Article in Japanese

A case of Currarino syndrome with presacral abscess: Radiological assessment was beneficial for identifying route of infection and evaluation of meningitis

Shun SAKATANI¹⁾, Takahiro MATSUSHIMA¹⁾, Hiroshi HATAYA¹⁾

Currarino syndrome is a rare autosomal dominant disorder consisting of a presacral mass, anorectal malformation, and sacral agenesis. Infection of the perilesional tissue or thecal cavity, possibly via a neuroenteric fistula, can lead to a presacral abscess or meningitis. The Currarino syndrome phenotype varies, potentially delaying diagnosis. Early diagnosis and multidisciplinary assessment are vital in preventing life-threatening complications such as meningitis. A 7-month-old female presented with fever that had persisted for two months. On admission, no abnormality was found on physical examination, except for anal stenosis. Currarino syndrome was diagnosed with triad and family history. Roentgen fluoroscopy revealed a fistula running backward from the rectum to the presacral mass from which pus was drained later. The presacral abscess was identified as the cause of the fever. An MRI of the presacral mass showed the thecal sac attached by a chord, but no connection between the presacral mass and cerebrospinal fluid. The absence of meningitis was confirmed. A culture of pus identified methicillin-susceptible Staphylococcus aureus, Escherichia coli, and Bacteroides caccae. Surgery was performed after perianal drainage and antibiotic treatment. Macroscopic examination showed no fistula or residual abscess. The patient was discharged without event. It is crucial to know that a presacral abscess and meningitis can cause fever in patients with Currarino syndrome. Meningitis is one of the most serious complications, due to its high mortality rate. Therefore, multidisciplinary radiological assessment is recommended to determine the most suitable treatment.

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1) Department of General Pediatrics, Tokyo Metropolitan Children's Medical Center

Key words	Currarino syndrome, presacral abscess, meningitis, radiological assessment
Received	March 14, 2019
Accepted	July 18, 2019

31 (4)：341─346,2019