Case Report Urinary Incontinence in Adulthood in a Course of Ectopic Ureter—Description of Two Clinical Cases with Review of Literature

Iga Kuliniec 1,International*, Przemysław Journal of Mitura 1, Paweł Płaza 1, Damian Widz 1, Damian Sudoł 1, Michał Godzisz 1, Environmental Research2 2 3 1 Aleksandra Kołodyńskaand Public Health , Marta Monist , Agata Wisz and Krzysztof Bar

Case Report 1 Department of Urology and Oncological Urology, Medical University of Lublin, Jaczewskiego 8, Urinary Incontinence20-954 inLublin, Adulthood Poland; [email protected] in a Course (P.M.); [email protected] of Ectopic (P.P.); Ureter—[email protected] of Two Clinical (D.W.); Cases [email protected] with (D.S.); [email protected] (M.G.); Review of [email protected] (K.B.) 2 2nd Department of Gynecology, Medical University of Lublin, Jaczewskiego 8, 20-954 Lublin, Poland; [email protected] (A.K.); [email protected] (M.M.) Iga Kuliniec 1,* , Przemysław Mitura 1 , Paweł Płaza 1, Damian Widz 1 , Damian Sudoł 1, Michał Godzisz 1, 3 Aleksandra Kołody ´nska 2 , Marta Department Monist 2, Agata of Diagnostic Wisz 3 and Imaging, Krzysztof Radiology Bar 1 and Nuclear Medicine, Faculty of Medical Science in Katowice, Medical University of Silesia, Medyków 16, 40-752 Katowice, Poland; [email protected]

1* DepartmentCorrespondence: of Urology and [email protected] Oncological Urology, Medical University of Lublin, Jaczewskiego 8, 20-954 Lublin, Poland; [email protected] (P.M.); [email protected] (P.P.); [email protected] (D.W.); [email protected] (D.S.); [email protected] (M.G.); Abstract:[email protected] Urinary (K.B.)tract pathologies are the most common congenital abnormalities. Duplex colleting 2 2nd Department of Gynecology, Medical University of Lublin, Jaczewskiego 8, 20-954 Lublin, Poland; [email protected] occurs at different (A.K.); stages [email protected] of completion (M.M.) and is usually asymptomatic. Ureteral ectopia is an Citation: Kuliniec, I.; Mitura, P.; 3associatedDepartment of anomaly Diagnostic Imaging, which Radiology may and manifest Nuclear Medicine, as continuous Faculty of Medical incontinence. Science in Katowice, The aim of this article is to Medical University of Silesia, Medyków 16, 40-752 Katowice, Poland; [email protected] Płaza, P.; Widz, D.; Sudoł, D.; *presentCorrespondence: two patients [email protected] with duplex kidney and ureteral ectopia. Both patients presented symptoms Godzisz, M.; Kołodyńska, A.; of continuous urinary incontinence and became symptomatic in the adult life.


Abstract: Urinary tract pathologies are the most common congenital abnormalities. Duplex colleting Monist, M.; Wisz,
 A.; Bar, K. Urinary system occurs at different stages of completion and is usually asymptomatic. Ureteral ectopia is an Incontinence in Adulthood in a Citation: Kuliniec, I.; Mitura, P.; associatedKeywords: anomaly duplex which maykidney; manifest ectopic as continuous ureter incontinence. in adulthood; The aim ureteric of this article enuresis; is to urinary incontinence Course of EPłaza,ctopic P.; Widz,Ureter D.;— Sudoł, D.; present two patients with duplex kidney and ureteral ectopia. Both patients presented symptoms of Godzisz, M.; Kołody´nska,A.; Description of Two Clinical Cases continuous urinary incontinence and became symptomatic in the adult life. Monist, M.; Wisz, A.; Bar, K. Urinary with ReviewIncontinence of Literature. in Adulthood Int. J. in a Keywords: duplex kidney; ectopic ureter in adulthood; ureteric enuresis; urinary incontinence Environ. Res.Course Public of Ectopic Health Ureter— 2021, 18, x. Description of Two Clinical Cases 1. Introduction https://doi.org/10.3390/xxxxxwith Review of Literature. Int. J. Environ. Res. Public Health 2021, 18, 1. IntroductionThe development of the urinary system is a highly complex process. It involves sev- 7084. https://doi.org/10.3390/ Academic Editors: Anna Maria ijerph18137084 eralThe consecutive development of steps the urinary that system need is ato highly be precisely complex process. maintained It involves several in order to achieve physiolog- Paoletti and Marian Klinger consecutiveical development. steps that need The to be development precisely maintained of inthe order urinary to achieve system physiological takes place between 4th and Academic Editors: Anna development. The development of the urinary system takes place between 4th and 10th Maria Paoletti and Marian Klinger gestational10th gestational week [1]. Kidneys week and [1]. ureters Kidneys arise from and the embryologicalureters arise structure from located the inembryological structure lo- Received: 1 May 2021 thecated dorsal in part the of thedorsal embryo part called of intermediate the embryo mesoderm called (Figure interm1)[2].ediate mesoderm (Figure 1) [2]. Accepted: 30Received: June 2021 1 May 2021 Accepted: 30 June 2021 Published: 2 July 2021 Published: 2 July 2021

