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Palpable Testes
Undescendedtestesmaybelocatedalongthelineofnormaldescentbetweenthe
abdomenandscrotumorinanectopicpositionthatismostcommonlythe
superficialinguinalpouch(anteriortotherectusabdominismuscle)or,morerarely,
inaperirenal,prepubic,femoral,peripenile,perineal,orcontralateralscrotalposition.
Carefulexaminationoftheseareasisneededtocorrectly

classifyatestisaspalpableornonpalpable,acriticalstepthatinfluencesfurther
diagnosisandtreatment.Everyeffortshouldbemadebytheexaminertodetermine
thelowestpositionattainablebyapalpableundescendedtestis.Manualdownward
pressurewithonehandalongtheipsilateralinguinalcanalfromtheanterioriliac
spinetothescrotumandpalpationwiththeoppositehandhelpstoidentifythelowest
positionofapalpabletestis.

Difficultyintheclinicalclassificationofcryptorchidismwhenthetestisispalpableis
relatedtobothdocumentationoftesticularpositionanddifferentiationoftruly
undescendedfromretractiletestes,complicatedbythefactthattheseentitiesmay
coexist.Thegoldstandardfordiagnosisremainscarefulexaminationofachildin
severalpositionsandconfirmationofincompletedescentofthetestistoadependent
scrotalpositionafterinductionofanesthesia.Prospectivestudiesofintraobserverand
interobservervariationshowmajordifferencesindocumentationoftesticularposition
betweenexaminers(Witetal,1987;Olsen,1989).Olsen(1989)notedcomplete
agreementbetweentwoexaminersonscaledmeasuresoftesticularpositionand
mobilityinonly5(13.5%,95%CI4.5%to28.8%)of37boys.Cendronand
coworkers(1993)reportedthatpreoperativetesticularpositioncorrelatespoorlywith
intraoperativefindings.Variationinobservedtesticularpositionpreoperativelyand
postoperativelymayinfluenceassessmentofprognosisandoutcomeinboyswith
cryptorchidism.

Nonpalpable Testes
Whenatestisisnonpalpable,possibleclinicalfindingsatsurgeryinclude(1)
abdominalortransinguinalpeepinglocation(25%to50%),(2)completeatrophy
(vanishingtestis,15%to40%),and(3)extraabdominallocationbutnonpalpable
duetobodyhabitus,testicularsize,and/orlimitedcooperationofthepatient(10%to
30%)(Cendronetal,1993;Ciseketal,1998;Kirschetal,1998;Radmayretal,2003;
Patiletal,2005).Ifbothtestesarenonpalpableandnotdistaltotheinternalinguinal
ringinageneticmale,atleast95%areabdominalwithbilateralvanishingtestis
occurringrarely(Cendronetal,1993;Mooreetal,1994).Ifneithervasnorspermatic
arteryisfoundatthetimeoflaparoscopy,laparoscopicorsurgicaldissectionofthe
paravesicalareaandretroperitoneumuptothelevelofthekidneyisrequiredto
excludethepresenceofatestis.Pararenalorotherabdominaltestesmaybeassociated
withmulticysticdysplasticorabsentipsilateralkidneysand/ornonunionofthetestis
andepididymis(Zaccaraetal,2004;Foleyetal,2005;Kimetal,2005).

Theetiologyofvanishingtestisismostlikelytorsionorvascularaccidentoccurring
aftercompletionofgenitalmasculinizationbutbeforefixationofthetestisinthe
scrotum.Evidencesupportingthisetiologyincludesthepresenceofhemosiderinin
remnanttesticularnubbinsexcisedatsurgery(Tureketal,1994)andreportedcases
ofcontralateralpostnataltorsion(Gongetal,1996).Anenlargedcontralateraltestis
(Huffetal,1992)andabsenceofpalpableintrascrotalappendagetissue(processus
vaginalis,wolffianstructures,orgubernaculum)arehighlypredictiveofvanishing
testis(Mesrobianetal,2002).However,diagnosisofavanishingtestisrequires
documentationofblindendingspermaticvesselsintheabdomen,inguinalcanal,
orscrotum.Endocrineevaluationincasesofsuspectedbilateralvanishingtestis
(anorchia)includeelevatedbasalserumgonadotropinlevelsandnoresponsetohCG
stimulation;however,gonadotropinsmaybeunexpectedlylowinmidchildhoodin
boyswhoarealsounresponsivetohCG(Lustigetal,1987;Lee,2000).Therefore
laparoscopicorsurgicaldocumentationofanorchiaiscriticaltoavoidleavingsmall
ordysgeneticabdominal

CHAPTER 132 Abnormalities of the Testis and Scrotum and Their


Surgical Management 3565

testesinsitu.AMHandinhibinBlevelsmaybeusefulindocumentingthepresence
offunctioningtesticulartissue(Grumbach,2005).

Inguinoscrotalultrasonographyandmagneticresonanceimaging(MRI)arenot
usuallyhelpfulinthediagnosisandmanagementofnonpalpabletestis(Elder,2002).
However,ifatestisisnonpalpablewhenevaluatedbyanexperiencedexaminer,the
sensitivityofultrasonographyinidentifyinginguinaltestesisreportedtobeashighas
95%to97%,andabdominaltestesarealsoseeninsomecases(Cainetal,1996;Nijs
etal,2007).Someofthesetestesarepeeping,however,andbestmobilizedviaa
laparoscopicapproach.Similarly,althoughMRIcanbeusefulinsomecasesto
identifynonpalpableabdominaltestes,itsaccuracyisvariableandtheprocedure
requiressedationinyoungerchildrenandoftenmaynotchangethemanagement
approach(Yeungetal,1999;Siemeretal,2000).Thesuperioraccuracyofmagnetic
resonanceangiographyinlocalizinganddifferentiatingviablefromvanishedtestes
(96%of23nonpalpabletestesin21boys,meanage2.5years)hasnotbeenreplicated
inarecentstudyofyoungerboys(57%of29testesin26boys,meanage13months)
(Yeungetal,1999;Desireddietal,2008).OneindicationforMRImaybe
identificationofanectopicabdominaltestisnotlocalizedbylaparoscopy.

Diagnosticlaparoscopyfollowedbylaparoscopicorchidopexyifthetestisis
abdominalhasbecomethepreferredapproachofmanyclinicians.Important
observationsincludesizeandpositionofthespermaticvesselsandvas;testicularsize,
quality,andposition;andpatencyoftheinternalinguinalring.Thecombinationofa
closedinternalringandablindendingspermaticarteryandvasismostconsistent
withanabdominalvanishingtestis,whereasaherniaisfrequentlybutnotalways
associatedwithaviableabdominalordistaltestis(Elder,1994;Mooreetal,1994).
Anatreticspermaticcordcoursingthroughaclosedinguinalringissuggestiveofa
distalvanishingtestis,butthisfindingmaybesubjective;and,conversely,normal
appearingvesselsmaybeassociatedwithbothviableandvanishingtestes(Zaccara
etal,2004).Moreover,thelaparoscopicviewmaysuggestabdominalblindending
vesselsdespiteatestisbeingpresentdistallyorinanectopicabdominalposition
(Zaccaraetal,2004;Kimetal,2005;EllsworthandCheuck,2009).

Theneedforexcisionandcontralateralscrotalorchidopexyincasesofvanishing
testisremainscontroversial.Germcellsand/ortubulesareconsistentlypresentin5%
to15%ofexcisedtesticularremnants(Mooreetal,1994;Tennenbaumetal,1994;
Tureketal,1994;Cortesetal,1995a;DeLunaetal,2003;Renzullietal,2005),but
theriskofmalignancyisunknown.Asinglecaseofcarcinomainsituwasreportedby
Rozanskiandassociates(1996)inatesticularremnant.Excisionmaybeappropriate
forremnantsdistaltoaninternalringtoallowconfirmationthataviabletestisis
absent,becausethelaparoscopicappearanceofthespermaticvesselsandprocessus
vaginalismaybedeceivingandmaynotreliablyexcludethepresenceofaninguinal
testis(EllsworthandCheuck,2009).Stormandcolleagues(2007)performedlaparo
scopicexcisionofvanishingtestesin56patientswithoutsignificantmorbidity,
althoughthepositionoftheremnantsinthesepatientswasnotclear.Becausethe
testicularremnantisofteninornearthescrotum,someadvocateaprimaryscrotal
approachwhencontralateraltesticularhypertrophy(testicularlength1.8cm)anda
palpablescrotalnubbinarepresent(BelmanandRushton,2003;Snodgrassetal,
2007).However,incasesofabdominalvanishingtestes,atransscrotalsearchistime
consumingandunproductive.Moreover,incasesoftesticularepididymal
dissociation,scrotalnubbinsmaybemistakenfortesticular

remnantsthatwouldotherwisebeidentifiedbylaparoscopy(Wolffenbutteletal,
2000;DeLunaetal,2003).Laparoscopyistheprocedureofchoicetoconfirmor
excludethepresenceofaviableorremnantabdominaltestis,unlessa
prominentscrotalnubbinispalpablewithotherclinicalsignsofmonorchism.

Theneedforcontralateralfixationofasolitarytestisincasesofmonorchismisnot
clear.Thepossibilitythatprenataltorsionistheetiologyofvanishingtestis(Gonget
al,1996)doesnotimplythatthecontralateraltestisislikelytoundergoasimilarfate
afterthepostnatalperiod.However,somesurgeonsrecommendcontralateral
fixationtoeliminatetheriskofsuchadevastatingcomplication(Rozanskietal,
1996)and/orbecauseacontralateralbellclapperdeformity(incompletetesticular
fixationtothetunicavaginalis)maybepresent(Bellinger,1985;AlZahemandShun,
2006).

