URETEROCELE WITH PROLAPSE THROUGH THE URETHRA JOHN L. EMMETT Section on Urology AND
GEORGE B. LOGAN Section on Pediatrics ivlayo Clinic, Rochester, Minnesota
An unusual case has recently been encountered at the Mayo Clinic which seems of sufficient interest to report. REPORT OF CASE
A Mexican girl, aged 2 years, was brought to the clinic with a rather curious history and complaint. Her father stated that since infancy she had suffered from urinary frequency and incontinence associated with dysuria which seemed to vary in intensity. Recently some blood had been present in the voided urine. Several days before admission to the clinic a mass had protruded from the region of the vagina. The father stated that the mass resembled a tomato in size and appearance. The physicians who examined the child were of the opinion that it was a hernia of the vagina. This condition had caused the patient considerable pain and had increased the urinary symptoms. Some time during the trip to the clinic the mass had disappeared but the urinary symptoms continued. On examination nothing could be found which could suggest the origin of the condition recounted by the father. Physical examination was essentially negative. Urinalysis revealed pus, graded 3 on a grading basis of 1 to 4, and erythrocytes, graded 3. Culture of the urine showed Escherichia coli. The excretory urogram (fig. 1) revealed a slight amount of pyelectasis and ureterectasis on the right side and a large filling defect in the bladder. The filling defect was similar to that seen in the bladder of a male when a large median lobe of the prostate gland is present. Cystoscopic examination was performed with the McCarthy infant cystoscope. A large mass was @.countered lying in the base of the bladder. The upper surface of the mass was necrotic and appeared to be the site of a previous slough. The mass which could be moved from side to side was attached to the right side of the base of the bladder, but was so large in relation to the size of the infant's bladder that it was difficult to view it satisfactorily. The most likely diagnosis seemed to be a large ureterocele which had prolapsed through the urethra and had sloughed sufficiently in one place to relieve the urinary back pressure. A Bugbee electrode with the distal tip cut off and the wire bared of insulation for a distance of 0.5 cm. was used; several cuts were made in the mass with the cutting diathermy current. There was no collapse of the mass so typical of that seen in the usual case of ureterocele. This observation further supported the assumption that the slough had ruptured the wall of the ureterocele and relieved the urinary obstruction. Nothing further was done at that time. Following this procedure the dysuria and frequency continued. Vesical 19
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JOHN L. EMMETT AND GEORGE B. LOGAN
spasm suggested that the bladder was trying to expel .a foreign body. It seemed likely that owing to long standing inflammation the sac of the ureterocele had become so thick and edematous that it was now acting like a foreign body and must be removed. Accordingly· cystoscopic examination was performed with the child under nitrous oxide anesthesia. By means of the 18 F. Thompson infant resectoscope the mass was completely resected and removed. At the conclusion of the resection the bladder was entirely clear and it could be seen that the point of attachment of the mass was in the region where the right ureteral orifice is normally situated. Following the operation a small retention urethral catheter
Fm. 1. Excretory urogram showing moderate right pyeloureterectasis and filling defect in bladder from right ureterocele.
was left in place for 48 hours. When it was removed, the child voided normally and all her urinary symptoms were gone. COMMENT
As far as we can determine, this is the first case of ureterocele with prolapse that has been encountered at the clinic. vVe were curious to know how common this lesion might be. Reviewing the literature we have found more than 37 cases reported. We say more than 37 because there were some articles that we have been unable to secure. All cases reported have been in females. As yet no case has been reported in which the ureterocele has been able to traverse the male urethra. Of the 37 cases we have been able to find, twelve were cases of children who ranged in age from 13 days to 14 years (table 1). Only 5 of these 12 children survived operation. The first patient reported in the literature
URETEROCELE WITH PROLAPSE THROUGH URETHRA
21
who had a prolapsed ureterocele and ,vho survived an operation was a girl 14 years of age. Patron1 referred to her as a "robust maiden." No doubt she had to be robust to survive the treatment, which consisted of ligation of the sac at the urethral orifice. Two of the older patients, who were between 20 and 30 years of age, had had prolapses of their ureteroceles when they were younger, one when she was only 8 years of age. One girl, a circus performer 19 years of age, had her first prolapse when she was fourteen and then continued to practice her profession of horseback riding until she was nineteen despite frequently recurring prolapses. She finally became unable to reduce the prolapse without digital manipulation and then consulted a physician. Two of the patients had had intermittent prolapses much longer than that, one for 12 years and one for 20 years. Caille in 1888 reported an interesting c·ase of a prolapsed ureterocele in an infant girl aged 2 weeks. The true nature of the lesion was not appreciated. The child died 12 hours following ligation of the sac which was thought to be a TABLE
1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12.