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Copyright: © 2021 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article Copyright: © 2021 by the authors. Li- distributed under the terms and FigureFigure 1. Structures1. Structures in the dorsalin the part dorsal of the embryo:part of (1)the intermediate embryo: mesoderm,(1) intermediate (2) paraxial mesoderm, (2) paraxial mes- censee MDPI,conditions Basel, of the CreativeSwitzerland. Commons mesoderm,oderm, (3) notochord, notochord, (4) neural (4) tube,neural (5) yolk tube, sac, (5) (6) lateral yolk plate. sac, (6) lateral plate. This articleAttribution is an open (CC BY) access license article (https:// creativecommons.org/licenses/by/ distributed 4.0/).under the terms and con- ditions of the Creative Commons At- During the process of embryogenesis, the metanephric mesenchyme and ureteric tribution (CC BY) license (http://crea- buds become ureters, renal pelvis, major and minor calyces and collecting tubules. These tivecommons.org/licenses/by/4.0/).Int. J. Environ. Res. Public Health 2021, 18st,ructures 7084. https://doi.org/10.3390/ijerph18137084 migrate cranially towards thehttps://www.mdpi.com/journal/ijerph final localization of the permanent kidney (Fig- ure 2) [3].

Int. J. Environ. Res. Public Health 2021, 18, x. https://doi.org/10.3390/xxxxx www.mdpi.com/journal/ijerph Int. J. Environ. Res.Int. J. Public Environ. Health Res. Public 2021 Health, 18,2021 x FOR, 18, 7084PEER REVIEW 2 of 8 2 of 8

Int. J. Environ. Res. Public Health 2021, 18, x FOR PEERDuring REVIEW the process of embryogenesis, the metanephric mesenchyme and ureteric buds 2 of 8 become ureters, renal pelvis, major and minor calyces and collecting tubules. These struc- tures migrate cranially towards the final localization of the permanent kidney (Figure2)[3] .