Associated Pathology Testicular


Maldevelopment
Manyobservationalstudiesofthehistologicdevelopmentofprepubertalcryptorchid
testeshavebeenpublished.Morethan40yearsago,Manciniandassociates(1965)
systematicallyreportedgermcellcountsandrelatedarresteddevelopmentof
spermatogoniawithprogressivelossincryptorchidtestes.Subsequently,several
largeseries,somewithadditionalnormalautopsy(Hedinger,1982)oraffected
(hernia,hydrocele)(Hadziselimovicetal,1987a)controldataandothersthatreferto
theseestablishedagedependentnorms(Schindleretal,1987;Huffetal,1993;
McAleeretal,1995;Graciaetal,2000;Cortesetal,2001),haveprovidedmainly
consistentfindingsincryptorchidboys.Thesedatashowthatthenumberof
spermatogoniapertubule(S/T)isreducedafterinfancyandfailstoincreasenormally
withageincryptorchidand,toalesserdegree,incontralateralscrotaltestes.The
frequencyofabnormalhistologyinthecontralateraltestisvariesbetweenstudies,
rangingfrom22%to95%,andislikelyreflectiveofdifferencesinpatient
populations,useofcontroldata,andmethodology.Moreover,variabilitywithinand
betweenbiopsyspecimensfromsingletestesisreported(Hedinger,1982;Schindleret
al,1987).However,thesedataprovidestrongevidencethatabnormalgermcell
developmentisoftenpresentafterearlyinfancyincryptorchidtestes.The
degreeofpathologywassimilarintrueectopic,superficialinguinalpouch,and
ascendingtestes(Herzogetal,1992;Hutchesonetal,2000b;Rusnacketal,2002)
andwasmoresevereinlimitedsamplesfrompatientswithmyelomeningocele,
posteriorurethralvalves,andprunebellysyndrome(Orvisetal,1988;Pateletal,
2008).Findingsaresimilarinretractedtestesafterherniarepair,suggestingprimary
insteadofsecondarycryptorchidism(Fenigetal,2001).Inotherstudies,higherS/T
countswerecorrelatedwithreducedagedependentinterstitialfibrosis(Suskindetal,
2008),lowerageatsurgery,andincreasedlikelihoodofpalpability(Tasianetal,
2009),butnotvolume(Nohetal,2000),ofcryptorchidtestes.Regionaldifferencesin
S/Tcountswererecentlyreported,potentiallyreflectingpopulationdifferencesin
environmentalexposures(Zivkovicetal,2009).

DetailedstudiesofperitubularmyoidandSertolicellsincryptorchidismarelacking,
buttheirabnormaldevelopmentorfunctionmaycontributetotheobservedgerm
cellabnormalities.TheavailabledatasuggestdisruptionofprepubertalSertolicell
morphology,failureofmaturationatpuberty,andevidenceforreduced

3566 SECTION XVII Pediatric Urologynumberafter4monthsofagein


cryptorchidtestes(Lackgrenand

Ploen,1984;Runeetal,1992;Regaderaetal,2001;ZivkovicandHadziselimovic,
2009).ReducedexpressionoftypeIVcollagen,aproductofbothSertoliandmyoid
cellsthatmayfunctionincellcellcommunication,wasreducedinbasement
membranesofundescendedandcontralaterallydescendedtestes(Santamariaetal,
1990).

Impairedtransformationofgonocytestospermatogoniaisreportedin
cryptorchidtestesandmaybeakeydeterminantoffertilitypotential.Innormal
testes,germcellnumberdecreasesafterbirthasgonocyteseitherdegenerateby
apoptosisormigratetothebasementmembraneanddifferentiateintospermatogonia
(Hadziselimovicetal,1986;Huffetal,2001).Althoughtheratioofgonocytesto
spermatogoniaappearstobenormalincryptorchidtestesatabout1.5monthsofage,
delayeddisappearanceofgonocytesandappearanceofadultdark(Ad)spermatogonia
occursintheundescendedascomparedwiththecontralateraldescendedtestis.Ad
spermatogoniaarelikelythereservestemcellsofthegermcellpool(Dymetal,
2009).Themechanismsinvolvedinmigrationand/ordifferentiationofgonocytesinto
stemcellsarenotclearlydefinedbutappeartoinvolveADAMintegrintetraspanin
complexes,retinoicacid,plateletderivedgrowthfactorreceptor,andcKIT(Culty,
2009).Hadziselimovicandcolleagueshypothesizethatfailureofgonocyte
differentiationincryptorchidismreflectsfailureofpostnatalactivationoftheHPG
axis(Hadziselimovicetal,1986);however,althoughtheyoccurconcurrentlythere
arenodataconfirmingadirectrelationshipbetweenthetwoevents.

Kollinandcolleagues(2006,2007)studiedtesticulargrowthinundescendedand
scrotaltestesandmeasuredtheeffectinorchidopexyontesticularsizeusingserial
ultrasonographyinprospectiverandomizedstudies.Theyshowedthatthe
undescendedtestisisnotsignificantlysmalleratbirthbutgrowslesswellthanthe
scrotaltestis,andorchidopexyatage9monthsallowspartialcatchupgrowthofthe
cryptorchidtestisupto4yearsofage.Incontrast,testesthatremainedcryptorchid
until3yearsofagedidnotgrowsignificantlybeforeorafterorchidopexy.The
cellularcomponentsresponsibleforthesedifferencesinvolumearenotknown.In
scrotaltestes,ethnicdifferencesintesticularsizeasreportedbyMainandassociates
(2006b)wereassociatedwithincreasedinhibinBlevelsandinterpretedtoprimarily
reflectdifferencesinSertolicellnumber(Sharpe,2006).

Anomalies of the Epididymis, Processus


Vaginalis, and Gubernaculum
Theepididymisisoftenabnormalinboyswithcryptorchidism(Marshalland
Shermeta,1979),withareportedfrequencyof35%to75%(Heathetal,1984;Gillet
al,1989;Mollaeianetal,1994).Anatomicfindingsindecreasingorderoffrequency
includeanomaliesoffusionbetweenthecaputand/orcaudaepididymis,elongation
and/orlooping,andatresia.Theoccurrenceofepididymalanomaliescorrelateswith
boththeseverityofcryptorchidismandthedegreeofclosureoftheprocessus
vaginalis(Elder,1992;BartholdandRedman,1996).Thedegreetowhichthese
anomaliescontributetotheriskofsubfertilityinmaleswithahistoryof
cryptorchidismremainsundefined.Similarly,failureofclosureoftheprocessus
vaginalisandattachmentofthegubernacularremnantarecommoninassociationwith
cryptorchidism.Intheirstudyof759patients,Cendronandcolleagues(1993)
identifiedapersistentlypatentprocessusvaginalisipsilateralto87%ofunilateral
and71%ofbilateralundescendedtestes.Specificnotationofthegubernacular
positionavailablefromthisandanotherlarge

studyindicatesaberrantattachmentlateraltothescrotumin66%to75%ofcases
(MoulandBelman,1988).Theprocessusispatentinapproximately50%ofboys
presentingwithascendingtestes,possiblyrelatedtoolderpatientageand/orreduced
severityofcryptorchidisminthisgroup(BartholdandGonzalez,2003).Anomaliesof
thetunicaandprocessusvaginalisincryptorchidismpredisposetodevelopment
oftesticulartorsionorclinicalhernia,respectively,inrarecases.Torsionofan
undescendedtestiscanoccuratanyage(reviewedbyZilbermanetal,2006)andmay
beconfusedwithanincarceratedinguinalhernia.Theriskoftorsionismuchhigher
inanundescendedascomparedwithadescendedtestisandmaybeparticularlyhigh
inchildrenwithneuromusculardiseasesuchascerebralpalsy.Delayindiagnosisis
common,andahighindexofsuspicionandeducationoffamilyandreferring
physiciansisneededtoreducethehighriskoftesticularloss.

Other Testicular Anomalies Associated


with Cryptorchidism
Severalrareanomaliesoftesticulardevelopmentassociatedwithcryptorchidism,each
with100150reportedcasesintheliterature,includepolyorchidism,splenogonadal
fusion,andtransversetesticularectopia.Becauseabdominalcryptorchidism
commonlyoccursinthesecases,laparoscopyisusefulinbothdiagnosisand
treatment.

