIe was Iarger on the right side, and both the right and Ieft side were suffrcientIy Iarge to produce biIatera1 obstructive uropathy. In this case the diagnosis was made endoscopicaIIy and confirmed by the characteristic “ haIo” on x-ray examination. We have not encountered any cases of proIapse of the ureter, We believe that what is commonIy referred to as “proIapse” is actuaIIy

Ureterocele

FIG. 7. The presence of other coincidental anomalies has been reported in from 2~ to 30 per cent of all cases. Dilation of the upper urinary tract in ureterocele associated with ureter4 duplication has been reported by many authors. Such findings favor a congenital origin.

323

UreteroceIe a manifestation of ureteroceIe. Ureteric proIapse is a true uretera proIapse, invoIving a11 the Iayers of the ureter. Winsbury-White3’ describes this condition as a true prolapse of the ureteric mucosa into the vesical cavity, a statement which we cannot accept. This author states, “In the earIier stages the orifice may appear swoIIen and edematous with its Iips congested and pouting. It is raised above the surface and the Iumen is central and puckered. The margins of the prolapse are found on cross section, to consist of two Iayers of ureteric mucosa. In the more advanced stages, a portion of the muscuIar Iayer may intervene, and the proIapse has come to resembIe an intussusception. Narrowing of the Iumen is not marked and may be accounted for aImost entireIy by the associated congestion and edema.” Hinman3* points out that in herniation or proIapse of the Iower ureter the orifice may be Iarge and the uretera mucosa turned out. EndoscopicaIIy there shouId be a smooth, no dificuIty in distinguishing rounded ureteroceIe from the engorged, edematous, irreguIarIy protruberant mass of a ureteral proIapse. AI1 who have visuaIized a ureteroceIe are impressed by the smoothness of the uretera mucosa which is baIIooned and presents the fine vascuIarity seen on norma bIadder mucosa. ProIapse of the ureter is not covered by vesica1 mucosa as is the ureteroceIe. Braasch3g stated in 1928, X-ray Diagnosis. “The appIication of urography to the diagnosis of ureteroceIe is of more scientific interest than practica1 vaIue, as ureterocele is easiIy recognized by ocuIar inspection of the bladder.” However, this was prior to the advent of excretion urography. Today ureteroceIe is frequentIy demonstrated by excretion urography or retrograde urography. Too often, even if radiographic data are obtained, the condition may be erroneousIy diagnosed or compIeteIy overIooked. Thompson and Greene2g do not beIieve that excretory urography is aIways of aid. They point out that in their series of twenty-one cases studied by means of excretory urography a diagnosis of ureteroceIe was estabIished without the necessity of cystoscopy in onIy four instances; and in one case the excretory urogram was mereIy suggestive of ureteroceIe. It is our beIief that the vaIue of excretory urography in the study of a patient who has a ureteroceIe must not be minimized. AIthough

324

we are not in accord with those authors who overemphasize the value of excretory urography, we beIieve that excretory urography may be of vaIue in confirming a diagnosis of ureterocele or in directing attention to its possibiIity. Furthermore, with the popuIarity of excretory pyeIography not onIy by uroIogists but aIso by internists and genera1 practitioners, we believe that emphasis on certain radiographic signs which may be produced by a ureteroceIe is of singuIar significance and importance. Since a ureteroceIe constitutes a menace to the upper urinary tract, excretory urography further aids the doctor to evaIuate and estimate the function of both kidneys and the presence or absence of any architectura1 changes resuIting from the ureteroceIe. Another advantage of excretory urograms is that since ureteroceIes are frequentIy associated with other types of congenita1 anomaIies, such abnormaIities can be identified by the excretory urogram. When the ureteroceIe contains a caIcuIus, attention is caIIed to the ureteroceIe by the Iocation of the caIcuIus, visibIe in a pIain radiogram at the site of the ureteric orifice. If no caIcuIus is present, a diagnosis of ureteroceIe can often be made or suspected by the fiIIing defect produced by the cystic mass. When, in addition, an excretory or retrograde urogram reveaIs a fiIIing defect surrounded by a thin haIo, one can make a diagnosis of ureteroceIe with certainty. (Fig. 3.) RandaII and CampbeI140 beIieve that the dye being concentrated in this area gives the appearance of a haI0 about the saccuIation. Rudhe41 states that the halo sign is significant onIy for ureteroceIes of smaI1, or moderate size, even though it has been described as typica1. We disagree with this statement. In our case of biIatera1 ureteroceIe (Fig. 3) the haI0 is present around the Iarge and smaIIer ureterocele with equal cIarity. In Figures 3 and 6, a case of biIatera1 ureteroceIe, no halo or radiographic evidence is present to confirm or make a diagnosis of biIatera1 ureteroceIe. We believe this evidence is Iacking in this case and in other simiIar cases onIy because of the minima1 enIargement of the ureteroceIe. As impIied before, we aIso believe that such cases fai1 to produce obstructive uropathy. Such was the experience in our series of cases. Some authors fai1 to mention the haIo; others mention it but fai1 to expIain whether it is a constant finding. UnfortunateIy,

