• Journal of Korean Neurosurgical Society
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• 제48권4호
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• pp.375-379
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• 2010

The Chiari 1.5 malformation is defined as a tonsillar hemiation within a Chiari I malformation with additional caudal descent of the brainstem through the foramen magnum. We describe a patient with Chiari I malformation who evolved to Chiari 1.5 malformation during longitudinal follow-up. A 15-year-old girl presented with neck pain during exercise for two years. She had been diagnosed with Chiari I malformation with mild hydrocephalus after minor cervical trauma at the age of six years. At that time, she was asymptomatic. After she complained of aggravated neck pain, neuroimaging (nine years after first imaging) revealed caudal descent of the brainstem and syringomyelia in addition to progression of tonsillar hemiation. Posterior fossa decompressive surgery resulted in complete resolution of neck pain. Based on neuroimaging and operative findings, she was diagnosed as Chiari 1.5 malformation. Neuroimaging performed seven months after surgery showed an increased anterior-posterior diameter of the medulla oblongata and markedly decreased syringomyelia. This case demonstrates progressive developmental process of the Chiari 1.5 malformation as an advanced form of the Chiari I malformation.

• Journal of Korean Neurosurgical Society
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• 제40권1호
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• pp.38-39
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• 2006

Perioperative lumbar drainage of cerebrospinal fluid is commonly used in neurosurgical practice. However, the relationship between lumbar drainage and acquired Chiari malformation is not well established. The authors present an unusual case of paraplegia as a result of acquired Chiari malformation after lumbar drainage. Acquired Chiari malformation can induce compression of cervicomedullary junction and syrinx formation. Foramen magnum decompression is recommended for the solution of such problems.

• 보험의학회지
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• 제27권1호
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• pp.37-38
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• 2008

The concept of Chiari malformations emerged toward the end of $19^{th}$ century from Chiari's initial descriptions of "alterations in the cerebellum resulting from cerebral hydrocephalus." In 1891, Hans Chiari(1851-1916) suggested cerebellar ectopia in which he classified Type I as "elongation of the tonsils and medial parts of the inferior lobes of the cerebellum into cone-shaped projections, which accompany the medulla oblongata into the spinal canal. The incidence of Chiari malformation has been found to be between 0.56% and 0.77% on MR imaging studies, as well as 0.62% in brain dissection studies. the definition of the adult Chiari malformation has varied with the evolution of neurodiagnositic capabilities and knowledge of physiopathology. This disorder can be associated with significant symptomatology, risk of secondary injury due to trauma, and the risk of progression and damage of the spinal cord due to associated Syringomyelia. Syringomyelia is found in 50 to 70% of Chiari I malformation. It is the clinical judgment of the physicians evaluating this disorder that is of the importance to avoid the therapeutic extremes of pursuing unnecessary surgery or withholding necessary treatment from patients.

• 한국임상수의학회지
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• 제23권2호
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• pp.91-95
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• 2006

사람에서, Chiari 1형 기형은 소뇌 탈출과 척수공동증이 특정인 발달성 장애이다. 이러한 사람의 Chiari 1형 기형과 유사한 질환이 cavalier King Charles spaniels에서 흔히 나타났다. 그러나, 이러한 Chiari 1형 유사 기형이 다른 종의 개에서 진단 보고된 증례가 거의 없는 실정이다. 이에 본 연구에서는 24 마리의 개에서 자기공명영상 장치를 이용하여 Chiari 1형 유사 기형으로 진단된 증례를 보고하고자 한다. 이 개들은 다양한 신경 증상과 다양한 정도의 소뇌 탈출, 척수공동증, 두개관내 거미막 낭종 또는 뇌수종의 병발 질환들을 나타내었다.

• Journal of Yeungnam Medical Science
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• 제9권1호
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• pp.203-209
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• 1992

저자들은 본 병원 산부인과에서 최근 Arnold-Chiari 기형 1례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

• Journal of Korean Neurosurgical Society
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• 제57권4호
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• pp.311-313
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• 2015

Chiari type I malformation is a tonsillar herniation more than 3 mm from the level of foramen magnum, with or without concurrent syringomyelia. Different surgical treatments have been developed for syringomyelia secondary to Chiari's malformations: craniovertebral decompression with or without plugging of the obex, syringo-subarachnoid, syringo-peritoneal, and theco-peritoneal shunt placement. Shunt placement procedures are useful for neurologically symptomatic large-sized syrinx. In this paper, authors define the first successful treatment of a patient with syringomyelia due to Chiari type I malformation using a pre-defined new technique of syringo-subarachnoid-peritoneal shunt with T-tube system.

