Two infants presenting within a period of weeks with gram negative meningitis and diagnosed with Curranino syndrome are reported from Wake Forest University, Winston-Salem, NC, and Eastern Virginia Medical School, Norfolk, VA. Case 1, a 5-month-old girl, had bacteremia 2 months before developing signs of Escherichia coli and Proteus mirabilis meningitis. MRI spine showed a sickle-shaped sacrum, an anterior sacral meningocele and gastrointestinal fistulous connection. Case 2, a 2-month-old girl, presented with recurrent Pseudomonas aeruginosa meningitis. MRI showed a sickleshaped sacrum, anterior meningocele, and fistulous tract to the rectum. Both patients were treated with antibiotics and surgical repair. 
COMMENT. The above letter was written in response to a Mayo Clinic case report of a 20-day-old female infant who presented with meningitis refractory to antibiotics, and was found to have a scimitar sacrum, recto-thecal fistula, anterior sacral meningocele, tethered spinal cord, anal stenosis, and neurogenic bladder. Sequencing of the causative gene for Currarino syndrome, HLXB9, on blood leukocytes was positive for a novel nonsense mutation. Family history was positive for the same mutation, mother had a history of Hirschprung’s disease, and a cousin had a presacral teratoma. 
A Currarino triad, anorectal malformation, sacrococcygeal defect, and a presacral mass, recognized as a caudal regression syndrome and neuroenteric malformation, is due to an anomaly of the notochord in early embryogenesis. The disorder is inherited as an autosomal dominant, and mutations of the gene HLXB9 are found in 50% of patients. The diagnosis should be suspected in infants with recurrent or polymicrobial, especially gram negative, meningitis, persistent CSF pleocytosis, and a history of bowel abnormality.