1.-Cases of prolapsed ureterocele in children less than fifteen years of age reported in available literature AUTHOR
YEAR
Patron ... Beach. Davies-Colley .... Caille .... Gecrdts. von Hibler. Gcipel and Wollenberg. Goertz .. Mayer ... Campbell. Campbell. Thomas ...
1857 1874 1879 1888 1887 1903 1905 1921 1922 1941 1941 1942
AGE OF CHILD
14 yrs. 5 mos. 1½ yrs. 2 wks. 3 wks. 6 wks. 13 days "Une petite fille" 14 yrs. 6 yrs. 20 mos. 2 yrs. 10 mos.
prolapse of a vesical diverticulum. Only at necropsy was the true condition appreciated. The etiology and pathogenesis of ureterocele have been widely discussed. The consensus seems to favor a congenital origin. As a result of congenital stenosis of the ureter, which also may sometimes be due to an inflammatory condition, and a probable congenital weakness of the wall of the lower part of the ureter, dilatation of this part of the ureter takes place and then ballooning out into the bladder occurs. Prolapse occurs when the lesion becomes large. The size and appearance of the prolapse have been described as that of a ripe fig or a 1 Patron's article was abstracted in Schmidt's Jahrbucher, 1858, vol. 100, pp. 222-227 by Streubel. Preceding this case report was the sentence "Vf. selbst hat im J. 1850 nachstehenden interessanten Fall beobachtet." Apparently this has always been interpreted as meaning that the case was observed by Streubel. The presence of the same case report in Patron's article clarifies this point. In Streubel's review reference is made to a case of a girl of two to three years of age reported by Crosse (Transact. of the prov. med. and surg. assoc. vol. 14, Nov. 2, 1846) who had a prolapse of a mass through the urethra. This may have been a prolapsed ureterocele but as cystoscopes were unknown and as the child did not die, we cannot be sure. The same applies to the reported case of Patron.
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JOHN L. EMMETT AND GEORGE B. LOGAN
tomato. Ureteroceles are lined on their bladder aspect by vesical mucosa and internally by ureteral mucosa, between which are fibrous tissue and some smooth muscle fibers. Prolapse of the ureterocele through the urethra is accompanied by pain, dysuria and bleeding. Anuria occurs if the tumor blocks the urethral opening completely. The diagnosis can frequently be made on seeing the lesion, providing that the physician is aware of the possibility of its occurrence. It can be confirmed by excretory urography and cystoscopic examination. TABLE
2.-Cases of prolapsed ureterocele occurring in adults reported in available literature AUTHOR
YEAR
AGE
years
1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14. 15. 16. 17. 18. 19. 20. 21. 22. 23. 24. 25.
Furni val. .......................................... . Simon ................ . Hartman ....... . Pietkiewicz. Von Franque ... Kolisko ... . Nelsen .. . Santrucek. Pendl* ... Schroeder ...... . Pribram....... . ....... . Kehrer ................ . Jeanbrau .... . Gifert. Goldberg. Davis and Owens. Runner ... . Runner .................. . Rhodes ................... . Herrick ..................... . Schultze-Rhonhof ......... . H. H. Schnid-Reichenberg ................ . Huth...... . .................. . Earlam ..... MacPherson ....................... .
190,1
mos HJll 1911 1913 1921 1921 1920 1921 1922 1925 1926 1927 1928 1932 1928 1935 1935 1936 1936 1938
16
52 Not given Not given Not given Not given Not given 33 Not given
1939 1940 1942
45 19 32 20 47 31 21 34 29 22 19 58 27 49 27
* Case demonstrated before Congress of Urology in Vienna in September, 1921.
