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Currarino Syndrome- Case Report University of Pecs/Hungary, Faculty of Medicine, Department of Paediatrics, Surgical Unit Attila M Vastyan, Andrew B Pinter,

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Presentation on theme: "Currarino Syndrome- Case Report University of Pecs/Hungary, Faculty of Medicine, Department of Paediatrics, Surgical Unit Attila M Vastyan, Andrew B Pinter,"— Presentation transcript:

1 Currarino Syndrome- Case Report University of Pecs/Hungary, Faculty of Medicine, Department of Paediatrics, Surgical Unit Attila M Vastyan, Andrew B Pinter, Zsolt Oberritter, Zsolt Juhasz, Peter Vajda

2 1981- G.Currarino - Currarino triad hemisacrum anorectal malformation presacral mass

3 hemisacrum caudal regression syndrome

4 Anorectal malformation rectourethral fistula rectovestibular fistula rectocloacal fistula rectoperineal fistula (anorectal stenosis)

5 Presacral mass anterior meningocele teratoma dermoid cyst rectal duplication other

6 Associated anomalies neural tube defects (tethered cord) cardiac anomalies urologic involvement gynecologic malformations Hirschsprung, dysganglionosis Currarino syndrome

7 Genetics autosomal dominant inheritance mutations or deletions in the coding sequence of HLXB9 gene phenotype variability

8 Case report 1 D.B (03/10/2001),girl constipation since birth, biopsy - no Hirschsprung, but anal stenosis Y-V plasty for anal stenosis at 9 months, no significant improvement laxatives, suppositories and enemas

9 Case report 2 bronchoscopy (2003)- stenosis left side change in walking ("limping") orthopedic & neurologic examination, x-ray, CT,MR

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15 Case report 3 cardiology- no abnormality detected MCU - "lazy bladder", no VUR AFP, beta HCG -norm. anal and uromanometry rectal digital examination Operation

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19 Thank you for your attention ! Visit Pecs, The Cultural Capital of Europe 2010 !


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