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Luy DD, Agarwal N, McDowell MM, Tonetti DA, Goldschmidt E, Friedlander RM. Acquired Chiari Type I Malformation Associated with Type IV Dural Arteriovenous Fistula: Case Report. J Neurol Surg A Cent Eur Neurosurg 2024; 85:94-99. [PMID: 35263802 DOI: 10.1055/a-1792-5000] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/18/2022]
Abstract
BACKGROUND Chiari malformations, usually congenital, can rarely be associated with arteriovenous (AV) fistulas. We present the first case involving a type IV dural AV fistula with a Chiari type I malformation. METHODS Retrospective chart review was performed to obtain pertinent details regarding history and examination, pathologic findings, and treatment course. RESULTS A 63-year-old woman with a 2-year history of migraines presented with 5 months of occipital, right-sided headaches and neck pain exacerbated by Valsalva maneuvers. Computed tomography (CT) and magnetic resonance imaging (MRI) of the head showed a possible right occipital AV malformation, bilateral cerebellar subdural hygromas, and tonsillar crowding at the foramen magnum indicating an acquired Chiari type I malformation. Angiography demonstrated a Cognardtype IV right posterior occipital dural AV fistula supplied by bilateral middle meningeal and posterior meningeal arteries. CONCLUSION After treatment of the dural AV fistula, hygroma evacuation, and decompression of the acquired Chiari malformation, the patient's Valsalva-induced headaches abated.
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Affiliation(s)
- Diego D Luy
- Department of Neurological Surgery, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, United States
| | - Nitin Agarwal
- Department of Neurological Surgery, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, United States
| | - Michael M McDowell
- Department of Neurological Surgery, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, United States
| | - Daniel A Tonetti
- Department of Neurological Surgery, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, United States
| | - Ezequiel Goldschmidt
- Department of Neurological Surgery, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, United States
| | - Robert M Friedlander
- Department of Neurological Surgery, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, United States
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Savage C, Hale AT, Parr MS, Hedaya A, Saccomano BW, Tsemo GB, Hafeez MU, Tanweer O, Kan P, Solomon LJ, Meila D, Dirks PB, Blount JP, Johnston JM, Rocque BG, Rozzelle CJ, Bhatia K, Muthusami P, Krings T, Jones J. Outcomes of endovascular embolization for Vein of Galen malformations: An individual participant data meta-analysis. Front Pediatr 2022; 10:976060. [PMID: 36245731 PMCID: PMC9561813 DOI: 10.3389/fped.2022.976060] [Citation(s) in RCA: 9] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/23/2022] [Accepted: 09/15/2022] [Indexed: 11/17/2022] Open
Abstract
Introduction Understanding outcomes after Vein of Galen malformation (VOGM) embolization has been limited by small sample size in reported series and predominantly single center studies. To address these limitations, we perform an individual-participant meta-analysis (IPMA) to identify risk factors associated with all-cause mortality and clinical outcome after VOGM endovascular embolization. Methods We performed a systematic review and IPMA of VOGM endovascular outcomes according to PRISMA guidelines. Individual patient characteristics including demographic, intra/post-operative adverse events, treatment efficacy (partial or complete occlusion), and clinical outcome were collected. Mixed-effects logistic regression with random effects modeling and Bonferroni correction was used (p ≤ 0.003 threshold for statistical significance). The primary and secondary outcomes were all-cause mortality and poor clinical outcome (moderate/severe developmental delay or permanent disabling injury), respectively. Data are expressed as (mean ± standard deviation (SD)) or (odds ratio (OR), 95% confidence interval (CI), I 2, p-value). Results Thirty-five studies totaling 307 participants quantifying outcomes after endovascular embolization for VOGM were included. Follow up time was 42 (±57) months. Our analysis contained 42% neonates (<1 month) at first embolization, 45% infants (1 month ≤2 years), and 13% children (>2 years). Complete occlusion was reported in 48% of participants. Overall all-cause mortality was 16%. Overall, good clinical outcome was achieved in 68% of participants. First embolization as a neonate [OR = 6.93; 95% CI (1.99-24.08); I 2 < 0.01; p < 0.001] and incomplete embolization [OR = 10.87; 95% CI (1.86-63.55); I 2 < 0.01; p < 0.001] were associated with mortality. First embolization as a neonate [OR = 3.24; 95% CI (1.47-7.15); I 2 < 0.01; p < 0.001], incomplete embolization [OR = 5.26; 95% CI (2.06-13.43); I 2 < 0.01; p < 0.001], and heart failure at presentation [OR = 3.10; 95% CI (1.03-9.33); I 2 < 0.01; p = 0.002] were associated with poor clinical outcomes. Sex, angioarchitecture of lesion, embolization approach (transvenous vs. transarterial), and single or multistage embolization were not associated with mortality or clinical outcome. Conclusions We identify incomplete VOGM embolization independently associated with mortality and poor clinical outcome. While this study provides the highest level of evidence for VOGM embolization to date, prospective multicenter studies are needed to understand the optimal treatment strategies, outcomes, and natural history after VOGM embolization.
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Affiliation(s)
- Cody Savage
- Heersink School of Medicine, University of Alabama at Birmingham, Birmingham, AL, United States
| | - Andrew T. Hale
- Department of Neurosurgery, University of Alabama at Birmingham, Birmingham, AL, United States
| | - Matthew S. Parr
- Department of Neurosurgery, University of Alabama at Birmingham, Birmingham, AL, United States
| | - Alexander Hedaya
- Department of Neurology, University of Alabama at Birmingham, Birmingham, AL, United States
| | - Benjamin W. Saccomano
- Department of Neurosurgery, University of Alabama at Birmingham, Birmingham, AL, United States
| | - Georges Bouobda Tsemo
- Department of Neurosurgery, University of Alabama at Birmingham, Birmingham, AL, United States
| | - Muhammad U. Hafeez
- Department of Neurology, Baylor College of Medicine, Houston, TX, United States
| | - Omar Tanweer
- Department of Neurosurgery, Baylor College of Medicine, Houston TX, United States
| | - Peter Kan
- Department of Neurosurgery, University of Texas Medical Branch at Galveston, Galveston, TX, United States
| | - Laurent J. Solomon
- Department of Obstetrics and Fetal Medicine, Paris Descartes University, Assistance Publique-Hôpitaux de Paris, Hôpital Necker Enfants, Paris, France
| | - Dan Meila
- Department of Interventional Radiology, Helois Klinikum Krefeld, Johanna-Etienne Hospital Neuss, Neuss, Germany
| | - Peter B. Dirks
- Division of Pediatric Neurosurgery, The Hospital for Sick Children, Toronto, ON, Canada
| | - Jeffrey P. Blount
- Department of Neurosurgery, University of Alabama at Birmingham, Birmingham, AL, United States
| | - James M. Johnston
- Department of Neurosurgery, University of Alabama at Birmingham, Birmingham, AL, United States
| | - Brandon G. Rocque
- Department of Neurosurgery, University of Alabama at Birmingham, Birmingham, AL, United States
| | - Curtis J. Rozzelle
- Department of Neurosurgery, University of Alabama at Birmingham, Birmingham, AL, United States
| | - Kartik Bhatia
- Department of Medical Imaging, Sydney Children’s Hospital Network, Westmead, NSW, Australia
| | - Prakash Muthusami
- Division of Interventional Radiology, University of Toronto and the Hospital for Sick Children, Toronto, ON, Canada
| | - Timo Krings
- Division of Interventional Radiology, University of Toronto and the Hospital for Sick Children, Toronto, ON, Canada
| | - Jesse Jones
- Department of Neurosurgery, University of Alabama at Birmingham, Birmingham, AL, United States
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Sinha A, Vankipuram S, Ellenbogen J. Management of Chiari 1 malformation and hydrocephalus in syndromic craniosynostosis: A review. J Pediatr Neurosci 2022; 17:S67-S76. [PMID: 36388008 PMCID: PMC9648655 DOI: 10.4103/jpn.jpn_49_22] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/07/2022] [Revised: 04/16/2022] [Accepted: 04/07/2022] [Indexed: 11/26/2022] Open
Abstract
Chiari 1 malformation and hydrocephalus are frequent findings in multi-suture and syndromic craniosynostosis patients. In this article, we review the pathogenesis, clinical significance, and management options for these conditions with comments from our own experience. The role of premature fusion of skull base sutures leading to a crowded posterior fossa and venous outflow obstruction resulting in impaired cerebrospinal fluid (CSF) absorption is highlighted. Management options are unique in this group and we advocate early (prior to 6 months of age) posterior vault expansion by distraction osteogenesis (DO) in the management of Chiari 1 malformation. Foramen magnum decompression is recommended for a select few either as part of posterior vault expansion or at a later date. Treatment of hydrocephalus, utilizing a ventriculoperitoneal (VP) shunt with preferably a programmable high-pressure valve and anti-siphon device, is required in a small percentage of cases despite successful posterior vault expansion. Patients need to be carefully selected and managed as hydrocephalus often serves as an important cranial vault growth stimulus. Further, they require careful monitoring and thought to ensure the management of these conditions and the timing of any intervention provides the optimal long-term outcome for the patient.
