Background: Delayed Chiari malformation with syringomyelia is an uncommon and sparsely reported complication after lumbar cisternal shunting. A number of treatments have been implemented with varying degrees of success. After review of these modalities, a treatment plan was devised and implemented.
Case Description: A 15-year-old girl was diagnosed with idiopathic intracranial hypertension (pseudotumor cerebri) and had placement of a Medtronic Spetzler lumboperitoneal shunt. She did well for 10 years and then developed a 3-week history of headache, balance problems, and sensory loss. Workup demonstrated a new Chiari malformation and cervical syrinx. She underwent initial placement of a ventriculoperitoneal shunt, followed by ligation of the lumboperitoneal shunt. She then had a suboccipital decompression and duraplasty. Follow-up at 6 months showed relief of her symptoms and MRI demonstrated resolution of the Chiari malformation and syrinx.
Conclusion: Delayed Chiari malformation and/or syringomyelia after lumbar shunting is best treated with placement of a ventricular device and ligation of the lumbar shunt. The Chiari malformation and/or syrinx should be surgically addressed for persistent symptoms or progression of the structural abnormality.
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http://dx.doi.org/10.4103/2152-7806.100369 | DOI Listing |
Brain Spine
November 2024
Neurosurgical Department, "KAT" General Hospital of Athens, Greece.
Introduction: Klippel-Trenaunay Syndrome (KTS) is a rare congenital condition characterized by vascular malformations, bone abnormalities, and limb overgrowth. The genetic basis of KTS is not fully understood, and the diagnosis relies on clinical features. Its clinical spectrum includes several neurosurgical diagnoses, such as cavernous hemangiomas, arteriovenous fistulas, and Chiari I malformation.
View Article and Find Full Text PDFFront Neurol
December 2024
Neuromuscular Imaging Research Lab, The Kolling Institute, North Sydney Local Health District, St Leonards, NSW, Australia.
Individuals with joint hypermobility and the Ehlers-Danlos Syndromes (EDS) are disproportionately affected by neuraxial dysfunction and Central Nervous System (CNS) disorders: such as Spontaneous Intracranial Hypotension (SIH) due to spinal cerebrospinal fluid (CSF) leaks, Upper Cervical Instability (UCI; including craniocervical or atlantoaxial instability (CCI/AAI)), Occult Tethered Cord Syndrome (TCS), Chiari Malformation (CM) and Idiopathic Intracranial Hypertension (IIH). The neuraxis comprises the parts of the nervous system (brain, nerves, spinal cord) along the craniospinal axis of the body. Neuraxial tissue includes all tissue structures that comprise, support, sheath, and connect along the neuraxis and peripheral nerves.
View Article and Find Full Text PDFJ Neurosurg Case Lessons
December 2024
Department of Neurosurgery, The Jikei University School of Medicine, Tokyo, Japan.
Background: Postoperative subdural hematoma (SDH) typically presents with headache, impaired consciousness, hemiplegia, gait disturbance, and aphasia but can also present with visual impairment.
Observations: A 52-year-old woman diagnosed with Chiari malformation type I and syringomyelia underwent foramen magnum decompression. Cerebrospinal fluid was lost due to arachnoid injury during the procedure.
Pak J Med Sci
December 2024
Dr Mahwish Shoaib, FCPS Radiology. Assistant Professor, Department of Diagnostic Radiology, Punjab Institute of Neurosciences, Lahore, Pakistan.
Background And Objective: Chiari I Malformation-associated syringomyelia (CM) and idiopathic syringomyelia (IS) are often confused together. They require different diagnostic approach and treatment modalities; it is important to distinguish between the two. We aimed to evaluate the radiological and morphologic characteristics of CM and IS in adult and pediatric patients in Pakistani population.
View Article and Find Full Text PDFJ Evid Based Med
December 2024
School of International Pharmaceutical Business, China Pharmaceutical University, Nanjing, Jiangsu, China.
Objective: This study aims to investigate the occurrence of adverse events associated with topiramate by analyzing data from the FDA Adverse Event Reporting System. The goal is to provide a basis for the safe clinical use of topiramate.
Methods: Adverse event data from the FDA Adverse Event Reporting System, from its inception through the first quarter of 2024, were extracted.
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