The Currarino syndrome is a rare triad that is a combination of a presacral mass, a congenital sacral bony abnormality and an
anorectal malformation. We present 4 children with complete Currarino triad who were diagnosed using MRI. Our aim was to report the neurosurgical management of Currarino syndrome in children. All of the patients had chronic
constipation and
pain in the lumbosacral region. In the plain radiograph, 3 patients had a sacral scimitar-shaped bony abnormality, and 1 patient had total
sacral agenesis. There was a narrow anal canal or narrow ventrally displaced anus in all patients. Their
anorectal malformations were characterized as anal
stenoses (4 patients), associated with
Hirschsprung's disease in 2 cases. In 3 patients, MRI showed
tethered cord syndrome in addition to the presacral mass. There was
hydrocephalus in 1 patient. Anal
stenosis was treated by anal dilatation. In 2 patients, rectal biopsy and temporary
colostomy (2 patients) had been performed previously due to
Hirschsprung's disease. We performed a posterior procedure via lumbar and sacral partial
laminectomy-
laminoplasty and transdural
ligation of the neck of the
meningocele for anterior sacral
meningoceles, or alternatively,
tumor excision for other types of presacral lesions. Histopathologically, 3 were cases of anterior sacral
meningoceles and 1 was a
teratoma. One of them also had a spinal
abscess. He required reoperation (twice) and appeared at the time to have improved with medical
therapy. All patients improved and stabilized. There were no additional neurological deficits and no recurrence of the presacral mass over the follow-up period (6 years, on average). The family pedigree did not reveal any familial transmission pattern. In cases of Currarino triad, MRI can allow the characterization of the presacral masses. If it is an anterior sacral
meningocele or a solid
tumor without severe
anorectal malformation, it can be managed with posterior lumbar and sacral procedures. Such approaches are performed easily by transdural
ligation of the neck of the anterior sacral
meningocele or through
tumor excision.