Ureteroceie

IO 9 portion of the ureter is well demonstrated in excretory urography. empties sIowIy from this portion of the ureter because of narrowing of the uretera orifice.

8

FIG. 8. The tower or terminal

Here the dye

FIG. g. This demonstrates compIete Iack of radiographic evidence of smaI1 right ureteroceIe, diagnosed by cystoscopic evidence. Whereas Figures 3 and 4 demonstrate the need of contrast substance in ureter and bladder, no halo is produced if ureterocele is of smalI size as demonstrated in Figures 5, 6 and g. FIG. 10. The cobra head is another radiographic the diIated portion of the ureter. See Figure8.

sign of ureterocele. -

It is caused by the intravesical

projection

of

diagnosed by cystoscopic examination. Figures 3 and 6 iIIustrate a case of biIatera1 ureteroceIe diagnosed endoscopicaIIy. In Figure 5 excretory urography faiIed to revea1 any haI0 despite repeated exposures. AIthough biIatera1 ureteroceIes were present (smaI1 and smaIIer than biIatera1 ureteroceIes demonstrated in Figure 3), we were unabIe to demonstrate any radiographic signs despite repeated attempts. Figure 6 represents the same case in which retrograde pyeIography aIso faiIed to demonstrate any radiologic evidence of biIatera1 ureteroceIes. The “cobra head” is another radiographic sign of ureteroceIe. It is caused by the intravesica1 projection of the diIated portion of the ureter. (Figs. 8 and IO.) In such instances the ureteroceIe is not of the spherica shape but is sausage-shaped. The Iower or termina1 portion of the ureter is we11 demonstrated in excretory urography onIy because the dye empties sIowIy from this portion of the ureter due to narrowing of the uretera orifice. This termina1 diIation and buIbous intravesica1 projection of the ureter resembIes a cobra head or spring onion. Braasch and Emmett42 point out that in

this pathognomonic sign is not aIways present. It wiI1 be demonstrated onIy if the exposure is taken when the ureteroceIe is HIed with contrast medium at the same time that contrast medium has reached the bIadder. The halo is produced by the thickened uretera and vesica1 mucosa which, Iying back-to-back, produces a definite difference in density. This difference in density is further accentuated by the presence of contrast medium in the ureteroceIe and in the bIadder. When the dye is not present in the bIadder and ureteroceIe, no haI0 is present. This is we11demonstrated in Figure 3, in which biIatera1 ureteroceIes are demonstrated by excretion urography with production of a distinct haIo. In Figure 4 (same case) no haI0 is demonstrabIe for there was a Iack of dye in the ureteroceIe and bIadder at the time of the exposure. We aIso wish to emphasize at this time that in addition to the foregoing factors essentia1 for the production of a haI0 it has been our experience that no haI0 is produced when the ureteroceIe is of smal1 size. This is we11 demonstrated in Figures 3, 6 and 9. Figure 9 demonstrates compIete Iack of radiographic evidence of smaI1 right ureteroceIe 325

Ureterocele some cases the ureteroceIe does not fiI1 well with contrast medium, and in such cases a “negative” shadow is cast which may easiIy be confused with a non-opaque vesical stone or tumor. We prefer the use of the term “spaceoccupying Iesion” to “negative” shadow. Regardless of the terminology utilized, we never accept such data as sufficient for accurate diagnosis without endoscopic examination. Such radiographic information is of vaIue only in arousing suspicion of a lesion and the need of a complete urologic examination. Urography does not suppIy concIusive evidence; and if one relies on this method aIone, he may miss the is diagnosis. 43 On the other hand, cystoscopy not always avaiIabIe, particuIarIy for chiIdren. It is of interest to note that in the series of Gross and Clatworthy 32 the correct diagnosis was made in ten of their twenty-one cases without cystoscopy but by carefu1 roentgenographic investigation. These authors are in agreement with most writers on the subject and say, “Cystoscopy is not required in a11 cases but generaIIy it is desirable to confirm the diagnosis which has been already made by roentgenographic means or to estabIish the diagnosis when other types of examinations have failed to clearly indicate the nature of the pathoIogy which is present.” We prefer prehminary radiographic investigation by means of excretory urography. It is then our procedure to cystoscope and foIIow with retrograde urograms. In those cases in which the meata opening will not permit passage of a catheter, urography can be accomplished onIy by the injection through the waI1 of the cyst of an opaque medium used for excretory urographic study, as first recommended by Mertz et a1.44 This may be accompIished by simple incision of the cyst waI1 and passage of a catheter through the new opening. SUMMARY