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Kim J, Patel VJ, El Ahmadieh TY, Olson DM, Swift DM. Hydrocephalus in achondroplasia: efficacy of endoscopic third ventriculostomy. J Neurosurg Pediatr 2021:1-8. [PMID: 34920430 DOI: 10.3171/2021.9.peds21242] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/07/2021] [Accepted: 09/17/2021] [Indexed: 11/06/2022]
Abstract
OBJECTIVE Ventriculoperitoneal shunts (VPSs) for hydrocephalus in patients with achondroplasia are known to have a higher failure rate than in other hydrocephalus populations. However, the etiology of hydrocephalus in this group is considered "communicating," and, therefore, potentially not amenable to endoscopic third ventriculostomy (ETV). ETV has, nonetheless, been reported to be successful in a small number of patients with achondroplasia. The authors aimed to investigate the long-term results of ETV in this population. METHODS Patients with achondroplasia who had undergone surgical treatment for hydrocephalus (ETV or VPS placement) were identified. In patients who had undergone ETV, medical records and neuroimages were reviewed to determine ventricular volumes and frontal and occipital horn ratios (FOHRs) pre- and postoperatively, as well as the incidence of surgical complications and reoperation. Patients who underwent VPS placement were included for historical comparison, and their medical records were reviewed for basic demographic information as well as the incidence of surgical complications and reoperation. RESULTS Of 114 pediatric patients with achondroplasia referred for neurosurgical consultation, 19 (17%) were treated for hydrocephalus; 10 patients underwent ETV only, 7 patients underwent VPS placement only, and 2 patients had a VPS placed followed by ETV. In patients treated with ETV, ventricular volume and FOHRs were normal, if measured at birth, and increased significantly until the time of the ETV. After ETV, all patients demonstrated significant and sustained decreases in ventricular measurements with surveillance up to 15 years. There was a statistically significant difference in rates of repeat CSF surgery between the ETV and VPS cohorts (0/12 vs 7/9, p < 0.001). CONCLUSIONS ETV was efficacious, safe, and durable in the treatment of hydrocephalus in patients with achondroplasia. Although many studies have indicated that hydrocephalus in these patients is "communicating," a subset may develop an "obstructive" component that is progressive and responsive to ETV.
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Affiliation(s)
- Jun Kim
- 1Department of Neurological Surgery, University of Texas Southwestern Medical Center, Dallas; and.,2Division of Pediatric Neurosurgery, Children's Medical Center Dallas, Dallas, Texas
| | - Vishal J Patel
- 1Department of Neurological Surgery, University of Texas Southwestern Medical Center, Dallas; and
| | - Tarek Y El Ahmadieh
- 1Department of Neurological Surgery, University of Texas Southwestern Medical Center, Dallas; and
| | - DaiWai M Olson
- 1Department of Neurological Surgery, University of Texas Southwestern Medical Center, Dallas; and
| | - Dale M Swift
- 1Department of Neurological Surgery, University of Texas Southwestern Medical Center, Dallas; and.,2Division of Pediatric Neurosurgery, Children's Medical Center Dallas, Dallas, Texas
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Van Der Veken J, Harding M, Hatami S, Agzarian M, Vrodos N. Syringomyelia intermittens: highlighting the complex pathophysiology of syringomyelia. Illustrative case. JOURNAL OF NEUROSURGERY: CASE LESSONS 2021; 2:CASE21341. [PMID: 35855301 PMCID: PMC9265193 DOI: 10.3171/case21341] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 06/03/2021] [Accepted: 07/12/2021] [Indexed: 11/30/2022]
Abstract
BACKGROUND Chiari Type I malformation (CM1) is a disorder recognized by caudal displacement of the cerebellar tonsils through the foramen magnum and into the cervical canal. Syringomyelia is frequently found in patients with CM1, but the pathophysiology of syringomyelia remains an enigma. As a general consensus, symptomatic patients should be treated and asymptomatic patients without a syrinx should not be treated. Mildly symptomatic patients or asymptomatic patients with a syrinx, on the other hand, pose a more challenging dilemma, as the natural evolution is uncertain. For many surgeons, the presence of a syrinx is an indication to offer surgery even if the patient is asymptomatic or mildly symptomatic. OBSERVATIONS The authors describe an illustrative case of a 31-year-old female with an incidental finding of a CM1 malformation and cervical syrinx in 2013. Conservative management was advocated as the patient was asymptomatic. Monitoring of the syrinx over a course of 8 years showed resolution, followed by reappearance and finally a complete resolution in 2021. A review of the literature and the possible pathophysiology is discussed. LESSONS The unusual course of this patient highlights the importance of guiding treatment by clinical symptoms, not radiological findings. Furthermore it reflects the complexity of the pathophysiology and the uncertain natural history of syringomyelia.
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Affiliation(s)
| | | | - Saba Hatami
- South Australia Medical Imaging, Flinders Medical Centre, Bedford Park, South Australia, Australia; and
- College of Medicine & Public Health, Flinders University, Bedford Park, South Australia, Australia
| | - Marc Agzarian
- South Australia Medical Imaging, Flinders Medical Centre, Bedford Park, South Australia, Australia; and
- College of Medicine & Public Health, Flinders University, Bedford Park, South Australia, Australia
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Spazzapan P, Bosnjak R, Prestor B, Velnar T. Chiari malformations in children: An overview. World J Clin Cases 2021; 9:764-773. [PMID: 33585622 PMCID: PMC7852648 DOI: 10.12998/wjcc.v9.i4.764] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/10/2020] [Revised: 12/04/2020] [Accepted: 12/26/2020] [Indexed: 02/06/2023] Open
Abstract
Chiari malformations encompass various radiological and clinical entities, sharing the herniation of the rhombencephalic structures through the foramen magnum as a common characteristic. They can be symptomatic or asymptomatic. The therapeutic strategies for these malformations differ on the basis of the diverse pathophysiologic processes that cause them. As Chiari malformations are caused by various pathophysiologic processes, they must be recognized promptly to select the best treatment for each single case.
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Affiliation(s)
- Peter Spazzapan
- Department of Neurosurgery, University Medical Centre Ljubljana, Ljubljana 1000, Slovenia
| | - Roman Bosnjak
- Department of Neurosurgery, University Medical Centre Ljubljana, Ljubljana 1000, Slovenia
| | - Borut Prestor
- Department of Neurosurgery, University Medical Centre Ljubljana, Ljubljana 1000, Slovenia
| | - Tomaz Velnar
- Department of Neurosurgery, University Medical Centre Ljubljana, Ljubljana 1000, Slovenia
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Thompson DNP. Chiari I-a 'not so' congenital malformation? Childs Nerv Syst 2019; 35:1653-1664. [PMID: 31292759 DOI: 10.1007/s00381-019-04296-9] [Citation(s) in RCA: 19] [Impact Index Per Article: 3.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/27/2019] [Accepted: 07/02/2019] [Indexed: 02/07/2023]
Abstract
The term Chiari I malformation (CIM) is imbedded in the paediatric neurosurgical lexicon; however, the diagnostic criteria for this entity are imprecise, its pathophysiology variable, and the treatment options diverse. Until recently, CIM has been considered to be a discrete congenital malformation requiring a uniform approach to treatment. Increasingly, it is recognised that this is an oversimplification and that a more critical, etiologically based approach to the evaluation of children with this diagnosis is essential, not only to select those children who might be suitable for surgical treatment (and, of course those who might be better served by conservative management) but also to determine the most appropriate surgical strategy. Whilst good outcomes can be anticipated in the majority of children with CIM following foramen magnum decompression, treatment failures and complication rates are not insignificant. Arguably, poor or suboptimal outcomes following treatment for CIM reflect, not only a failure of surgical technique, but incorrect patient selection and failure to acknowledge the diverse pathophysiology underlying the phenomenon of CIM. The investigation of the child with 'hindbrain herniation' should be aimed at better understanding the mechanisms underlying the herniation so that these may be addressed by an appropriate choice of treatment.
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Affiliation(s)
- Dominic N P Thompson
- Department of Paediatric Neurosurgery, Great Ormond Street Hospital for Children NHS Trust, Great Ormond Street, London, WC1N 3JH, UK.