Polyorchidismisthepresenceofasupernumerarytestisthatismorecommonly
unilateralandontheleftside,withrarecasesofbilateralduplicationortriplication
reportedinacomprehensivereviewofthepediatricandadultliterature(Bergholzand
Wenke,2009).Thecauseisunknown,butmostauthorsspeculatethatthisanomalyis
relatedtoduplicationordivisionofthegenitalridgewithorwithoutthewolffian
duct,asillustratedbyDanradandcolleagues(2004).Testesarereportedtobescrotal,
inguinal,andabdominalin75%,20%,and5%ofcases,respectively(Kumaretal,
2008).Affectedindividualsarefrequentlyasymptomatic,andthepolyorchidismis
identifiedatthetimeoforchidopexyorherniarepair,althoughascrotaloringuinal
massandpainwithorwithouttorsionmayoccurandpersistentmllerianremnants
maycoexist.Variousclassificationschemeshavebeenproposed,witharecenttrend
towardcategorizingtestesbasedonepididymalandvasalconfiguration(Bergholzet
al,2007;Khedisetal,2008;Kumaretal,2008).Kumarandcolleagues(2008)
suggestaclassificationthatdifferentiatesbetweentestesthataredrainedbyavas
deferens:typeA1separateepididymisandvas,typeA2separateepididymis,and
typeA3sharedepididymisandvasfromthosewithnovasaldrainage:typeB1
epididymispresentandtypeB2noepididymisorvas.Thisclassificationcanaidin
managementdecisionsthatshouldbebasednotonlyontheanatomyofthe
accessoryductsbutalsotheposition,size,andattachmentsofthetestis.Observation
andperiodicselfexaminationwithoutsurgeryshouldbeconsideredfor
sonographicallynormalscrotaltestesandorchidopexyfortestesthatareundescended
butwithintactductaldrainage(Sprangeretal,2002;Bergholzetal,2007;Khediset
al,2008).Occasionalcasesoftesticulartumorhavebeenreportedinsupernumerary
testes,butitisunclearifthisisariskrelatedtopolyorchidismperseortoassociated
cryptorchidismorpersistentmllerianductsyndrome(Sprangeretal,2002;Ghoseet
al,2007).

Splenogonadalfusionisadefectcharacterizedbycontinuousordiscontinuous
fibrousunionbetweensplenictissueandthegonad,aconditionmuchmore
commonlyrecognizedinmales(KhairatandIsmail,2005).Approximately30%of
affected

CHAPTER 132 Abnormalities of the Testis and Scrotum and Their


Surgical Management 3567

individualshavecryptorchidism,withthemajorityofcasesabdominalandbilateral
(59%)and65%and26%involvingtheleftandrightsides,respectively(Cortesetal,
1996).Thecontinuousformofsplenogonadalfusionismorecommonlysyndromic,
associatedwithlimbdefects,micrognathia,microglossia,analatresia,andpulmonary
hypoplasia(McPhersonetal,2003),whereascryptorchidismisassociatedwithboth
continuousanddiscontinuousformsandmostcommonlycoexistswithbilateral
amelia(Cortesetal,1996).Thepathogenesisoftheanomalyisnotunderstoodbut
basedontheconstellationofdefectsobservedishypothesizedtorepresenta
developmentalfielddefectwithaberrantmigrationofspleencellsoccurringat5to8
weeksgestation.Mostcasespresentincidentallyatthetimeoforchidopexyor
inguinalherniarepairorwithscrotalswellingrelatedtoillnessrelatedreactive
changeswithinthesplenictissue.Testicularmalignancyisreportedrarelyin
associationwithcryptorchidismandnotlikelyrelatedtothesplenicanomaly.
Treatmentshouldfocusonrecognitionofthedefectatthetimeoforchidopexyand
avoidanceofunnecessaryorchiectomy.

Transversetesticularectopiamayoccurasanisolatedanomalyinotherwisenormal
maleswithcryptorchidismorvanishingtestesorbeassociatedwithpersistent
mllerianductsyndromein20%to50%ofcases(DeLunaetal,2003;Wuerstleet
al,2007;ThambidoraiandKhaleed,2008).Theclassicpresentationisinguinalhernia
withcontralateralnonpalpabletestis,althoughbothtestesmaybepalpableinthesame
hemiscrotum.Theetiologymayberelatedtomechanicalhindrancetodescentby
fusionofwolffianductderivatives(Chackoetal,2006)orpersistentmllerianducts
ortoaprimarygubernaculardefect.Interestingly,completelossofthegubernacular
attachment,transverseectopia,and/ortorsionwereobservedintransgenicmicenull
forInsl3(NefandParada,1999;Zimmermannetal,1999).Orchidopexymaybe
performedusingopensurgicalorlaparoscopictechniques,butincasesofvasalfusion
theinvolvedtestisismobilizedipsilaterallyandatransseptalapproachisusedto
placethetestisinthecontralateralscrotum(Chackoetal,2006;Thambidoraiand
Khaleed,2008).

Treatment
Correctionofcryptorchidismisindicatedtooptimizetesticularfunction,
potentiallyreduceand/orfacilitatediagnosisoftesticularmalignancy,provide
cosmeticbenefits,andpreventcomplicationssuchasclinicalherniaortorsion.
Exceptincertaincasesofassociatedcomplexmedicalillnessorinthepostnatal
period,treatmentshouldproceedafterconfirmationofthediagnosis.Aflowdiagram
(Fig.1324)showstherecommendedapproachtopalpableandnonpalpabletestesin
patientsconfirmedtohaveundescendedtestisbyanexperiencedexaminer.

Ininfants,observationisindicatedforthefirst6postnatalmonthstoallow
spontaneoustesticulardescent.Ifdescentdoesnotoccurinthepostnatalperiod,
presentconsensussupportssurgicaltreatmentat6monthsofage.Supportforthis
approachisbasedonthefollowingrationale:(1)descentisunlikelyinfulltermmales
afterage6months(Wenzleretal,2004);(2)testiculargrowthisrestoredafterearly
orchidopexy(Kollinetal,2007);(3)hormonetherapyisnotconsideredefficacious
(Ritzenetal,2007);and(4)orchidopexyforabdominaltestesmaybefacilitatedin
younginfantssoonafterthehormonalsurge.Inboyswithahistoryofprematurity,
spontaneousdescentmaybedelayedandthereforecontinuedobservationfor6
monthsbeyondtheexpecteddateofdeliveryor,especiallyiftesticular

positionismarginal,untilayearofagemaybewarranted.

However,evenifspontaneousdescentoccurs,continuedobservationisneeded
becauseoftheriskforrecurrentcryptorchidism,orreascentofa
spontaneouslydescendedtestis.

Recently,twogroupsfromtheNetherlandspublishedreportsoftheoutcomeofboys
withacquiredundescendedtesteswhowereobservedexpectantly(Sijstermansetal,
2006;Eijsboutsetal,2007).Theseauthorsreportedspontaneousdescentin75of132
(57%)and98of129(75%)testes,respectively,mostbymidpuberty.Themean
volumeofdescendedtestesinthestudybyEijsboutsandcoworkerswascloserto
thoseofnormalcontralateraltestesascomparedwithboyswhounderwentorchi
dopexy.However,inbothoftheseserieshighscrotaltestescomprisedthemajority
andofthosethatdescendedandlowscrotalunstabletestes(likelyretractile)were
alsoincluded.Ontheotherhand,Eijsboutsandcoworkersreportedthat19of82
unilateralcaseshadpreviouscontralateralorchidopexyandthat10boysrequired
orchidopexybecauseofclinicalhernia,pain,ortorsion,suggestingahigherlikelihood
oftruecryptorchidisminthesecases.Becausethesestudieswerenotrandomized
andprovidenolongtermdataregardingtesticularoutcome,observationisnot
recommendedforacquiredcryptorchidism.

Medical Therapy
Hormonaltherapyhasbeenusedforavarietyofindicationsinpatientswith
cryptorchidism,includingdifferentiationofretractilefromtrueundescendedtestes,
stimulationoftesticulardescentorgermcellmaturation,andasanadjunctto
abdominalorchidopexy.Thesetherapiesmaybeusedsporadically,butnoneiscon
sideredthestandardofcare,likelybecauseoflimitedavailabilityofsome
medications,lackofrigorousdatasupportingtheirefficacy,andconcernsabout
possibleadverseeffects(Thorssonetal,2007).

Severalpublishedreportsaddresstheusefulnessofhormonaltherapyin
distinguishingretractilefromtrueundescendedtestes.Inprospectiveseriesreporting
theresponseofputativeretractiletestestohCG,successratesvaryfrom58%to100%
andmaybedependentnotonlyonage,degreeofretractility,andaccuracyof
diagnosisbutalsoonthedosageregimenused(Rajferetal,1986;Milleretal,2003;
Metinetal,2005).InpatientstreatedbyMillerandassociates(2003),nonresponders
undergoingsurgeryhadtypicalfindingsassociatedwithtruecryptorchidism,
includingapatentprocessusvaginalisandsuperficialinguinalpouchlocation,and
followupwasshort(6to12months).ThesedatasuggestthathCGfailstoreliably
distinguishretractilefromcryptorchidtestesandthereforedoesnoteliminate
theneedforserialexaminationsinthesepatients.

