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

• Journal of Korean Neurosurgical Society
• /
• v.54 no.5
• /
• pp.405-410
• /
• 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
• /
• v.59 no.5
• /
• pp.512-517
• /
• 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
• /
• v.66 no.4
• /
• pp.393-399
• /
• 2023

Objective : Chiari II malformation (CM II) is still the main cause of severe morbidity and mortality in children with open neural tube defects (ONTDs). The goal of this study was to validate a CM II model in late-stage chick embryos with surgically induced ONTDs. Methods : To make the chick embryo model of ONTD, their neural tubes were opened for a length of 5-6 somites at the thoracic level in Hamburger and Hamilton stage 18 chick embryos (n=150). They were reincubated in ovo up to a total age of 17-21 days. A total of 19 embryos survived and were assigned to either the postoperative day (POD) 14-15 group (n=6) or the POD 17-18 group (n=13). Magnetic resonance imaging (MRI) and histopathologic findings of embryo heads with spinal ONTDs were compared with age-matched normal chick embryos. Results : The chick embryos with ONTDs demonstrated definite and constant structural changes, such as downward displacement of the cerebellum to just above the foramen magnum and narrow and small cerebrospinal fluid spaces in the crowded small posterior fossa. These morphologic features were more prominent in the POD 17-18 group than in the POD 14-15 group. Conclusion : This is the first description of CM II with spinal ONTD in a late-stage chick embryo model with MRI and histopathological analysis. The morphological changes of the posterior fossa in this study mimic those of CM II associated with spinal ONTD in humans. This model will facilitate investigation of the pathogenesis of CM II.

• Journal of Korean Neurosurgical Society
• /
• v.65 no.6
• /
• pp.841-845
• /
• 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 the Korean Orthopaedic Association
• /
• v.55 no.6
• /
• pp.534-539
• /
• 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.

• Perinatology
• /
• v.29 no.4
• /
• pp.185-188
• /
• 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 Veterinary Clinics
• /
• v.34 no.6
• /
• pp.449-453
• /
• 2017

A 3-year-old castrated male Maltese dog, weighing 4.8 kg was referred with hindlimb ataxia and right forelimb proprioceptive deficits were shown for 20 months. Chiari-like malformation and syringomyelia diagnosed through MRI at a local animal hospital and Knuckling of right forelimb and reluctance to walk were managed with steroid. The medical management was getting ineffective to manage for the symptoms one month before referred. Physical and neurological examinations, radiography, computed tomography, and magnetic resonance imaging were performed and diagnoses of caudal occipital malformation syndrome (COMS) and subsequent syringomyelia (SM) were made. Given that pharmacological treatment was previously ineffective, surgical intervention was recommended. Foramen magnum decompression with duraplasty using $Lyoplant^{(R)}$ was performed. Three days post-surgery, the dog showed improved gait and activity. After 2 months, the dog received no additional prescription medications. At the 12-month follow-up after surgery, the dog showed no clinical problems or recurrences, despite complete cessation of pharmacological treatment. In present report, we applied $Lyoplant^{(R)}$ as a dural graft has been carried out in a dog with COMS. Surgical decompression with $Lyoplant^{(R)}$ was an effective long-term (12-month) treatment for COMS without the need for any pharmacological treatment.

• Journal of Korean Neurosurgical Society
• /
• v.40 no.3
• /
• pp.169-174
• /
• 2006

Objective : This study was conducted to examine the correlation between clinical outcomes and the size of the syrinx in post-operative magnetic resonance imaging[MRI] and symptom duration. Methods : The authors investigated twelve patients who underwent various operations for syringomyelia from January 1995 to December 2003. The authors retrospectively analyzed medical records. pre- and post-operative MRI findings, features and durations of symptoms, and the method of surgical treatments. The clinical outcomes were assessed on Prolo scale at 6 months of post-operative period. Results : Neurologic symptoms did not promptly disappear after the shrinkage of syrinx, but post-operative MRI demonstrated most patients showed reductions in the size of the syrinx. There is no statistical relationship between clinical improvements and decrements of the syrinx size. However, patients who underwent surgical treatment within 2 years from the symptom onset had more favorable outcome than those who had operations after 2 years from the onset of symptoms. Conclusion : Change in the size of the syrinx in post-operative MRI is not directly proportional to favorable clinical outcomes. However, symptom duration before surgical treatment has considerable impact on the clinical outcomes.

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