Cervicomedullary compression in young patients with achondroplasia: Value of comprehensive neurologic and respiratory evaluation

Reid, Cheryl S.; Pyeritz, Reed E.; Kopits, Steven E.; Maria, Bernard L.; Wang, Henry E.; McPherson, Robert W.; Hurko, Orest; Phillips, John A.; Rosenbaum, Arthur E.

doi:10.1016/s0022-3476(87)80542-5

Cited by 133 publications

(76 citation statements)

References 21 publications

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“…In comparison, the foramen magnum in achondroplasia is small and can va'ry in shape, having a keyhole or elongated appearance. and mortality associated with foramen magnum stenosis in achondroplasia Hecht et al, 1985Hecht et al, , 1987Reid et al, 1987;Nelson et al, 19881. previous study: Drs. C. I. Scott, E. R. Wassman, C .…”

Section: Discussionmentioning

confidence: 99%

“…Neurologic and respiratory complications caused by the abnormal structure of the base of the skull and upper cervical spine have recently been demonstrated in achondroplasia [Reid et al, 1987;Nelson et al, 19881. Of particular importance is the small foramen magnum, which has been implicated in an excess of sudden deaths in those less than age 4 years Hecht et al, 19871.…”

Section: Introductionmentioning

confidence: 99%

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Growth of the foramen magnum in achondroplasia

et al. 1989

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Foramen magnum growth curves in achondroplasia and in the general population are presented. The achondroplastic foramen magnum is small at birth, and during the first year it has a severely impaired rate of growth especially in the transverse dimension. This markedly diminished growth results not only from abnormal endochondral bone growth but also because of abnormal placement and premature fusion of the synchondroses. Evaluation of the foramen magnum in achondroplasia should address absolute size of the transverse and sagittal dimensions, shape, and growth centers to determine growth potential of this area. KEYWORDS: foramen magnum stenosis, craniocervical stenosis, foramen magnum normal curves, foramen magnum achondroplasia curves

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Growth of the foramen magnum in achondroplasia

et al. 1989

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“…2,8,10,19,21 Unfortunately, there continues to be controversy over the degree of risk for sudden death in children with achondroplasia, how often to operate, the indications for decompression, and surgical methods that should be used. Criteria for decompression in large surgical series 17 have not provided a fail-safe method for prospectively identifying patients who are likely to die or experience severe neurological complications without surgical decompression.…”

Section: Discussionmentioning

confidence: 99%

“…8,12,19 Criteria for decompression have been previously described in large surgical series. 17,20 Unfortunately, there has never been a study that provided a fail-safe method for prospectively identifying patients who are likely to die or experience severe neurological complications if decompression surgery was not performed.…”

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

Mukherjee

Pressman

Krakow

et al. 2014

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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.