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8
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Spontaneous Resolution of Syringomyelia with a 16-Year Serial Magnetic Resonance Imaging Follow-Up: A Case Report and Literature Review. World Neurosurg 2019; 130:432-438. [DOI: 10.1016/j.wneu.2019.07.138] [Citation(s) in RCA: 13] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/23/2019] [Revised: 07/16/2019] [Accepted: 07/17/2019] [Indexed: 11/18/2022]
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Ghali GZ, Zaki Ghali MG, Ghali EZ, Srinivasan VM, Wagner KM, Rothermel A, Taylor J, Johnson J, Kan P, Lam S, Britz G. Intracranial Venous Hypertension in Craniosynostosis: Mechanistic Underpinnings and Therapeutic Implications. World Neurosurg 2018; 127:549-558. [PMID: 30092478 DOI: 10.1016/j.wneu.2018.07.260] [Citation(s) in RCA: 23] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/06/2018] [Revised: 07/27/2018] [Accepted: 07/28/2018] [Indexed: 11/20/2022]
Abstract
Patients with complex, multisutural, and syndromic craniosynostosis (CSO) frequently exhibit intracranial hypertension. The intracranial hypertension cannot be entirely attributed to the craniocephalic disproportion with calvarial restriction because cranial vault expansion has not consistently alleviated elevated intracranial pressure. Evidence has most strongly supported a multifactorial interaction, including venous hypertension along with other pathogenic processes. Patients with CSO exhibit marked venous anomalies, including stenosis of the jugular-sigmoid complex, transverse sinuses, and extensive transosseous venous collaterals. These abnormal intracranial-extracranial occipital venous collaterals might represent anomalous development, with persistence and subsequent enlargement of channels normally present in the fetus, either as a primary defect or as nonregression in response to failure of the development of the jugular-sigmoid complexes. It has been suggested by some investigators that venous hypertension in patients with CSO could be treated directly via jugular foraminoplasty, venous stenting, or jugular venous bypass, although these options are not in common clinical practice. Obstructive sleep apnea, occurring as a consequence of midface hypoplasia, can also contribute to intracranial hypertension in patients with syndromic CSO. Thus, correction of facial deformities, as well as posterior fossa decompression, could also play important roles in the treatment of intracranial hypertension. Determining the precise mechanistic underpinnings underlying intracranial hypertension in any given patient with CSO requires individualized evaluation and management.
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Affiliation(s)
- George Zaki Ghali
- United States Environmental Protection Agency, Arlington, Virginia, USA; Department of Toxicology, Purdue University, West Lafayette, Indiana, USA
| | - Michael George Zaki Ghali
- Department of Neurological Surgery, Houston Methodist Hospital, Houston, Texas, USA; Department of Neurological Surgery, Baylor College of Medicine, Houston, Texas, USA.
| | - Emil Zaki Ghali
- Department of Medicine, Inova Alexandria Hospital, Alexandria, Virginia, USA; Department of Urological Surgery, El Gomhoureya General Hospital, Alexandria, Egypt
| | - Visish M Srinivasan
- Department of Neurological Surgery, Baylor College of Medicine, Houston, Texas, USA
| | - Kathryn M Wagner
- Department of Neurological Surgery, Baylor College of Medicine, Houston, Texas, USA
| | - Alexis Rothermel
- Division of Plastic and Reconstructive Surgery, Penn State Hershey Medical Center, Hershey, Pennsylvania, USA
| | - Jesse Taylor
- Division of Plastic and Reconstructive Surgery, Children's Hospital of Philadelphia, University of Pennsylvania, Philadelphia, Pennsylvania, USA
| | - Jeremiah Johnson
- Department of Neurological Surgery, Baylor College of Medicine, Houston, Texas, USA
| | - Peter Kan
- Department of Neurological Surgery, Baylor College of Medicine, Houston, Texas, USA
| | - Sandi Lam
- Department of Neurological Surgery, Baylor College of Medicine, Houston, Texas, USA
| | - Gavin Britz
- Department of Neurological Surgery, Houston Methodist Hospital, Houston, Texas, USA
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Raybaud C, Jallo GI. Chiari 1 deformity in children: etiopathogenesis and radiologic diagnosis. HANDBOOK OF CLINICAL NEUROLOGY 2018; 155:25-48. [PMID: 29891063 DOI: 10.1016/b978-0-444-64189-2.00002-0] [Citation(s) in RCA: 26] [Impact Index Per Article: 3.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/19/2022]
Abstract
The metamerically associated normal hindbrain and normal posterior fossa are programmed to grow together in such a way that the tonsils are located above the foramen magnum and surrounded by the cerebrospinal fluid (CSF) of the cisterna magna. This allows the pulsating CSF to move freely up and down across the craniovertebral junction (CVJ). A developmental mismatch between the rates of growth of the neural tissue and of the bony posterior fossa may result in the cerebellar tonsils being dislocated across the foramen magnum. The cause of this may be, rarely, an overgrowth of the cerebellum. More commonly, it is due to an insufficient development of the posterior fossa, possibly associated with a malformation of the craniocervical joint. When it is not due to a remediable cause, such a herniation is called a Chiari 1 deformity. This definition is anatomic (descent of the tonsils below the plane of the foramen magnum) and not clinical: many patients with the deformity are and will remain asymptomatic. Most authors consider that a descent of 5 mm or more is clinically significant but other factors, such as the diameter of the foramen magnum and the degree of tapering of the upper cervical "funnel," are likely to be as important. Morphologic markers of severity on magnetic resonance imaging are, beside the degree of descent, the peg-like deformity of the tonsils, the obstruction of the surrounding CSF spaces (at the craniocervical junction and in the whole posterior fossa), a compression of the cord, an abnormal signal of the cord, and a syringomyelia, typically cervicothoracic. The syringomyelia is assumed to be explained by the "Venturi effect" that is associated with the increased velocity of the CSF across the restricted CSF spaces. Radiologically, the etiopathogenic assessment should address the size and morphology of the posterior fossa, and the functional status of the craniocervical flexion joint. The posterior fossa is best evaluated on sagittal cuts by the posterior fossa pentagon proportionality associated with the line of Chamberlain, and on coronal cuts, by showing a possible shallowness of the posterior fossa. The functional status of the craniocervical joint is altered in case of a proatlantal hypoplasia, as this condition results in a cranial shift of the joint that brings the tip of the dens and of the flexion axis in front of the medulla, that is, in a situation of osteoneural conflict. Less commonly, similar conflicts may also occur when an abnormal craniocervical segmentation results in an instability of the joint.
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Affiliation(s)
- Charles Raybaud
- Neuroradiology, Hospital for Sick Children, University of Toronto, Toronto, ON, Canada.
| | - George I Jallo
- Division of Pediatric Neurosurgery, Johns Hopkins Hospital, Baltimore, MD, United States
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Lasjaunias P, Alvarez H, Rodesch G, Garcia-Monaco R, terBrugge K, Burrows P, Taylor W. Aneurysmal Malformations of the Vein of Galen. Interv Neuroradiol 2016; 2:15-26. [DOI: 10.1177/159101999600200102] [Citation(s) in RCA: 52] [Impact Index Per Article: 5.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/15/1996] [Accepted: 01/20/1996] [Indexed: 11/16/2022] Open
Abstract
The Vein of Galen Aneurysmal Malformation (VGAM) is regarded as a lesion with high morbidity and mortality, both spontaneously and under treatment, in part due to an incomplete appreciation of the effects of the lesion on the specific physiology of young children. In addition, various techniques have been applied over a short period of time to treat the lesion rather than the disorders it creates. We report experience with 120 consecutive cases of VGAM managed over the past ten years: 24 were diagnosed antenatally; 50 presented in the neonatal period with haemodynamic disturbance; 35 in infants presented with secondary hydrovenous disorders (macrocrania, subependymal atrophy and ventricular dilatation); 12 were seen in children. We were unable to follow 10% of the patients because the referring teams decided not to follow our therapeutic advice. Treatment was contra-indicated in 17% of cases (with early brain damage and a rapidly fatal outcome). There were five (4%) which thrombosed spontaneously (but only two of these were neurologically normal). Embolisation was performed in 78 patients via a transarterial femoral approach using glue. Anatomical cure has already been achieved in 53%. Of the treated patients, even when treatment has not been completed, 80% are growing neurologically normal. Permanent neurological deficit or irreversible neurocognitive delay related to the natural history of the lesion were evident in 8.5%. Overall mortality was 9%. These figures confirm that previous views on the prognosis of VGAM should be revised. Endoarterial embolisation, is the preferred treatment to date.
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Affiliation(s)
| | | | | | | | | | - P. Burrows
- Hospital for Sick Children; Toronto, Ontario, Canada
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12
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Grillner P, Söderman M, Holmin S, Rodesch G. A spectrum of intracranial vascular high-flow arteriovenous shunts in RASA1 mutations. Childs Nerv Syst 2016; 32:709-15. [PMID: 26499346 DOI: 10.1007/s00381-015-2940-y] [Citation(s) in RCA: 19] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/29/2015] [Accepted: 10/15/2015] [Indexed: 12/15/2022]
Abstract
PURPOSE AND BACKGROUND We describe three paediatric cases with different intracranial fast-flow shunts presenting early in life, all with capillary malformation-arteriovenous malformation syndrome and RASA1 verified mutations. Intracranial arteriovenous fast-flow shunts are rare vascular malformations typically presenting early in life and have been associated with cutaneous capillary malformations, characterized as capillary malformation-arteriovenous malformation syndrome. Heterozygous RASA1 gene mutations have been found to be disease causing with high penetrance for the typical cutaneous findings, but only some individuals with the syndrome have intracranial lesions. CASES One infant presented with a vein of Galen malformation responsible for hydrodynamic disorders, one neonate suffered from severe cardiac insufficiency related to a superior sagittal sinus dural malformation with high-flow fistulas, and one baby was treated at infant age of a choroidal arteriovenous fistula discovered antenatally. RESULTS AND CONCLUSIONS We report the follow-up of these three cases with RASA1 gene mutation and comment on the possible role of evaluation for vascular lesions and capillary malformation-arteriovenous malformation syndrome in patients and their families, with intracranial fast-flow shunts.
