• 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.

• Journal of Veterinary Clinics
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• v.23 no.2
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• pp.91-95
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• 2006

Chiari type 1 malformation is a developmental condition characterized by cerebellar herniation and syringohydromyelia in human beings. It has been reported as a common condition in the cavalier King Charles spaniel that is similar to human Chiari type 1 malformations. However, there are few documentations of diagnosed Chiari type 1 like malformation in other breed dogs. Abnormalities compatible with such a malformation were identified by magnetic resonance imaging in 24 dogs with neurologic signs in this study. The dogs were 15 females and 9 males. Their breed were variable, and 6 of the dogs were maltese, 5 were shih-tzu. The dogs had a variety of neurological signs and the severity of cerebellar herniation, syringohydromyelia, intracranial intra-arachnoid cyst or hydrocephalus.

• Journal of Korean Neurosurgical Society
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• v.65 no.6
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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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• 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.

• Korean Journal of Veterinary Research
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• v.63 no.3
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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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• 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 Life Science
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• v.15 no.4 s.71
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• pp.664-667
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• 2005

Hydrosyringomyelia is a dilation of the spinal cord central canal. In human it may be caused by congenital malformations such as Dandy-Walker syndrome and Chiari malformations or may be acquired as a result of infection, trauma or neoplasia. Hydrocephalus is an excessive accumulation of cerebrospinal fluid within the ventricles and occipital dysplasia is the dorsal extension of the foramen magnum. Hydrosyringomyelia and hydrocephalus can be confirmed by computed tomography or magnetic resonance imaging (MRI). A 3-year-old male maltese was presented with a history of long-term seizure. Blood examination was all unremarkable. On rostrodorsal-caudoventral oblique radiograph of the skull showed severe occipital dysplasia. On brain sonography through the persistent fontanelle, severe lateral ventriculomegaly was revealed. MRI examination revealed hydrocephalus and hydrosyringomyelia. Diuretic therapy didn't reduce clinical symptoms and surgical decompression was conducted. The dog responded well with ventriculo-peritoneal shunting. MRI is the most superior modality to diagnose hydrocephalus and hydrosyringomyelia, to plan therapy and to determine the prognosis.