LHreleasinghormone(LHRH)and/orhCGhavebeenusedashormonaltherapyto
inducedescentoftestesformorethan70yearsbasedonthepremisethatandrogens
promotetesticulardescent,buttheefficacyofthistherapyisquestionable(Pyoralaet
al,1995;Hennaetal,2004;Thorssonetal,2007).Evenwhenanalysisislimitedto
randomized,placebocontrolledtrialsthereliabilityofthedataislimitedinthatmany
seriesdidnotconfirmexclusionofretractileornonpalpable(potentiallyabsent)
testes,adequacyofrandomizationprocedures,and/orlongtermefficacyofthe
treatment.Althoughtheefficacyofeitherhormonaltreatmentisabout20%and
superiortoplaceboinrandomizedtrials,thiseffectisnotclearlyclinicallysignificant.
Althoughlowertesticularpositionshowsaclearcorrelationwithresponseto
therapy,noconsistentdifferencesinresponsebasedonageorlaterality

3568

SECTION XVII Pediatric Urology

Testis examination by experienced observer


Nonpalpable

Palpable

Distal to external ring


Consider US Overweight Uncooperative

Retractile

Consider scrotal orchidopexy


Large contralateral testis >2 SD No palpable appendage

Normal contralateral testis No intrascrotal structures

Yearly exams and parental observation

Inguinal orchidopexy with or w/o hernia repair

Patent PV


Scrotal nubbin

Laparoscopy


Scrotal excision

? Atretic vessels Vanishing testis

Vessels pass internal ring

Abdominal testis

Long-term F/U Counseling the family and self-exam

? Inguinal repair


Consider contralateral testicular fixation

No further intervention

Inguinal exploration

Solitary testis

Near ring

High abdominal


Counsel family about prosthesis

Viable testis

Consider microvascular orchidopexy

Laparoscopic orchidopexy

1- or 2-stage Fowler-Stephens orchidopexy

Hypotrophic, short vas, dysgenetic, or postpubertal

Orchidopexy

? Bilateral

Orchiectomy

Consider staged approach

Figure 1324. Algorithm for management of the undescended testis. This


is the recommended approach to diagnosis and treatment of palpable and
nonpalpable testes in patients confirmed to have undescended testis by
an experienced examiner. F/U, follow-up; PV, processus vaginalis; US,
ultrasonography.

havebeenreportedinrandomizedtrials.Overall,theevidencefromrigorous
studiesindicatesthatLHRHtherapyforcryptorchidismisonlymarginallymore
effectivethanplacebo;althoughnotstudiedinrandomizedplacebocontrolled
trialsbecauseofitsrouteofadministration,hCGalsoshowslimitedefficacy.
OtherusesofhCGincludingtreatmentofacquiredcryptorchidismandtofacilitate
palpabilityand/ortreatmentoftheabdominaltestis(Polasciketal,1996;Bakeretal,
2001;Bukowskietal,2001)alsohavelimitedefficacy.

ThequestionoftheeffectoftherapeuticdosesofhCGorLHRHongermcell
developmenthasbeenaddressedinseveralconflictingstudiesthatarelimitedby
smallsamplesize,absentorsuboptimalrandomization,andvariableavailabilityof
biopsydata(Ongetal,2005).Insmall,retrospectivestudies,hCGtreatmentwas
associatedwithincreasedgermcellapoptosisatbiopsyandloweradulttestisvolume
(Dunkeletal,1997)andprevioushCGorLHRHtherapywasassociatedwithreduced
S/Tcountsin1to3yearoldboysascomparedwithsurgeryalone(Cortesetal,
2000).Incontrast,Schwentnerandassociatesrandomizedyoungboys(meanage33
months,21pergroup)toLHRHornohormonaltherapybeforesurgeryandreported
thatmeanS/Tcountwashigher(1.050.71)inLHRHtreatedascomparedwith
nontreated(0.520.39)testes(Schwentneretal,2005).Inviewofa

lackoflargeprospectivestudies,itisunclearifhormonetherapyforcryptorchidismis
beneficialorharmfultogermcellsintheshortorlongterm.

Hadziselimovicandcolleagues(1987b)haveadvocateduseoflowdose,longterm
(everyotherdayfor6months)LHRHanalogue(buserelin)therapyforstimulation
ofgermcelldevelopmentinconjunctionwithorchidopexy.Inaretrospectivestudyof
nonrandomized,nonagematchedpatientsreceivingbuserelinversussurgeryonly,
S/Tcountsweresignificantlyhigherinthetreatedgroup.Asubsetofpatientsfrom
thissamecohortwiththemostseveretesticularhistologyunderwentrebiopsyafter
completionoftherapyandwerecomparedwithagroupof8boysofunknownage
whorequiredreoperativeorchidopexy(Hadziselimovicetal,1987a).Asignificant
improvementinmeanS/Tratiowasseeninthebuserelintreatedbutnotthesurgical
group.Similarly,inasmallerselectgroupofboystreatedwithlowdosesofarelated
LHRHagonist,nafarelin,improvedhistologywasobservedinoneorbothtestesat
rebiopsyin8of12boys.Inarandomizedstudyofclinicallymatchedboysreceiving
buserelinandhCG,placeboandhCG,orsurgeryalone(19to25/group),S/Tcounts
werealsosignificantlyhigherinthosetreatedwithbuserelin(Bicaand
Hadziselimovic,1992).Morerecently,Hadziselimovic(2008)publisheda
nonrandomizedretrospectivestudyshowingthat

CHAPTER 132 Abnormalities of the Testis and Scrotum and Their


Surgical Management 3569

buserelintreatedmales(mostalsoreceivedhCG)withahistoryofunilateral
cryptorchidismandpoorpretreatmentS/Tcountshadmuchhigherspermcountsthan
patientswhounderwentsurgeryonly(n=15pergroup).Unfortunately,thesetwo
groupsweresmallandnotclinicallymatchedprospectivelytolimitotherpotential
confoundingfactors,suchastesticularposition,andspermcountsinthesurgeryonly
groupwerelowerthantypicalforunilateralcryptorchidism.Overall,thesestudies
providepreliminary,suggestiveevidencethatbuserelinmayhavebothshortand
longtermeffectsontesticularhistologyand/orfertilitypotential.However,the
suboptimaldesignofthestudiesonwhichthisevidenceisbasedmandatesthatfuture
welldesignedprospectivestudiesarenecessarybeforeroutineuseofbuserelin
treatmentincryptorchidism.

Insummary,littleifanyhighqualityevidenceexistsshowingabenefitofhormonal
therapyforcryptorchidismorforstimulationofgermcells.Arecentstatementby
theNordicConsensusgrouprecommendsthattesticularbiopsyandhormonal
therapynotbeusedinstandardclinicalcareofboyswithcryptorchidism(Ritzen
etal,2007),althoughnotallcliniciansconcur(HadziselimovicandZivkovic,2007).

Surgical Approach to the Palpable Testis


Thestandardtreatmentforpalpabletestesisinguinalorchidopexywithrepairofan
associatedherniaifpresent(Hutchesonetal,2000a),althoughinrecentyearsa
primaryscrotalapproachasoriginallydescribedandadvocatedbyBianchiand
colleagues(BianchiandSquire,1989;Iyeretal,1995)hasbeenadvocatedbysome.
Therecommendedageforsurgicalinterventionhasgraduallydeclinedoverthe
yearsandpresentlyisage6monthsinfulltermmalesinwhomthetesteshavefailed
todescend.However,theaverageageoforchidopexyremainsabout4yearsinmany
series,likelyowingtothefrequencyofmilderacquiredcasesaswellasdelayed
referralofsomecasesdiagnosedatbirth(BartholdandGonzalez,2003).Anoption
forpubertalandpostpubertalboysisorchiectomy,especiallyifthetestisisabdominal
ordifficulttomobilizebecausepoorspermatogenesisandhypotrophyareusually
presentandtheriskofcarcinomainsituandtorsionexist(Rogersetal,1998).

Inguinal Orchidopexy. Afterinductionofanesthesia,thepatientis


reexaminedtoconfirmthatthetestisispalpableandtoidentifythelowesttesticular
position.Inthestandardinguinalapproach,alowtransverseincisioninLangerslines
atorbelowtheinguinalcreaseismadesuperolateraltothepubictubercle.Dissection
ofthesubcutaneoustissueshouldincludeasearchforatestiswithinthesuperficial
inguinalpouch.Theexternalobliquefasciaisincisedtoexposethecanalwithcareto
avoidinjurytotheilioinguinalnerve.Testispositionisrecordedrelativetothe
inguinalcanal.Thespermaticcordisisolated,andthetestisisdissecteddistallytoits
attachmenttothegubernacularremnant.Transectionofthegubernaculumdistalto
thesacwillavoidpotentialinjurytoalongloopingvas.Longitudinalincisionofthe
internalspermaticfasciaallowsfreemobilizationofanintactherniasac,ifpresent,
andminimizesskeletonizationofthevasandspermaticvessels.Aftertransection,the
sacismobilizedtotheleveloftheinternalinguinalringandsutureligated.Incisionof
theinternalspermaticandtransversalisfasciaattheleveloftheringfacilitates
additionalretroperitonealmobilizationofthevasandvessels,ifneeded.Further
maneuverstoprovidespermaticcordlengthincludetransectionoflateralfascial
bandsalongthecord,cranialretroperitonealdissection,medialtranspositionofthe
testisbeneaththeepigastricvessels(Prentissmaneuver)and,ifrequired,cranial
extensionoftheincision.Veryrarely,thetestiscannotbe

broughttodependentscrotalpositionafterthesemaneuversandatwostage
proceduremaybeconsideredasanalternativetoorchiectomy,whichispreferentially
reservedforgrosslyabnormaloratrophictestes.Dessantiandassociates(2009)
describeanoveltwostagetechniqueforhighcanalicularorabdominaltestesthat
involvesplacementofapolytetrafluoroethylenemembranearoundthemobilized
cord,fixationofthetestistotheinvaginatedscrotumwithapledget,followedbya9
to12monthdelaythatallowedspontaneousscrotallocalizationofthetestisin82%of
45testes.

