Congenital stridor in the context of Chiari malformation type II: the etiological role of vernix caseosa granulomatous meningitis.

The authors describe the case of a late preterm infant girl who presented prenatally with a low lumbar neural tube defect and features of Chiari malformation type II (CM-II). At birth, she exhibited stridor and underwent surgical repair of a lumbosacral myelomeningocele on Day 2 of life. The prognosis was deemed to be poor, and hence a "Chiari decompression" procedure was not undertaken. The patient was subsequently extubated and died on Day 10. Postmortem findings included a rarely described but characteristic granulomatous meningitic reaction to vernix caseosa, which presumably entered the subarachnoid space and spinal cord syrinx antenatally via the open neural tube defect. The significance of congenital stridor in the context of CM-II and in particular the role of vernix caseosa granulomatous meningitis are examined. The antenatal repair of myelomeningoceles, as championed by some, may prevent this ominous meningitic complication.