unrepaired imperforate anus in a 9-year-old child with ... · unrepaired imperforate anus in a...
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UnrepairedImperforateAnusina9-Year-OldChildwithDownSyndrome
SaniYamout MDPediatricSurgeonKaiserPermanenteOakland,CA
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CaseHistory
• 9-year-oldborninPhilippines(11/28/2008)• Trisomy21withAVcanal• Colostomyatbirth(doublebarrel)• Abd surgeryviaRUQincisionat4daysoflife(?Duodenalatresia)
• MovedtoUSA2014• RepairofAVcanalin2015(LCPHStanford,CA)– ResidualpulmonaryHTN
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Exam
• RUQincision• Doublebarrelcolostomy• Wellformedanaldimplewithnoidentifiablefistulainperineum
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Workup
• Echo• MRIspine:– borderlinelowconus(L2-L3level)
– Nosegmentalabnormalities
• U/Skidneynormal• Colostogram:
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Workup
• Echo• MRIspine:– borderlinelowconus(L2-L3level)
– Nosegmentalabnormalities
• U/Skidneynormal• Colostogram:
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Workup
• Echo:• MRIspine:– borderlinelowconus(L2-L3level)
– Nosegmentalabnormalities
• U/Skidneynormal• Colostogram:
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Challenges
• ChildwithDownSyndromewhowillneedtolearntobe“pottytrained”at9yearsofage
• Risk/Benefitdiscussion– HigheroperativeriskduetocardiacconditionandpulmonaryHTN
– Unclearpotentialforcontinence
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TreatmentOptions
• Donothing– Colostomyfunctioningwell– Colostomysimpletotakecare
• PSARPandrepairoftrueimperforateanus– Higherriskpatient– Whatispotentialforcontinence?– Ifincontinentandneedstobemadeartificiallyclean,whatisbetterhavingacolostomyorneedingante-gradeenemastobeadministeredbyparentspossiblyforlife
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Questions
• Wouldanyonestronglyrecommenddoingnothing?
• Wouldanyonetapertherectumatthetimeofrepair