Arnold-Chiari deformity

PURPOSE: To describe curve patterns in patients with Chiari malformation I (CIM) without syringomyelia, and compare to patients with Chiari malformation with syringomyelia (CIM + SM). METHODS: Review of medical records from 2000 to 2013 at a single institution was performed to identify CIM patients with scoliosis. Patients with CIM were matched (1:1) by age and gender to CIM + SM. Radiographic curve patterns, MRI-based craniovertebral junction parameters, and associated neurological signs were compared between the two cohorts...

BACKGROUND CONTEXT: Chiari malformation, Type 1, with syringomyelia (CIM+SM) is often associated with spinal deformity. The safety of scoliosis surgery this population is controversial and has never been directly compared with adolescent idiopathic scoliosis (AIS). PURPOSE: The purpose of this study was to compare the safety and subjective outcomes of spinal deformity surgery between patients with Chiari malformation Type 1-associated scoliosis and a matched AIS cohort...

Patients with multisutural craniosynostosis can develop anomalous venous connections between the intracranial sinuses and cutaneous venous system through enlarged emissary veins. Cranial vault remodeling in this subset of patients carries the risk of massive intraoperative blood loss and/or occlusion of collateral draining veins leading to intracranial venous hypertension and raised intracranial pressure, increasing the morbidity of cranial expansion. The authors report the use of spring-mediated expansion as a technique for cranial reconstruction in which the collateral intracranial venous drainage system can be preserved...

A subset of patients with Chiari Type I malformation may develop neurological dysfunction secondary to an abnormally obtuse clivoaxial angle (CXA) and clivoaxial deformity causing deformative stress injury to the neural axis. Clivoaxial deformity can occur after initial standard suboccipital craniectomy, duraplasty, and C-1 laminectomy for brainstem compression, or severe clivoaxial deformity may be present in conjunction with a Chiari malformation. Clivoaxial deformity and abnormal CXA can be treated with an occipitocervical fusion (OCF)...

PURPOSE: Chiari malformation type I (CMI) is characterized by deformed hindbrain. This study aimed to quantitatively evaluate the alterations in position of hindbrain after Posterior fossa decompression (PFD), and to identify the factors associated with syrinx resolution in pediatric patients with CMI. METHODS: Eighty-seven patients, aged from 5 to 18 years, who underwent PFD for CMI between September 2006 and September 2012 were retrospectively reviewed. On mid-sagittal MR images, the position of medulla oblongata and cerebellum was quantitatively evaluated preoperatively and at follow-up...

Spinal deformity is an important clinical manifestation of Chiari I malformation (CM-I) and syringomyelia. Here we report the result of an 8-year follow-up of a 13-year-old girl with severe scoliosis associated with Chiari malformation and a large syringomyelia. The patient presented at our hospital at the age of 13 with a 68° scoliosis. Magnetic resonance imaging showed Chiari malformation and a large syringomyelia. Neurosurgical treatment involved foramen magnum decompression and partial C1 laminectomy, but the scoliosis still progressed...

of morphological and tomographic (CT) studies of the skull that was found in the crypt of the Silesian Piasts in the St. Jadwiga church in Brzeg (Silesia, Poland) are presented and discussed here. The established date of burial of probably a 20-30 years old male was 16th-17th century. The analyzed skull showed premature obliteration of the major skull sutures. It resulted in the braincase deformation, similar to the forms found in oxycephaly and microcephaly. Tomographic analysis revealed gross pathology. Signs of increased intracranial pressure, basilar invagination and hypoplasia of the occipital bone were observed...

A 48-year-old man was admitted for the management of congenital anomalies: Arnold-Chiari type I malformation combined with odontoid upward migration. He also had degenerative stenosis of the spinal canal by spurs at C2/C3 and C3/C4 levels. Osseous deformities caused ischaemic changes of the brainstem as well as spinal cord compression. Authors used the Biocage - interbody cage covered by bioresorbable layer to fill the surgically created gap after removal of the right part of C3 vertebral body. Twenty-seven months after implantation, the implant was extruded through posterior pharyngeal wall...

OBJECT: Chiari malformation Type I (CM-I) is a developmental abnormality often associated with a spinal syrinx. Patients with syringomyelia are known to have an increased risk of scoliosis, yet the influence of specific radiographically demonstrated features on the prevalence of scoliosis remains unclear. The primary objective of the present study was to investigate the relationship of maximum syrinx diameter and tonsillar descent to the presence of scoliosis in patients with CM-I-associated syringomyelia...

BACKGROUND CONTEXT: Acute correction of severe spinal deformities significantly increases the risk of neurologic complications. Previously used methods to safely correct these deformities include halo-traction devices and internal distraction rods. PURPOSE: This report introduces a novel method for gradual correction of severe spinal deformity by using the magnetically controlled growing rod (MCGR). STUDY DESIGN: This is a case report. METHODS: A 12-year-old girl with severe kyphoscoliosis and concurrent syringomyelia and Arnold-Chiari Type I malformation underwent implantation of the MCGR...

