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Currarino Syndrome- Case Report

Currarino Syndrome- Case Report. Attila M Vastyan, Andrew B Pinter, Zsolt Oberritter, Zsolt Juhasz, Peter Vajda. University of Pecs/Hungary, Faculty of Medicine , Department of Paediatrics , Surgical Unit. 1981- G.Currarino - Currarino triad. hemisacrum anorectal malformation

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Currarino Syndrome- Case Report

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

  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")- 2004 • orthopedic & neurologic examination, x-ray, CT,MR

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

  11. Thank you for your attention ! • Visit Pecs, The Cultural Capital of Europe 2010 !

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