Figure 2. Physiological development of renal system. (a) Outgrowth of ureteric bud. (b) The metanephric mesenchyme and the ureteric bud migrate cranially toward the final localization of the permanent kidney. Mesonephros atrophies and degenerates. Mesonephric duct migrates caudally and acquires reproductive function [4]. Movement of the structures Figureindicated 2. PhysiologicalwithFigure blue 2. Physiologicalarrows. development (c) developmentDeveloped of renal ofurinary renal system. system. system. (a (a) )Outgrowth Outgrowth of of ureteric ureteric bud. bud. (b) The (b metanephric) The metanephric mesenchyme mesenchyme and the uretericand thebud ureteric migrate bud migratecranially cranially toward toward the the final final localization localization of of the the permanent permanent kidney. kidney. Mesonephros Mesonephros atrophies and atrophies and degenerates.degenerates. Mesonephric Mesonephric duct migratesAn duct abnormal migrates caudally caudally interaction and and acquires acquires between reproductivereproductive the metanephric function function [4]. Movement [4] mesenchyme. Movement of the structures andof the the structures ureteric bud indicated with blue arrows. (c) Developed urinary system. indicated with blue arrows.may (c) Developed lead to congenital urinary system. abnormalities of the kidney and urinary tract (CAKUT). Those constitute Ana wide abnormal range interaction of anatomical between the and metanephric histopathological mesenchyme and pathologies the ureteric budwhich include deformatioAnmay abnormal leadn of to the congenital interaction urinary abnormalities tract between such of theas the kidney metanephric kidney andagenesis, urinary mesenchyme polycystic tract (CAKUT). and kidneys, Those the ureteric horseshoe bud constitute a wide range of anatomical and histopathological pathologies which include may lead to congenital abnormalities of the kidney and urinary tract (CAKUT). Those kidney,deformation duplex collecting of the urinary system tract such and as kidneyduplicated agenesis, ureters polycystic [5]. kidneys,Depending horseshoe on the kind of constituteabnormalkidney, interactiona wide duplex range collecting between of systemanatomical the and metanephric duplicated and histopathological ureters mesenchyme [5]. Depending andpathologies onthe the ureteric kind which of bud include differ- deformatioent formsabnormal ofn CAKUTof interaction the urinary may between be tract presented the metanephricsuch as clinically. kidney mesenchyme agenesis, As a and result the polycystic ureteric of abnormal bud kidneys, different gene horseshoe signaling kidney,in the formsureteric duplex of CAKUT bud, collecting a may patient be system presented may andpresent clinically. duplicated with As a resultduplicated ureters of abnormal [5]. ureter. Depending gene A signaling complete on in the or kindincom- of the ureteric bud, a patient may present with duplicated ureter. A complete or incomplete abnormalplete duplicationduplication interaction of theof the ureter between ureter with duplex thewith metanephric ofduplex the kidney of the may mesenchyme kidney occur as amay result and occur of partialthe as ureteric ora result total bud of partialdiffer- entor total formsdivision division of CAKUT of the of ureteric the may ureteric bud be (Figure presented bud3)[ (Figure6,7]. clinically. 3) [6,7] As. a result of abnormal gene signaling in the ureteric bud, a patient may present with duplicated ureter. A complete or incom- plete duplication of the ureter with duplex of the kidney may occur as a result of partial or total division of the ureteric bud (Figure 3) [6,7].

Figure 3. DevelopmentFigure 3. Development of the duplex of the duplex kidney. kidney. (a) (Outgrowtha) Outgrowth ofof two two ureteric ureteric buds. buds. (b) The (b metanephric) The metanephric mesenchyme mesenchyme and and tureteric budstureteric migrate buds cranially migrate cranially toward toward the final the final localization localization of thethe permanent permanent kidney kidn iney accordance in accordance with Weigert—Mayer with Weigert—Mayer law. Mesonephroslaw. Mesonephros atrophies atrophies and degenerates. and degenerates. Mesonephric Mesonephric duct duct migrates migrates caudally caudally and acquires and reproductive acquires reproductive function [4]. function Movement of the structures indicated with blue arrows. (c) Developed duplex kidney with ureteral ectopia. Figure[4]. Mov 3.ement Development of the structures of the duplex indicated kidney. with (a blue) Outgrowth arrows. (ofc) Developedtwo ureteric duplex buds. (kidneyb) The withmetanephric ureteral mesenchymeectopia. and tureteric buds migrate cranially toward the final localization of the permanent kidney in accordance with Weigert—Mayer law. Mesonephros atrophies andDuplex degenerates. systems Mesonephric may have duct a broad migrates spectrum caudally of and manifestation acquires reproductive including function ectopic ure- [4]. Movement of the structurester and indicated continuous with blue urinary arrows. leakage (c) Developed [8]. Although duplex kidney these withpathologies ureteral ectoare pia.well known and described in pediatric patients, limited cases have been reported in adults until now [9,10]. We describeDuplex systems two cases may with have ureteral a broad duplication spectrum of and manifestation ureteric enuresis including which ectopic became ure- tersymptomatic and continuous in adulthood. urinary leakageThese data [8]. Althoughshould be these taken pathologies into account are in well the knowndifferential and describeddiagnosis inof pediatricurinary incontinence patients, limited in women. cases have been reported in adults until now [9,10]. We describe two cases with ureteral duplication and ureteric enuresis which became symptomatic2. Case Reports in adulthood. These data should be taken into account in the differential diagnosis2.1. Case Study of urinary No. 1 incontinence in women.