Acromesomelic dysplasias are autosomal recessive osteochondrodysplasias. Acromesomelic dysplasia Maroteaux-type (AMDM), also known as St Helena dysplasia, is of two types: The classical and the mild variety. About 50 cases of AMDM have been reported till date, most of them being the classical variety. There is scarcity of literature on anesthesia for such patients. We are reporting a case of general anesthetic management of AMDM, associated with hydrocephalus, Arnold Chiari malformation type-1 and syringomyelia...

Fixation at the craniovertebral junction (CVJ) is necessary in a variety of pediatric clinical scenarios. Traditionally an occipital bone to cervical fusion is preformed, which requires a large amount of hardware to be placed on the occiput of a child. If a patient has previously undergone a posterior fossa decompression or requires a decompression at the time of the fusion procedure, it can be difficult to anchor a plate to the occipital bone. The authors propose a technique that can be used when faced with this difficult challenge by using the occipital condyle as a point of fixation for the construct...

We report Ogilvie's syndrome following posterior spinal arthrodesis on a patient with thoracic and lumbar scoliosis associated with intraspinal anomalies. Postoperative paralytic ileus can commonly complicate scoliosis surgery. Ogilvie's syndrome as a cause of abdominal distension and pain has not been reported following spinal deformity correction and can mimic post-surgical ileus. 12 year old female patient with double thoracic and lumbar scoliosis associated with Arnold-Chiari 1 malformation and syringomyelia...

OBJECT: Phase contrast-cine MRI (PC-cine MRI) studies in patients with syringomyelia and Chiari malformation Type I (CM-I) have demonstrated abnormal CSF flow across the foramen magnum, which can revert to normal after craniocervical decompression with syrinx shrinkage. In order to investigate the mechanisms leading to postoperative syringomyelia shrinkage, the authors studied the hydrodynamic changes of CSF flow in the craniocervical junction and spinal canal in patients with scoliosis associated with syringomyelia after one-stage deformity correction by posterior vertebral column resection...

PURPOSE: Rates of neural axis abnormalities in infantile and juvenile idiopathic scoliosis may be as high as 50 %. We aimed to determine the rates of neural axis abnormalities in early onset idiopathic scoliosis patients in a British population. METHODS: This retrospective study at two British spinal deformity clinics identified 72 patients satisfying the inclusion criteria of: (1) age at diagnosis of 7 years and younger, (2) idiopathic scoliosis and (3) magnetic resonance imaging of the neuraxis...

PURPOSE: This study sought to quantify the frequency of previously unidentified spinal cord anomalies identified by routine preoperative magnetic resonance imaging (MRI), in patients planned for surgical scoliosis correction. METHODS: Our study group comprised 206 patients with idiopathic scoliosis who underwent deformity correction from 1998 to 2008. Clinical records of all the patients were retrospectively reviewed to ascertain the proportion having a neural abnormality on preoperative MRI scan...

OBJECT: Chiari malformation Type I (CM-I) is characterized by hindbrain deformity. We investigated the effects of craniocervical decompression surgery on the anatomical features of hindbrain deformity with a prospective MRI study of patients with CM-I. METHODS: A prospective longitudinal study was conducted in 48 patients with CM-I (39 with syringomyelia) treated with craniocervical decompression. Clinical examinations and cervical MRI were performed before surgery and 1 week, 3-6 months, and annually after surgery...

BACKGROUND: Knowledge of the occurrence of malformations of cortical development (MCDs) and its relationship to hindbrain deformity in Chiari II malformation (CIIM) is limited. OBJECTIVE: To assess malformations of cortical development and its relationship to hindbrain deformity regarding Chiari II malformation. MATERIALS AND METHODS: Brain and cervical spinal MRI from 66 children (age range, 1-256 days; mean age, 22.3 days) with Chiari II malformation were retrospectively reviewed...

Myelomeningocele (MMC), one of the most common congenital malformations, can result in severe lifelong disabilities, including paraplegia, hydrocephalus, Arnold-Chiari II malformation, incontinence, sexual dysfunction, skeletal deformations, and mental impairment. MMC was the first nonlethal anomaly to be treated by fetal surgery. Studies in animals provide compelling evidence that the primary cause of the neurological deficit associated with MMC is not simply incomplete neurulation but rather chronic mechanical injury and amniotic-fluid-induced chemical trauma that progressively damage the exposed neural tissue during gestation...

Poland syndrome is characterized by hypoplastic unilateral chest wall structures. These chest wall deformities may be associated with upper extremity anomalies. The association of Poland syndrome with either intercostal liver herniation or a spinocerebral deformity has been described, but there is no report of both findings encountered simultaneously. This is the first report of a newborn child with Poland syndrome associated with an intercostal liver segment herniation and thoracic myelomeningocele with features of an Arnold-Chiari II cerebral malformation...