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Presacral mature cystic teratoma associated with Currarino syndrome in an adolescent with androgen insensitivity: illustrative case

Grant Koskay, Patrick Opperman, Frank M. Mezzacappa, Joseph Menousek, Megan K. Fuller, Linden Fornoff, and Daniel Surdell

BACKGROUND

Currarino syndrome is a rare disorder that classically presents with the triad of presacral mass, anorectal malformation, and spinal dysraphism. The presacral mass is typically benign, although malignant transformation is possible. Surgical treatment of the mass and exploration and repair of associated dysraphism are indicated for diagnosis and symptom relief. There are no previous reports of Currarino syndrome in an androgen-insensitive patient.

OBSERVATIONS

A 17-year-old female patient presented with lack of menarche. Physical examination and laboratory investigation identified complete androgen insensitivity. Imaging analysis revealed a presacral mass lesion, and the patient was taken to surgery for resection of the mass and spinal cord untethering. Intraoperative ultrasound revealed a fibrous stalk connecting the thecal sac to the presacral mass, which was disconnected without the need for intrathecal exploration. The presacral mass was then resected, and pathological analysis revealed a mature cystic teratoma. Postoperatively, the patient recovered without neurological or gastrointestinal sequelae.

LESSONS

Diagnosis of incomplete Currarino syndrome may be difficult but can be identified via work-up of other disorders, such as androgen insensitivity. Intraoperative ultrasound is useful for surgical decision making and may obviate the need for intrathecal exploration during repair of dysraphism in the setting of Currarino syndrome.