• 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 Korean Neurosurgical Society
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• 제65권6호
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• pp.841-845
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• 2022

Objective : Chiari III malformations are extremely rare hindbrain malformations that are associated with a high early mortality rate, or severe neurologic deficits in the survivors. The treatment is early operative closure and cerebrospinal fluid diversion (CSF) shunting. Methods : We operated on 15 patients by repair and excision between July 2014 till June 2020 and retrospective data collection was done. Only one patient doesn't need ventriculoperitoneal (VP) shunt and the other 14 patients need a VP shunt. We described stepwise dissection and untethering of the cerebellum from the bony edge to regrowth and herniation of cerebellum again into this potential space and simple dural closure or repair with graft was done. Results : We started with VP in eight patients (53%) and the other seven patients (46.7%) started with excision and then six patients need VP shunt later on because four patients developed CSF leak and two patients developed increased high intracranial tension. Only four patients (26.7%) needed a blood transfusion. Conclusion : There are variations in the outcome and not all cases of Chiari malformation III will die and severe developmental delay is not a must. Proper management and repair carry a good prognosis.

• 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.

• 대한수의학회지
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• 제63권3호
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• pp.26.1-26.5
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• 2023

Syringobulbia is a rare neurological disorder characterized by a fluid-filled cavity in the brainstem. In this study, clinical signs, features on magnetic resonance imaging (MRI), and the diseases present concurrently with syringobulbia were investigated in 33 small breed dogs. Most dogs (97%) had concurrent syringomyelia, and some dogs (24%) presented with vestibular or cranial nerve symptoms associated with the medulla oblongata. MRIs revealed slit-like, bulbous, and vertical linear shapes of the cavities on T2-weighted hyperintense and T1-weighted hypointense signals similar to the cerebrospinal fluid. Chiari-like malformations were identified in all dogs. This study highlights the association of syringobulbia with syringomyelia and Chiari-like malformations in small breed dogs with or without brainstem-associated clinical signs.

• 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.

• 생명과학회지
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• 제15권4호
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• pp.664-667
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• 2005

척수공동증(hydrosyringomyelia)은 척수내의 중심관(central canal)이 확장되는 질환으로 사람에 있어서는 Dandy-walker 증후군이나 Chiari 기형과 같은 선천적인 기형에 의해 발생되거나 감염, 외상 및 종양과 관련되어서도 발생된다. 뇌수두증(hydrocephalus)은 뇌척수액이 뇌실내에 과도하게 축적되는 것을 말하며 후두골 이형성증(occipital dysplasia)은 후두공이 과도하게 위쪽으로 연장되어 열린 질병이다. 척수공동증과 뇌수두증은 컴퓨터단층촬영이나 자기공명영상 검사에 의해 확진되어질 수 있다. 3세령의 수컷 말티즈가 장기간의 발작 증세를 주증으로 내원하였다. 혈액검사상 특이소견은 없었으나 rostrodorsal-caudoventral oblique의 두개골 방사선 검사에서 심한 두개골 이형성증 소견이 확인되었고 열려진 천문을 통한 초음파 검사상 양측 외측 뇌실이 모두 심하게 확장된 것이 확인되었다. 자기공명영상 검사에서 뇌수두증과 척수공동증이 진단되었다. 이뇨제를 중심으로 한 내과적 처치에 반응을 하지 않아 외과적 감압술(ventriculo-peritoneal shunting)을 시행하였고 현재까지 잘 유지되고 있다. 자기공명영상검사법은 뇌수두증과 척수공동증을 진단하고 그에 따른 치료계획을 수립하며 예후를 평가하는데 있어 가장 우수한 진단 기법이다.