Currarino syndrome in an adult presenting with a presacral abscess: a case report

Abstract

Introduction: Currarino syndrome (Currarino triad) was described in 1981 as a triad syndrome with a common embryogenesis in infants and with three characteristics: anorectal stenosis, a defect in the sacral bone, and a presacral mass. We describe here an unusual case of Currarino syndrome in an adult presenting with a presacral abscess but no meningitis.

Case presentation: A 32-year-old Japanese man presented with fever, arthralgia and buttock pain. A digital rectal examination showed mild rectal stenosis with local warmth and tenderness in the posterior wall of his rectum. Computed tomography showed a scimitar-shaped deformity of his sacrum and an 8cm presacral mass, which continued to a pedicle of his deformed sacrum. This was diagnosed as Currarino syndrome with a presacral abscess. The abscess was drained by a perianal approach with our patient treated with antibiotics. His symptoms soon disappeared. After three months, an excision was performed through a posterior sagittal approach. His postoperative course was uneventful and he was discharged 10 days after surgery. A histopathological examination revealed an infected epidermoid cyst. He has been free from recurrence as of four years and six months after surgery.

Conclusions: We report a case of Currarino syndrome in an adult who presented with a presacral abscess but no meningitis. Abscess drainage followed by radical surgery resulted in a successful outcome.

Figure 1

Computed tomography imaging findings. (A) A scimitar-shaped deformity of the sacrum (black arrow). (B) An 8cm presacral mass (white arrow) containing air displaced the rectum (white arrow heads) ventrally. (C) The mass appeared to continue to the spinal canal through the anterior wall of the sacrum (asterisk).

Figure 2

Sigmoidoscopy findings. The posterior wall of the rectum was compressed extramurally (asterisk).

Figure 3

Magnetic resonance imaging findings. (A) Axial T1-weighted image showing a low density of the presacral mass (white arrow). (B) A tethered cord could not be revealed on a sagittal T2-weighted image. (C) The mass was not enhanced on contrast-enhanced magnetic resonance imaging. The cystic wall was thick, surrounding a fuzzy tissue.

Figure 4

Myelography and postmyelography computed tomography findings. (A) Sagittal myelography showed no apparent communication between the presacral mass and the thecal sac. (B, C) Coronal and sagittal postmyelography computed tomography demonstrated that the terminus of the thecal sac formed some processes. The drain (black (A) and white (B, C) triangles) was placed via a perianal insertion.

Figure 5

Intraoperative phase. Exploration through a posterior sagittal approach demonstrated a silvery white epidermoid tumor (white arrow) occupying the presacral space. The tumor was poorly circumscribed and firmly adhered to surrounding tissues containing bilateral S3 sacral nerve roots (white arrow heads) and dura (asterisk).