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Affiliation(s)
- Pernilla Grillner
- Department of Neuropediatrics, Astrid Lindgren Children's Hospital, Karolinska University Hospital, Stockholm, 171 76, Sweden.
| | - Micheal Söderman
- Department of Clinical Neuroscience, Karolinska Institutet, Stockholm, Sweden.,Department of Neuroradiology, Karolinska University Hospital, Stockholm, Sweden
| | - Staffan Holmin
- Department of Clinical Neuroscience, Karolinska Institutet, Stockholm, Sweden.,Department of Neuroradiology, Karolinska University Hospital, Stockholm, Sweden
| | - Georges Rodesch
- Service de Neuroradiologie Diagnostique et Thérapeutique, Hôpital Foch, Suresnes, France
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Winston KR, Stence NV, Boylan AJ, Beauchamp KM. Upward Translation of Cerebellar Tonsils following Surgical Expansion of Supratentorial Cranial Vault: A Unified Biomechanical Explanation of Chiari Type I. Pediatr Neurosurg 2015; 50:243-9. [PMID: 26367858 DOI: 10.1159/000437146] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/11/2015] [Accepted: 06/23/2015] [Indexed: 11/19/2022]
Abstract
Cerebellar tonsils moved significantly upward in 3 patients with Chiari type I who underwent supratentorial cranial vault expansion to alleviate intracranial pressure related to multisutural craniosynostosis. The Chiari type I deformities in these patients were the biomechanical consequence of posterior fossa-cerebellar disproportion caused by supratentorial craniocerebral disproportion secondary to multisutural craniosynostosis. The authors postulate that all cases of Chiari type I deformity share the sine qua non feature of posterior fossa-cerebellar disproportion.
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Pencovich N, Ben-Sira L, Kesler A, Constantini S. Acquired and reversible Chiari-like descent following a single lumbar puncture: case report. Childs Nerv Syst 2012; 28:1269-71. [PMID: 22457165 DOI: 10.1007/s00381-012-1738-4] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/17/2012] [Accepted: 03/14/2012] [Indexed: 10/28/2022]
Affiliation(s)
- N Pencovich
- Department of Pediatric Neurosurgery, Dana-Dwek Children's Hospital, Tel-Aviv Medical Center, Tel Aviv University, Israel, 6 Weizman Street, Tel Aviv 64239, Israel
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15
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Spontaneous resolution of syringomyelia in an adult patient with tight cisterna magna. Neurol Sci 2012; 33:1463-7. [PMID: 22258363 DOI: 10.1007/s10072-012-0946-8] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/28/2010] [Accepted: 01/09/2012] [Indexed: 12/27/2022]
Abstract
Spontaneous resolution of syringomyelia in adult patients with Chiari malformation is exceptionally rare, with only 10 cases having been reported. A 21-year-old man working as a carpenter presented with a 1-year history of paresthesias in his right arm. A magnetic resonance imaging scan disclosed a cervicothoracic syrinx associated with tight tonsillar impaction of the cisterna magna without herniation. The patient left the carpentry job and underwent close monitoring with serial clinical and neuroradiological controls. The patient's symptoms gradually disappeared and magnetic resonance imaging studies revealed progressive shrinkage of the syrinx despite persistence of crowding of posterior fossa structures at the level of the foramen magnum. This case suggests that spontaneous resolution of syringomyelia can occasionally be triggered by the cessation of daily physical strain in patients with tight cisterna magna. Health care professionals should be aware that strenuous physical activities could affect the natural history of syringomyelia.
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Strahle J, Muraszko KM, Buchman SR, Kapurch J, Garton HJL, Maher CO. Chiari malformation associated with craniosynostosis. Neurosurg Focus 2012; 31:E2. [PMID: 21882907 DOI: 10.3171/2011.6.focus11107] [Citation(s) in RCA: 72] [Impact Index Per Article: 5.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/06/2022]
Abstract
OBJECT Chiari malformation (CM) Type I is frequently associated with craniosynostosis. Optimal management of CM in patients with craniosynostosis is not well-established. The goal of this study was to report on a series of pediatric patients with both craniosynostosis and CM and discuss their management. METHODS The authors searched the medical records of 383 consecutive patients treated for craniosynostosis at a single institution over a 15-year period to identify those with CM. They recorded demographic data as well as surgical treatment and outcomes for these patients. When MR imaging was performed, cerebellar tonsillar descent was recorded and any other associated findings, such as hydrocephalus or spinal syringes, were noted. RESULTS A total of 29 patients with both CM and craniosynostosis were identified. Of these cases, 28% had associated occipital venous abnormalities, 45% were syndromic, and 52% also had hydrocephalus. Chiari malformation was more likely to be present in those patients with isolated lambdoid synostosis (55%), multisuture synostosis (35%), and pansynostosis (80%), compared with patients with coronal synostosis (6%) or sagittal synostosis (3%). All patients underwent surgical repair of craniosynostosis: 16 had craniosynostosis repair as well as CM decompression, and 13 patients did not undergo CM decompression. Of the 7 patients in whom craniosynostosis repair alone was performed, 5 had decreased tonsillar ectopia postoperatively and 5 had improved CSF flow studies postoperatively. Both patients with a spinal syrinx had imaging-documented syrinx regression after craniosynostosis repair. In 12 patients in whom CM was diagnosed after primary craniosynostosis repair, 5 had multiple cranial vault expansions and evidence of elevated intracranial pressure. In 5 cases, de novo CM development was documented following craniosynostosis repair at a mean of 3.5 years after surgery. CONCLUSIONS Chiari malformation is frequently seen in patients with both multi- and single-suture lambdoid craniosynostosis. Chiari malformation, and even a spinal cord syrinx, will occasionally resolve following craniofacial repair. De novo development of CM after craniosynostosis repair is not unusual.
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Affiliation(s)
- Jennifer Strahle
- Department of Neurosurgery, University of Michigan, Ann Arbor, Michigan 48709-5338, USA
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17
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Swift D, Nagy L, Robertson B. Endoscopic third ventriculostomy in hydrocephalus associated with achondroplasia. J Neurosurg Pediatr 2012; 9:73-81. [PMID: 22208325 DOI: 10.3171/2011.10.peds1169] [Citation(s) in RCA: 19] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/06/2022]
Abstract
Hydrocephalus in patients with achondroplasia is thought to be due to increased dural sinus venous pressure resulting from narrowing of the jugular foramen. In this setting, where hydrocephalus is presumed to be "vascular" in origin and therefore communicating, endoscopic third ventriculostomy (ETV) would seem contraindicated. The authors describe 3 patients in whom ETV was successfully performed, resulting in MR imaging-documented decreases in ventricle size. The patients were 11 months, 33 months, and 13 years at the time of surgery. All patients had serial preoperative MR images demonstrating progressive hydrocephalus in a "triventricular" pattern with a small fourth ventricle but an open aqueduct. All patients had undergone suboccipital decompression for foramen magnum stenosis prior to the treatment of hydrocephalus. Preoperative retrograde venography revealed variable pressure gradients across the jugular foramen. It is postulated that the increase in intracranial venous pressure resulting from jugular foramen stenosis may lead to disproportionate venous engorgement of the cerebellum and some degree of obstructive hydrocephalus amenable to ETV. The authors discuss the role of suboccipital decompression in the progression of hydrocephalus in patients with achondroplasia.
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Affiliation(s)
- Dale Swift
- Neurosurgeons for Children, Children’s Medical Center, Dallas, USA.
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18
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Abstract
The diagnosis of Chiari type I malformation (CIM) is more and more frequent in clinical practice due to the wide diffusion of magnetic resonance imaging. In many cases, such a diagnosis is made incidentally in asymptomatic patients, as including children investigated for different reasons such as mental development delay or sequelae of brain injury. The large number of affected patients, the presence of asymptomatic subjects, the uncertainties surrounding the pathogenesis of the malformation, and the different options for its surgical treatment make the management of CIM particularly controversial.This paper reports on the state of the art and the recent achievements about CIM aiming at providing further information especially on the pathogenesis, the natural history, and the management of the malformation, which are the most controversial aspects. A historial review introduces and explains the current classification. Furthermore, the main clinical, radiological, and neurophysiological findings of CIM are described to complete the picture of this heterogeneous and complex disease.