Although ureterocele is not a common disorder, it is probabIy more prevaIent than available statistics indicate. Like other congenita1 anomabes, and there is more evidence for its congenita1 origin than acquired, its presence may not necessariIy mean a disease. Its potentiality as a factor in obstructive uropathy, however, cannot be overlooked. The young cystoscopist should become familiar with the endoscopic picture of ureteroceIe. The more experienced cystoscopist shouId be

wary of its misleading appearance, particuIarIy during the phase of emptying of the ureterocele. It has been our experience that radiographic signs as described in the literature are rarely encountered and that the diagnosis of ureteroceIe rests entirely upon the endoscopic picture. We have further noted and demonstrated in this report that when a ureteroceIe is smal1, even if present biIateraIIy, no radiographic sign is present. When the ureterocele is large, the radiographic clue will be in the form of either a halo or cobra head. REFERENCES I. LECHLER. Fall einer doppelten Harnblase. Med. Car.-Bl. d. wiirttemb. iirztl. Ver., 4: 23-24, 1834. 2. LILIENFELD. Beitraege zur Morphologie und Entwicklungsgeschichte der GeschIectorgane und Beschreibung einer interessanten Missbildung. InauguraI Dissertation, Marburg, 1856. 3. STRUBEL, cited by RIBA, L. W. UreteroceIe: with case reports of bilateral ureteroceIe in identical twins. Brit. J. Ural., 8: 119131, 1936. 4. WRANY. PathoIogisch-anatomische Mitteilungen aus dem Praeger Franz Joseph KinderhospitaI. Oesterr. Jabrb. j. Paediat., I: 1870. 5. WEIGERT, C. Zwei FaeIIe von Missbildung eines Ureter. Arcb.f. pad. Anat., 104: 10-20, 1886. 6. FREYER. Obscure cystic tumor of the bladder containing two caIcuIi. Tr. Roy. M. ti S. Sot., g: ‘897. 7. LIPMANN-WULF. Beobachtungen bei einer vor 14 Jahren Nephrectomierten. CentralbLj. d. Krankb. d. Harn-u. Sex.-Org., IO: g, I 899. 8. GROSGLIK, S. Zur Kenntnis und chirurgischen BehandIung der angeborenen Harnleiteranomalien. M&atscbr. j. fJrol., 6: 575-625, 1901. 9. COHN, T. Ueber Cysternartige Erweiterung des HarnIeiters innerhaIb der Blase. Beitr. z. klin. Cbir., 41: 45-79, 1904. IO. MARMIER, R. Contribution a I’etude de Ia diIatation kystique intravesicaIe de I’extremite inferieure de I’uretere. These de doct., Paris, 1913, J. d’urol., 4: 503, 1913. 1 I. CAILLE, A. Prolapse of the inverted portion of the right ureter through the urethra in a chiId two weeks old. Am. J.-M. SC., 95: 481-486, 1888. I 2. STOEKEL, cited by WILENIUS, R. UreteroceIe. Ann. cbir. et gynaec..Fenniae, 39: 78, 1950. 13. LESHNEW, N. F. Ureterozele. Abstr. in: Zentralbl. j. Cbir., p. 156, 1912. 14. ENGLISCHE, J. Ueber cysternartige Erweiterung des BIasenendes des Harnleiters. Centralbl. f. d. Krankb. d. Harn-u. Sex.-Org., 9: 373-406, -1898. 15. FENWICK, E. H. Ureteric meatoscopy in obscure diseases of the kidney. Lance& p. 1246, 1903. 16. BLUM, V. Die intrav&ikaIe bIasige Erwditerung und der Prolaos des unteren Harnleiterendes. Arcb.j. klin. C&r., 113: 13~168, 1920. 17. KOTZENBERG. Beitraexre zur kasuistik der Zvsten des vesikalen Ureterostium. Med. K&n., p. 104, ‘9’4. ”

