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syrinx development

Elizabeth C Clarke, David F Fletcher, Lynne E Bilston
Syringomyelia (a spinal cord cyst) usually develops as a result of conditions that cause cerebrospinal fluid (CSF) obstruction. The mechanism of syrinx formation and enlargement remains unclear, though previous studies suggest that the fluid enters via the perivascular spaces (PVS) of the penetrating arteries of the spinal cord, and that alterations in the CSF pulse timing and pressure could contribute to enhanced PVS inflow. This study uses an idealised computational model of the PVS to investigate the factors that influence peri-arterial fluid flow...
October 7, 2016: Computer Methods in Biomechanics and Biomedical Engineering
Kristiina Kyrklund, Mikko P Pakarinen, Seppo Taskinen, Reetta Kivisaari, Risto J Rintala
OBJECTIVE The goal of this study was to determine the significance of spinal cord anomalies (SCAs) in patients with anorectal malformations (ARMs) by comparing the outcomes for bowel function, lower urinary tract symptoms (LUTS), and lower-limb neurological abnormalities to these outcomes in patients with similar ARMs and a normal spinal cord. METHODS The spinal cord MRI records of female patients treated for vestibular and perineal fistula (VF/PF) and male patients with rectourethral fistula (RUF) at a single center between 1983 and 2006 were reviewed...
July 22, 2016: Journal of Neurosurgery. Spine
Tatsuya Yasuda, Tomohiko Hasegawa, Yu Yamato, Sho Kobayashi, Daisuke Togawa, Tomohiro Banno, Hideyuki Arima, Shin Oe, Yukihiro Matsuyama
STUDY DESIGN: Retrospective case series. PURPOSE: To investigate the relationship between tumor location and clinical characteristics. OVERVIEW OF LITERATURE: Hemangioblastoma is a rare disease that develops in the central nervous system. Magnetic resonance imaging (MRI) is useful to evaluate hemangioblastomas. Hemangioblastoma's location is designated as intramedullary, intramedullary+extramedullary, or extramedullary by MRI. METHODS: We analyzed 11 patients who underwent surgery for spinal hemangioblastoma...
April 2016: Asian Spine Journal
Albert Tu, Alexander R Hengel, D Douglas Cochrane
OBJECTIVE Although patients with lumbosacral lipomas may be asymptomatic at presentation, most develop neurological symptoms over time. Given the challenges in examining infants, the authors sought to determine whether MRI would be helpful in identifying patients who are more likely to deteriorate early in life and who would potentially benefit from early surgical intervention. METHODS A retrospective review of all patients with lumbosacral lipomas who were seen at the authors' institution between 1997 and 2013 and who were managed without prophylactic surgery was performed...
August 2016: Journal of Neurosurgery. Pediatrics
Devorah Segal, Robert F Heary, Sanjeev Sabharwal, Maureen T Barry, Xue Ming
The authors present the case of a child with megalencephaly-capillary malformation syndrome who developed a rapidly progressive holocord syringomyelia that was treated surgically. A 3-year-old boy with megalencephaly-capillary malformation-polymicrogyria (MCAP) syndrome presented with several months of right leg weakness, worsening scoliosis, and increased seizures. An MRI study of the brain demonstrated a Chiari I malformation and massively dilated syringomyelia extending from C-2 to the conus medullaris. The patient underwent an urgent suboccipital craniectomy with C1-3 laminectomies to relieve the CSF outflow obstruction with significant clinical improvement...
July 2016: Journal of Neurosurgery. Pediatrics
Johnny H Y Wong, Xin Song, Sarah J Hemley, Lynne E Bilston, Shaokoon Cheng, Marcus A Stoodley
OBJECTIVE The pathogenesis of posttraumatic syringomyelia remains enigmatic and is not adequately explained by current theories. Experimental investigations require a reproducible animal model that replicates the human condition. Current animal models are imperfect because of their low reliability, severe neurological deficits, or dissimilar mechanism of injury. The objective of this study was to develop a reproducible rodent model of posttraumatic syringomyelia using a spinal cord impactor that produces an injury that more closely mimics the human condition and does not produce severe neurological deficits...
May 2016: Journal of Neurosurgery. Spine
Gurpreet S Gandhoke, Sabri Yilmaz, Lorelei Grunwaldt, Ronald L Hamilton, David J Salvetti, Stephanie Greene
While spinal epidural arteriovenous malformations, fistulas, and shunts are well reported, the presence of a venous malformation in the spinal epidural space is a rare phenomenon. Herein, the authors report the clinical presentation, imaging findings, pathological features, and the outcome of surgical and percutaneous interventional management of a mediastinal and spinal epidural venous malformation in a young woman who presented clinically with neurogenic claudication from presumed venous hypertension precipitating the formation of a syrinx...
