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The Chiari I malformation

JNSPG 75th Anniversary Invited Review Article

Samuel G. McClugage III and W. Jerry Oakes

As with many pathologies, the course of our understanding of the Chiari I malformation (CIM) has developed extensively over time. The early descriptions of the Chiari malformations by Hans Chiari in 1891 opened the door for future classification and research on this topic. However, even over a long timeframe, our understanding of the pathophysiology and, more importantly, treatment, remained in its infancy. As recently as the 1970s, CIM was not discussed in popular neurology textbooks. Syringomyelia is listed as a degenerative disorder with no satisfactory treatment. Radiation therapy was considered an option in treatment, and surgery was thought to play no role. During the last 40 years, equivalent to the duration of a neurosurgical career, our understanding of the pathophysiology and natural history of CIM, coupled with modern MRI, has improved the treatment paradigm for this patient population. More importantly, it has given us evidence confirming that CIM is a disorder responsive to surgical intervention, giving patients once thought to be destined for lifelong disability a comparatively normal life after treatment. The purpose of this article is to offer a review of CIM and its important associated entities. The authors will discuss the evolution in understanding of the Chiari malformation and, importantly, distinguish between symptomatic CIM and asymptomatic tonsillar ectopia, based on imaging and presenting symptomatology. They will discuss techniques for surgical intervention, expected outcomes, and complications after surgery. Proper patient selection for surgery based on appropriate symptomatology is tantamount to achieving good surgical outcomes in this population, separating those who can be helped by surgery from those who are unlikely to improve. While our knowledge of the Chiari malformations continues to improve through the efforts of clinical and basic science researchers, surgeons, and patients, our current understanding of these entities represents a monumental improvement in patient care over a relatively short time period.

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Brandon G. Rocque, Matthew C. Davis, Samuel G. McClugage III, Dang Anh Tuan, Donald T. King III, Nguyen Thi Huong, Nguyen Thi Bich Van, Pongkiat Kankirawatana, Cao Vu Hung, Le Nam Thang, James M. Johnston and Nguyen Duc Lien


The purpose of this report was to describe an international collaboration model to facilitate the surgical treatment of children with epilepsy in Vietnam.


This model uses three complementary methods to achieve a meaningful expansion in epilepsy surgery capacity: US-based providers visiting Hanoi, Vietnam; Vietnamese providers visiting the US; and ongoing telecollaboration, including case review and real-time mentorship using internet-based communication platforms.


Introductions took place during a US neurosurgeon’s visit to Vietnam in 2014. Given the Vietnamese surgeon’s expertise in intraventricular tumor surgery, the focus of the initial visit was corpus callosotomy. After two operations performed jointly, the Vietnamese surgeon went on to perform 10 more callosotomy procedures in the ensuing 6 months with excellent results. The collaborative work grew and matured in 2016–2017, with 40 pediatric epilepsy surgeries performed from 2015 through 2017. Because pediatric epilepsy care requires far more than neurosurgery, teams traveling to Vietnam included a pediatric neurologist and an electroencephalography (EEG) technologist. Also, in 2016–2017, a neurosurgeon, two neurologists, and an EEG nurse from Vietnam completed 2- to 3-month fellowships at Children’s of Alabama (COA) in the US. These experiences improved EEG capabilities and facilitated the development of intraoperative electrocorticography (ECoG), making nonlesional epilepsy treatment more feasible. The final component has been ongoing, i.e., regular communication. The Vietnamese team regularly sends case summaries for discussion to the COA epilepsy conference. Three patients in Vietnam have undergone resection guided by ECoG without the US team present, although there was communication via internet-based telecollaboration tools between Vietnamese and US EEG technologists. To date, two of these three patients remain seizure free. The Vietnamese team has presented the results of their epilepsy experience at two international functional and epilepsy surgery scientific meetings.


Ongoing international collaboration has improved the surgical care of epilepsy in Vietnam. Experience suggests that the combination of in-country and US-based training, augmented by long-distance telecollaboration, is an effective paradigm for increasing the capacity for highly subspecialized, multidisciplinary neurosurgical care.