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Affiliation(s)
- L Massimi
- Pediatric Neurosurgery, Catholic University Medical School, Rome, Italy
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19
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20
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Ganesan D, Hayward RD, Thompson DN. Evolution of tonsillar ectopia associated with frontal encephalocoele. Childs Nerv Syst 2009; 25:889-93. [PMID: 19238404 DOI: 10.1007/s00381-009-0830-x] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/13/2008] [Revised: 12/09/2008] [Indexed: 12/31/2022]
Abstract
INTRODUCTION Chiari I malformation has been traditionally considered a congenital malformation. However, there is growing clinical evidence suggesting that it is an acquired phenomenon as also exemplified by this case. CASE Fetal magnetic resonance imaging (MRI) at 28th week gestation revealed a frontonasal encephalocoele with no hindbrain abnormalities. Post-natal MRI of brain and cervical spine of the 7-week-old infant showed the presence of tonsillar ectopia in the absence of hydrocephalus. The normally developed cerebellar tonsil has herniated through the foramen magnum during the third trimester and neonatal period. DISCUSSION We hypothesise that the presence of the encephalocoele resulted in dampening expansile forces, produced by the growing brain as well as the cerebrospinal fluid pulsation required to stimulate of the skull growth. As a result, cranial growth is diminished producing a small posterior fossa. The subsequent growth spurt of the cerebellum at the end of the third trimester and during the neonatal period has resulted in cerebellar tonsillar ectopia consequent upon the state of cephalocranial disproportion.
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Affiliation(s)
- Dharmendra Ganesan
- Department of Neurosurgery, Great Ormond Street Children's Hospital, Great Ormond Street, London, WC1N 3JH, UK
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Abstract
Patients with the congenital neurocutaneous disorder PHACES are at a markedly increased risk of ischemic infarction during childhood. Although intracranial arterial anomalies have been well described, venous abnormalities have not been documented. The authors report on a unique case of a 3-month-old girl with PHACES and a skull base osteodural arteriovenous fistula. A separate arteriovenous shunt at T-5 may also have been present. Imaging findings and treatment strategies are discussed.
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Affiliation(s)
- Huan Wang
- Department of Neurosurgery, Children's Hospital Boston, Brigham & Women's Hospital, Harvard Medical School, Boston, Massachusetts 02115, USA
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22
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Novegno F, Caldarelli M, Massa A, Chieffo D, Massimi L, Pettorini B, Tamburrini G, Di Rocco C. The natural history of the Chiari Type I anomaly. J Neurosurg Pediatr 2008; 2:179-87. [PMID: 18759599 DOI: 10.3171/ped/2008/2/9/179] [Citation(s) in RCA: 142] [Impact Index Per Article: 8.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
Abstract
OBJECT Since the advent of MR imaging, an increasing number of asymptomatic or oligosymptomatic patients have been diagnosed with Chiari malformation Type I (CM-I). The decision of whether or not to operate is more difficult in these patients than in those with clear symptoms because of the lack of information about the natural course of this disease. METHODS The authors report on their experience in a series of 22 patients with CM-I who were evaluated at the authors' institution, and for whom a conservative approach to treatment was adopted. The patients ranged in age from 1 to 16 years (mean 6.3 years) at diagnosis. Neuroradiological and complete clinical examinations were performed in all patients 6 months after the first observation and every year thereafter. The follow-up period ranged from 3 to 19 years (mean 5.9 years). RESULTS Chiari malformation Type I was incidentally detected on MR images in 11 of 22 patients. The remaining 11 patients had minimal clinical signs at presentation that were not regarded as necessitating immediate surgical treatment. Seventeen patients (77.3%) showed progressive improvement in their symptoms or remained asymptomatic at the last follow-up whereas 5 patients (22.7%) experienced worsening, which was mild in 2 cases and required surgical correction in the remaining 3 cases. On MR imaging a mild reduction in tonsillar herniation was appreciated in 4 patients (18.18%), with complete spontaneous resolution in 1 of these. In 16 patients, tonsillar herniation remained stable during follow-up. CONCLUSIONS The authors' data confirm the common impression that in both asymptomatic and slightly symptomatic patients with CM-I, a conservative approach to treatment should be adopted with periodic clinical and radiological examinations.
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Affiliation(s)
- Federica Novegno
- Department of Pediatric Neurosurgery, Catholic University Medical School, Largo A. Gemelli 1, Rome, Italy.
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Iizuka Y, Suzuki M, Komura S, Shimoji K, Tsutsumi Y, Konishi Y, Kaneko K. Hydrovenous Disorder in Vein of Galen Aneurysmal Dilatation: Special Focus on Tonsilar Prolapse. Neuroradiol J 2008; 21:57-64. [DOI: 10.1177/197140090802100107] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/07/2007] [Accepted: 12/12/2007] [Indexed: 11/17/2022] Open
Abstract
We analyzed five cases of vein of Galen aneurysmal dilatation (VGAD) and 12 cases of vein of Galen aneurysmal malformation (VGAM) using sagittal T1-weighted images. Tonsilar prolapse (TP) was confirmed in three cases, which were all VGAD. Two of these cases were treated by endovascular procedures. In one of these two cases, elevation of TP was observed after intervention. There were no signs of TP in the control group of 12 VGAMs. Venous hypertension interferes with cerebrospinal fluid (CSF) resorption resulting in increased brain fluid. TP frequently develops in high flow AVFs and is reversible if therapeutic intervention is performed by an experienced clinical team when appropriately indicated. We consider that TP in VGAD is mainly the result of cerebellar swelling due to chronic venous hypertension.
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Affiliation(s)
- Y. Iizuka
- Division of Neuroradiology, Department of Radiology, Juntendo University, School of Medicine; Tokyo, Japan
| | - M. Suzuki
- Division of Neuroradiology, Department of Radiology, Juntendo University, School of Medicine; Tokyo, Japan
| | - S. Komura
- Division of Neuroradiology, Department of Radiology, Juntendo University, School of Medicine; Tokyo, Japan
| | - K. Shimoji
- Division of Neuroradiology, Department of Radiology, Juntendo University, School of Medicine; Tokyo, Japan
| | - Y. Tsutsumi
- Department of Radiology, National Center for Child Health and Development; Tokyo, Japan
| | - Y. Konishi
- Department of Neurosurgery, Kyorin Medical University; Tokyo, Japan
| | - K. Kaneko
- Department of Pediatric, Juntendo Urayasu Hospital; Chiba, Japan
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Alvarez H, Garcia Monaco R, Rodesch G, Sachet M, Krings T, Lasjaunias P. Vein of galen aneurysmal malformations. Neuroimaging Clin N Am 2007; 17:189-206. [PMID: 17645970 DOI: 10.1016/j.nic.2007.02.005] [Citation(s) in RCA: 109] [Impact Index Per Article: 6.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/31/2022]
Abstract
Different types of malformations share a dilated vein of Galen, but only one of them is a true vein of Galen aneurysmal malformation (VGAM). The optimal window of opportunity for treatment is between 4 and 5 years of months [corrected], because this allows the child to grow and mature. Heart failure and hydrocephalus respond favorably to embolization. Cerebrospinal fluid ventricular shunting, if needed, should be performed after the embolization. The transvenous approach carries significantly elevated morbidity and mortality and is rarely indicated. Anatomic cure of the VGAM is not the main goal of treatment; the ultimate goal is control of the malformation to allow the brain to mature and develop normally.
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Affiliation(s)
- H Alvarez
- Service de Neuroradiologie Diagnostique et Thérapeutique, Hôpital Bicêtre 78, rue du Général Leclerc, 94275 Le Kremlin-Bicêtre, Paris, France
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25
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Rodesch G, Otto B, Mouchamps M, Born J. Reversible tonsillar prolapse and syringomyelia after embolization of a tectal arteriovenous malformation. J Neurosurg 2007; 107:412-5. [PMID: 17695398 DOI: 10.3171/jns-07/08/0412] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/06/2022]
Abstract
✓ The authors report the case of a 21-year-old woman who presented with headaches, frequent sensations of loss of equilibrium, and intermittent strabismus. A tectal arteriovenous malformation (AVM) was diagnosed based on magnetic resonance (MR) imaging findings. The AVM drained toward the straight sinus and was associated with a tonsillar prolapse (Chiari malformation Type I [CM-I]) and cervical syringomyelia. The tectal AVM was embolized with N-butyl cyanoacrylate, and disconnection of about 80% of the lesion was obtained. All clinical symptoms resolved after embolization, and radiosurgery was proposed to treat the malformation remnant. A control MR image confirmed the regression of the tonsillar prolapse and the disappearance of the syrinx. This report emphasizes that CM-I and syringomyelia may be acquired and related to hydrovenous disorders.
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Affiliation(s)
- Georges Rodesch
- Department of Diagnostic and Therapeutic Neuroradiology, Hôpital Foch, Suresnes, France.
