Monday, October 29, 2007

Seven distinct coexistent cranial and spinal anomalies

Emmez H, Tokgoz N, Dogulu F, Yilmaz MB, Kale A, Baykaner MK. Seven distinct coexistent cranial and spinal anomalies. Pediatric Neurosurgery. 2006;42(5):316-9.

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.

PMID: 16902346

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