African Journal of Urology 201622,193–195Official journal of the Pan African Urological Surgeon’s Association web page of the journal www.ees.elsevier.com/afju www.sciencedirect.com Pedi
Trang 1African Journal of Urology (2016)22,193–195
Official journal of the Pan African Urological Surgeon’s Association
web page of the journal www.ees.elsevier.com/afju www.sciencedirect.com
Pediatric Urology
M.S Arslana,b,∗, S Arslana,b, H Zeytuna,b, E Basuguya,b,
M Ozkirc, B Aydogdua,b, M.H Okura,b, I Uyguna,b
Received19June2015;receivedinrevisedform3September2015;accepted13September2015
Availableonline27July2016
KEYWORDS
Female epispadias;
Patulous urethra;
Bifid clitoris;
Urinary incontinence
Abstract
Casesofisolatedfemaleepispadias(IFE)withoutexstrophyofthebladderarequiterare.Theclinical symptomsofIFEareabnormalexternalgenitaliawithprimaryurinaryincontinence
Ourpatientwasa7-year-oldgirlwithtotalurinaryincontinence.Physicalexaminationrevealeda pat-ulousurethra, bifidlabiaminora, and abifidclitoris Thevaginaand hymenappeared tobenormal Uroflowmetrydemonstrated anoverflow-typepathologicalvoidingpattern withlowvoidingpressure
Avoidingcystourethrogramshowednoindicationsofreflux
TotreatourIFEpatient,weperformedasinglestagegenitalapproachreconstructionoftheurethra,clitoris, andlabiaminora.Followupwiththepatientensuredthatshewascontinentandthatherexternalgenitals tookonanacceptableappearance
© 2016 Pan African Urological Surgeons’ Association Production and hosting by Elsevier B.V All rights reserved.
∗Correspondingauthor.Presentaddress:DepartmentofPediatricSurgery,
Cincinnati Children’s Hospital, University of Cincinnati, 3333 Burnet
Avenue, MLC 11025, Cincinnati, OH 45229-3039, USA.
E-mail address: mserif.arslan@dicle.edu.tr (M.S Arslan).
Peer review under responsibility of Pan African Urological Surgeons’
Association.
Introduction
Studiesofisolatedfemaleepispadias(IFE)withoutexstrophyof thebladderarefoundonlyinverylimitednumbersintheliterature andaregenerallypublishedonlyintheformofcasereports.The incidenceofIFEisonly1inevery448,000births[1].Ingeneral theremaybedelaysinthediagnosesofthesecasesastheir defor-mitiesare coveredbythelabiamajora.Theclinicalpresentation
ofIFEisdefectintheanatomicappearanceofthegenitalregion
http://dx.doi.org/10.1016/j.afju.2015.09.008
1110-5704/© 2016 Pan African Urological Surgeons’ Association Production and hosting by Elsevier B.V All rights reserved.
Trang 2194 M.S.Arslanetal.
Figure 1 (a)Afterplacingsilksuspensorysutureinthelabia:bifid
clitoris,patulousurethralmeatuswithdeficientdorsalwall,depressed
mons.(b)Suspensionoftheurethra;reconstructionofanewurethra
withasmileincision
accompanied by urinary incontinence Anatomically, the most
frequentlyobservedparametersofthisanomalyareanepispadiac
urethra, awide and open bladder neck,and bifidclitoris,along
withanormalhymenandanormalvagina[2].Therecommended
treatmentfor IFE is a single stageoperation reconstructing the
bladderneck,urethra,andbifidclitorisinordertoachievenormal
external genital appearance and urinary continence.We set out
inthisstudytopresentthecaseofapatientwhowasadmittedto
ourclinicbecauseofurinaryincontinence,whomwesubsequently
diagnosedwithIFEandtreatedwithasinglestageoperation
Case
A7-year-oldgirlwasadmittedtoouroutpatientclinic
complain-ing of afailure to develop bladder control sincebirth and skin
lesions in the genital area.Physicalexam revealed eruptions in
theskin ofthe perineumfromcontinueddiaperusage.The
ure-thrawasepispadiacin appearance, anda bifidclitoris and bifid
labia minora were observed (Fig 1a) The vaginal orifice and
hymenwerenormal.Voidingcystourethrographyshowedthe
blad-dercapacitytobe220ml(withanormalage-basedvalueof270ml)
Therewereno indicationsofvesicourethrealreflux
Uroflowme-trydemonstratedoverflow-typevoidingwithlowurethralpressure
Cystourethroscopyindicated that the urethrawaswide and very
short,withameasuredlengthof0.5cm.Thebladderneckwasfully
open.Theurethralorificeandbladdermucosaappearednormal
Forthesurgicalprocedure,thepatientwaspositionedinthe
litho-tomyposition,andsilksuspensorysutureswereplacedinthefront
walloftheurethraatcloseintervals.Asmileincision(from9to
3ontheclock)wasusedtofreethefrontwalloftheurethrafrom
theclitorisandthesurroundingtissues(Fig.1b).Theplanebetween
theurethraandthevaginawasnotdisturbed.Fullmobilizationof
theurethraandthebladderneckwasachievedinfront.Becausethe
neckofthebladderwassowide,apieceof full-thicknesstissue
Figure 2 (a)Finalappearanceaftercompletereconstruction,with the12Frcatheterinplace.(b)Appearanceoftheexternalgenitaliasix weeksaftersurgery
intheshapeofrectanglewasexcised,consistingofapproximately
