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Chiari type 1 deformity case reports


A 16-year-old male patient who presented with severe headache and gait instability. A micro-decompression of the suboccipital bone and posterior arch osteotomy of C1 through a 2-cm midline incision was performed under surgical microscope magnification. A duraplasty was performed through the same approach. The patient was discharged home after 2 days in the hospital and returned to regular activities at school 3 weeks after surgery. The minimally invasive technique presented here is a viable option for the posterior decompression of the CVJ in patients with CM-1 using a low-cost self-retaining retractor 1).


2 patients with CM-1 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 2).


A 13-year-old obese boy with a 3-week history of headaches, neck pain, torticollis and progressive visual deterioration was admitted. Bilateral chronic papilledema and decrease in visual acuity were found in the presence of a previously diagnosed CMI.

Intracranial pressure monitoring demonstrating increased pressure levels was followed by a suboccipital decompression, C1 laminectomy and duroplasty. Post-operatively, the boy improved markedly, the 6 months follow-up opthalmological examination demonstrated resolution of papilloedema, but consecutive bi-lateral optic nerve atrophy.

IH with progressive visual deterioration represents one of the varying clinical presentations of CMI and may be classified as a secondary form of idiopathic intracranial hypertension (IH). Neuro-ophthalmological examination in all patients with CMI is recommended to identify the real incidence of this presentation. Altered CSF dynamics, venous hypertension and obesity as co-factors may be causative pathophysiologic factors 3).

Quillo-Olvera J, Navarro-Ramírez R, Quillo-Olvera D, Quillo-Reséndiz J, Kim JS. Minimally Invasive Craniocervical Decompression for Chiari 1 Malformation: An Operative Technique. J Neurol Surg A Cent Eur Neurosurg. 2019 Apr 24. doi: 10.1055/s-0039-1685. [Epub ahead of print] PubMed PMID: 31018228.
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.
Kurschel S, Maier R, Gellner V, Eder HG. Chiari I malformation and intra-cranial hypertension:a case-based review. Childs Nerv Syst. 2007 Aug;23(8):901-5. Epub 2007 May 8. PubMed PMID: 17486353.
chiari_type_1_deformity_case_reports.txt · Last modified: 2019/04/25 20:56 by administrador