2. CaseIn 2006Reports a 55 -year-old female patient was admitted to the Department of Gynecology due to urinary incontinence. She had reported continuous leakage of urine during the 2.1. Case Study No. 1 In 2006 a 55-year-old female patient was admitted to the Department of Gynecology due to urinary incontinence. She had reported continuous leakage of urine during the

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Duplex systems may have a broad spectrum of manifestation including ectopic ureter and continuous urinary leakage [8]. Although these pathologies are well known and described in pediatric patients, limited cases have been reported in adults until now [9,10]. We describe two cases with ureteral duplication and ureteric enuresis which became symptomatic in adulthood. These data should be taken into account in the differential Int. J. Environ. Res. Public Health 2021, 18, x FOR PEER REVIEWdiagnosis of urinary incontinence in women. 3 of 8

2. Case Reports 2.1. Case Study No. 1 night over a period ofIn few 2006 years. a 55-year-old On daily female basis patient the patient was admitted used diaper to the pants, Department changi ofng Gynecology them 3–4 times a dueday. to She urinary gave birth incontinence. 5 times vaginally She had reported and the continuouslast menstruation leakage occurred of urine during the at the age of 48. nightDuring over the a periodhospitalization, of few years. due On to daily complete basis the prolapse patient usedof the diaper uterus, pants, a changing transvaginal hysterectomythem 3–4 times without a day. appendages She gave birth was 5 timesperformed. vaginally At andthe thesame last tim menstruatione, a dou- occurred ble TOT mesh wasat theinstalled. age of 48.The During patient the was hospitalization, discharged home due to in complete good condition. prolapse ofOne the uterus, a month postoperativelytransvaginal the patient hysterectomy was admitted without appendagesto the hospital was due performed. to urgency At the with same uri- time, a double nary leakage. BasedTOT on mesh urine was analysis installed. the The urinary patient wastract discharged infection homewas diagnosed in good condition. and an One month antibiotic therapypostoperatively was introduced the (Furazidinum, patient was admitted Metronidazole, to the hospital herbal OTC). due to Diagnostic urgency with urinary leakage. Based on urine analysis the urinary tract infection was diagnosed and an antibiotic cystoscopy was normal. The patient was discharged deciding to postpone further treat- therapy was introduced (Furazidinum, Metronidazole, herbal OTC). Diagnostic cystoscopy ment and continuewas using normal. diaper The pants. patient was discharged deciding to postpone further treatment and In 2012 the patientcontinue decided using diaper to continue pants. the diagnostic process. The gynecological ex- amination was normal,In 2012but due the patientto the continuous decided to continue urine leakage the diagnostic into vagina, process. it was The de- gynecological cided to perform examinationdouble dye test was with normal, methylene but due blue. to the During continuous the examination urine leakage no into vesico vagina,- it was vaginal fistula wasdecided visualized, to perform however double the dye urine test-like with discharge methylene was blue. still During visible the in examination the no vagina. After thevesico-vaginal urological consultation, fistula was visualized, it was decided however to the perform urine-like additional discharge testing, was still visible in such as CT scan andthe vagina. cystoscopy. After theThe urological CT scan consultation,showed a duplex it was decidedcollecting to performsystem on additional the testing, left side with a dilationsuch as CTof scanthe upper and cystoscopy. moiety ureter. The CT Both scan showedCT and a diagnostic duplex collecting cystoscopy system on the left did not reveal vesicoside- withvaginal a dilation fistula. of The the upperpatient moiety was then ureter. admitted Both CT andto the diagnostic Department cystoscopy of did not reveal vesico-vaginal fistula. The patient was then admitted to the Department of Urology Urology for further diagnostic and possible treatment. Urography was carried out and for further diagnostic and possible treatment. Urography was carried out and revealed revealed ectopic ectopicureter o ureterpening opening into the into urethra the urethra (Figure (Figure 4).4 Renal). Renal scintigraphy scintigraphy was was performedper- and formed and confirmedconfirmed good good function function of ofthe the upper upper moiety moiety of the leftleft kidney. kidney. The The patient patient was qualified was qualified for forthe the surgery surgery and and underwent underwent Leadbetter Leadbetter-- Politano Politano ureterocystoneostomy. ureterocystoneostomy. A DJ stent was A DJ stent was leftleft in in the the transplanted transplanted ureter. One One month month after after the the surgery surgery the the patient patient was was admitted to the admitted to the outpatientoutpatient clinic ofof thethe DepartmentDepartment of of Urology Urology and and the the DJ wasDJ was removed. removed. She confirmed She confirmed completecomplete continence. continence.

