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 presacral mass

hemisacrum caudal regression syndrome

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

Presacral mass anterior meningocele teratoma dermoid cyst rectal duplication other

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

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

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

Case report 2 bronchoscopy (2003)- stenosis left side change in walking ("limping")- 2004 orthopedic & neurologic examination, x-ray, CT,MR

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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