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26
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Lasjaunias PL, Chng SM, Sachet M, Alvarez H, Rodesch G, Garcia-Monaco R. The management of vein of Galen aneurysmal malformations. Neurosurgery 2007; 59:S184-94; discussion S3-13. [PMID: 17053602 DOI: 10.1227/01.neu.0000237445.39514.16] [Citation(s) in RCA: 150] [Impact Index Per Article: 8.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/14/2022] Open
Abstract
OBJECTIVE The vein of Galen aneurysmal malformation (VGAM) is a choroidal type of arteriovenous malformation involving the vein of Galen forerunner. This is distinct from an arteriovenous malformation with venous drainage into a dilated, but already formed, vein of Galen. Reports of endovascular treatment of VGAM in the literature approach the disease from a purely technical viewpoint and often fail to provide satisfactory midterm results. To focus the therapeutic challenge to a strictly morphological goal overlooks the fundamental aspects of neonatal and infant anatomy and fluid physiology. During the past 20 years, our approach to VGAM has remained the same. Our experience, based on 317 patients with VGAM who were studied in Hospital Bicêtre between October 1981 and October 2002, allows us to describe the angioarchitecture, natural history, and management of VGAM in neonates, infants, and children. METHODS Of our cohort of 317 patients, 233 patients were treated with endovascular embolization; of these, 216 patients were treated in our hospital. The treatment method of choice was a transfemoral arterial approach to deliver glue at the fistulous zone. RESULTS Of 216 patients, 23 died despite or because of the embolization (10.6%). Twenty out of the 193 (10.4%) surviving patients were severely retarded, 30 (15.6%) were moderately retarded, and 143 (74%) were neurologically normal on follow-up. CONCLUSION Our data demonstrate that most treated children survive and undergo normal neurological development; an understanding of the clinical, anatomical, and pathophysiological features of VGAM has, therefore, reversed the former poor prognosis. Our level of understanding about the lesion allows us to predict most situations and remedy them by applying a strict evaluation protocol and working within an optimal therapeutic window. Patient selection and timing remain the keys in the management of this condition. It is more important to restore normal growth conditions than a normal morphological appearance, with the primary therapeutic objective being normal development in a child without neurological deficit.
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Affiliation(s)
- Pierre L Lasjaunias
- Department of Diagnostic and Interventional Neuroradiology, Hopital de Bicêtre, Le Kremlin Bicêtre, France.
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27
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Ozisik PA, Hazer B, Ziyal IM, Ozcan OE. Spontaneous resolution of syringomyelia without Chiari malformation. Neurol Med Chir (Tokyo) 2006; 46:512-7. [PMID: 17062993 DOI: 10.2176/nmc.46.512] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/20/2022] Open
Abstract
A 30-year-old woman presented with a cervical syrinx manifesting as hemihypesthesia. Neuroimaging found no evidence of Chiari malformation or tight cisterna magna. Serial magnetic resonance imaging studies over a 6-year period demonstrated spontaneous and complete resolution of the syrinx accompanied by an asymptomatic clinical course. The natural history of syringomyelia is highly unpredictable. The outcome of surgical treatment for patients with syringomyelia is not always satisfactory, so the indications for surgery are controversial. Spontaneous resolution of syringomyelia unrelated with foramen magnum lesion has various causes. Close follow up of the patient is necessary to monitor for recurrence.
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Affiliation(s)
- Pinar Akdemir Ozisik
- Department of Neurosurgery, Faculty of Medicine, Hacettepe University, Ankara, Turkiye.
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Hamlat A, Helal H, Carsin-Nicol B, Brassier G, Guegan Y, Morandi X. Acute presentation of hydromyelia in a child. Acta Neurochir (Wien) 2006; 148:1117-21; discussion 1121. [PMID: 16944053 DOI: 10.1007/s00701-006-0875-5] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/27/2006] [Accepted: 07/20/2006] [Indexed: 11/26/2022]
Abstract
The authors document a rapid development, within 3 weeks, of hydromyelia in a 12 year-old boy. The boy was admitted to a local hospital because of drowsiness and persistent severe neck pain. Neurological examination disclosed a lethargic boy with no neurological deficit other than Parinaud's sign. During his transfer to our department, he presented a cardio-respiratory arrest with coma and bilateral mydriasis. External ventricular drain and craniocervical decompression achieved excellent clinical and neuroradiological outcomes. The development of hydromyelia in this case is caused by obstruction to the natural cerebrospinal fluid pathway at the craniocervical junction and the cardio-respiratory arrest is provoked by a brain stem compression against the clivus and odontoid process. This report illustrates that hydromyelia may complicate acute obstructive hydrocephalus due to acquired Chiari malformation.
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Affiliation(s)
- A Hamlat
- Department of Neurosurgery, CHRU Pontchaillou, Rennes, France.
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Cinalli G, Spennato P, Sainte-Rose C, Arnaud E, Aliberti F, Brunelle F, Cianciulli E, Renier D. Chiari malformation in craniosynostosis. Childs Nerv Syst 2005; 21:889-901. [PMID: 15875201 DOI: 10.1007/s00381-004-1115-z] [Citation(s) in RCA: 172] [Impact Index Per Article: 8.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/01/2004] [Indexed: 02/06/2023]
Abstract
INTRODUCTION Chiari malformation (CM) is a frequent finding in multisutural and syndromic craniosynostosis, occurring in 70% of patients with Crouzon's syndrome, 75% with oxycephaly, 50% with Pfeiffer's syndrome and 100% with the Kleeblattschädel deformity. The pathogenesis of this condition and rationale for treatment are still controversial. DISCUSSION Since its first description in 1972, several factors have been cited to play a role in inducing CM. In the light of recent publications, the roles of premature fusion of cranial vault and cranial base sutures, of congenital anomalies of the cerebellum and brain stem, of raised intracranial pressure, of venous hypertension and of hydrocephalus are reviewed. Evaluation and management of CM are also discussed. CONCLUSION Chiari malformation appears to be an acquired and progressive condition that develops in the first months of life, because of a disproportion between hindbrain growth and an abnormally small posterior fossa, a consequence of the premature fusion of lambdoid and cranial base sutures. Venous hypertension caused by stenosis of the jugular foramen can also be present in these patients, resulting in intracranial hypertension and/or hydrocephalus. Careful MRI evaluation is recommended for the forms of craniosynostosis at a high risk of developing hindbrain herniation. The selection of posterior cranial vault expansion as the first surgical procedure is advocated. In selected cases, treatment of the posterior cranial deformity by occipital vault remodelling and treatment of the Chiari-like deformity by suboccipital decompression can be carried out using the same surgical procedure.
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Affiliation(s)
- Giuseppe Cinalli
- Department of Pediatric Neurosurgery, Santobono-Pausilipon Children's Hospital, Naples, Italy.
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Abstract
INTRODUCTION This article addresses the relevance of venous hypertension to children born with, in particular, the "syndromic" forms of craniosynostosis. The development of the human cerebral venous system is summarised and the patterns of anomalous venous drainage seen in children with complex forms of craniosynostosis associated with raised intracranial pressure are described. AETIOLOGY The aetiology of these abnormal patterns is discussed under the general headings of "The constriction theory", "A primary role for FGFR mutations" and "Persistence of the foetal pattern of intracranial venous drainage."Venous hypertension (along with hydrocephalus, cranio-cerebral disproportion and airway obstruction) has an important role in the aetiology of raised intracranial pressure in severely affected children. TREATMENT The inter-relationship between these factors is explained and then the treatment of those children whose raised intracranial pressure is considered to be due predominantly to venous hypertension is described.
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31
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Rossi A, De Biasio P, Scarso E, Gandolfo C, Pavanello M, Morana G, Venturini PL, Tortori-Donati P. Prenatal MR imaging of dural sinus malformation: a case report. Prenat Diagn 2005; 26:11-6. [PMID: 16378320 DOI: 10.1002/pd.1347] [Citation(s) in RCA: 29] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/09/2022]
Abstract
OBJECTIVE To describe prenatal magnetic resonance imaging (MRI) findings of dural sinus malformation (DSM), a very rare, congenital form of dural arteriovenous shunt (DAVS), typically affecting newborns. METHODS Ultrasound (US) and MRI were performed at 34 weeks' gestation, and the findings of these examinations were compared with postnatal MRI studies performed at 2 days and 1 month. RESULTS US showed an anechoic, midline posterior fossa collection with irregular internal echodensities. Color Doppler showed prominent arterial vascularity at the lesion margins. The prenatal MRI showed a large, profoundly hypointense, midline retrocerebellar mass. Postnatal MRI, complemented with magnetic resonance (MR) angiography, showed the lesion to be a giant dural venous pouch fed by multiple mural arteriovenous shunts. Follow-up MRI at 1 month suggested latent venous hypertension and prompted endovascular treatment. CONCLUSION Prenatal MR imaging is useful to establish the diagnosis, to assess complications such as hydrocephalus and tonsillar prolapse, and to help plan perinatal management, postnatal follow-up, and treatment decision-making.
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Affiliation(s)
- Andrea Rossi
- Department of Pediatric Neuroradiology, G. Gaslini Children's Hospital, Genoa, Italy.
