Seven distinct coexistent cranial and spinal anomalies

Pediatr Neurosurg. 2006;42(5):316-9. doi: 10.1159/000094070.

Abstract

Existence of multiple cranial and spinal anomalies in spina bifida is well known; however, coexistence of seven different severe anomalies is extremely rare. The location of the anomalies, the patient's age and presentation are other interesting aspects of the presented case.

Case report: A 1-year-old girl with an enlarged head and big, infected lumbosacral myelomeningocele (MMC) was admitted to the emergency department in a comatose state. Further investigations revealed hydrocephalus, Chiari malformation, syringohydromyelia, split cord malformation, dermal sinus tract, lumbosacral MMC, and tethered cord. Unfortunately, the patient died 3 days after admission because of meningitis and sepsis.

Discussion: Coexistence of seven different craniospinal anomalies is extremely unusual. Further investigations with magnetic resonance imaging for associated anomalies, early shunt placement and MMC repair are required to prevent this life-threatening condition in spina bifida.

Publication types

  • Case Reports

MeSH terms

  • Cephalometry
  • Fatal Outcome
  • Female
  • Head / abnormalities*
  • Humans
  • Hydrocephalus / complications
  • Infant
  • Meningitis / complications
  • Neural Tube Defects / complications*
  • Sepsis / complications
  • Spinal Cord / abnormalities*
  • Syringomyelia / complications*