Chiari type 1 deformity Natural History

2 patients with Chiari type 1 deformity progressed in the degree of tonsillar herniation, whereas increasing in prolapse of tonsillar herniation after diagnosis is extremely uncommon.

The first patient aged 17 years was diagnosed with CM-1 in 2009 and was operated due to progression of 5 mm radiologically and worsening symptoms in 2014. The second 5-month-old patient initially showed just low-settled tonsillar localization at the borderline, then it descended by 2 cm when the patient reached 3 years of age, yet could not be operated because of parents’ objection.

Due to lack of reports on increasing tonsillar descent in the literature, these case reports will contribute to natural history and management of CM-1 1).


The natural history of mild symptomatic and asymptomatic CM-1 in adults is relatively benign and nonprogressive; the decision to perform surgical decompression should be based on severity and duration of a patient’s symptoms at presentation. It is reasonable to observe a patient with mild or asymptomatic symptoms even in the presence of significant tonsillar descent or syringomyelia 2).


Strahle et al., identified 147 patients in whom CM-I was diagnosed on MR imaging, who were not offered surgery at the time of diagnosis, and in whom at least 1 year of clinical and MR imaging follow-up was available after the initial CM-I diagnosis. These patients were included in an outcome analysis.

Patients were followed clinically and by MR imaging for a mean duration of 4.6 and 3.8 years, respectively. Of the 147 patients, 9 had new symptoms attributed to the CM-I during the follow-up interval. During this time, development of a spinal cord syrinx occurred in 8 patients; 5 of these patients had a prior diagnosis of a presyrinx state or a dilated central canal. Spontaneous resolution of a syrinx occurred in 3 patients. Multiple CSF flow studies were obtained in 74 patients. Of these patients, 23 had improvement in CSF flow, 39 had no change, and 12 showed worsening CSF flow at the foramen magnum. There was no significant change in the mean amount of cerebellar tonsillar herniation over the follow-up period. Fourteen patients underwent surgical treatment for CM-I. There were no differences in initial cerebellar tonsillar herniation or CSF flow at the foramen magnum in those who ultimately underwent surgery compared with those who did not.

In patients with CM-Is that are selected for nonsurgical management, the natural history is usually benign, although spontaneous improvement and worsening are occasionally seen 3).


Chiari type 1 deformity was incidentally detected on MR images in 11 of 22 patients. The remaining 11 patients had minimal clinical signs at presentation that were not regarded as necessitating immediate surgical treatment. Seventeen patients (77.3%) showed progressive improvement in their symptoms or remained asymptomatic at the last follow-up whereas 5 patients (22.7%) experienced worsening, which was mild in 2 cases and required surgical correction in the remaining 3 cases. On MR imaging a mild reduction in tonsillar herniation was appreciated in 4 patients (18.18%), with complete spontaneous resolution in 1 of these. In 16 patients, tonsillar herniation remained stable during follow-up.

Data confirm the common impression that in both asymptomatic and slightly symptomatic patients with CM-I, a conservative approach to treatment should be adopted with periodic clinical and radiological examinations 4).

References

1)

Börcek AÖ, Aslan A. Unexpected Progression of Tonsillar Herniation in Two Pediatric Cases with Chiari Malformation Type I and Review of the Literature. Pediatr Neurosurg. 2018 Dec 21:1-6. doi: 10.1159/000495066. [Epub ahead of print] PubMed PMID: 30580335.
2)

Langridge B, Phillips E, Choi D. Chiari Malformation Type 1: A Systematic Review of Natural History and Conservative Management. World Neurosurg. 2017 Aug;104:213-219. doi: 10.1016/j.wneu.2017.04.082. Epub 2017 Apr 21. Review. PubMed PMID: 28435116.
3)

Strahle J, Muraszko KM, Kapurch J, Bapuraj JR, Garton HJ, Maher CO. Natural history of Chiari malformation Type I following decision for conservative treatment. J Neurosurg Pediatr. 2011 Aug;8(2):214-21. doi: 10.3171/2011.5.PEDS1122. PubMed PMID: 21806365.
4)

Novegno F, Caldarelli M, Massa A, Chieffo D, Massimi L, Pettorini B, Tamburrini G, Di Rocco C. The natural history of the Chiari Type I anomaly. J Neurosurg Pediatr. 2008 Sep;2(3):179-87. doi: 10.3171/PED/2008/2/9/179. PubMed PMID: 18759599.

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