Figure 4.Figure(a) Contrasted 4. (a) Contrasted ectopic ureter ectopic (white ureter arrow). (white (b) arrow). Contrasted (b) proximalContrasted ectopic proximal ureter ectopic (white arrow). ureter ((whitec) Contrasted distal ectopicarrow). ureter (c) Contrasted (white arrow). distal (d) ectopic Contrasted ureter duplex (white kidney arrow). on the (d left) Contrasted side (white duplex arrows) kidney and normal on the pelvicalyceal left systemside on the (white right side.arrows) and normal pelvicalyceal system on the right side.

2.2. Case Study No. 2 A 36-year-old female patient diagnosed in childhood with asymptomatic bilateral duplex collecting system with bilateral complete ureteric duplication and ectopic ureter of the upper moiety of the left kidney was admitted the Department of Urology for further evaluation because of urinary incontinence. Since 2007 the patient was diagnosed and treated several times due to bilateral neph- rolithiasis, using both URSL and PCNL. The last CT scan was performed in 2014 and con- firmed bilateral duplication of collecting system and bilateral complete duplication of ure- ters, with poorly contrasted upper moiety of the left kidney and its ureter on the left side. The CT scan did not reveal the ectopic ureteral insertion. The patient was asymptomatic until October 2015 when she underwent a cesarean section due to the pelvic position of

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2.2. Case Study No. 2 A 36-year-old female patient diagnosed in childhood with asymptomatic bilateral duplex collecting system with bilateral complete ureteric duplication and ectopic ureter of the upper moiety of the left kidney was admitted the Department of Urology for further Int. J. Environ. Res. Public Health 2021, 18, x FOR PEER REVIEWevaluation because of urinary incontinence. 4 of 8

Since 2007 the patient was diagnosed and treated several times due to bilateral nephrolithiasis, using both URSL and PCNL. The last CT scan was performed in 2014 and confirmed bilateral duplication of collecting system and bilateral complete duplication of the fetus. The surgeryureters, went withwell poorly and contrastedthere was upper no early moiety postoperative of the left kidney andcomplication. its ureter on the How- left side. The CT scan did not reveal the ectopic ureteral insertion. The patient was asymptomatic ever, during postpartumuntil Octoberperiod, 2015 patient when shereported underwent urinary a cesarean incontinence section due to symptoms, the pelvic position which of the she had never reportedfetus. before. The surgery Urinary went i wellncontinence and there was appeared no early postoperative occasionally, complication. few times However, per year, subsiding beforeduring menstruation postpartum period, and intensifying patient reported after urinary the incontinence menstrual symptoms, bleeding. which The she patient was treated forhad few never years reported conservatively before. Urinary incontinencewith the use appeared of muscarinic occasionally, receptors few times per an- year, subsiding before menstruation and intensifying after the menstrual bleeding. The patient tagonists achieving mediocrewas treated eff forect. few years conservatively with the use of muscarinic receptors antagonists In 2020 her symptomsachieving worsened mediocre effect. and became continuous without urgency nor SUI. The abdomen CT scan wasIn 2020repeated her symptoms and showed worsened bilateral and became duplex continuous kidneys without with urgency complete nor SUI. The abdomen CT scan was repeated and showed bilateral duplex kidneys with complete duplication of ureters.duplication The upper of ureters. moiety The of upper the moietyleft kidney of the left showed kidney showedpoor function poor function and and its its ureter was dilated (Figureureter was5). dilated (Figure5).

