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

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

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