先天的女子尿道憩室あるいは, 拡張尿道は極めて稀である.これまでに, 尿管瘤に伴って, 憩室様の変化を示した症例は数例報告されているが, その大多数が, 尿道内に開口する拡張した尿管を誤って憩室と判定したものであり, 他は, 尿管瘤のpoor backingのためのcvcrsionや, Gartner's cystとの交通によるものである.今回われわれは, 3カ月の女児で, 特に右側尿道の著明な拡張を示し, この憩室様の拡張が尿管瘤内に翻転を示す症例を経験したので報告した, 本症例は内視鏡的にも手術時の所見からも, 尿管瘤は膀胱頸部以下には認められず, 発生学上右側後部尿道の形成不全が関与したものと考えられた.A rare case of wide urethra in a female infant mimicking as a urethral diverticulum in association with ectopic ureterocele is reported. The ureterocele terminated just below the bladder neck, but the diverticulum-like structure everted from the wide urethra, protruding towards the ureterocele. Weakness of the urethral wall, distal to the actual ureterocele may produce such an abnormal radiologic finding. The pertinent literature is reviewed

KONAMI, Teruo

TAKEUCHI, Hideo

TOMOYOSHI, Tadao

WAKABAYASHI, Yoshihiko

Kyoto University Research Information Repository

Title Female wide urethra masquerading as a urethral diverticulumin association with ectopic ureteroceleAuthor(s)KONAMI, Teruo; WAKABAYASHI, Yoshihiko;TAKEUCHI, Hideo; TOMOYOSHI, TadaoCitation泌尿器科紀要 (1988), 34(8): 1437-1441Issue Date1988-08URL http://hdl.handle.net/2433/119667RightType Departmental Bulletin PaperTextversionpublisherKyoto UniversityActa Urol. Jpn. 34: 1437-1441, 1988 1437 FEMALE WIDE URETHRA MASQUERADING AS A URETHRAL DIVERTICULUM IN ASSOCIATION WITH ECTOPIC URETEROCELE Teruo KONAMI, Yoshihiko WAKABAYASHI, Hideo TAKEUCHI and Tadao TOMOYOSHI From the Department of Urology, Shiga University of Medical Sciellce (Dirlftor: Prof. T. Tomoyoshi) A rare case of wide urethra in a female infant mimicking as a urethral diverticulum in association with ectopic ureterocele is reported. The ureterocele terminated just below the bladder neck, but the dil·erticulum·like structure everted from the wide urethra, ~ protruding towards the ureterocele. Weakness of the urethral wall, distal to the actual ureterocele may produce such an abnormal radiologic finding. The pertinent literature is reviewed. Key words: Ectopic ureterocele, Wide urethra, Female, Urethral diverticulum INTRODUCTION An ectopic ureterocele may demonstrate variable radiologic abnormalities of the lower urinary tract. Urethral dilatation in association with prolapsed and/or balloon-ing ureterocele or one due to distal urethral stenosis is well known. However, in females, megalourethra and urethral diverticulum are uncommon and usually considered as an acquired lesion. Although there are several reported cases which present as a urethral diverticulum asso-ciated with an ectopic ureterocele l - 6), true diverticulum of the urethra is extremely rare. The case reported herein had a wide atonic urethra without distal urethral stenosis and with diverticulum-like dilata-tion of its posterior wall, distal to the actual ureterocele. We could find only case simulating that of William'sD in the per-tinent literature. Possible explanations of this abnormality are discussed. CASE REPORT M.K., a 3-month-old female infant was hospitalized for recurrent attacks of pye-lonephritis, vomiting and failure to thrive. Urine specimens revealed many white cells with significant growth of Escherichia coli. Physical examination was normal except for the patulous external urethral meatus, opening above the vaginal introi-tus (Fig. 1). An excretory urogram (IVP) demonstrated a faint right pelviocalyceal system, which shows a drooping flower sign, with a filling defect in the bladder. Transurethral catheterization was difficult because of resistance about 3 em proximal to the external urethral meatus. Injection of the contrast media showed a wide urethra with a round diverticulum-like shadow protruded up toward the bladder (Fig. 2. A, B). By sounding the urethra, a l2F, Folley catheter could be introduced Fig. I. Patulous external urethral meatus open-ing above the vaginal introitus. 