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  • By Author: Mortazavi, Martin M. x
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Joshua J. Chern, Amber J. Gordon, Martin M. Mortazavi, R. Shane Tubbs and W. Jerry Oakes

Object

In 1998 the authors identified 5 patients with syringomyelia and no evidence of Chiari malformation Type I (CM-I). Magnetic resonance imaging of the entire neuraxis ruled out other causes of a syrinx. Ultimately, abnormal CSF flow at the foramen magnum was the suspected cause. The label “Chiari 0” was used to categorize these unique cases with no tonsillar ectopia. All of the patients underwent posterior fossa decompression and duraplasty identical to the technique used to treat patients with CM-I. Significant syrinx and symptom resolution occurred in these patients. Herein, the authors report on a follow-up study of patients with CM-0 who were derived from over 400 operative cases of pediatric CM-I decompression.

Methods

The authors present their 12-year experience with this group of patients.

Results

Fifteen patients (3.7%) were identified. At surgery, many were found to have physical barriers to CSF flow near the foramen magnum. In most of them, the syringomyelia was greatly diminished postoperatively.

Conclusions

The authors stress that this subgroup represents a very small cohort among patients with Chiari malformations. They emphasize that careful patient selection is critical when diagnosing CM-0. Without an obvious CM-I, other etiologies of a spinal syrinx must be conclusively ruled out. Only then can one reasonably expect to ameliorate the clinical course of these patients via posterior fossa decompression.

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Martin M. Mortazavi, R. Shane Tubbs, Maja Andrea Brockerhoff, Marios Loukas and W. Jerry Oakes

Few are familiar with the neurological contributions of the German pathologist Theodor Langhans. Even fewer are aware of his significant and early contributions to the study of what is now known as the Chiari I malformation. In at least 4 cases, Langhans described the association between tonsillar ectopia and syringomyelia. Moreover, this early pioneer speculated that there was a cause and effect with hindbrain herniation resulting in improper flow at the craniocervical junction and consequent development of syringomyelia. These cases were reported prior to Hans Chiari's descriptions, and Langhans' theory of impeded foramen magnum flow as a cause of syringomyelia was novel and preceded the current understanding of this mechanism by almost a century. The authors discuss the life of Langhans and translate excerpts from his 1881 work regarding tonsillar ectopia and syringomyelia.

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Martin M. Mortazavi, R. Shane Tubbs, Daniel Harmon and W. Jerry Oakes

Chronic emesis may result from a variety of causes. To the authors' knowledge, compression of the area postrema by regional vessels resulting in chronic emesis has not been reported.

The authors report on a child who presented with chronic medically intractable emesis and significant weight loss requiring jejunostomy feeding. Surgical exploration of the posterior cranial fossa found unilateral compression of the area postrema by the posterior inferior cerebellar artery. Microvascular decompression resulted in postoperative and long-term resolution of the patient's emesis.

Although apparently very rare, irritation of the area postrema from the posterior inferior cerebellar artery with resultant medically intractable chronic emesis may occur. Therefore, the clinician should be aware of this potential etiology when dealing with such patients.