Alargeclamporafingercanbeusedtocreateatunneljustanteriortothepubisanda
scrotalorsubdartospouchcreatedaftertransverseincisionofthescrotalskin(Ritchey
andBloom,1995).Thetestisispassedthroughanopeninginthedartoswithout
twistingofthespermaticcord.Existingappendagesshouldbeexcisedandthe
epididymisinspectedandanyanomaliesrecorded.Recordingoftesticularvolume
bydirectcalipermeasurementinthreedimensionsandsimilar(estimated)mea
surementsofcontralateraltesticularvolumecanestablishabaselinefor
postoperativeassessment.Securefixationofthetestiswithinthepouchcanbe
achievedbytensionfreeclosureoftheopeninginthedartosaroundthecord,
incorporatingthecutedgeofthetunicavaginalis.Ifneeded,additionalabsorbable
fixationsuturescanbeplacedbetweenthevisceraltunicavaginalisandthedartos.
Suturesthroughthetunicaalbugineaofthetestisarenotrecommendedbecauseof
possibleinjurytothetestisviainflammatoryorvascularinsult;suturefixationofthe
testisisinanyeventnotneededifmobilizationisadequateandasubdartospouch
techniqueisused.Closureiscompletedwithabsorbablesutures.Supplementallocal
orregionaltechniquesforperioperativepaincontrolareadvisableandmayinclude
localanestheticinfiltrationorcaudalanesthesia;thelatterisparticularlyusefulin
youngerpatientsundergoingbilateralinguinalorconcomitantpenilesurgery.

Testicularbiopsyhasbeenperformedroutinelyinsomecentersandisadvocatedby
Hadziselimovicandcolleagues(2007)asamethodtodetermineprognosisfor
fertility.Thisapproachiscontroversialandnotrecommendedoutsideofresearch
protocolsbecauseitdoesnotchangethecurrentapproachtotreatment(Ritzenetal,
2007;BeckersandvanderHorst,2008).Therisktothecryptorchidtestisfrombiopsy
istheoretical;althoughlongtermeffectsdonotappeartoincludeincreasedriskof
microlithiasisorantispermantibodyformation(Pateletal,2005),othermoresubtle
effectscannotbeexcludedfromtheavailabledata.Biopsyisindicatedincasesof
sexualambiguityorifclinicalevidenceoftesticulardysgenesisispresent.

Aminimumof6monthsfollowupisneededtodeterminetestispositionandsize
oncestabilized,andlongtermfollowupisindicatedforcounselingofthepatient
regardingfertilityissues,riskoftesticularmalignancy,andselfexamination.
Complicationsofinguinalorchidopexyforapalpabletestisareuncommon;thoseof
greatestfrequencyandconcernincludetesticularretractionoratrophy.Ina
comprehensiveliteraturereviewin1995,Docimo(1995)concludedthattheoverall
riskofatrophyornonscrotalpositionwasapproximately15%overallinpublished
reports,significantlyhigherinabdominalorpeepingtestes(24%)comparedwith
thosedistaltotheinternalring(10%)andhigherinboysoperatedafter6yearsofage.
Torsionofascrotaltestisafterorchidopexyhasbeenreportedbutisveryrare,and
theriskmaybeminimizedbyusingasubdartospouch.Ifcompleteintrascrotal
testicularatrophyoccurspostoperatively,furtherinterventionisnotneededbutthe
optionoftesticularprosthesisplacementshouldbeofferedtothepatientandfamily
(Bodiwalaetal,2007).

3570 SECTION XVII Pediatric Urology

Implantationofatesticularprosthesisshouldoccuratleast6monthsafterany
inguinoscrotalprocedureorafterpubertyandisbestperformedthroughaninguinal
approach.Closureofthescrotalfasciaabovetheimplantusingapursestring
nonabsorbablesutureisrequired.Risksincludingdisplacement,pain,orinfection
occurinlessthan5%ofcases.Clinicalexperiencesuggeststhatcryptorchidboys
mayrequestprosthesisimplantationlessfrequentlythanmaleswithacutetesticular
lossafterpuberty(Bodiwalaetal,2007).Elderandassociates(1989)recommended
thatprepubertalprosthesisplacementbeconsidered,butitisnotclearwhetherthis
approachispreferabletoplacementafterpuberty.

Reoperationisindicatedifatestisisnonscrotalafterorchidopexy.Ifthetestisis
prescrotal,aprimaryscrotalapproachcanbeconsideredandmayallowadequate
mobilizationofthetestis.Ifinguinalexplorationisneededtoprovidesufficientcord
length,severalapproachesareavailable.Redman(2000)describedausefultechnique
forprimaryorsecondaryorchidopexythatinvolvesalateralapproachtothecordafter
mobilizationoftheexternalobliqueandcremasterfasciae.Thisapproachavoids
traversalofthepreviouslyscarredlayersanteriortothecordandaclearerviewofthe
anatomy.Cartwrightandcolleagues(1993)describedmobilizationofthe
intracanicularcordwithanoverlyingpatchofexternalspermaticfascia.The
importanceofcorrectingapersistentlypatentprocessusvaginalisand/orofadequate
retroperitonealmobilizationofthecordincasesofhighrecurrentcryptorchidismhas
beenstressed(Redman,2000;Pesceetal,2001;Ziylanetal,2004).Theresultsof
secondaryorchidopexyappeartobesimilartotheprimaryprocedure,althoughthe
riskofvascularandvasalinjuryistheoreticallyhigher(Pesceetal,2001).

Transscrotal Orchidopexy. Aprimaryscrotalapproachcanbeconsidered


whenthetestisispalpable(BianchiandSquire,1989;Iyeretal,1995),althoughsome
surgeonsreservethisapproachfortestesthatareclosetoorcanbedrawnintothe
scrotum(Russinkoetal,2003;Rajimwaleetal,2004;Basseletal,2007;Takahashiet
al,2009).Anincisionalongthesuperiorscrotalborderismadeandthedistalsacand
overlyingcremastermobilized.Oncedissectedfree,thesaccanbeplacedon
tractionandfreedasfarcraniallyaspossible,highabovetheinguinalcanal(Iyeret
al,1995),althoughsomecasesrequireconversiontoaninguinalapproach(Parsonset
al,2003;Dayancetal,2007).Rajimwaleandcoworkersconfirmedinseveralcases
thattheherniasachadbeeneffectivelyligatedabovetheinternalringviathescrotal
incisionwhenasecondaryinguinalincisionwasrequiredforfurthermobilizationof
thetestis(Rajimwaleetal,2004).Fixationsuturesthroughthetunicaalbugineawere
usedinmanyseriesofscrotalorchidopexy(Jawad,1997;Russinkoetal,2003;Bassel
etal,2007;Dayancetal,2007;Takahashietal,2009).Theriskoftesticularretraction
oratrophywasreportedas0%to2%inmostoftheseseriesafterfollowupranging
from1monthto3years.However,postoperativeherniawasreportedinabout3%of
casesintwoseriescontainingalargerproportionofcanaliculartestes(Dayancetal,
2007;AlMandiletal,2008)andin2%ofherniasrepairedbyBianchisgroupusinga
scrotalapproach(Iyeretal,1995).Althoughtheriskofthiscomplicationappearslow,
followupisnotsufficientinreportedseriestofullydefinetherisk.

Surgical Approach to the Abdominal Testis


Onceanabdominaltestishasbeenidentifiedbylaparoscopyorothermeans,a
decisionismadewhethertoproceedwithanopenorlaparoscopic,oneortwostage
orchidopexywithorwithout

spermaticvesseltransection.Orchiectomyisappropriateforpatientswithtestesthat
arepoorlyviableand/orathigherriskfortumor,suchasverysmallordysgenetic
prepubertalorinpostpubertalpatients,andisbestperformedlaparoscopically.

Open Transabdominal Orchidopexy. Extensivedissectionofthevasand


vesselsisfacilitatedbyalongitudinalopeningoftheinternalobliqueandperitoneum
throughanextendedinguinalincision(Kirschetal,1998)orviaahigherincision
medialtothepubictubercleandapreperitonealapproach(Gheileretal,1997;Jones
andBagley,1979).IntheproceduredescribedbyJonesandBagleytheinternalringis
approachedviaamusclesplittingincision,theperitoneumisopened,thetestisis
delivered,andthevasandvesselsarefreedfromtheirperitonealattachments.A
tunneliscreatedtothescrotum,andthetestisissecuredinplaceasforaninguinal
orchidopexy.Thereportedsuccessrateforthisprocedureforabdominaltesteswas
95%(Gheileretal,1997).

Laparoscopic Orchidopexy and Fowler-Stephens


Orchidopexy.

Operativelaparoscopyemergedover15yearsagoastheprocedureofchoicefor
abdominalorchidopexy(CaldamoneandAmaral,1994;JordanandWinslow,1994),
andthebasicsurgicalapproachandhighsuccessrateshavestoodthetestoftime
(Table1321).ThefeasibilityofprimaryversusFowlerStephensorchidopexy
dependsonthelengthofthevasandvessels,presenceorabsenceofloopingductal
structures,andageofthepatient.Althoughlaparoscopyallowsthesurgeontoassess
someofthesefeaturesbeforechoosingaspecificsurgicalprocedure,thechoicemay
bedifficult(Yuceletal,2007).Observedtesticularpositionalonemaycorrelate
poorlywiththeultimatelengthofthecordaftermobilization.