1438 Acta Urol. Jpn . Vol. 34, No.8, 1988 Fig. 2. Retrograde urethrograms. A ; Dilatation of the urethra was demonstrated with round protrusion of the right upper portion toward the bladder. B ; More contrast media instill ed into the ure· thra. Urethral dil atation was clearer. White ar row shows the catheter in troduced into the vagina. Fig. 3. Cystography shows massive VUR to the lower kidney. Intubated catheter is dis-placed to the left and wide urethra with round head is demonst rated. into the bladder. The catheter was displa-ced to the left and a massive vesicoureteral reflux into the lower segment of the right kidney was demonstrated by the cystogra-phy (Fig. 3) . The vaginogram showed no abnormal findings. Urethrocystoscopy re-vealed a wide urethra with a bulging of Fig. 4. Urethroscopy shows « bulging of the thinner wall with gutter formation at the right posterior aspect of the urethra, clearly demarcated from the normal urethral mucosa. Black arrow shows the internal urethral meatus. Fig. 5. Intraoperative ureterography, injected of contrast media through needle punc· ture into the upper ureter, demonstrates a large ureterocele producing a dome-like shadow defect at the base of its, which lumen is communicating with the urethra. the thinner wall sectioned into several compartments by gutter formation at the right posterior aspect of the urethra, clearly demarcated from the main urethral wall (Fig. 4) . This bulging gradually disappeared and the urethral wall extended posteriorly, when the urethral cavity was filled with water. Cystoscopy confirmed Konami et a1.: Wide urethra, Ectopic ureterocele 1439 ilie ,presence of a ureterocele which termi-nates. just. below the bladder nack but did not. disclose the exact site of the orifice of-tke. ureterocele. Because repeated renal sci~tigl1aphies showed no uptake of the right kidney and recurrent urinary tract infection continued, right nephrourete-rectomy was performed without removal of the ureterocele. During the operation, contrast m,edia instilled through the upper yreterdemonstrated a large ureterocele producing a dome-like shadow defect at _ the base of bladder, which lumen had communication with urethra (Fig. 5). The bladder was opened, and a large ureterocele was found on the right side extending to the bladder neck, where a small orifice was found at the bladder neck. The surgical specimen showed that the upper pole kidney was dysplastic and lower pole kidney showed remarkable pyelonephritic change. At the age of 3 she was readmitted for evaluation of urinary continence and cystourethroscopy. She voided with slight difficulty but urinary continence was almost completly preser-ved. Cy~tourethroscopy revealed the same bulging of urethral wall as the previous examination but ureterocele significantly decreased its size being located at the lateral vesical wall. DISCUSSION Possible anatomical conditions, which present as a urethral diverticulum in association with ectopic ureterocele are listed in the table. The first possibliity is a false urethral diverticulum. Occasion-ally a dilatated ectopic orifice located in the urethra with dilatated ureter may Table I. Possible anatomical conditions of case presenting as a urethral diver-ticulum associated with ectopic ureterocele. 1 . False urethral diverticulum. 2 . Everting ureterocele. 3 . Cecoureterocele. 4 . Ruptured ureterocele. 