• 대한소아치과학회지
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• 제28권4호
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• pp.649-653
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• 2001

Chiari malformation은 하부 뇌간과 소뇌가 대공 속으로 들어가 하향 편위의 소견을 보이는 중추 신경계의 기형으로 3가지 type으로 분류된다. 본 증례는 7세 10개월 된 여환이 10일전부터 칫솔질시 상악 우측 어금니가 아프고 얼굴이 약간 붓고 아프다는 주소로 내원하였는데, 임상구강검사결과 상악 우측 제1대구치 원심에 치은낭이 형성되어 있었고 동요도와 동통이 존재하였다. 악골 방사선사진검사결과 상악 구치부의 골밀도가 정상에 비해 낮으며, 특히 좌우측 제1대구치 하방의 골의 부재가 보였다. 유년성 치주염의 가진 하에 치주치료 중 전신질환과의 연관성 문진으로 Chiari malformation의 의과적 병력을 확인한 후 3차원 전산화단층촬영을 시행하였다. 두개골이 전반적으로 않으며 다수의 골결손이 관찰되고 대공이 다소 커져있으며 후두골과 상악골의 골밀도가 감소되어 있었고 특히 상악 구치부의 치조골이 거의 없어 상악 좌우측 제1대구치가 부유치처럼 보였다. 이에 본원 신경외과로 협의진료를 의뢰하여 계속적으로 관찰하고 있는 중이다. 소아에서 치주염이 의심될 때 leukemia, hystiocytosis X, hypoposphatasia 등의 잠재된 전신질환과 연관되어 있을 수 있으므로 적절한 검사를 시행해야 하며 전신질환의 근본적인 원인을 치료할 수 있도록 고려해야 한다.

• Clinical and Experimental Pediatrics
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• 제59권sup1호
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• pp.149-151
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• 2016

Chiari malformations are a congenital anomaly of the hindbrain. The most common, Chiari malformation type I (CM-I), is characterized by herniation of the cerebellar tonsils extending at least 3 mm below the plane of the foramen magnum. Consequently, CM-I is associated with hydrocephalus and symptoms involving compression of the cervicomedullary junction by ectopic tonsils. Several studies have reported the clinical symptoms associated with CM-I, including suboccipital headache, weakness in the upper extremities, facial numbness, loss of temperature sensation, ataxia, diplopia, dysarthria, dysphagia, vomiting, vertigo, nystagmus, and tinnitus. Syncope is one of the rarest presentations in patients with CM-I. There are many hypotheses regarding the causes of syncope in patients with CM-I; however, the mechanisms are not clearly understood. Although surgical decompression for CM-I in patients with syncope has yielded good clinical results in some studies, such cases are rarely reported. We report a case of orthostatic syncope in a patient with CM-I who was treated with surgical intervention.

• Clinical and Experimental Pediatrics
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• 제53권6호
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• pp.718-721
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• 2010

Rubinstein-Taybi syndrome (RTS) is a congenital disorder characterized by typical facial features, broad thumbs and toes, with mental retardation. Additionally, tumors, keloids and various congenital anomalies including congenital heart defects have been reported in RTS patients. In about 50% of the patients, mutations in the $CREB$ $binding$ $protein$ ($CREBBP$) have been found, which are understood to be associated with cell growth and proliferation. Here, we describe a typical RTS patient with Arnold-Chiari malformation. A mutation in the CREBBP gene, c.4944_4945insC, was identified by mutational analysis.

• Journal of Korean Neurosurgical Society
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• 제59권3호
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• pp.197-203
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• 2016

Craniosynostosis is defined as the premature fusion of one or more cranial sutures resulting in skull deformity. Characteristically, this disorder can cause diverse neurosurgical problems, as well as abnormal skull shape. Intracranial hypertension, hydrocephalus, Chiari malformation and neuropsychological dysfunction are the major neurosurgical concerns in children with craniosynostosis. In this review article, we investigate pathophysiology, characteristics and proper neurosurgical management of these neurosurgical issues, respectively.