PMC4997085_01
Abdominal radiograph for abdominal distention evaluation. It shows significantly dilated air and fecal filled bowel loops with fecaliths. There is a sickle-shaped sacral defect (arrow), not reported initially.
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An abdominal radiograph was interpreted as stool filled significantly dilated descending and sigmoid colon (Figure 1). In retrospect, the sacral defect was seen on the abdominal radiograph (Figure 1).
Head CT on day 2 for evaluation of meningitis. (a) Axial head CT shows subdural effusion over the right cerebellar hemisphere abutting the sigmoid sinus (arrowhead) and large air in the prepontine cistern. (b) Sagittal reformat demonstrates large pneumocephalus over the left frontal lobe and in the prepontine cistern (arrows). There is mild lateral ventricle dilatation (arrowhead).
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CT head showed nontraumatic large pneumocephalus, too disproportionate to be explained by lumbar puncture (Figure 2).
Abdominal CT on day 4 for evaluation of progressive abdominal distention. (a) Axial CT slice through the lower pelvis shows hypoplasia of the sacrum and an indeterminate presacral mass extending into the spinal canal (arrow). The mass contains heterogeneous material and air and the contents have a similar appearance to the fecal material in the bowel. (b) Sagittal abdominal CT reformat again shows the presacral mass in continuity with the sacral canal having a few fecaliths. Notice few fecaliths (arrow) and air (arrowhead) in the spinal canal, suggesting communication with the anal canal.
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It demonstrated a presacral mass, sickle-shaped sacral defect, coccyx agenesis, and a fistulous tract between the sacral canal and the anal canal (Figure 3). The contents of the mass had similar appearance to the fecal material in distended bowel loops and some fecaliths were also seen in the spinal canal suggesting fistulous connection (Figure 3).
Spine MRI on day 5 to further characterize the presacral mass. (a) Sagittal T2 weighted MR image of the lower spine shows complex presacral mass with a small separate hyperintense cyst communicating with the thecal sac, which could be meningocele (thin arrow). Spinal canal shows epidural fluid collection (arrow) with multiple air foci (arrowheads). There is low-lying conus and a tethered cord, reported in 70% of Currarino syndrome cases (curved arrow). (b) Sagittal T1 weighted MR image of the spine shows the communication of the larger presacral mass with the anal canal (arrow). The contents of the mass have similar intensity to the contents of the dilated sigmoid colon (asterix).
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The distal thecal sac was in communication with the heterogenous presacral mass containing a hyperintense cyst (Figure 4). Multifocal tiny artifacts from air were seen in the thecal sac and epidural collection suggestive of the connection of the mass with the spinal canal and explaining the source of large pneumocephalus and central nervous system infection (Figure 4).
Brain MRI on day 5 for meningitis evaluation. Axial diffusion weighted MR image of the brain shows restricted diffusion over the right frontal lobe and left medial occipital lobe and in the ventricles suggesting subdural empyemas and intraventricular abscesses (arrows).
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MRI brain demonstrated cerebritis, meningitis, subdural empyema, and intraventricular abscesses (Figure 5).
Abdominal ultrasound on day 7 to evaluate the neuroenteric fistula. (a) Gray scale sagittal image demonstrates a complex presacral mass with heterogeneous echogenicity and multiple echogenic foci representing air. There is a hypoechoic cystic structure within it which is in continuity with the sacral canal (arrow). (b) Subsequent transverse gray scale US image shows echogenic foci representing air in the small inferior hypoechoic cyst with the presacral mass (within calipers) suggesting continuous fistulous connection (arrow).
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Pelvic ultrasound to evaluate the fistula reaffirmed the neurenteric connection through the presacral mass (Figure 6).