5 . Ectopic ureter into the Gartner's duct or cyst. 6 . Weakness of urethral wall. be mistaken for a urethral diverticulum1- S). As Emmet7) suggested, the dilatation in such instances may result from infection. The second possibility is the eversion of the ureterocele. An everting ureterocele is a relatively new concept, characterized by a diverticulum-like protrusion outside the general contour of the bladder or urethraS,9). Williams and associates2) have described that occasionally the ectopic ureterocele exhibits not only ureterocele dilatation within the bladder but a second saccular dilatation lying posterior to the urethra. Koyanagi and associates9) have classified the everting ureterocele into 2 types; type I is designated when ureterocele is everting intraluminally into dilatated ureter, which is issuing in blad-der or urethral wall through enlarged hiatus, type 2 is designated when an ureterocele is everting exteriorly along craniolateral aspect of ureteral wall through loose hiatus. The eversion is clinically not uncommon, but a protrusion solely of the urethra, not of the uretero-cele, can not be found in the pertinent literature. The third is the cecoureterocele, uncommon variety of ureterocele termed by StephenslO,\l) characterized by upward migration of the developing large and incompetent ectopic ureteral orifice leav-ing a tongue of more distal ureter under-neath the urethral mucosa demonstrating the diverticulum-like appearance in the urethra. The fourth is the ruptured urete-rocele, which may be distinguishable by urethroscopy and or urethrography. Ano-ther possible anatomical condition which presents as a urethral diverticulum is a congenital urethral diverticulm. Urethral diverticuli in females are believed to be acquired lesions from infection and abscess formation in the periurethral glands I2 ). Although several cases of ure-teral ectopia into a Wolffian ducts remnant (Gartner's duct or cyst) has been reported 13-IS), almost all of these cases are associated with single ectopic ureter, and the communication with bladder or urethra is rare I7). The case reported herein can not be classified into any of the entities described above. The weakness 1440 Acta Urol. Jpn. Vol. 34, No.8, 1988 of the right urethral wall and the bladder neck may have resulted in a marked protrusion of the urethra outside and posterior with a patulous external urethral meatus, resembling megalourethra or urethral diverticulum. A review of the literature revealed only one case like that of William'sl), which presents similar, bizarre dilatation distal to the actual ureterocele. Ectopic ureterocele sometimes involves distortion or dissection of trigone or bladder neck muscle. Several cases of the diverticulum of the floor are reported as a result of muscular defect, which produced secondary obstruction of the bladder outlet during the act of void ing19- 20) • Morphological studies of the ureterocele and ureter were provided by Tokunaka and associates2D , who showed a lack of thick microfilaments in the dome of the ureterocele, concluding that the uretero-cele is a segmental embryonal arrest of the most distal portion of the ureter. However, the anatomical details of the urethra associated with an ectopic uretero-cele are not well understood. Weakness of the urethral wall, clinically encountered in a prolapsed ureterocele, has been generally believed to be a result of a progressive enlargement of the ureterocele within the urethral wall!9l. Gomez and Stephens lll studied the struc-ture of the ureterocele and the accom-panying vesicourethral sphincter in an autopsy case, which had a right cecoure· terocele and a left sphincteric ureterocele. They demonstrated that the bladder neck and the both involuntary and voluntary sphincters were attenuated or devoid of muscle in the quadrant beneath the urete-rocele and its cecal extension. We suspect that maldevelopment of the urethral wall are not so uncommon in cases with a large ectopic