May 2016: Journal of Neurosurgery. Pediatrics
E Najafi, M A Stoodley, L E Bilston, S J Hemley
Post-traumatic syringomyelia (PTS) is a serious neurological disorder characterized by fluid filled cavities that develop in the spinal cord. PTS is thought to be caused by an imbalance between fluid inflow and outflow in the spinal cord, but the underlying mechanisms are unknown. The ion channel Kir4.1 plays an important role in the uptake of K(+) ions from the extracellular space and release of K(+) ions into the microvasculature, generating an osmotic gradient that drives water movement. Changes in Kir4...
March 11, 2016: Neuroscience
Fawaz S Almotairi, Magnus Tisell
BACKGROUND: Cerebrospinal fluid disturbance (CSFD) is a well-known complication after occipitocervical decompression (OCD) in patients with Chiari malformation type I (CMI). There is scarce data focusing on preoperative patients' factors predisposing to development of CSF disturbance. The aim of this study is to evaluate a prognostic value of some patients' factors in the prediction of CSFD after OCD in CMI patients. MATERIALS AND METHODS: We undertook a 10-year (2003-2013) retrospective study of all OCD in patients with CMI performed at Sahlgrenska IC, Sahlgrenska University Hospital, Sweden...
March 2016: Acta Neurochirurgica
Liyong Sun, Stephan Emich, Wenzhuo Fu, Zan Chen, Wu Hao, Feng Ling, Fengzeng Jian
A retrocerebellar arachnoid cyst causing syringomyelia is extremely rare without tonsillar herniation. The authors present a 44-year-old woman with symptoms of foramen magnum compression and syringomyelia. Magnetic resonance imaging demonstrated a large retrocerebellar arachnoid cyst with a large cervicothoracic syrinx but no signs of tonsillar herniation or hydrocephalus. The patient underwent a foramen magnum decompression with C1 laminectomy, microsurgical fenestration of the cyst, and duraplasty. After successful reconstruction of CSF flow, the patient experienced a relief of symptoms and a significant reduction of the syrinx...
April 2016: Neurosurgical Review
Claudio De Tommasi, Aaron E Bond
BACKGROUND: Pseudomeningocele is a recognised complication after posterior fossa decompression for Chiari malformation. Its management can be challenging and treatment options vary in literature. CASE DESCRIPTION: A difficult-to-treat case of a pseudomeningocele after posterior fossa decompression for a Chiari I malformation is presented. A 34-year-old woman underwent an initial decompression followed by multiple revision surgeries after the development of a symptomatic pseudomeningocele and a low-grade infection...
April 2016: World Neurosurgery
Zezhang Zhu, Huang Yan, Xiao Han, Mengran Jin, Dingding Xie, Shifu Sha, Zhen Liu, Bangping Qian, Feng Zhu, Yong Qiu
STUDY DESIGN: A retrospective radiographic analysis. OBJECTIVE: The aim of this study was to investigate the scoliosis curve patterns/features and magnetic resonance imaging (MRI) tonsillar ectopia characteristics in Chiari I malformation without syringomyelia (CMI-only). SUMMARY OF BACKGROUND DATA: The development of scoliosis associated with a CMI typically has been ascribed to the presence of syringomyelia. However, a subset of CMI patients with scoliosis may not have a concomitant syrinx...
March 2016: Spine
Matthew T Bender, Geoffrey P Colby, Judy Huang
Hydrocephalus has varied presentations in patients with a history of spinal dysraphism. This is a unique case of post-subarachnoid hemorrhage hydrocephalus presenting as cauda equina syndrome. We report on a 32-year-old woman with remotely repaired spinal defect who experienced subarachnoid hemorrhage and underwent anterior communicating artery aneurysm clipping. Post-operatively, she developed urinary and fecal incontinence as the sole presenting symptom of communicating post-hemorrhagic hydrocephalus. New neurological deficits in this population can also be attributed to recurrent cord tethering or syrinx, both of which were demonstrated on her lumbar spine MRI, but her incontinence resolved with external ventricular drain placement and cerebrospinal fluid diversion...