FigureFigure 5. (a(a) Bilateral) Bilateral duplex duplex kidney. kidney. Exertion of Exertion the contrast of from the both contrast segments from of right both kidney segments (green brackets) of right and lowerkidney (greensegment brackets) of the left kidneyand lower (blue bracket). segment No of exertion the left of the kidney contrast (blue from upper bracket). moiety No of the exertion left kidney of (red the bracket). contrast from(b) Bilateral upperduplication moiety of of the the ureter.left kidney Two contrasted (red bracket). ureters of ( theb) rightBilateral kidney duplication (green arrows). of Contrasted the ureter. ureter Two of the con- left kidney (blue arrow) and dilated, non-contrasted ureter of the upper moiety of the left kidney (red arrow). trasted ureters of the right kidney (green arrows). Contrasted ureter of the left kidney (blue arrow) and dilated, non-contrastedDuring ureter diagnostic of the upper cystoscopy moiety two of orifices the left were kidney found (red on the arrow). right side of the bladder trigon whereas only one orifice was found on the left side. The patient was diagnosed with During diagnosticureteric cystoscopy enuresis and two qualified orifices for were heminephrectomy found on ofthe the right upper side moiety of ofthe the bladder left kidney. trigon whereas only one orifice was found on the left side. The patient was diagnosed with ureteric enuresis and qualified for heminephrectomy of the upper moiety of the left kidney. In the early postoperative days patient reported minimal urinary leakage. Due to that fact a diagnostic cystoscopy was performed again. During the procedure the ectopic orifice of the dissected ectopic ureter was finally located below the bladder neck on the left side and successfully probed with SJ catheter (Figure 6). Purulent fluid was obtained. Examination of the vagina using specula was normal. SJ catheter was removed after one week and the patient was discharged home in good general condition. The patient no longer showed symptoms of urinary incontinence.

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In the early postoperative days patient reported minimal urinary leakage. Due to that fact a diagnostic cystoscopy was performed again. During the procedure the ectopic orifice of the dissected ectopic ureter was finally located below the bladder neck on the left side and successfully probed with SJ catheter (Figure6). Purulent fluid was obtained. Examination Int. J. Environ. Res. Public Health 2021of, 18 the, x vaginaFOR PEER using REVIEW specula was normal. SJ catheter was removed after one week and the 5 of 8 patient was discharged home in good general condition. The patient no longer showed symptoms of urinary incontinence.