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32
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Mallinger B, Marson F, Sévely A, Loubes-Lacroix F, Catalaa I, Manelfe C. [Spontaneous resolution of syringomyelia in a child with Chiari I malformation: a case report]. JOURNAL DE RADIOLOGIE 2004; 85:1943-6. [PMID: 15602417 DOI: 10.1016/s0221-0363(04)97764-5] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 05/01/2023]
Abstract
Cervicothoracic syringomyelia is a frequent feature in Chiari I malformation. It can be symptomatic or not, and is well demonstrated by magnetic resonance imaging (MRI). Its spontaneous resolution is uncommon. The authors report a case of spontaneous resolution of a thoracic syrinx in an 18-year-old patient with a Chiari I malformation. MRI study performed 6 years previously because of worsening headaches demonstrated a Chiari I malformation associated with a syrinx cavity. The cavity disappearance was noted after improvement of the symptoms.
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Affiliation(s)
- B Mallinger
- Service de Neuroradiologie diagnostique et thérapeutique, Hôpital Purpan Place du Docteur Baylac, 31059 Toulouse Cedex 9, France
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Abstract
Intracranial pathological changes can occur as a result of impaired craniocervical venous return. Thrombosis of central venous access catheters was demonstrated in two neonates born at 38 and 27 weeks' gestation. Neither infant developed hemorrhage of prematurity as confirmed on cranial ultrasonography. Clinical evidence of vena cava thrombosis and associated spontaneous intraventricular hemorrhage developed on Day 24 and 36, respectively, and these findings were confirmed on imaging studies. In one infant the hemorrhage was accompanied by communicating hydrocephalus.
The cause of the intracranial disease was attributable to the retrograde cerebral venous congestion. This, together with the primitive venous bed developing in the periventricular region, was associated with the spontaneous hemorrhage in the region of the foramen of Monro.
To the authors' knowledge, this is the first report in the English-language literature of spontaneous neonatal intra-cerebral hemorrhage, due to thrombosis of the superior or inferior vena cava.
The natural history of this condition is resolution without sequelae after appropriate therapeutic intervention for the vena cava thrombosis.
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Affiliation(s)
- Sanjay N Misra
- Department of Neurosurgery, Denver Health Medical Center, University of Colorado Health Sciences Center, Denver, Colorado 80204, USA.
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Duckwiller G. Pediatric Pial AVF. Interv Neuroradiol 2003; 9:41-6. [DOI: 10.1177/15910199030090s205] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/06/2003] [Accepted: 08/08/2003] [Indexed: 11/15/2022] Open
Affiliation(s)
- G. Duckwiller
- Radiology Department, UCLA, School of Medicine, Los Angeles; USA
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Kyoshima K, Bogdanov EI. Spontaneous resolution of syringomyelia: report of two cases and review of the literature. Neurosurgery 2003; 53:762-8; discussion 768-9. [PMID: 12943593 DOI: 10.1227/01.neu.0000079629.05048.a2] [Citation(s) in RCA: 51] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/16/2002] [Accepted: 05/14/2003] [Indexed: 11/19/2022] Open
Abstract
OBJECTIVE AND IMPORTANCE The natural history of syringomyelia is highly unpredictable, and some patients experience improvement or stabilization without surgery. However, the mechanisms of the formation and spontaneous resolution of syringomyelia remain controversial. This report concerns two patients with syringomyelia who demonstrated spontaneous reductions in syrinx size, accompanied by symptomatic improvement. CLINICAL PRESENTATION One patient was a 10-year-old girl with syringomyelia associated with a tight cisterna magna and basilar impression, who demonstrated a spontaneous decrease in syrinx size, accompanied by symptomatic improvement, in 22 months. The other patient was a 39-year-old man with syringomyelia associated with a Chiari I malformation, who demonstrated a spontaneous reduction in syrinx size and neurological improvement, accompanied by elevation of the cerebellar tonsils, 6 months after diagnosis. INTERVENTION The patients were monitored. CONCLUSION The mechanisms of spontaneous resolution of syringomyelia, as well as the factors leading to the cerebrospinal fluid flow disturbances that cause syringomyelia, may vary. Resolution of foramen magnum lesion-related syringomyelia may be the result of spontaneous correction of the abnormal cerebrospinal fluid flow, as observed in our cases, or of cavity fluid drainage into the spinal arachnoid space because of spinal cord fissuring.
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Affiliation(s)
- Kazuhiko Kyoshima
- Department of Neurosurgery, Shinshu University School of Medicine, Matsumoto, Japan
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Hartmann A, Mast H, Stapf C, Koch HC, Marx P. Peripheral Hemodialysis Shunt With Intracranial Venous Congestion. Stroke 2001. [DOI: 10.1161/str.32.12.2945] [Citation(s) in RCA: 23] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
Abstract
Background
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Intracranial venous congestion is often caused by local venous thrombosis or brain arteriovenous fistulas. Hemodialysis shunts are known to cause venous enlargement in the arm or chest but have not been related to intracranial vascular pathology.
Case Description
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A 59-year-old woman who presented with increasing headache, gait instability, and memory loss was a renal transplant recipient who still carried a left upper arm shunt. Cranial CT scan showed enlarged veins in the posterior fossa with incipient hydrocephalus. Extracranial duplex sonography revealed reversed flow in the left internal jugular vein, which normalized on cuff inflation around the shunt-carrying arm. The reversed flow, intracranial venous congestion, and neurological status improved after surgical shunt ligation.
Conclusions
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To our knowledge, this is the first case description of an intracranial venous outflow obstruction caused by a peripheral arteriovenous shunt.
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Affiliation(s)
- Andreas Hartmann
- From the Departments of Neurology, Stroke Unit (A.H., C.S., P.M.), and Radiology (H-C.K.), Universitätsklinikum Benjamin Franklin, Freie Universität Berlin, and Department of Neurology, Stroke Unit, Berufsgenossenschaftliche Kliniken der Stadt Halle Bergmannstrost, Halle (H.M.), Germany
| | - Henning Mast
- From the Departments of Neurology, Stroke Unit (A.H., C.S., P.M.), and Radiology (H-C.K.), Universitätsklinikum Benjamin Franklin, Freie Universität Berlin, and Department of Neurology, Stroke Unit, Berufsgenossenschaftliche Kliniken der Stadt Halle Bergmannstrost, Halle (H.M.), Germany
| | - Christian Stapf
- From the Departments of Neurology, Stroke Unit (A.H., C.S., P.M.), and Radiology (H-C.K.), Universitätsklinikum Benjamin Franklin, Freie Universität Berlin, and Department of Neurology, Stroke Unit, Berufsgenossenschaftliche Kliniken der Stadt Halle Bergmannstrost, Halle (H.M.), Germany
| | - Hans-Christian Koch
- From the Departments of Neurology, Stroke Unit (A.H., C.S., P.M.), and Radiology (H-C.K.), Universitätsklinikum Benjamin Franklin, Freie Universität Berlin, and Department of Neurology, Stroke Unit, Berufsgenossenschaftliche Kliniken der Stadt Halle Bergmannstrost, Halle (H.M.), Germany
| | - Peter Marx
- From the Departments of Neurology, Stroke Unit (A.H., C.S., P.M.), and Radiology (H-C.K.), Universitätsklinikum Benjamin Franklin, Freie Universität Berlin, and Department of Neurology, Stroke Unit, Berufsgenossenschaftliche Kliniken der Stadt Halle Bergmannstrost, Halle (H.M.), Germany
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Klekamp J, Iaconetta G, Samii M. Spontaneous resolution of Chiari I malformation and syringomyelia: case report and review of the literature. Neurosurgery 2001; 48:664-7. [PMID: 11270558 DOI: 10.1097/00006123-200103000-00044] [Citation(s) in RCA: 59] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/05/2023] Open
Abstract
OBJECTIVE AND IMPORTANCE Indications for surgery and the surgical technique of foramen magnum decompression for patients with Chiari I malformation and syringomyelia are controversial issues. This case report supports the view that observation may be adequate for patients without progressive symptoms or with mild clinical symptoms. CLINICAL PRESENTATION A 37-year-old woman presented with a 3-month history of burning dysesthesias and hypesthesia in her right arm. A neurological examination revealed hypesthesia in the right trigeminal distribution. A magnetic resonance imaging scan revealed a Chiari I malformation with syringomyelia between C2 and T2. No hydrocephalus was observed. CLINICAL COURSE Because the patient's symptoms regressed spontaneously, surgery was not performed. Thirty-two months after her initial examination, the patient was asymptomatic. A second magnetic resonance imaging scan was obtained, which demonstrated complete spontaneous resolution of the Chiari I malformation and syringomyelia. CONCLUSION We attribute the regression of the patient's symptoms to spontaneous recanalization of cerebrospinal fluid pathways at the foramen magnum, which most likely was due to rupture of the arachnoid membranes that had obstructed cerebrospinal fluid flow.