April 2016: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
I Jonathan Pomeraniec, Alexander Ksendzovsky, Ahmed J Awad, Francis Fezeu, John A Jane
OBJECT The natural and surgical history of Chiari malformation Type I (CM-I) in pediatric patients is currently not well described. In this study the authors discuss the clinical and radiological presentation and outcomes in a large cohort of pediatric CM-I patients treated with either conservative or surgical management. METHODS The authors retrospectively reviewed 95 cases involving pediatric patients with CM-I who presented between 2004 and 2013. The patients ranged in age from 9 months to 18 years (mean 8 years) at presentation...
March 2016: Journal of Neurosurgery. Pediatrics
A Thompson, N Madan, J R Hesselink, G Weinstein, A Munoz del Rio, V Haughton
BACKGROUND AND PURPOSE: The cause of syringomyelia in patients with Chiari I remains uncertain. Cervical spine anatomy modifies CSF velocities, flow patterns, and pressure gradients, which may affect the spinal cord. We tested the hypothesis that cervical spinal anatomy differs between Chiari I patients with and without syringomyelia. MATERIALS AND METHODS: We identified consecutive patients with Chiari I at 3 institutions and divided them into groups with and without syringomyelia...
April 2016: AJNR. American Journal of Neuroradiology
K Santosh Mohan Rao, Chidambaram Balasubramaniam, K Subramaniam
Syringohydromyelia is a frequent finding in cases of tethered cord syndrome. The classical teaching is that the development and progression of a syrinx is a chronic process. We present a case report of an acute onset syringomyelia in an infant, who underwent an excision of a lumbosacral transitional lipoma and detethering of the cord. Immediately after recovery, the infant was found to have flaccid paraplegia. An emergency magnetic resonance imaging revealed a large acute onset syringomyelia for which he underwent an emergency midline myelotomy and release of fluid from the syrinx...
July 2015: Journal of Pediatric Neurosciences
Anne Kathleen Ganal-Antonio, Dino Samartzis, Cora Bow, Kenneth M C Cheung, Keith D K Luk, Yat-Wa Wong
BACKGROUND CONTEXT: Disappearing bone disease (DBD) is a rare idiopathic musculoskeletal disorder that is distinguished by bone resorption without bone formation, vascular or lymphatic vessel proliferation, and soft-tissue swelling. Long-term follow-up of a patient with DBD has rarely been reported in the literature. PURPOSE: The following is a case report of a female patient with DBD of the humerus and the spine who was followed for 42 years, documenting the progression of the disease and outcomes...
February 2016: Spine Journal: Official Journal of the North American Spine Society
Thomas J Mitchell, Susan P Knowler, Henny van den Berg, Jane Sykes, Clare Rusbridge
BACKGROUND: Syringomyelia (SM) is a painful neurological condition, prevalent in brachycephalic toy breeds including the Cavalier King Charles Spaniel (CKCS). In these breeds, SM is typically secondary to Chiari-like Malformation (CM). There has been much debate in the scientific and veterinary communities to what extent head shape is indicative of either pathology, especially as certain craniosynostosis syndromes in humans (highly associated with CM) have characteristic facial and cranial morphologies...
2014: Canine Genetics and Epidemiology
Kavya I Rao, John Hesselink, Doris A Trauner
OBJECTIVE: To determine the relative incidence of Chiari I malformations in children with cystinosis compared with those in the general population. STUDY DESIGN: Magnetic resonance imaging scans were performed on 53 patients with nephropathic cystinosis and 120 controls, age range 3-18 years. RESULTS: Ten of 53 (18.9%) patients with cystinosis had Chiari I or tonsillar ectopia, and only 2 of 120 controls (1.6%) had a similar finding. At least 2 of the patients had symptoms or signs thought to be related to the malformation, and one had surgical decompression...
November 2015: Journal of Pediatrics
Mukul Mohindra, V-K Gautam, Lalit Maini, Santosh Kumar, Saurabh Verma
Subacute posttraumatic ascending myelopathy is a rare disorder, unrelated to syrinx formation or mechanical instability, which may gradually emerge within the first 1-2 weeks after a spinal cord injury and may lead to diagnostic and prognostic dilemmas. We present a case of 24-year-old female with unstable wedge compression fracture of L1 vertebrae with signal changes in the upper lumbar cord causing complete paraplegia below D9 with bladder and bowel involvement. In the subsequent week, she developed a delayed progressively increasing neurological deficit with cord signal abnormality on MRI extending cephalad from the injury site to the upper dorsal cord...
2015: Chinese Journal of Traumatology, Zhonghua Chuang Shang za Zhi
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