FigureFigure 6. 6.Ectopic Ectopic orifice orifice located located below bladder below neck bladder probed neck with SJprobed catheter. with SJ catheter. 3. Discussion 3. DiscussionDuplex collecting system (DCS) is one of the most common congenital renal system abnormalities.Duplex Itcollecting occurs in less system than 1% (DCS) of the is population one of the and most is more common common amongcongenital renal system femaleabnormalities. population [It11 ].occurs in less than 1% of the population and is more common among Duplex colleting systems have a variety of different phenotypes. The abnormality canfemale be presented population as a completely [11]. duplicated system (CDS)—a duplex kidney with dou- bled collectingDuplex systems colleting that systems drain into twohave ureters a variety with separate of different ureteral phenotypes. orifices and as The abnormality incompletelycan be presented duplicated as system a completely (IDS) with duplicated either partial uretericsystem duplication (CDS)— (Y—shapeda duplex kidney with dou- ureter) or incomplete ureteric duplication (V—shaped ureter). H—shaped ureter, inverted Y—shapedbled collecting ureter or systems blind ending that ureter drain are into very raretwo variations ureters ofwith ureteric separate fusions [ureteral6]. orifices and as incompletelyBilateral CDS duplicated occurs 5 times system less often (IDS) than with IDS. Duplexeither kidneyspartial oftenureteric coexist duplication with (Y—shaped otherureter) abnormalities or incomplete of the urinary ureteric tract. duplication Vesicoureteral (V reflux—shaped (VUR) and ureter). ureterocele H— aresha theped ureter, inverted mostY—shaped common anomaliesureter or associated blind ending with CDS. ureter Other are possible very findings rare variations include urinary of tractureteric fusions [6]. infections, hydronephrosis, pelvi-calyceal dilatation, cortical scarring or caliculi. Duplex kidneysBilateral are CDS asymptomatic occurs in5 times most cases less [ 12often]. than IDS. Duplex kidneys often coexist with other abnormalitiesAn ectopic ureter of the (EU) urinary is a ureter tract. that terminates Vesicoureteral outside of refluthe trigonx (VUR) of the bladder.and ureterocele are the Accordingmost common to Weigert—Meyer anomalies law associated it usually concerns with theCDS. upper Other segment possible ureter of findings duplex include urinary kidney.tract infections, The orifice hydronephrosis, of an ectopic ureter pelvi may- becalyceal located dilatation, not only in thecortical bladder, scarring but or caliculi. Du- also in different parts of genitourinary system such as bladder neck, urethra, ejaculatory track,plex vaskidneys deferens, are seminal asymptomatic vesicles, vagina in most or uterus cases [13[12].]. During the embryological separation/duplicationAn ectopic ureter of the (EU) ureteric is a bud,ureter the that future terminates lower pole separatesoutside of earlier the andtrigon of the bladder. According to Weigert—Meyer law it usually concerns the upper segment ureter of duplex kidney. The orifice of an ectopic ureter may be located not only in the bladder, but also in different parts of genitourinary system such as bladder neck, urethra, ejaculatory track, vas deferens, seminal vesicles, vagina or uterus [13]. During the embryological separa- tion/duplication of the ureteric bud, the future lower pole separates earlier and migrates superiorly and laterally. Simultaneously, the urogenital sinus migrates cranially and be- comes the upper pole. As a result, the ureteric orifice of the upper pole ureter is located more medially and inferiorly on the bladder wall, whereas the ureteric orifice of the lower pole ureter is located more laterally and superiorly to the other one (Figure 3) [14]. Ureteral ectopia may manifest itself as continuous incontinence that commonly ap- pears in early childhood. In their article, Jain et al. analyzed 9 patients with EU. According to their results, the symptoms of EU depended on the position of the ectopic ureter orifice and at 8 out of 9 patients it was urinary incontinence [15]. In many cases the localization of the ectopic ureter insertion may be problematic or even impossible and the diagnostic process often requires computed tomography with urographic phase, MRI urography or urethrocystography [16]. Finally, a cystoscopic ex- amination may confirm the localization of the ectopic ureteral orifice [17]. Hanson et al.

