Presentation
Several days of fever. Evaluate for infectious source.
Patient Data
There is a bony defect within the anterior aspect of the lower sacrum and coccyx. Arising from the bony defect are multiple loculated low density cystic structures. One of these cystic lesions appears to be contiguous with the thecal sac.
There is a cyst demonstrating direct extension into the spinal canal. Inferior to this, there is another distinct, mass with multiple cysts. The masses appear to displace the rectosigmoid colon anteriorly and slightly superiorly. Additionally, the anus is narrowed and is anteriorly displaced.
Case Discussion
This is a case of Currarino syndrome (sacrococcygeal anomaly, anorectal malformation, and a presacral soft tissue mass). The patient's father also has Currarino syndrome (autosomal dominant inheritance).
The patient was taken to the operating room for de-tethering of the spinal cord, excision of presacral mass, and closure of the anterior meningocele. During the operation, the coccyx was noted to be short, malformed, and deviated to the right. A fatty appearing mass anterior to the sacrum contained what appeared to be a large cyst containing keratin material. The mass abutted the meningeal coverings of the meningocele. The mass was resected and sent for pathological examination.
Grossly, the mass contained white tissue and fatty tissue. Histopathologic examination determined that the presacral mass contained benign squamous epithelium and respiratory epithelium-lined fibrocystic areas. Taken together, the specimens represented a mature cystic teratoma, consisting of a ruptured benign dermoid cyst, with areas of additional respiratory epithelium and mature central nervous system tissue. No immature elements were identified.
Co-authors:
Alannah McCann
Travis Bevington, MD
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