• Journal of Korean Neurosurgical Society
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• v.57 no.4
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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.

• The Journal of the Korean life insurance medical association
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• v.27 no.1
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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.

• Clinical and Experimental Pediatrics
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• v.59 no.sup1
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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.

• Journal of Korean Neurosurgical Society
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• v.59 no.5
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• pp.512-517
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• 2016

Objective : The pathophysiology of idiopathic Chiari malformation (CM) type 1 is disturbance of free cerebrospinal fluid (CSF) flow and restoration of normal CSF flow is the mainstay of treatment. Additional migration of the medulla oblongata in pediatric patients is referred to as CM type 1.5, but its significance in adult patients is unknown. This study is to compare surgical outcomes of adult idiopathic CM type 1.5 with that of type 1. Methods : Thirty-eight consecutive adult patients (M : F=11 : 27; median, 33.5; range, 18-63) with syringomyelia due to idiopathic CM type 1 were reviewed. Migration of the medulla oblongata was noted in 13 patients. The modified McCormick scale (MMS) was used to evaluate functional status before and one year after surgery. All patients underwent foramen magnum decompression and duroplasty. Factors related to radiological success (${\geq}50%$ decrease in the diameter of the syrinx) were investigated. The follow-up period was $72.7{\pm}55.6$ months. Results : Preoperative functional status were MMS I in 11 patients and MMS II in 14 of CM type 1 and MMS I in 8 and II in 5 of CM type 1.5. Of patients with MMS II, 5/14 patients in group A and 3/5 patients in group B showed improvement and there was no case of deterioration. Radiological success was achieved in 32 (84%) patients and restoration of the cisterna magna (p=0.01; OR, 46.5) was the only significant factor. Conclusion : Migration of the medulla oblongata did not make a difference in the surgical outcome when the cisterna magna was restored.

• Journal of Korean Neurosurgical Society
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• v.54 no.5
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• pp.405-410
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• 2013

Objective : The objective of this study was to investigate changes in the posterior cranial fossa in patients with symptomatic Chiari malformation type I (CMI) compared to a control group. Methods : We retrospectively reviewed clinical and radiological data from 12 symptomatic patients with CMI and 24 healthy control subjects. The structures of the brain and skull base were investigated using magnetic resonance imaging. Results : The length of the clivus had significantly decreased in the CMI group than in the control group (p=0.000). The angle between the clivus and the McRae line (p<0.024), as the angle between the supraocciput and the McRae line (p<0.021), and the angle between the tentorium and a line connecting the internal occipital protuberance to the opisthion (p<0.009) were significantly larger in the CMI group than in the control group. The mean vertical length of the cerebellar hemisphere (p<0.003) and the mean length of the coronal and sagittal superoinferior aspects of the cerebellum (p<0.05) were longer in the CMI group than in the control group, while the mean length of the axial anteroposterior aspect of the cerebellum (p<0.001) was significantly shorter in the CMI group relative to control subjects. Conclusion : We elucidate the transformation of the posterior cranial fossa into the narrow funnel shape. The sufficient cephalocaudal extension of the craniectomy of the posterior cranial fossa has more decompression effect than other type extension of the craniectomy in CMI patients.

• Journal of Korean Neurosurgical Society
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• v.58 no.5
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• pp.454-461
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• 2015

Objective : In this study, we aimed to investigate the underlying ethiological factors in chiari malformation (CM) type-I (CMI) via performing volumetric and morphometric length-angle measurements. Methods : A total of 66 individuals [33 patients (20-65 years) with CMI and 33 control subjects] were included in this study. In sagittal MR images, tonsillar herniation length and concurrent anomalies were evaluated. Supratentorial, infratentorial, and total intracranial volumes were measured using Cavalieri method. Various cranial distances and angles were used to evaluate the platybasia and posterior cranial fossa (PCF) development. Results : Tonsillar herniation length was measured $9.09{\pm}3.39mm$ below foramen magnum in CM group. Tonsillar herniation/concurrent syringomyelia, concavity/defect of clivus, herniation of bulbus and fourth ventricle, basilar invagination and craniovertebral junction abnormality rates were 30.3, 27, 18, 2, 3, and 3 percent, respectively. Absence of cisterna magna was encountered in 87.9% of the patients. Total, IT and ST volumes and distance between Chamberlain line and tip of dens axis, Klaus index, clivus length, distance between internal occipital protuberance and opisthion were significantly decreased in patient group. Also in patient group, it was found that Welcher basal angle/Boogard angle increased and tentorial slope angle decreased. Conclusion : Mean cranial volume and length-angle measurement values significantly decreased and there was a congenital abnormality association in nearly 81.5 percent of the CM cases. As a result, it was concluded that CM ethiology can be attributed to multifactorial causes. Moreover, congenital defects can also give rise to this condition.

• The Korean Journal of Pain
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• v.19 no.2
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• pp.241-243
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• 2006

Central pain is defined as pain associated with lesions of the central nervous system, and is among the most intractable of chronic pain syndromes. A 47 year-old-female, who had right arm and shoulder pain, was diagnosed with syringomyelia of the Arnold Chiari malformation type I and received foramen magnum decompression and a syringo-subarachnoid shunt. After the operation, the evoked pain was improved, but she complained of a continuous burning pain, coupled with cold and tactile allodynia. This symptom failed to fully subside on administration of oral medicine; therefore, brachial plexus block was performed, which relieved her pain transiently. Through repeated trials, a gradual decrease in the pain intensity and frequency was found. However, the way in which brachial plexus block improves spinal central pain is not completely known.