• Journal of Korean Neurosurgical Society
• /
• v.48 no.4
• /
• pp.375-379
• /
• 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
• /
• v.40 no.1
• /
• pp.38-39
• /
• 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.

• The Journal of the Korean life insurance medical association
• /
• v.27 no.1
• /
• pp.37-38
• /
• 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
• /
• v.23 no.2
• /
• pp.91-95
• /
• 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 Yeungnam Medical Science
• /
• v.9 no.1
• /
• pp.203-209
• /
• 1992

The Arnold-Chiari malformation(ACM) is an anomaly of the hindbrain consisting of two components : a variable displacement of a tongue of tissue derived from the inferior cerbellar vermis in the upper cervical cannal and a similar caudal dislocation of the medulla and fourth ventricle. Hydrocephalus and meningomyelocele are another abnormalities that associated frequently. We present a case of Arnold-Chiari malformation with hydrocephalus and meningomyeolcele and a brief review of the literatures was added.

• Journal of Korean Neurosurgical Society
• /
• v.57 no.4
• /
• pp.311-313
• /
• 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.

• Journal of the korean academy of Pediatric Dentistry
• /
• v.28 no.4
• /
• pp.649-653
• /
• 2001

The Chiari malformation is a deformation within the central nervous system which the lower brain stem and the cerebellum migrate into the foramen magnum causing herniation. In 1891, Arnold Chiari classified such symptoms into 3 categories. This case report is of a 8-year-old female with the complaint of a slight facial swelling and pain on the upper right molar during tooth brushing since 10 days before. Clinical examination showed gingival pocket formation on distal of the upper right first molar with pain and mobility of the tooth. Radiographic examination showed generalized low bone density in the upper molar area, and especially no bone support above the upper right and left first molars were noted. With a temporary diagnosis of Early-onset periodontitis, consultations with medical doctors for the possibility of an underlying systemic disease were made during periodontal treatment. 3D CT was taken with after a final diagnosis of Chiari malformation. Generalized thinning and defect of the cranial bone was noted and the foramen magnum was slightly enlarged. The occipital and maxillary bone was low in density, and the alveolar bone of maxillary posterior teeth was especially almost non-existing causing the upper right and left first molar to be floating. For this, the patient went under consultation with the department of neurosurgery and is still under observation. Periodontitis in childreren is very rare. When symptoms of periodontitis appear in a child, due to the possibility of an underlying systemic disease such as leukemia, histiocytosis X, and hypophosphatasia, proper examinations should be carried out so that the primary factor the symptoms can be treated.

• Clinical and Experimental Pediatrics
• /
• v.59 no.sup1
• /
• pp.149-151
• /
• 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
• /
• v.53 no.6
• /
• pp.718-721
• /
• 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
• /
• v.59 no.3
• /
• pp.197-203
• /
• 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.