Afterinductionofanesthesia,afurtherattempttopalpatethetestisismade,although
alaparoscopicapproachmaybeconsideredformobilizationofhighcanalicular
testesaswell.Afterdecompressionofthebladderandstomach,aninfraumbilical5
mmtrocarisplacedforpassageofa30degreelensandbothinternalringsare
visualized.AnopenHassonorBaileztechniqueispreferableforumbilicaltrocar
placementinthepediatricagegrouptominimizetheriskofinjury(FrancGuimondet
al,2003).CO2pneumoperitoneumtoamaximumpressureof8to12mmHgisused.
Thesizeandpositionofthetestiswithintheabdomenisdeterminedbeforefurther
decisionmaking.Forsinglestagelaparoscopicorchidopexy,additional2or3mm
trocarsareplacedintherightandleftlowerquadrantstotriangulatewiththeumbili
cusandipsilateralinternalringorinthemidclavicularlineatthelevelofthe
umbilicusbilaterally.Themajorstepsaremobilizationofanystructuresextending
distaltotheinternalring,includingepididymis/vasandgubernacularremnant,
transectionoftheperitoneumlateraltothevesselsanddistaltothevas,and
proximalmobilizationofthevesselswhilemaintainingcollateralbloodsupply
betweenthevasandspermaticvesselsifaFowlerStephensmaneuverbecomes
necessary.Samadiandassociates(2003)advocateinitialmobilizationofthe
gubernaculumtobeusedasahandleforfurthermobilizationofthetestis,and
minimaluseofcauteryduringthismaneuver.Abilitytomobilizethetestistothe
oppositeinternalringhasbeenusedasameasureofadequatelengthforplacementin
thescrotumbutisnotpredictableinsomeseries.Oncemobilized,thetestisisbrought
throughanewhiatusatthelevelofthemedialumbilicalligamentorthroughthe
existinginternalinguinalring.Thismaneuvercanbecompletedusingatransscrotal
clamporport.Withtensionontheextraabdominaltestis,peritonealattachments
overlyingthecordcanbemoreeasilytransected,thusprovidingadditionallength.In
somecasesthetestiscanonlybebroughtintotheupperscrotum;thelongterm
adequacyofthisapproachisnotclear.Excessivetensionon

Table 1321.

Results of Laparoscopic Orchidopexy*


CHAPTER 132 Abnormalities of the Testis and Scrotum and Their
Surgical Management 3571

PATIENTS
PROCEDURE SERIES AGE FOLLOW-UP
(TESTES)

Laparoscopic
Baker et al, 2001 178 (208) 36 mo 7.7 mo (mean
orchidopexy

Samadi et al, 2003 139 ? 6 mo

Handa et al, 2005 58 (76) ? 2.2 yr (media

El-Anany et al, 2007 46 5 yr 3 yr (mean)

Kaye and Palmer, 2008 19 (38) 9 mo (median) 6-12 mo

Yucel et al, 2007 34 ~12 mo (median) 5-8 mo (medi

Radmayr et al, 2003 28 (28) 1.9 yr 6.2 yr

Denes et al, 2008 24 (26) 6.4 yr 6 mo

Esposito et al, 2002 25 (25) 3.9 yr (median) 23 mo (media

Laparoscopic Esposito and Garipoli, 1997 33 (33) 3-10 yr (range) 30 mo (mean


one-stage FS

Baker et al, 2001 25 (28) 31 mo 8.6 mo (mean

Chang et al, 2001 20 1.5 yr (median) 6 mo

Laparoscopic
Baker et al, 2001 63 (74) 55 mo 20 mo
two-stage FS

Lotan et al, 2001 59 (66) 14 mo 3-12 mo

El-Anany et al, 2007 47 5 yr 3 yr (mean)

Radmayr et al, 2003 29 (29) 1.9 yr 6.2 yr (mean)

Robertson et al, 2007 21 (25) 36 mo (mean) 6 & 18 mo

Denes et al, 2008 15 (21) 6.4 yr 6 mo

Hay et al, 1999 20 ? 6 mo

*Reported results of abdominal, one- and two-stage Fowler-Stephens (FS) orchidopexy


in.series with at least 20 treated testes. Age is mean for entire series unless otherwise
noted. High position refers to testes not in dependent scrotal position.

Position within scrotum not clearly documented. Overall success refers to the frequency of
nonatrophic testes in satisfactory scrotal position according to criteria used by the authors.

thevesselsduringplacementofthetestisshouldbeavoided,however,becauseinjury
oravulsionofthespermaticvesselsmayoccur(Espositoetal,2002).Akeystrategy
shouldbepreservationofthebloodsupplybetweenthevasandspermaticartery
duringdissectionsothattheFowlerStephensprocedurecanbeperformedif
necessary.

Formalclosureofthedissectedinternalring,irrespectiveofwhetherthemobilized
testespassesthroughit,doesnotappeartobenecessary(Handaetal,2005;Riquelme
etal,2007);indeed,previousexperiencewithopenherniarepairsuggeststhatligation
isnotneedediftheinternalringisdissected(Mohtaetal,2003).Acontralateral
patentprocessusvaginaliswasidentifiedin9%ofboysundergoinglaparoscopic
orchidopexyinoneseries(PalmerandRastinehad,2008),andlaparoscopicrepairwas
performedandrecommended.Thenecessityforthisapproachinpreventingclinical
herniaformationisquestionablebasedonstudiesofboysundergoinglaparoscopicor
opencontralateralherniarepair(seeSchier,2007b,anddiscussioninthelatersection
HerniasandHydroceles).

Fortestesthatarenotnear(variablydefinedas2to4cmabove)theinternalinguinal
ring,transectionofthespermaticvesselsasoriginallydescribedbyFowlerand
Stephens(1959)maybenecessary;alongloopingvasfacilitatesbutisnotrequired
fortesticularmobilizationtothescrotum.TheFowlerStephensprocedureisnow
typicallyperformedlaparoscopicallywithspermaticvesselclipping(Bloom,1991)
followedbylaparoscopicoropentesticularmobilization6monthslaterorinone
stage.Althoughmostsurgeonstransectthespermaticvessels1.5to3cmabovethe
testis,KoffandSethi(1996)proposedthatligationclosetothetestisispreferable.
Thisgroupsubsequentlystudiedtheeffectoflowversushightransectionofthe
vesselsinprepubertalratsandshowedareductioninadulttesticularspermnumbers
thatwassimilarinbothgroups(Srinivasetal,2005).Inhumanstudies,testicular
biopsiesbeforeandafterspermaticvesseltransectionalsoshowedareductioninS/T
count,afindingthatwassignificantinyoungerboys(Thorupetal,1999;Rositoet
al,2004).Ingeneral,thepreferredapproachisavoidanceofspermaticvessel
transectionwheneverpossible;theavailabledatasuggestthisis

3572 SECTION XVII Pediatric Urology

possibleinthemajorityofcasesofabdominalorchidopexy.Inrarecases,particularly
ifthetestisisretrovesical,thevasistooshorttoallowscrotalplacementofthetestis
andorchiectomyisultimatelyrequired(PerovicandJanic,1997).

ThesuccessratesforlaparoscopicproceduresasshowninTable1321(Jordanand
Winslow,1994;EspositoandGaripoli,1997;Hayetal,1999;Bakeretal,2001;
Lotanetal,2001;Espositoetal,2002;Radmayretal,2003;Samadietal,2003;
Handaetal,2005;Robertsonetal,2007;Yuceletal,2007;ChangandFranco,2008;
Denesetal,2008;KayeandPalmer,2008)appeartocomparefavorablywiththe
corresponding74%,63%,and77%overallsuccessratesforopensurgicalandone
andtwostageFowlerStephensprocedures,respectively,reportedbyDocimo(1995).
Variationinreportedresultsintheseseriesmayreflectinherentselectionbiasdueto
differencesinpatientage,testicularposition,lengthoffollowup,and/orcriteriaused
todefinesuccess,suchasintrascrotalversusdependentscrotalposition.Some
authorsrecommendthatultrasoundbeusedtoconfirmtesticularviability
postoperatively(Espositoetal,2002).Othercomplicationsoflaparoscopic
orchidopexyappeartoberareandpotentiallyincludebladderorvascularinjury,
hypercapnia,anddelayedsmallbowelobstruction(Espositoetal,2003;Hsiehetal,
2009).

Laparoscopictechniquesmaybeapplicableinunusualcases,includingbilateral
orchidopexy,abdominalwalldefects,polyorchidism,splenogonadalfusion,and
transversetesticularectopiawithorwithoutpersistentmllerianducts.Manyauthors
recommendsimultaneousbilateralabdominalorchidopexy(KayeandPalmer,
2008),butthesurgeonshouldconsiderastagedapproachifbothtestesareveryhigh
ortheviabilityofatestisisquestionedduringthecourseoforchidopexy.Depending
ontheoutcomeofthefirstprocedureat6monthsfollowup,thesurgeoncanchoose
anoperativeapproachtothecontralateralsidethatwouldappeartominimizetherisk
ofbilateraltesticularatrophy(Thorupetal,2007).Somesurgeonshaveconsidered
microvascularorchidopexytobeapreferredapproachtothesolitaryabdominaltestis,
particularlywithhistoricalsuccessratesof88%ascomparedwithlowerratesfor
openprocedures(Docimo,1995).Atacenterwithsubstantialexperienceusingthe
microvascularapproach,longtermsuccessratesof96%forstandardand88%for
laparoscopicallyassistedautotransplantationwerereported(Bukowskietal,1995b;
Tackettetal,2002).Theadvantageofthisapproachispreservationofthespermatic
vessels,atthecostoflongeroperativetimeandrequirementsforanexperienced
microvascularsurgeonandhospitalstay.
PrognosisRisk of Subfertility
Althoughthereisstrongevidencethatahistoryofcryptorchidismisassociatedwith
subfertilityinindividualpatients,theeffectsofageatdiagnosis,typeoftreatment,
and/orseverityofdiseaseonoutcomeremainincompletelydefined.Majorlimitations
intheinterpretationofcryptorchidismoutcomestudiesincludeselectionbiasdueto
incompletefollowupoflargepatientcohorts,heterogeneityofdiagnosisand
timing/typeoftreatment,andothermethodologicconcerns,includinglackofage
matchedcontrols,failureofabstinencebeforesemenanalysis,and/oranalysisofa
singlesemensample.Inalargereviewofretrospectivestudiespublishedinthe50
previousyearsthatdidnottaketheseconcernsintoconsiderationanddidnotinclude
astatisticalmetaanalysis,Chilversandcolleagues(1986)reportedoverallratesof
oligospermiaand/orazoospermiain75%offormerlybilaterallyand43%