ureterocele, sometimes mak-ing prolapse into the posterior urethra or external urethral orifice. It is important to distinguish the ana-tomical and functional abnormalities of the urethra, such as descrived above, when consider the operative management in the cases, which present as a urethral diver-ticum associated with ureterocele. While in our case, the base of the ureterocele was thin and attached to the urethral wall closely, unroofing may be dangerous injuring the urethral wall or causing a more pronounced protrusion >of the urethra. REFERENCES I) Williams DI and Woodard JR: Problems in the management of ectopic ureteroceles. J Urol 92: 635-651, 1964 2) Williams DI, Fay R and Lillie JG: The functional radiology of ectopic ureterocele. Br J Urol 44: 417-433, 1972 3) Willmarth CL : Ectopic ureteral orifice within an urethral diverticulum: report of a case. J Urol 59: 47-49, 1948 4) Vanhoutte JJ: Ureteral ectopiajinto aWol. ffian duct remnant (Gartner's ducts or cysts) presenting as a urethral diverticulum in two girls. AJR 110: 540-545, 1970 5) Curry NS: Ectopic ureteral orifice masque-rading as u urethral diverticulum. AJR 141: 1325-1328, 1983 6) Wyly JB and Lebowitz RI: Reftuxing ure-thral ectopic ureters: recognitions by the cyclic voiding cystourethrogram. AJR 142: 1263-1267, 1984 7) Emmett JL: Clinical Urography, vol. 2, 4th ed. Philadelphia: Saunders, 1977, p.7oo 8) Weiss RM and Spackman TJ: Everting ectopic ureteroceles. J Urol 111: 538-540, 1974 9) Koyanagi T, Hisajima S, Goto T, Tokunaka S and Tsuji I: Everting ureteroceles: radiographic and endoscopic observation, and surgical management. J Urol 123: 538-543, 1980 10) Stephens D: Cecoureterocele and concepts on the embrylogy and etiology of uretero-celes. Aust NZJ Surg 40: 239-248, 1971 II) Gomez F and Stephens FD: Cecoureterocele: morphology and clinical correlations. J Urol 129: 1017-1019, 1983 12) Ginsburg DS and Genadry R: Suburethral diverticulum in the female. Obstet Gynecol Surv 39: 1-5, 1984 13) Schulman CC: The single ectopic ureter. Eur Urol 2: 64-69, 1976 14) David MD and Duckett Jr JW: Single vaginal ectopic ureter: a case report. J Urol 120: 493-495, 1978 15) Kjaeldgaard A, Fianau S, Thorgeirsson T, Ekman P: Single bifid ectopic left ureter presenting clinically as a vaginal cyst. Diag. Konamieta1.:Wideurethra,EctopicureteroceleGynecolObstct3:321-324,198116)sorensoncWJrandMiddletonAwJr=Thcsingleectopicuretcr=3casereports.IUrol129=132-134,198317)KjaeldgaardA,FianauS=Classificationandembryo夏ogicalaspectsofectopicureterscommunicatingwitllGartner,scysts.DiagGynecolObstet4=429-273,198218)EllerkerAG:Theextravesicalectopicureter.BrJSurg145;344-353,195819)LeadbetterGWJr=Ectopicureteroceleasa1441causeofurinaryincontinence。Jurollo3:222-226,197020)AsllcraftKWandHendrenWH:Bladderoutletobstructionafteroperationforurete-rocelc.Jpediatsurg14:819-824,197921)TokunakaS,GotohT,KoyanagiTandTsujiI=Morphologicalstudyoftheurete・rocele:apossiblecllletoitsembryogenesisasevidencedbyalocallyarrcstedmyogenesis.IUrol1261726-729,1981(Acceptedforpublicat董onAugust17,1987)和文抄録異所性尿管瘤に伴い憩室様拡張を示した女児 wide urethra の1例滋賀医科大学医学部泌尿器科学教室(主任:友吉唯夫教授)神波 照夫,若 林 賢彦,竹 内 秀雄,友吉 唯夫先天的 女子 尿 道 憩室 あ る い は,拡 張尿 道 は極 め て 稀であ る.こ れ まで に,尿 管瘤 に 伴 って,憩 室 様 の変 化を示 した 症例 は 数 例 報 告 され て い る が,そ の 大多 数が,尿 道 内 に開 口す る拡 張 した 尿 管 を誤 って 憩 室 と判 定 した もの であ り,他 は,尿 管 瘤 のpoorbackingのため のcvcrsionや,Gartner'scystとの 交 通 による もので あ る.今 回わ れ われ は,3カ 月 の 女児 で,特に右側尿道の著明な拡張を示し,この憩室様の拡張が尿管瘤内に翻転を示す症例 を経験 したので報告した,本症例は内視鏡的にも手術時の所見からも,尿管瘤は膀胱頸部以下には認められず,発生学上右側後部尿道の形成不全が関与したものと考えられた.(泌尿紀要34:1437-1441,l988)

異所性尿管瘤に伴い憩室様拡張を示した女児wide urethra の1例

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異所性尿管瘤に伴い憩室様拡張を示した女児wide urethra の1例

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