Currarino Syndroma, initially misdiagnosed and treated like megacolon congenitum

• Authors: Ivona Djordjevic , Danica Jovanovic , Blagoje Grujic
• Languages of publication: EN

Abstracts

EN

Currarino syndrome was first described as a triad by Guido Currarino, an American radiologist in 1981. It is an autosomal dominant hereditary disease known by the triad of anorectal stenosis, anterior sacral defect, and a presacral mass that is most often an anterior sacral meningocele.We represent a 3 year boy with refractory constipation from birth, which had been wrongly diagnosed and treated as Hirschsprung’s disease since early childhood. The patient underwent urgent colostomy because of water intoxication due to bowel irrigation, and following investigations (CT, MRI) revealed anorectal anomaly and presacral mass compatible with Curarrino’s syndrome.Definitive operation was done with perineal approach, posterior sagittal anorectoplasty, with interval colostomy closure 2 months after perineal operation. An uncomplicated postoperative recovery ensued. The patient’s bowel habits successfully returned to normal soon after the operation. A multidisciplinary diagnostic and therapeutic protocol is presented. Main points are rectal examination, sacrum x-Ray, molecular genetic diagnosis, radiologic evaluation of every member of Currarino syndrome families, CT scan, magnetic resonance (MR) evaluation of patient spine and pelvis and suction rectal biopsies. If the mass is a meningocele, colostomy and neurosurgical exploration should precede anoplasty due to the risk of meningitis.

Keywords

EN

Currarino’s triad; Constipation; Anterior meningocela

• Publisher: De Gruyter Open
• Journal: Open Medicine
• Year: 2011
• Volume: 6
• Issue: 6
• Pages: 723-726

Dates

published

1 - 12 - 2011

online

8 - 10 - 2011

Contributors

author

Ivona Djordjevic

• Clinic for Pediatric Surgery and Orthopedics, 18000, Nis, Serbia and Montenegro

author

Danica Jovanovic

• Institute for mother and child “Vukan Cupic”, 11000, New Belgrade, Serbia and Montenegro

author

Blagoje Grujic

• Institute for mother and child “Vukan Cupic”, 11000, New Belgrade, Serbia and Montenegro

References

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Identifiers

DOI

10.2478/s11536-011-0091-4