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Affiliation(s)
- J Klekamp
- Department of Neurosurgery, Nordstadt Krankenhaus, Medizinische Hochschule, Hannover, Germany
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Taylor WJ, Hayward RD, Lasjaunias P, Britto JA, Thompson DN, Jones BM, Evans RD. Enigma of raised intracranial pressure in patients with complex craniosynostosis: the role of abnormal intracranial venous drainage. J Neurosurg 2001; 94:377-85. [PMID: 11235939 DOI: 10.3171/jns.2001.94.3.0377] [Citation(s) in RCA: 147] [Impact Index Per Article: 6.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/06/2022]
Abstract
OBJECT In this study the authors investigated whether patterns of intracranial venous drainage in children with complex craniosynostosis associated with raised intracranial pressure (ICP) were abnormal and, thus, could support the theory that venous hypertension is a major contributor to raised ICP that can lead to impaired visual function or even blindness in these patients. METHODS The authors analyzed the anatomy of intracranial venous drainage as demonstrated in the results of 24 angiography studies obtained in 23 patients, all of whom had either a craniosynostosis-related syndrome (18 patients) or a nonsyndromic multisutural synostosis (five patients). Twenty-one patients had experienced raised ICP (in 19 patients diagnosis was based on invasive ICP monitoring and in two patients on clinical grounds alone) 1 to 6 weeks before undergoing angiography. Of the two remaining patients (both with Apert syndrome) whose ICP monitoring was normal immediately before angiography, each had undergone two previous cranial vault expansion procedures. On results of 18 angiography studies a 51 to 99% stenosis or no flow at all could be observed in the sigmoid-jugular sinus complex either bilaterally (11 patients) or unilaterally (seven patients). In 11 of these patients a florid collateral circulation through the stylomastoid emissary venous plexus was also seen. Two angiography studies were performed in one patient with Crouzon syndrome. A comparison of the two studies demonstrated a progression of the abnormal venous anatomy in that case. The authors found no obvious correlation between each patient's baseline ICP and the degree of abnormality of their venous anatomy, as judged on the basis of a venous-phase angiography severity score. CONCLUSIONS Based on their findings, the authors assert that in children with complex forms of craniosynostosis in whom other factors, such as hydrocephalus, are absent, abnormalities of venous drainage that particularly affect the sigmoid-jugular sinus complex produce a state of venous hypertension that, in turn, is responsible for the majority of cases of raised ICP. The incidence of these changes is unknown, but an analysis of the ages of the children in this study indicated that the period of particular vulnerability to the effects of venous hypertension lasts until the affected child is approximately 6 years old. After that age the collateral venous drainage through the stylomastoid plexus will likely become sufficient to allow ICP to normalize.
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Affiliation(s)
- W J Taylor
- Department of Neuroradiology and Craniofacial Surgery, Great Ormond Street Hospital for Children, London, United Kingdom
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Agostinis C, Caverni L, Montini M, Pagani G, Bonaldi G. "Spontaneous" reduction of tonsillar herniation in acromegaly: a case report. SURGICAL NEUROLOGY 2000; 53:396-9. [PMID: 10825527 DOI: 10.1016/s0090-3019(00)00179-8] [Citation(s) in RCA: 13] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 10/18/2022]
Affiliation(s)
- C Agostinis
- Department of Neuroradiology, Ospedali Riuniti di Bergamo, Bergamo, Italy
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40
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Sun JC, Steinbok P, Cochrane DD. Spontaneous resolution and recurrence of a Chiari I malformation and associated syringomyelia. Case report. J Neurosurg 2000; 92:207-10. [PMID: 10763693 DOI: 10.3171/spi.2000.92.2.0207] [Citation(s) in RCA: 14] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/06/2022]
Abstract
The spontaneous resolution of syringomyelia has been reported infrequently. In patients with Chiari I malformations, resolution of the syringomyelia has sometimes been associated with improvement of their malformation. The authors present a case of spontaneous resolution followed by recurrence of syringomyelia and a corresponding change in the Chiari malformation. This case is of interest in light of the theories postulated to explain spontaneous resolution of syringomyelia.
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Affiliation(s)
- J C Sun
- Division of Pediatric Neurosurgery, British Columbia's Children's Hospital, Vancouver, Canada
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Abstract
OBJECTIVE Certain neurosurgical procedures require sectioning of the tentorium cerebelli. The presence of venous sinuses within the tentorium makes these procedures difficult. The aim of this study was to investigate the incidence, size, location, configuration, and pattern of venous drainage of these sinuses. METHODS The tentorium cerebelli was studied in 80 fresh cadavers. After the skull cap and the supratentorial portion of the brain were removed, the tentorium was inspected for the presence of venous sinuses. Their location, size, configuration, and pattern of venous drainage were noted. Subsequently, the infratentorial structures were removed via the tentorial incisura. The tentorial sinuses were again studied. In certain cases, the sinus was opened and a probe passed inside to confirm its presence. RESULTS The tentorium cerebelli was revealed to contain sinuses in 86% of the cadavers. These sinuses were classified into the following three types: Type I sinuses constituted 25% of the total and were most often located in the medial one-third of the tentorium. They were larger than the other types, frequently occurring with a branching "stag-horn" configuration and a tendency to drain into the straight sinus, the torcular herophili, and the medial one-third of the transverse sinus. Type II sinuses constituted 25% of the total and were most often located in the lateral one-third of the tentorium. They were smaller than the other types, and tended to drain into the duction of the transverse sinus and superior petrosal sinus and into the lateral one-third of the transverse sinus. Type III sinuses constituted 50% of the total and were located in the medial one-third of the tentorium. Their size ranged from small to medium. Unlike Type I sinuses, no branching pattern was observed. These sinuses tended to drain into the straight sinus, the torcular herophili, and the medial one-third of the transverse sinus. In the present study, the medial one-third of the tentorium was observed to be the most vascular part. No venous sinus was observed in the anterior part of the tentorium. CONCLUSION Venous sinuses are common in the tentorium cerebelli. In this study, they were observed in 86% of the cases. They can be classified into three types, based on their location, size, configuration, and pattern of drainage. The medial one-third of the tentorium is the most vascular part. A knowledge of these sinuses may be helpful while sectioning the tentorium. The importance of these sinuses in treating vascular and neoplastic diseases of the brain is highlighted. A brief review of the embryology of these sinuses is also presented.
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Affiliation(s)
- N Muthukumar
- Department of Neurosurgery, Madurai Medical College, India
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Abe T, Matsumoto K, Kiyota K, Tanaka H. Vein of Galen aneurysmal malformation in an adult: a case report. SURGICAL NEUROLOGY 1996; 45:39-43. [PMID: 9190697 DOI: 10.1016/0090-3019(95)00340-1] [Citation(s) in RCA: 15] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 02/04/2023]
Abstract
We report a 19-year old woman with a choroidal-type vein of Galen aneurysmal malformation who presented with hydrocephalus. This venous malformation was fed by multiple fistulas and drained into the superior sagittal sinus through a persistent falcine sinus (precursor of the straight sinus). The hydrocephalus was treated by ventriculoperitioneal shunting, and the patient remained well for 9 years. At this time, magnetic resonance imaging (MRI) and magnetic resonance angiography demonstrated a vein of Galen aneurysmal malformation in the right thalamus, with a Chiari I appearance. This case may provide valuable data regarding the natural history of such venous malformations.
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Affiliation(s)
- T Abe
- Department of Neurosurgery, Showa University School of Medicine, and Showa General Hospital, Tokyo, Japan
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Affiliation(s)
- W Taylor
- Lysholm Department of Radiology, National Hospital for Neurology and Neurosurgery, London
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Watban JA, Rodesch G, Alvarez H, Lasjaunias P. Transarterial embolization of vein of Galen aneurysmal malformation after unsuccessful stereotactic radiosurgery. Report of three cases. Childs Nerv Syst 1995; 11:406-8. [PMID: 7585669 DOI: 10.1007/bf00717406] [Citation(s) in RCA: 15] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/26/2023]
Abstract
The authors present three cases of vein of Galen aneurysmal malformations (VGAMs) diagnosed in infancy and submitted by the referring teams for stereotactic radiosurgery as the initial therapy (therapeutic doses ranging between 20-25 Gy and 40-50 Gy to the peak dose). After the conventional follow-up of 18-24 months, no change could be detected in the angioarchitecture of the lesions. All three cases were then referred for endovascular treatment and underwent embolization by the transarterial route using liquid adhesives (N-butyl cyanoacrylate). This resulted in complete anatomical exclusion of the lesion. Regardless of the theoretical efficiency of radiosurgery in the management of brain arteriovenous malformations, the present authors believe that transarterial embolization remains the treatment of choice in VGAMs. It offers a high rate of morphological cure and the best chances for normal neurocognitive development. The time required by radiosurgery to achieve a significant result is too long for developing and maturing brain and may not prevent the negative effects of the lesion, mainly in regard to hemo- and hydrodynamic disorders (atrophy, subcortical calcifications, etc.) created by the VGAM, thus leading to irreversible mental retardation.
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Affiliation(s)
- J A Watban
- Neuroradiologie Vasculaire Diagnostique et Thérapeutique, Hôpital Bicêtre, Le Kremlin Bicêtre, France
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