offormerlyunilaterallycryptorchidmen.Thelimitedavailabledatacomparingearlier
(age<9years)andlatertreatmentdidnotshowdifferencesinthefrequencyof
subfertilityafterunilateral(281cases)orbilateral(123cases)orchidopexy.Similarly,
subsetanalysisfailedtoidentifyanyeffectofhCGtreatment.Twosubsequentlarge
studiesofsemenparametersinmenwhounderwentorchidopexyinchildhoodalso
founddifferencesbetweenbilateralandunilateralcryptorchidismbutlessconsistent
overallresults.Okuyamaandassociates(1989)reportednormalspermdensityin0%,
72%,77%,and42%ofmenafterbilateralorchidopexy(61patients),unilateral
orchidopexy(149patients),unilateralorchiectomy(26patients),andnotreatment
(38patients)foringuinaltesteswithouthormonetherapy.Allofthesesubjectshad
threesemenanalyses.Incontrast,Graciaandcolleagues(2000)reportednormal
semensamplesin10of55(18%)menwithahistoryofbilateraland57of171(33%)
menwithpreviousunilateralcryptorchidism.Themajorityoftestesinthisseries
werecanalicular,and80%ofsubjectsreceivedpreoperativehCGtherapy.These
authorsnotednodifferencesbasedontesticularposition,andsemenqualitywasnot
correlatedwithageofsurgeryineitherseries.Inacohortof91patientswith
unilateralcryptorchidismwhounderwentorchidopexyaftertheonsetofpuberty(age
14to29),theriskofazoospermiaoroligospermiawas84%(Grassoetal,1991),a
trendinkeepingwiththedatareportedpreviously(Okuyamaetal,1989).Puriand
ODonnell(1988)studied142menwhounderwentunilateral(119men)orbilateral
(23men)orchidopexyatage7yearsorolderandreportednormalspermdensityin
84%and50%ofcases,respectively.

MeanS/Tcountsobtainedatbiopsyshowcorrelationwithlongtermfertility
potentialasmeasuredbymeansemenanalysisparametersinseriesbutmaynotbe
predictiveinindividuals(Engeleretal,2000;Cortesetal,2003a;Rusnacketal,
2003).OtherdatasuggestabettercorrelationbetweenthenumberofAd
spermatogoniaincryptorchidtestesandspermcountinadulthoodafterprevious
unilateralorbilateralorchidopexywithorwithoutpriorhormonaltherapy
(Hadziselimovicetal,2007;HadziselimovicandHoecht,2008).Innonhormonally
treatedcases,totalspermcountwasnormal(>40million/ejaculate)in84%of25men
withAdspermatogoniapresentinbothtesticularbiopsyspecimens,whereasitwas
subnormalinall18men(10ofthe19meninthisserieshadahistoryofbilateral
cryptorchidism)inwhombiopsieswerenegativeforAdspermatogonia.Totalgerm
cellcountswerereportedlynotpredictiveofspermconcentrationinthisseries
(HadziselimovicandHoecht,2008).BothAdspermatogoniacountandgermcell
absenceappearpromisingasmeasuresoffertilityprognosis,buttheir
predictivevaluehasnotbeenconfirmedinadditionalstudiestodate.Further
prospectivestudiesoftheseparametersareindicated.

Outcomestudiesofsemenanalysisinmenwithahistoryofcryptorchidismare
believedtoprovideusefulinformationthatpredictsfertilitypotential.However,a
largepopulationstudyoffertileandinfertilemenwithfertilepartnerssuggeststhat
thereislargeoverlapbetweensemenparametersinmenwithandwithoutproven
paternity(Guzicketal,2001).Inthisstudy,theauthorsestablishedlowerinfertile
thresholdlevelsfordensity(13.5106/mL),motility(>35%),andnormal
morphology(>9%)thanhadbeenestablishedbyWorldHealthAssociationcriteria.
About3%offertilemeninthisserieshadaspermdensityoflessthan10106/mL,
andmeasurementsbetween13.4and48106/mLwereconsideredindeterminate.
Repeatedsemenanalyses,rarelyobtainedinstudiesofformerlycryptorchidmen,are
neededtoprovidereliabledatainnormalmen(Oshioetal,2004).

CHAPTER 132 Abnormalities of the Testis and Scrotum and Their


Surgical Management 3573

Consequently,determinationofpaternitystatusisanalternativemeasureoffertility
thatshouldbeconsideredwhendeterminingprognosis.Limitationsofthisapproach
includepaternaldiscrepancyandvariabilityinthetiminganddegreeofinterestin
attemptsatpaternity.Althoughofconcernandnotethicallyretrievable,arecent
review(Bellisetal,2005)foundthatthemedianlevelofpaternaldiscrepancyin17
studiesofunselectedpopulationsinEuropeandtheAmericasisonly3.7%(interquar
tilerange,2%to9.6%).

Tworetrospectivecohortstudiesofmenwithpreviouscryptorchidismassessed
paternityin145(Gilhoolyetal,1984)and40(Cendronetal,1989)cases.Together,
thesestudiesidentifiedsuccessfulpaternityin100of123(81%)menwithahistoryof
unilateraland19of54(35%)ofmenwithahistoryofbilateralcryptorchidism.Lee
andcolleaguespublishedaseriesofwelldesignedcasecontrolstudiesoffertilityin
cryptorchidism(Leeetal,1996,1997,2000;Coughlinetal,1999;LeeandCough
lin,2001,2002b;Bellis,2005;Lee,2005)Questionnaire,hormone,semenanalysis,
andpaternitydatawereanalyzedforalargecohortofmenwhounderwent
orchidopexybetween1955and1975andacontrolgroupofsimilaragewhowere
matchedfortimingofunrelatedsurgery.Forallmarriedorcohabitatingmen,32of88
(36%)formerbilateral,322of609(53%)formerunilateral,and413of708(58%)
controlshadfatheredchildren.Ofthoseattemptingpaternity,32of49(65%)
formerbilateral,322of359(90%)formerunilateral,and413of443(93%)
controlsweresuccessful.Therewerenosignificantdifferencesbetweenthe
unilateralandcontrolgroupsandnodifferencesbetweengroupsinthefrequencyof
attemptedpaternityorinotherlifestylefactorsthatmayadverselyaffectfertility.The
frequencyofsuccessfulpaternitydidnotdifferbetweenmenwithprevious
unilateralcryptorchidismwhohadundergoneorchiectomyandthecontrolgroup.
RelativeriskforinfertilitywasincreasedafterhCGtreatment(RR4.7,P=.002)but
notwithhighertesticularpositionorageatorchidopexy.Spermdensitywaslessthan
orequalto13106/mLinall8patientswithbilateralcryptorchidismthatwere
studied;however,3ofthesemenhadfatheredchildren(LeeandCoughlin,2001).In
contrasttopreviousstudies,83%ofmenintheunilateralgrouphadnormalsperm
densityandspermmotilityandmorphologydidnotdifferfromcontrolvalues.
Althoughhormonelevelsalonedidnotcorrelatedirectlywithfertility,abnormal
levelsofseruminhibinB,FSH,and/orspermdensityprovidedcumulativeriskof
decreasedfertility.However,theauthorsconcludethatpredictionofinfertilityis
difficultintheabsenceofazoospermiaorsevereoligospermia.Theseinvestigators
alsofounddifferencesinbasalandstimulatedLHandinserumtestosteronelevels
whencomparingfertileandinfertileorsubfertileformerlycryptorchidmenand
suggestthatglobaltesticulardysfunctionoccursincryptorchidmales.Moreover,
thereissomeevidencefromthesestudiesofarelationshipbetweenimproved
testosterone,inhibinB,andFSHlevelsinmaleswhounderwentearlierorchidopexy
(Coughlinetal,1999;LeeandCoughlin,2002a).