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migrates superiorly and laterally. Simultaneously, the urogenital sinus migrates cranially and becomes the upper pole. As a result, the ureteric orifice of the upper pole ureter is located more medially and inferiorly on the bladder wall, whereas the ureteric orifice of the lower pole ureter is located more laterally and superiorly to the other one (Figure3)[14]. Ureteral ectopia may manifest itself as continuous incontinence that commonly ap- pears in early childhood. In their article, Jain et al. analyzed 9 patients with EU. According to their results, the symptoms of EU depended on the position of the ectopic ureter orifice and at 8 out of 9 patients it was urinary incontinence [15]. In many cases the localization of the ectopic ureter insertion may be problematic or even impossible and the diagnostic process often requires computed tomography with urographic phase, MRI urography or urethrocystography [16]. Finally, a cystoscopic examination may confirm the localization of the ectopic ureteral orifice [17]. Hanson et al. were able to identify ectopic ureter in all cases where they used a CT scan as a diagnostic tool. More than 20% of ectopic ureteral orifices were found in cystoscopy. They concluded that a CT scan with delayed contrast phase was highly sensitive, quick, affordable and available method for diagnosing ectopic ureters and renal systems [18]. The treatment of ureteral ectopia resulting in urinary incontinence is surgery. The most common surgical approach in case of dysplastic, poor functioning upper moiety is heminephrectomy. For kidneys with a preserved upper moiety function the ureteral reimplantation can be considered [19]. In 2019 Toia at al. published the results of their work devoted to diagnosis and out- comes of treatment of ectopic ureters in adults. They presented 10 cases (9 women and 1 man). All women complained of lifelong urinary leakage. The predominant diagnostic tool in this study was MRI. They also performed a video-urodynamic examination which additionally revealed stress urinary incontinence (SUI) in 5/9 women, urge urinary incon- tinence in 1/9 and mixed urinary incontinence in 2/9 women. The locations of ectopic ureteral orifice were the bladder neck in 4 cases, the subsphincteric urethra in 2 cases and the vagina in 3 cases. Further treatment depended on the kidney or its upper moiety function and included nephroureterectomy, heminephrectomy, heminephrectomy with distal ureterectomy and reimplantation of the ectopic ureter with or without bladder neck reconstruction. Additionally, other procedures like colposuspention, rectus facia sling, reg- ular Botox injections and artificial urethral sphincter implantation have been shown to be essential in the treatment of previously diagnosed urinary incontinence. Toia et al. pointed out the role of ectopic ureter in differential diagnosis of the lifelong urinary incontinence. They recommended MRI as an examination of choice in the diagnostic process and showed that patients with ectopic ureters might require additional treatments in order to achieve continence [10]. We performed an electronic search of PubMed using a combination of keywords “urinary incontinence” and (“ectopic ureter” or “ureteral ectopia”) and “adult” limiting the results only to articles written in English over the last 10 years. We only found 20 results, most of which were single case reports. Urinary leakage, reported in most of case reports, was lifelong and related to vaginal insertion [20–24]. Only one article of Fichtenbaum at al. presented a case of new onset of urinary incontinence in adult life. They published a case of 61 years old female who presented with new onset urinary incontinence after undergoing organ prolapse repair surgery. During a cystoscopic evaluation the ectopic ureteral orifice was found at the bladder neck. They hypothesized that the patient might have been able to maintain continence in her young life due to the pelvic floor muscle strength. Once she got older and the muscle strength weakened, she could still maintain continence as a result of the acquired organ prolapse which created urethral kink. After the prolapse correction, the urethral kink subsided, and the urinary incontinence appeared. The patient underwent an open extra-vesical left ureteroneocystostomy and restored continence [9]. Both our patients developed urinary incontinence due to ectopic ureter in adulthood. Patient No. 1 developed syndromes of urinary incontinence as an older adult with a prolapse component. Standard procedures were performed without success. The persis- Int. J. Environ. Res. Public Health 2021, 18, 7084 7 of 8

tence of symptoms of urinary incontinence prompted further extended diagnosis which was finally carried out a few years later. In patient No. 2 bilateral duplex collecting sys- tem with bilateral complete ureteric duplication was diagnosed earlier and ectopic ureter with ureteric enuresis was suspected and finally confirmed. We hypothesize that both patients might have been able to maintain urinary continence until adulthood because of the strength of their pelvic floor muscles and probably due to the presence of a plug of exfoliated urothelial cells occluding the distal ureteral lumen. When the muscles weaken, either as a result of the aging process or as a result of an injury such as pregnancy and childbirth, the cell plug may be released, and symptoms of urinary incontinence appear. The severity of incontinence depends on the function of the kidney or its segment from which the ectopic ureter originates.

4. Conclusions The outbreak of urinary incontinence in a course of ectopic ureter in adulthood is an extremely rarely described phenomenon, still of uncertain cause. The ectopic ureter and duplex kidney are very uncommon findings in adults and should always be taken into consideration during differential diagnosis of urinary incontinence.

Author Contributions: Conceptualization, I.K. and P.M.; investigation, I.K., M.G. and D.S.; visu- alization, I.K. and A.W.; resources, I.K., A.K. and M.M.; writing—original draft preparation, I.K.; writing—review and editing, P.M., P.P. and D.W.; supervision, K.B. All authors have read and agreed to the published version of the manuscript. Funding: This research received no external funding. Institutional Review Board Statement: The study was conducted according to the guidelines of the Declaration of Helsinki, and approved by the Institutional Ethics Committee of Medical University of Lublin (KE-0254/86/2021). Informed Consent Statement: Informed consent was obtained from all subjects involved in the study. Written informed consent has been obtained from the patient to publish this paper. Data Availability Statement: Not applicable. Conflicts of Interest: The authors declare no conflict of interest.

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