CompletePrimaryRepairofExstrophy:Pitfallsand

PracticalitiesDouglasA.Canning,MDJosephG.Borer,MDDanaA.Weiss,MD

TheHendren Project,February28,2018

SessionStructure

• Topic1Discussion:• GoalsofCompletePrimaryRepairofExstrophy• PitfallsofCPRE

• Casediscussion:• Case1:nearmiss– glansischemia• Case2:Protocolchangetoaddressthenearmiss

• Topic2Discussion:• Utility/Futilityofureteralreimplant duringCPRE

• CaseDiscussion:• Case1:Cephalo-trigonalreimplants attimeofsurgery• Case2:Delayedcephalo-trigonalreimplants

Hendren ProjectWebinarGoals

• Audienceparticipation!• Makessessionmoredynamic,interesting,andvaluable• ParticipateintheQuestion polls--allanswersareanonymous!

• PleaseusetheCOMMENTS&QUESTIONSbox(locatedontherightsideofthescreen)toaskasmanyquestionsandtoofferasmanyopinionsaspossible!

Background:TheDelayedCompleteRepair

• CPREdescribedin1998byMitchell• Closureofbladder• Reconstructionofbladderneck• Urethroplasty• Epispadiasrepair

• Goal:• Createappropriatebladderoutletresistance• Allowfornormalbladdercyclingfromearlyage• Allowforoptimalbladdergrowthanddevelopment

Background– ConcernswithCPRE

• Immediateconcerns:• Dehiscence• PenileIschemiaà Glans/CorporalInjury• Balancebetweentootightandtooloose(bladderneck)

• Laterconcerns:• Highvolumereflux• Persistentincontinence

Question1:

• Howdoyoucloseaprimarybladderexstrophy?A. CPREB. ModernstagedrepairC. KellyprocedureD. Primarydiversion(MainzII)

Question2:

• WhatisyourbiggestconcernduringaCPRE?A. BladderdehiscenceB. GlansischemiaC. UrethralfistulaD. Penopubic fistulaE. Lackofbladdergrowthovertimeduetopooroutlet

resistance

Topic1:Penileischemia

EarlyExperiences:BigConcerns• Twodehiscences• bothinboyswithoutosteotomy

• Twoglans/corporalinjuries• onefollowingseparation• onein6montholdwithepispadiaswhohadthesymphasis splitaspartoftherepairwithoutosteotomy

• Takeaways:• CPREcanbereallydangerous• Osteotomyiscriticaltosafeclosure

Glans/corporalinjury

Husman,Gearhart:J.Urol.172(4Pt2):1696-700;discussion1700-1,2004Oct.

Newbornwithepispadias:• Primaryclosurewithepispadiasrepair• Noosteotomy• Bladderneckreconstruction• Completedissassembly withanatomicalclosure

• Whatdoyoudotopreventglansandcorporalischemia?A. Don’tclosepubicsymphysisB. PosteriorosteotomyC. AnteriorosteotomyD. LeaveagapinpubisifthereissignofischemiaE. Sprinklepapaverine onpenisduringclosure

Question3:

“BeNo”Event

• Howtoavoidthe”BeNo”Event• KeepGlansintact• Temperedclosureofpubis• Inciseglanstowatchforbleeding• BEAWAREofthepotential,andopenthepubisiftheglansappearsdusky

ACautionaryTale

Topic2:CPREwithBilateralUreteral

Reimplant

Theconcurrentre-implantation

• Cephalotrigonal technique• DescribedbyCanningandJeffs in1993

• DoneattimeofCPRE• DescribedbyBragaandPippi Sallein2010

ConcurrentCPRE-BURoutcomes

• Doyoudoaureteralreimplantation atthetimeofCPRE?A. YesB. NoC. DependsonbladdersizeD. Dependsonbladderpolyps

Question4:

There-implantquestion

PRO• Allowsbladdertocycleefficiently• Avoidsprogressiveureteraldilationduringcycling• MaypreventUTI’s

Con• Smallbladderplatemaynotallowforlongtunnel• Persistentrefluxmayoccur• Addstimetoalreadylongprocedure

BURatCPRE– caseforandagainstinBoyandGirl

Thankyouforyourparticipation!