UnrepairedImperforateAnusina9-Year-OldChildwithDownSyndrome

SaniYamout MDPediatricSurgeonKaiserPermanenteOakland,CA

CaseHistory

• 9-year-oldborninPhilippines(11/28/2008)• Trisomy21withAVcanal• Colostomyatbirth(doublebarrel)• Abd surgeryviaRUQincisionat4daysoflife(?Duodenalatresia)

• MovedtoUSA2014• RepairofAVcanalin2015(LCPHStanford,CA)– ResidualpulmonaryHTN

Exam

• RUQincision• Doublebarrelcolostomy• Wellformedanaldimplewithnoidentifiablefistulainperineum

Workup

• Echo• MRIspine:– borderlinelowconus(L2-L3level)

– Nosegmentalabnormalities

• U/Skidneynormal• Colostogram:

Workup

• Echo• MRIspine:– borderlinelowconus(L2-L3level)

– Nosegmentalabnormalities

• U/Skidneynormal• Colostogram:

Workup

• Echo:• MRIspine:– borderlinelowconus(L2-L3level)

– Nosegmentalabnormalities

• U/Skidneynormal• Colostogram:

Challenges

• ChildwithDownSyndromewhowillneedtolearntobe“pottytrained”at9yearsofage

• Risk/Benefitdiscussion– HigheroperativeriskduetocardiacconditionandpulmonaryHTN

– Unclearpotentialforcontinence

TreatmentOptions

• Donothing– Colostomyfunctioningwell– Colostomysimpletotakecare

• PSARPandrepairoftrueimperforateanus– Higherriskpatient– Whatispotentialforcontinence?– Ifincontinentandneedstobemadeartificiallyclean,whatisbetterhavingacolostomyorneedingante-gradeenemastobeadministeredbyparentspossiblyforlife

Questions

• Wouldanyonestronglyrecommenddoingnothing?

• Wouldanyonetapertherectumatthetimeofrepair