• Journal of Yeungnam Medical Science
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• v.9 no.1
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• pp.203-209
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• 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
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• v.40 no.1
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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.

• Clinical and Experimental Pediatrics
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• v.53 no.6
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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 the korean academy of Pediatric Dentistry
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• v.28 no.4
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• pp.649-653
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• 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.

• 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.

• Perinatology
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• v.29 no.4
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• pp.185-188
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• 2018

Jarcho-Levin syndrome is a congenital disorder characterized by several vertebral and costal anomalies. Other abnormalities have also been described, including neural tube defects, Arnold-Chiari malformation, renal/urinary tract abnormalities, hydrocephalus, hydroureteronephrosis, and meningomyelocele. We describe a spondylocostal dysplasia form of Jarcho-Levin syndrome that was prenatally diagnosed at 11 weeks of gestation and surviving. Although the patient had sporadic-type Jarcho-Levin syndrome, with normal karyotype and no family history of disease, the assessment of inheritance patterns and genetic counseling for the parents was important to inform them about the potential risks.

• Journal of the Korean Orthopaedic Association
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• v.55 no.6
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• pp.534-539
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• 2020

The management of severe scoliosis remains a challenge to spine surgeons. The rapid intraoperative correction of severe scoliosis may increase the risk of perioperative complications, such as neurological compromise and implant failure. To minimize these risks, various preoperative traction methods have been employed to achieve partial correction before performing definitive corrective surgery. On the other hand, some studies have shown that one of the complications associated with halo traction could lead to cranial nerve palsy, with the sixth nerve (abducens nerve) being most commonly affected. To reduce the complications, gradual increases in the traction weight and detailed neurological examinations are needed, particularly for patients who have previously undergone brain or cervical surgery. The authors report a case of sixth cranial nerve palsy by preoperative halo-pelvic traction in patients with severe scoliosis who underwent previous decompression surgery for a Chiari I malformation with a review of the relevant literature.

• 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.