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Mondini malformation associated with diastematomyelia and presenting with recurrent meningitis

Amira Masri1, Faris G Bakri, Ralf Birkenhäger

  • 1Department of Pediatrics, Division of Child Neurology, Jordan University Hospital, Amman, Jordan. amasri@ju.edu.jo

Journal of Child Neurology
|March 23, 2011
PubMed

Insights

Recurrent meningitis in a child was linked to a spinal defect and inner ear anomaly. This case highlights the first known association between Mondini dysplasia and diastematomyelia, offering new insights into complex pediatric neurological conditions.

Area of Science:

  • Pediatric Neurology
  • Developmental Biology
  • Otolaryngology

Background:

  • Recurrent meningitis poses significant diagnostic and therapeutic challenges in pediatric patients.
  • Congenital spinal anomalies, such as diastematomyelia and tethered cord, can predispose individuals to infections.
  • Inner ear malformations, like Mondini dysplasia, are less commonly associated with central nervous system infections.

Observation:

  • A 5-year-old girl experienced four episodes of recurrent meningitis.
  • Initial investigations identified a lumbosacral dermoid sinus with diastematomyelia and a tethered cord, which underwent surgical correction.
  • Despite surgical intervention, meningitis recurred, and a temporal bone scan revealed left Mondini dysplasia.

Findings:

  • This case represents the first documented instance of Mondini dysplasia co-occurring with diastematomyelia.
  • The findings suggest a potential, previously unrecognized association between these congenital anomalies.
  • The complex interplay between spinal and inner ear malformations may contribute to recurrent meningitis.

Implications:

  • This report expands the understanding of rare congenital conditions and their clinical manifestations.
  • It underscores the importance of comprehensive evaluation in cases of recurrent meningitis, considering potential associated anomalies.
  • Further research is warranted to explore the embryological basis and clinical significance of this combined presentation.

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