Limitedevidencesuggeststhatdefectivespermatogenesismayexistinsome
adultpatientswithpersistentlyretractiletestisorwithmilderformsofacquired
cryptorchidismwithorwithoutapparentspontaneousdescentofthetestisat
puberty.Insmall,retrospectiveoutcomestudies,PuriandNixon(1977)reported
74%paternityandnormaltesticularvolumeinaseriesof43adultswithuntreated
retractiletestesinchildhood.Conversely,NistalandPaniagua(1984)andCaroppo
andcolleaguesidentified23and34males,respectively,frominfertilityclinicdata
andidentifiedpoorsemenparametersinthe

majorityofcases,butthedurationandseverityofretractilitywaspoorlydocumented
intheseseries.Thesestudiesdonotprovidesufficientevidenceforinfertilityriskin
uncomplicatedcasesofretractiletestis.Twoseriesreportedvaryingdegreesof
abnormalgermorSertolicelldevelopmentinretractiletestesofboyswhounderwent
electiveorchidopexyascomparedwithboyswithdescendedtestes;differenceswere
qualitativelysimilartofindingsincryptorchidtestes(Hadziselimovicetal,1987a;
Cintietal,1993).However,smallsamplesizepersubgrouplimitsinterpretationof
thesedata.Hanandassociates(1999)compared61retractilewith83cryptorchid
testisbiopsiesandnotedsimilartrendsbutdidnotincludeacontrolgroup.Ina
retrospectiveseriesof45adultswithspontaneousdescentofbilaterallyundescended
testesafterage10byhistory,BrenholmRasmussenandcoworkers(1988)observed
testicularvolumesoflessthan15mLin62%andspermcountsbelow20million/mL
in44%ofpatients.Large,prospectivestudiesofpersistentlyretractiletestesandcases
ofacquiredcryptorchidismareneededtobetterdefinediagnosisandprognosisin
thesegroupsofpatients.

Insummary,availabledataprovidestrongevidencethatfertilitypotentialis
compromisedinmenwithahistoryofbilateralcryptorchidismbutthe
frequencyofabnormalhormonalandspermparametersinunilateralcasesis
higherthantherelativeriskofinfertilityasmeasuredbypaternitydata.
Unfortunately,thenumberofformerlybilaterallycryptorchidmenwhohavebeen
comprehensivelystudiedislimited.Althoughdatasuggestsomeassociation
betweenageatsurgeryorhormonetherapyandriskofinfertility,furtherstudiesare
neededtoelucidatetherelationshipsbetweenthesefactors.

Risk of Malignancy
Theincreasedriskoftesticulargermcelltumor(TGCT)inmaleswithahistoryof
cryptorchidismhasbeenknownformanyyears.Bothseminomaand
nonseminomatousgermcelltumors(NSGCT)developfromcarcinomainsitu(CIS)
ofthetestis,alsocalledintratubulargermcellneoplasia,unclassified(ITGCNU),and
arebelievedtobedevelopmentalinorigin(RajpertdeMeytsandHoeiHansen,
2007).ThehypothesisthatpersistentgonocytesaretheprecursorsoftesticularCIS
hasexistedforsometime,andrecentgeneexpressiondataindeedsupportacommon
originforthetwocelltypes(Sonneetal,2009).Thehistologicdatasuggestingthat
gonocytesfailtotransformnormallyincryptorchidtestesmaycoincidewitheventual
transformationofthesepersistentcellsintoCISandTGCT.However,todateno
evidencedirectlylinksgermcellmaldevelopmentinindividualpatientstoeventual
malignanttransformation.Usingplacentalikealkalinephosphatase(PLAP)asa
markerofITGCNU,Engelerandassociates(2000)identifiedPLAPpositivecellsin
5%of440patients,most(82%)youngerthan3yearsofage,whohadundergone
testicularbiopsyandorchidopexyseveralyearsearlier.Whereasupto50%ofadults
withITGCNUareexpectedtodevelopTGCTovertime,notumorwasdetectedinthe
15of22affectedindividualsthattheauthorswereabletoevaluateamedianof21
yearslater.However,PLAPimmunopositivitymaybeanormalfindingininfants
duringthefirstyearoflife(Jorgensenetal,1993).Similarly,Cortesandcoworkers
(2003b)identifiedmultinucleatedspermatogoniain13(8%)of163consecutive
patientsundergoingbiopsyatthetimeoforchidopexy.Thisfindingoccurredin
youngerboysandwasassociatedwithanS/Tcountthatwasusuallynormaland
higherthanthemeanforthemajorityofcases.Althoughnotidentifiedinnormal
boys,therelevanceofthisfindingtotumorriskremainscompletelyunknown.

3574 SECTION XVII Pediatric Urology

Recently,intheirexhaustiveanalysisoftheliteratureWoodandElder(2009)
clarifiedthenatureofincreasedTGCTriskinthepreviouslycryptorchidand
contralateraldescendedtestis.Menwithahistoryofcryptorchidismcompriseabout
10%ofthosepresentingwithTGCT.Therelativeriskofmalignanttrans
formationinanundescendedtestisis2.5to8overalland2to3inboys
undergoingprepubertalorchidopexy,whichislowerthanhistoricalestimates.
ThisincidencecorrelateswiththereportedriskofCISof2%to3%inpreviously
cryptorchidmen(Giwercmanetal,1989);amuchlowerrisk(0%to0.4%)was
reportedinchildrenwithnonsyndromiccryptorchidism(Cortesetal,2001;
Husmann,2005).TGCTmayoccurinthecontralateraldescendedtestisofmenwitha
historyofunilateralcryptorchidism,butWoodandElder(2009)concludethatthe
relativeriskofonly1to2indicatesalevelcomparabletothegeneralpopulationthat
isnotrelatedtocryptorchidismperse.However,arecentmetaanalysisshowsthat
therelativeriskof6.3(95%CI,4.30to9.31)intheipsilateraland1.7(95%CI,1.01
to2.98)inthecontralateraltestisissignificantcomparedwithcontrols(Akreetal,
2009).Inanothermetaanalysis,Walshandassociates(2007)determinedthatthe
relativeriskoftesticularcancerwas5.8(95%CI,1.8to19.3)inmenwhounderwent
orchidopexyafterage10to11ascomparedwiththoseundergoingearliercor
rection.However,recentpopulationbaseddataareconflicting,showingtwicetherisk
ofmalignancyinorchidopexycasesoperatedatorafterage13inoneseries
(Petterssonetal,2007)butnoagedependentdifferencesinanother(Myrupetal,
2007),possiblyrelatedtoascertainmentbiasinthelatterseries.Furtherstudiesare
neededtoclarifytherelationshipbetweenageatorchidopexyandriskoftesticular
cancer.Reviewoftumorpathologyintreatedversusuntreatedcryptorchidismshows
thatseminomaisassociatedwithpersistentlycryptorchidtestes(74%)and
nonseminomaispresentinthemajorityofscrotaltestes(63%)(WoodandElder,
2009).

CertainsubgroupswithundescendedtestisareatincreasedriskforTGCT,including
thosewithchromosomaldefectsandothergenitalanomalies(Cortesetal,2001;
Husmann,2005).Husmann(2005)recommendsthatbiopsybeperformedinthese
individualsandinboysolderthan12undergoingorchidopexy,althoughtheage
cutoffandusefulnessofbiopsyduringpubertalorchidopexyhavenotbeenclearly
defined.Orchiectomyshouldbeconsideredthepreferredtreatmentofcryptorchid
testesfrompubertytotheageof50(WoodandElder,2009).Swerdlowand
associates(1997)reportedinaretrospectivecohortstudythattesticularbiopsyat
orchidopexywasassociatedwitharelativeriskof6.7forfutureTGCTcomparedwith
orchidopexywithoutbiopsy,buttheindicationsforbiopsyinthisserieswerenot
clearlyknown.AsubsequentreportfromalargeScandinaviancohortshowedthat
universalbiopsydidnotappeartoincreasetheriskforTGCTbeyondwhatis
expectedforpreviouslycryptorchidmen(Molleretal,1998).

Testicularmicrolithiasis,characterizedbymultiplespectralcalcificationswithinthe
testicularparenchyma(Fig.1325),ismorefrequentlypresentinmenwithITGCNU
andgermcelltumorsbutisalsopresentin5%to10%ofthenormalpopulationandin
asimilarproportionofpreviouslycryptorchidmen(Pateletal,2005;vanCasterenet
al,2009).AlthoughconcernexiststhattheriskforTGCTmaybehigherwhen
cryptorchidismcoexistswithmicrolithiasisinindividualpatients,the
appropriatefollowupstrategyremainsundefined.Evenlesswelldefinedisthe
significanceofmicrolithiasisingeneral,whichisnotclearlyshowntobean
independentriskfactorforTGCT.Arecentpopulationbasedanalysisofthe
prevalenceof

Testicular microlithiasis found incidentally in a 13-year-old boy with early


puberty (Tanner stage II) and a clinical appendix testis torsion. The
remainder of the study showed normal Doppler flow to testis, increased
epididymal flow, and no visible appendage. The examination findings
normalized within 1 week.

microlithiasisinprimarilywhiteboysshowsthattheprevalenceis4.2%andincreases
withage(Goedeetal,2009).Inabouthalfofthesecases,thedegreeofmicrolithiasis
waslimited,definedaslessthanfivelesionspertestis,andnotconsideredclinically
significant.Slaughenhouptandassociates(2009)reportedacaseandreviewed
pediatricreportsofTGCToryolksactumorinboyswithmicrolithiasisbutwithout
otherriskfactors;theyrecommendperiodicexaminationandultrasonographyinall
cases.Incontrast,biopsiesareperformedinadultswithmicrolithiasisandoneother
riskfactorfortumor,suchascryptorchidism,butifthebiopsyisnegativethenno
specificfollowupotherthanselfexaminationisrecommended(vanCasterenetal,
2009).Testicularselfexamination,whichrequireseducationandcounselingof
thepatient,remainsamainstayoftesticularcancerscreening.Adescriptionexists
(http://www.cwpeds.com/pdfs/adolescents/TesticularExam.pdf),anditshouldbe
taughttoallpatientswithahistoryofcryptorchidismaftertheyreachpuberty,
preferablybythepediatricurologistoroperatingsurgeoninadditiontotheprimary
carepractitioner.

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