A late-recognized Currarino syndrome in an adult revealed by an anal fistula.
BACKGROUND
Currarino syndrome (CS) is characterized by the triad of anorectal malformations, sacral bone defects, and presacral mass in which an autosomal dominant inheritance has been described. The surgical community has a little no knowledge of CS in adults, apart from, perhaps, a small number of paediatric surgeons. Therefore, we sought to describe this unusual cause of anal fistula.


METHODS
A 55-year-old man was referred with an anal fistula. The patient was scheduled for drainage of multiple collections and an anal fistulectomy. Cytological results were confirmed that the cyst was dermoid (and non abscess). One month after surgery, the patient informs us of his CS. MRI was performed and it revealed an anterior sacral mass. It was to decide to realize an exeresis of this mass by coelioscopy. The patient experienced severely constipated and urinary retention. After therapy by Peristeen anal irrigation and self Intermittent catheterization (six times daily), there was a good improvement in symptoms.


CONCLUSIONS
This is an extremely rare case of CS revealed in an adult. MRI is a sensitive non-invasive diagnostic tool, and could be performed on any patient with long-standing anal fistula.


CONCLUSIONS
We recommend an early and multidisciplinary approach of CS is suspected in a patient. The surgeon must always be alert to the possibility of pelvic nerves injury during an exeresis of a retrorectal tumour fistulized.
Department of Digestive Surgery, CHU Hospital, Jean Monnet University, Saint Etienne, France. Electronic address: Radwankassir42@hotmail.Fr.
Radwan
R
Kassir
Department of Digestive Surgery, clinique chirurgicale mutualiste, Saint Etienne, France.
David
D
Kaczmarek
Anal fistula; Currarino syndrome; Genetic disease; Presacral mass; Sacrococcygeal agenesis; Surgery