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UreteroceIe 18. PLESCHNER. Fall von dippeIseitiger zystischer Dilatation des vesikalen Ureterendes. (Phimosis des Ureters.) Arch. f. klin. Cbir., 108: 49-51, 1917. 19. GUTIERREZ, R. Modern surgica1 treatment of ureteroceIe. Surg., Gynec. c~ Obst., 68: 611-630,

der UreteroceIe 31. MARTIUS, H. Zur BehandIung VesicaIis. Zentralbl. f. Gyniik., 5 I : 327, 1927. 32. GROSS, R. E. and CLATWORTHY, H. W., JR. Ureterocele in infancy and chiIdhood. Pediatrics, 5: 6877, 1950. 33. CAMPBELL, M. The diIated ureter in chiIdren. A brief consideration of its causes, diagnosis and treatment. Am. J. Surg., 39: 438-451, 1938. 34. SMITH, E. C. and ORKIN, L. A. CIinicaI and statistical study of 471 congenita1 anomalies of kidney and ureter. J. Ural., 53: I I, 1945. 35. LAZARUS, J. A. Cystic dilatation of the Iower end of the ureter; specia1 reference to the transurethra1 treatment with the high frequency cutting current; report of two cases. J. Ural., 36: 139-149, 1936. C., BERANBAUM, L. S. and HAMILTON, 36. GO~LIEB, H. R. Radiographic features of ureterocele. Radiology, 60: 64, 1953. of Gen37. WINSBURY-WHITE, H. P. A Text-book ito-urinary Surgery. Edinburgh, 1948. E. S. Livingstone. 38. HINMAN, F. The Principles and Practice of UroIogy. Philadelphia, 1937. W. B. Saunders Co. p. 468, 2nd ed. 39. BRAASCH, F. W. Urography, Philadelphia, 1928. W. B. Saunders Co. 1936. 40. CABOT, H. Modern UroIogy. PhiIadeIphia, Lea & Febiger. 41. RUDHE, U. A typica picture of very Iarge ureteroceIes. Acta radiol., 29: 396402, 1948. 42. BRAASCH, W. F. and EMMETT, J. L. CIinicaI Urography. Philadelphia, 1951. W. B. Saunders Co. simuIating bIadder 43. BURGER, A. J. S. Ureterocele caIcuIus. South African M. J., 24: 298-300, 1950. 44. MERTZ, H. O., HENDRICKS, J. W. and GARRETT, R. H. Cystic ureterovesica1 protrusion; report of four cases in chiIdren and two in aduIts. J. Ural., 61: 506, 1949.

1939. 20. MACALPINE, J. B. Cystoscopy and Urography, pp. Baltimore, 1949. WiIIiams & WiIkinS 234-242. co. 2 I. GO~LIEB, I. G. Zurt Frage ueber Pathogenese und Therapie der Ureterocele. Ztscbr. f. urol. Cbir., 19: 345-367, 1926. 22. CHWALLA, R. Eine bemerkenswerte AnomaIie der Harnblase bei einem menschlichen Embryo von 32.5 mm. St. Sch. L. Vircbows Arch. f. patb. Anat., 263: 632448, 1927. 23. PIERSOL, A. G. Human Anatomy, 6th ed. PhiIadeIphia, 1907. J. B. Lippincott Co. 24. EISENDRATH, D. N. and ROLNICK, H, C. UroIogy, p. 548, 4th ed. PhiIadeIphia, 1938. J. B. Lippincott Co. 25. CAMPBELL, M. UreteroceIe. Surg., Gynec. Ed Oh., 93: 705-718, 1951. 26. CAULK, J. R. Ureterovesical cysts. An operative procedure for their relief. J. A. M. A., 61: 16851688, 1913. 27. PETILLO, D. UreteroceIe: cIinica1 significance and process of formation. Report of 4 cases. Surg., Gynec. 0 Obst., 40: 811-818, 1925. 28. O’CONOR, V. J. and JOHNSON, A. B. UreteroceIe; a clinica study of nineteen cases. J. Ural., 23: 33-42, 1930. 20. THOMPSON. G. J. and GREENE. L. F. UreteroceIe: a clinica study and a report of thirty-seven cases. J. Ural., 47: 800-809, 1942. 30. PATCH, F. S. UreteroceIe, with report of case. J. Ural., 16: 125, 1926.

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