In most of the cases reported in recent years in which an accurate preoperative diagnosis has been made, open surgical methods of treatment have been employed. The most common procedure is to open the bladder suprapubically and excise the protruding ureterocele. Most ureteroceles can now be cared for transurethrally. In the average case simple excision of the ureterocele with the cutting diathermy current ,vill prove sufficient as the collapsed sac left behind ·will atrophy or slough away. In the occasional case, however, as in the one reported herein, the walls of the sac may become so thickened from inflammation, trauma and edema that it may be of sufficient bulk to act as a foreign body of more or less solid consistency. In such a case complete resection of the sac with the resectoscope will be necessary,
URETEROCELE WITH PROLAPSE THROUGH URETHRA
23
REFERENCES BEACH, FLETCHER: Bladder containing a pouch, which opens into a dilated coiled tube (a ~hird ureter), communicating with the right kidney by a smooth walled cavity, which 1s shut off from the remaining portion of the kidney. Tr. Path. Soc. London, 25: 185-186, 1874. CAILLE, AUGUSTUS: Prolapse of the inverted lower portion of the right ureter through the the urethra in a child two weeks old. Am. J.M. Sc., 95: 481-486, 1888. CAMPBELL, M. F.: Uretcrocele. J. Urol., 45: 598-611, 1941. GIBERT, J.: Dilatation kystique de l'uretere etranglee au meat uretral. J. d'Urol., 25: 468-470, 1928. DAvrns-CoLLEY, N.: Specimen of malformation and disease of the ureter and bladder in a female child 18 months of age. Lancet, 1: 372-373, 1879. DAVIS, EDWIN AND OWENS, C. A.: Prolapse of the ureter through the urethra. Tr. Am. A. Genito-Urin. Surg., 21: 367-372, 1928. EARLAM, M. S.S.: Double ureterocele. Australian & New Zealand J. Surg., 10: 80-82, 1940. VON FRANQUE, OTTO: Uber den Vorfall des Harnleiters durch die Harnrohre nebst Bemerkungen zur Histologie des Odenia bullosum. Monatschr. f. Geburtsh. u. Gynak., (Ergnzshft.), 38: 115-129, 1913. FuRNIVAL, F. H.: Cystic dilation of lower end of ureter. Australasian M. Gaz., 23: 394-396, 1904. GEIPEL AND W OLLJDNBERG: U eber den Pro laps der Blassenartig in die Harnblasse vorgewolbten blinden Ureterenendigung durch die Harnrohre. Arch. f. Kinderh., 40: 57-67, 1905. GEERDTs: Quoted by Schwarz, Carl: Ueber abnorme Ausmundungcn der Ureteren und deren chirurgische Behandlung (nebst Bemerkungen ubcr die doppelte Harnblase), 15: 159-244, 1895. _ GoERTz: Quoted by PoLLETT:_A propos de dcux cas de dilatation kystique intravesicale de l'extremite inferieure de l'ureterc traites par l'etincelage. J. d'urol., 11: 15-21, 1921. GOLDBERG, VIKTOR: Cystische Dilatation des vesikalen Ureterendes. Zugleich ein Beitrag zur Physiopathologie dcr oberen Harnwege. Ztschr. f. urol., Chir., 35: 1-25, 1932. HARTMAN: Quoted by KEHRER, E. HERRICK, F. C.: Large ureterocele with herniation through the urethra. J. Urol., 35: 643-645, 1936. VON HIBLER, E.: Vorfall cines zystich crweiterten Ureters durch Harnblase und Urethra in die Vulva bei einem 6 Wochen alten !Vfadchen. Wien. klin. Wchnschr., 16: 506-507, 1903. RUNNER, G. L.: Ureterocele: report of ten cases. Urol. & Cutan. Rev., 39: 755-768, 1935. HUTH, P. E.: A large ureterocele which extruded from female bladder on voiding. J. Urol., 42: 534-537, 1939. JEANBRAU, EMILE: Dilatation kystique de l'uretere etranglee au meat urinaire chez une accouchee_._ J. d'urol., 23: 255-261, 1927. KEHRER, E.: Uber den V orfall einer U reterocele vesicalis durch die Harnrohre. Zentralbl. f. Gynak., 50: 905-914, 1926. KoLrsKo: Quoted by POLLETT. See GOERTZ. IVIAcPHERSON, IAN: Ureterocele with prolapse through the external urinary meatus. Brit. J. Surg., 2_9: 294--298, 1942. MAYER, A.: Uber Vorfall des divertikelartig erweiterten Ureters durch die Harnrohre. Zcntralbl. f. Gynak., 46: 296-304, 1922. NELSEN: Quoted by POLLETT. See GOERTZ. PA TRON, JosEPH: Du renversement de la muqueuse de l'urethrc ct de la muqueuse vesicale. Arch. gen. de med. (s.5) 10: 689-709, 1857. PENDL: Quoted by KEHRER, E. PIE.TKIEWICZ: Quoted by KEHRER, E. PRIBRAM, EGoN: Uber den Prolaps von Urcterdivertikeln. Arch. f. Gynec., 124: 726-732, 1925. RHODES, J. S.: The clinical importance of ureterocele. J. Urol., 35: 300-308, 1936. SANTRUCEK, K.: Zur Diagnose der cystichcn Dilatation der Ureteren (Ureterokele). (Abstr.) Gynak., 44: 307, 1920. ScHNID-REICHENBERG, H. H.: Quoted by ScHULTZE-RHONHOF, F. SCHROEDER: Quoted by KEHRER, E. ScHuL·rzE-RHoNHOF, F.: Prolaps der Ureterocele vesicalis. Zentralbl. f. Gynak., 62: 191-196, 1938. SIMON, MAx: Vorfall und Gangran des erweiterten Ureterendes. Zentralbl. f. Gynak., 29: 76-81, 1905. THOMAS, J.M. R.: A ureterocele prolapsed through the urethra in a child. Brit. J. Urol., 14: 24-27, 1942.