Abstract
We report a case of a 14-month-old girl with undiagnosed Currarino triad presenting acutely with meningitis caused by enteric commensals. Head CT demonstrated a large pneumocephalus. A fistulous neurenteric tract through a presacral mass was present on spine MRI and abdominal CT. The patient had a history of constipation for the last three months. However, an underlying diagnosis of Currarino triad had not been suspected. In retrospect, a sickle-shaped sacral anomaly was present on a previous abdominal radiograph. The patient succumbed to complications of meningitis. The purpose of the case report is to highlight the potentially fatal complication of Currarino triad and sensitize radiologists to look actively for sacral anomalies on abdominal radiographs, especially of children with chronic constipation.
Highlights
Currarino syndrome is a rare congenital complex disorder characterized by triad of partial sacral agenesis, anorectal malformation, and presacral mass [1]
The mutations identified in most familial and some sporadic cases are in the homeobox gene, HLXB9, called MNX1 gene at chromosome 7q36 [4, 5]
We emphasize the importance of careful evaluation of lower spine on abdominal radiographs of children with chronic constipation or urogenital complaints
Summary
Currarino syndrome is a rare congenital complex disorder characterized by triad of partial sacral agenesis, anorectal malformation, and presacral mass [1]. The sacral abnormality is most often identified on an abdominal or pelvic radiograph. In older children, these radiographs may have been obtained for workup of chronic constipation or urogenital problems which are the most common symptoms of this syndrome [6,7,8]. It is important to identify this syndrome early to prevent future complications like meningitis, which occurs in 7–11% of cases and has a high mortality of 50% [6, 7]
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