The Chiari 3 Malformation and a Systemic Review of the Literature

Pediatric Neurosurgery
Richard M YoungJohn S Myseros

Abstract

Chiari type 3 is a rare hindbrain malformation that has been reported in the literature primarily as case reports and case series. Radiological, pathophysiological and surgical definitions of the malformation are inconsistent in the literature and subsequently can be confusing, and outcomes have also been uniformly poor. The definition of this rare malformation will be clarified through a case presentation. A retrospective review of prior publications in the PubMed and MEDLINE databases was performed looking for reports of 'Chiari 3 +/- malformation' and 'occipital encephalocele'. Relevant papers were reviewed and compiled into table format with associated descriptions of a Chiari type 3 malformation. A case illustration is presented with radiological and intraoperative imaging to reinforce and clarify the definition. Upon review of the prior publications in the detail of the descriptions and imaging associated with each article, there is a wide range of variability in the description of what is considered a Chiari 3 malformation. Occipital, occipitocervical and high cervical defects have all been described as Chiari 3 malformation. Our case illustration presents a patient with an occipitocervical encephalocele with neural elem...Continue Reading

Citations

Apr 12, 2016·Seminars in Ultrasound, CT, and MR·Andrea PorettiThierry A G M Huisman
Feb 7, 2019·Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery·Serge Eddy MbaBrighton Nyamapfene
May 4, 2021·Journal of Pediatric Neurosciences·Mohammad ElbaroodyMohamed Gabr
Aug 15, 2021·Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery·Tatiana ProtzenkoJosé Francisco M Salomão

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