3cmofthebladderneckandbladderfrombeginningfromthe ure-thraup.Theneckofthebladderandtheurethraweretubularized with5/0PDSsuturesovera12FrsiliconFoleycatheter,forminga urethraofapproximately3–4cminlength.Thebifidclitoral struc-tureswerecombinedatthe midline.Thesurgicalprocedurewas completedwiththereconstructionofthelabiaminora(Fig.2a).The Foleycatheterintheurethrawasremovedonthe10thpostoperative day.Urethralcalibrationwasobservedtobenormal
Thepatientbeganurotherapyinpostoperativemedicaltreatment, whichtaughtherbiofeedbackand kegelexercises Atacheckup performedafterthreemonths,uroflowmetrywithsimultaneous elec-tromyography indicated a normalvoiding pattern and abladder capacityof250ml.Inthecourseoffollowingupwiththepatient,
it wasnotedthatshehadgainedurinarycontinenceandthatthe perinealregioneruptionshadclearedup(Fig.2b)
Discussion
IFEisanuncommoncongenitalanomalyofthelowerurogenital tract Theliteraturereports anincidenceof 1in484,000female patients, withcasesranging fromneonatesto 39-year-old adults
[3].Thesepublishedcasesalsoexhibitedvariousdegreesofurinary incontinence[4].Similarlyinourowncase,weobservedurinary incontinenceaccompaniedbyalowbladdercapacity
Davisclassifiedfemaleepispadiasintothreegroupsaccordingto appearance: mild,moderate,and severe.Mild casesmay exhibit onlyapatulousurethra Inmoderatecases,thedorsalsurfaceof theurethraissplit.Inseverecasestheentirelengthoftheurethra
Trang 3Acaseofisolatedfemaleepispadiasandourapproachtotreatment 195 and the neck of the bladder are quite wide and short,
render-ingthesphinctericmechanismdeficient[5].Mildcasesarequite
rareintheliterature[6].Itisextremelychallengingtorecognize
these cases as they are continent It would be very difficult to
diagnosethesecaseswithoutseparatingthelabiamajoraand
per-forming a careful physicalexam However, usuallythe external
appearanceisdistinctive.Inseverecases,becausethesphincteric
mechanismisdeficientandtheneckofthebladderispoorly
devel-oped,bladdercapacityisgenerallynotedto below.However,in
thesepatientsthe vaginaand thehymenarenormallydeveloped
[4].ThemostfrequentlyencounteredcausesofincontinenceinIFE
areincompletebladderneck,lowurethralpressure,andlowbladder
capacity[7]
Surgicalrepairisnecessarytocreateanacceptablecosmetic
appear-anceinthegenitalregion,to restorefunction,and toprotectthe
upperurinarysystem.Urethralreconstructiontogetherwithclitoris
andlabiaminorareconstructionresultinanacceptableappearance
andrestorationoffunction.Intheliterature,thetraditionaldefinitive
IFEoperationispresentedasatwostageoperation.Traditionally,
thefirststageconsistedofgenitourethralreconstruction,followed
after1–2yearsbytherepairofthebladdernecktorestore
conti-nence[8].Thefirstsinglestagereconstructionusingtheperineal
and transvesicalapproach wasdescribedin threecases by
Hen-dren.Intheir6-casestudy,theyperformedsinglestagesurgeryon
3patients,andontheotherthreetheyusedthetraditional
combi-nationprocedure[9].ThecurrentIFEtreatmentmethodsetsoutto
freethepatulousurethrawithanincisionfromthe9to3positions
ontheclock,immediatelyfollowedbythecreationofafunctional
newurethraandgenitoplastyinthesamesteptocreateanacceptable
cosmeticappearance[2–4,8,10,11]
Ourpatientwasaseverecaseaccordingto theDaviscriteria.In
hercase,urethralresistancewaslow,theneckofthebladderwas
incomplete,andthebladdercapacitywaslow.Inonesurgicalsession
withthis patient,wewereableto forma3–4cmurethra witha
bladderneckandurethralreconstruction.Becausewewereableto
providenormalurethralresistance,weobservedinourfollow-up
thatthepatient’ssymptomsofincontinencehadclearedup,andthat
thepatientwascontinent
Inconclusion,infemalechildrenexhibitingincontinence,toavoid
an insufficient diagnosisone must never overlook the necessity
of a careful physicalexamination of the genitals In diagnosed
casesofIFE,werecommendsinglestepurethroplasty,bladderneck
reconstruction,andperineoplastyinordertoachieveanacceptable
cosmeticappearanceandafunctionalurethra
Authors’ contributions
MehmetSerifArslan(mserif.arslan@dicle.edu.tr):Interpretationof
data,manuscriptwriting,criticalrevision
Serkan Arslan (drserkanarslan@hotmail.com): Interpretation of
data
HikmetZeytun(hzeytun333@hotmail.com):Criticalrevision ErolBasuguy(erbas.80@hotmail.com):Criticalrevision
MariahOzkir(mariah.ozkir@gmail.com):Englishlanguagereview BahattinAydogdu(bahattinaydogdu@hotmail.com):Critical revi-sion
MehmetH Okur(m.hanifi-okur@hotmail.com): Conceptionand designofthestudy
IbrahimUygun(iuygun@hotmail.com):Conceptionanddesignof thestudy
Ethical committee approval
ApprovedbytheEthicscommitteeofHumanofDicleUniversity, schoolofmedicine.Ethicalcommitteenumberis14/33
Conflict of interest
Theauthorsdeclarethattheyhavenoconflictofinteresttodisclose
Funding
None
References
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