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

Abstract

IntroductionCurrarino 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 presentationA 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.ConclusionsWe 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.

Currarino syndrome with intramedullary spinal cord abscess related communication between the tethered cord and a presacral mass: A case report

“…Furthermore, meningitis is one of the most severe complications in CS and occurred in 7e11% of all cases, including familial cases in previous reports [9]. The communication between the spinal canal and the presacral tumor through the tethered cord is considered to be the cause of the patient's meningitis and spinal cord abscess [1,14,15]. The mortality due to meningitis in CS is surprisingly high at 56% [10].…”

“…It is hypothesized that meningitis is caused by a Tethered cord communicating between the presacral space and intramedullary space [1,14,15]. In severe cases, the cavity of the abscess extends to the intramedullary space and leads to a lifethreatening septic condition.…”

Currarino syndrome in an adult woman

“…Currarino syndrome is a rare autosomal dominant hereditary disorder [4]. Since the Currarino triad was first described by Currarino in 1981, approximately 300 cases have been reported [345]. However, the exact incidence of this condition is unknown owing to its multiple phenotypes and clinical presentations [5].…”

“…Since the Currarino triad was first described by Currarino in 1981, approximately 300 cases have been reported [345]. However, the exact incidence of this condition is unknown owing to its multiple phenotypes and clinical presentations [5]. Recent studies have reported an association between Currarino syndrome (primarily sacral agenesis) and the HLXB9 gene located on chromosome 7q36 [4].…”

Primary intradural sacral epidermoid in a nondysraphic spine: Case report and review of literature

“…[ 1 ] Their occurrence in the sacral region is unusual. [ 1 2 3 4 5 6 7 8 9 ] Most of the sacral SEs have been reported either in conjunction with anterior sacral meningoceles, as a component of Currarino syndrome (CS),[ 10 11 12 13 14 ] or as sequelae to lumbar punctures. [ 9 15 ] This report of a patient with a primary, nonsyndromal sacral SE in the absence of coexistent spinal dysraphism or preceding trauma is the third of its kind in indexed literature.…”