Dynamic cervicomedullary cord compression and alterations in cerebrospinal fluid dynamics in children with achondroplasia: review of an 11-year surgical case series

Clinical article

Debraj Mukherjee M.D., M.P.H.1, Barry D. Pressman M.D.2, Deborah Krakow M.D.4, David L. Rimoin M.D., Ph.D.3, and Moise Danielpour M.D.1
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  • 1 Maxine Dunitz Neurosurgical Institute,
  • | 2 S. Mark Taper Foundation Imaging Center,
  • | 3 Medical Genetics Research Institute, and
  • | 4 Pediatric Infectious Disease, Cedars-Sinai Medical Center, Los Angeles, California
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Object

Achondroplasia may be associated with compression at the cervicomedullary junction. Determining which patients are at greatest risk for neurological complications of cervicomedullary compression can be difficult. In the current study the authors reviewed their records to determine the incidence and clinical significance of dynamic cervicomedullary stenosis and obstruction of CSF flow along with surgical outcomes following posterior fossa decompression.

Methods

The authors reviewed 34 consecutive cases involving symptomatic children with achondroplasia undergoing cervicomedullary decompression performed by a single surgeon over 11 years. Of these patients, 29 had undergone preoperative dynamic MRI of the cervicomedullary junction with cine (cinema) CSF flow studies; 13 of these patients underwent postoperative dynamic MRI studies. Clinical outcomes included changes in polysomnography, head circumference percentile, and fontanel characteristics. Radiographic outcomes included changes in dynamic spinal cord diameter, improvement in CSF flow at the foramen magnum, and change in the Evans ratio.

Results

Patients were predominantly female, with a mean age at presentation of 6.6 years and mean follow-up of 3.7 years (range 1–10 years).

All patients had moderate to excellent improvement in postoperative polysomnography, slight decrease in average head circumference percentile (from 46.9th percentile to 45.7th percentile), and no subjective worsening of fontanel characteristics. The Evans ratio decreased by 2%, spinal cord diameter increased an average of 3.1 mm, 5.2 mm, and 0.2 mm in the neutral, flexed, and extended positions, respectively, and CSF flow improved qualitatively in all 3 positions. There were no postoperative infections, CSF leaks, or other major complications. None of the patients undergoing initial foramen magnum decompression performed at our medical center required reoperation.

Conclusions

Patients with achondroplasia and symptomatic cervicomedullary compression have increased risk of dynamic stenosis at the foramen magnum evident upon dynamic cine MRI. Operative decompression may be offered with low risk of complications or need for reoperation.

Abbreviations used in this paper:

CMJ = cervicomedullary junction; VP = ventriculoperitoneal.

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