A prospective study was undertaken in 1985 to better understand how the surgical manipulation of hindbrain herniation affected abnormal spinal curvature. Eleven patients under 16 years of age with Chiari malformation (not associated with myelodysplasia) and scoliosis of at least 15 degrees were studied. The mean curve angle at the time of original treatment was 29 degrees, with the convexity to the right in seven patients. The curvature was rapidly progressing in four patients. The most common presenting signs were myelopathy and weakness. Investigative procedures included spine radiographs with the patient standing and magnetic resonance (MR) imaging of the brain, spinal cord, and craniovertebral junction. Eight children had associated hydrosyringomyelia. Surgical intervention consisted of a dorsal posterior fossa decompression in all patients and a transoral ventral decompression of the cervicomedullary junction in five. All patients were followed at 3, 6, and 12 months, and at yearly intervals thereafter with clinical evaluations, spine radiographs in the standing position, and postoperative MR imaging. The mean follow-up period was 35 months. The scoliosis improved in eight patients, stabilized in one, and progressed in two. Only one child required postoperative spinal fusion and instrumentation for progression of scoliosis. Hematomyelia or hematobulbia was associated with persistent scoliosis in two patients. The presence of hydrosyringomyelia and bone erosion did not preclude curve improvement. All patients under 10 years of age had resolution of their scoliosis, despite preoperative curves of more than 40 degrees. These findings emphasize the importance of early surgical intervention, with the restoration of normal cerebrospinal fluid dynamics at the craniovertebral junction in children with symptomatic Chiari malformations.
Download full-text PDF |
Source |
---|---|
http://dx.doi.org/10.3171/jns.1992.77.1.0069 | DOI Listing |
A A Pract
January 2025
Département d'Anesthésie, Hôpital Antoine Béclère, APHP.Université Paris-Saclay, Clamart, France.
We describe a patient with severe Arnold Chiari Malformation and syringomyelia who underwent gynecological laparoscopy in an emergency context; no brain imaging was available. We here report the successful use of optic nerve sheath diameter (ONSD) and middle cerebral artery (MCA) velocity measurements as surrogate monitoring for cerebral blood flow and intracranial pressure, respectively. MCA velocity was low when assessed after peritoneal insufflation and ONSD increased to 6.
View Article and Find Full Text PDFMed Sci Monit
January 2025
Department of Neurosurgery, Istanbul Training and Research Hospital, Istanbul, Turkey.
BACKGROUND Chiari malformation type 1 occurs when the cerebellar tonsils are pushed into the spinal canal, which can result in syringomyelia. This retrospective study from a single center evaluated outcomes in 89 patients with Chiari malformation type-I (CM-I) and syringomyelia treated with an arachnoid-preserving technique between 2016 and 2023. MATERIAL AND METHODS A retrospective analysis was conducted at a tertiary referral center, involving 88 adult patients and 1 adolescent patient aged 14 to 61 years, with diagnosis by MRI and treated for CM-I with syringomyelia between 2016 and 2023, using the arachnoid-preserving technique.
View Article and Find Full Text PDFChilds Nerv Syst
January 2025
Department of Global Health, Faculty of Health Sciences, McMaster University, 1280 Main St W, Hamilton, ON, L8S 4L8, Canada.
Background: A giant encephalocele associated with Chiari malformation is a rare congenital anomaly from a cephalad neural tube defect. Early prenatal diagnosis and parental counseling are essential; as early surgical intervention can improve outcomes.
Methods: Between 2010 and 2023, twenty-seven newborns out of 43,815 delivered at our institution were diagnosed with encephaloceles, including seven cases of giant encephalocele associated with Chiari malformation type III.
Objective: While the association of a syrinx with a tethered spinal cord in the context of VACTERL (vertebral defects [V], imperforate anus or anal atresia [A], cardiac malformations [C], tracheoesophageal defects [T] with or without esophageal atresia [E], renal anomalies [R], and limb defects [L]) association is known, the incidence of idiopathic syrinxes among these patients has not previously been reported. The authors aimed to characterize the incidence of syrinxes and the pattern of congenital anomalies in pediatric patients with VACTERL association, with a specific focus on the presence of idiopathic syrinxes in this population.
Methods: An institutional database was retrospectively queried for all pediatric patients with VACTERL association.
Brain Sci
December 2024
Department of Neurosurgery, IRCSS Humanitas Research Hospital, Via Alessandro Manzoni 56, 20089 Rozzano, Milan, Italy.
Background: Congenital craniovertebral junction anomalies (CCVJAs) encompass a diverse range of conditions characterized by distorted anatomy and significant variation in the pathways of neurovascular structures. This study aims to assess the safety and feasibility of tailoring posterior fixation for CCVJAs through intraoperative CT-based navigation.
Methods: An in-depth retrospective analysis was conducted on eight patients diagnosed with CCVJAs (excluding Arnold-Chiari malformation).
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!