Reversible tonsillar prolapse and syringomyelia after embolization of a tectal arteriovenous malformation

2007 ◽  
Vol 107 (2) ◽  
pp. 412-415 ◽  
Author(s):  
Georges Rodesch ◽  
Bernard Otto ◽  
Micheline Mouchamps ◽  
Jacques Born

✓ 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.

2011 ◽  
Vol 31 (3) ◽  
pp. E5 ◽  
Author(s):  
Uwe Max Mauer ◽  
Andreas Gottschalk ◽  
Carolin Mueller ◽  
Linda Weselek ◽  
Ulrich Kunz ◽  
...  

Object The causal treatment of Chiari malformation Type I (CM-I) consists of removing the obstruction of CSF flow at the level of the foramen magnum. Cerebrospinal fluid flow can be visualized using dynamic phase-contrast MR imaging. Because there is only a paucity of studies evaluating CSF dynamics in the region of the spinal canal on the basis of preoperative and postoperative measurements, the authors investigated the clinical usefulness of cardiacgated phase-contrast MR imaging in patients with CM-I. Methods Ninety patients with CM-I underwent preoperative MR imaging of CSF pulsation. Syringomyelia was present in 59 patients and absent in 31 patients. Phase-contrast MR imaging of the entire CNS was used to investigate 22 patients with CM-I before surgery and after a mean postoperative period of 12 months (median 12 months, range 3–33 months). In addition to the dynamic studies, absolute flow velocities, the extension of the syrinx, and tonsillar descent were also measured. Results The changes in pulsation were highly significant in the region of the (enlarged) cistern (p = 0.0005). Maximum and minimum velocities (the pulsation amplitude) increased considerably in the region where the syrinx was largest in diameter. The changes of pulsation in these patients were significant in the subarachnoid space in all spinal segments but not in the syrinx itself and in the central canal. Conclusions The demonstration of CSF flow pulsation can contribute to assessments of surgical outcomes. The results presented here, however, raise doubts about current theories on the pathogenesis of syringomyelia.


2011 ◽  
Vol 31 (2) ◽  
pp. E6 ◽  
Author(s):  
Mónica Rivero-Garvía ◽  
F. Javier Márquez-Rivas ◽  
Antonia García-Iglesias ◽  
Raquel Gutiérrez-González

Craniometaphyseal dysplasia (CMD) is a very rare bone disorder characterized by abnormally developed metaphyses in long bones and sclerosis of the craniofacial bones. In this paper, the authors report 2 cases of children diagnosed with CMD and chronic intracranial hypertension with deletion in exon 9 of the human ANK gene (ANKH). After intracranial monitoring, a different treatment was chosen for each patient. One of the patients was treated using CSF shunting because ventriculomegaly in the absence of a Chiari malformation was also observed on cerebral MR imaging. The other patient underwent cranial expansion and decompressive craniotomy of the posterior fossa, because ventriculomegaly was excluded after cerebral MR imaging and cervical MR imaging showed a Chiari malformation Type I. The origin of intracranial hypertension in CMD is multifactorial. Previous intracranial pressure monitoring and a thorough understanding of neuroimaging studies are essential to achieve an accurate diagnosis and effective treatment.


2018 ◽  
Vol 27 (1) ◽  
pp. 71-73
Author(s):  
Lindolfo Carlos Heringer ◽  
Matheus Fernandes De Oliveira ◽  
Ulysses Oliveira De Sousa ◽  
Wanderley Cerqueira De Lima ◽  
Ricardo Vieira Botelho

Introduction. The association between hydrocephalus and Chiari malformation (CM) has not been described frequently. Ventricular dilation affects 7% to 10% of patients with CM, but the ideal choice of surgical treatment is controversial. Wereport a case of a patient with Chiari malformation and hydrocephalus with improvement in clinical symptoms and ventricular dilatation. Case Description. A 19-year-old male complaining of frontal headache when coughing, laughing and during valsalva maneuvers, associated with dizziness for 2 months. Magnetic resonance (MR) showed hydrocephalus and small posterior fossa with overcrowding of contents, characterizing Chiari malformation type I, with cerebellar tonsils protruding through magnum foramen. Patient underwent surgery with posterior fossa decompression in a semi-sitting position and removal of the arc C1.After 3 months of follow-up, headache disappeared becoming asymptomatic. Control MR showed improvement of hydrocephalus with restoration of the cisterna magna and CSF flow. Discussion. Hydrocephalus has been related to CM for a long time. In our case, we performed treatment with intradural and intra-arachnoidal approach with bilateral tonsillectomy without placing ventricular shunt. The cisterna magna was “recreated”. There was improvement of hydrocephalus with decreased Evans ratio index and symptoms disappearance. Although there is no other studies addressing such matter, in this case, the improvement suggests that the CSF compression at the foramen magnum was the cause of associated hydrocephalus with Chiari malformation. 


Author(s):  
Elizabeth N Alford ◽  
Travis J Atchley ◽  
Tofey J Leon ◽  
Nicholas M. B Laskay ◽  
Anastasia A Arynchyna ◽  
...  

OBJECTIVE In Chiari malformation type I (CM-I), a variety of imaging findings have been purported to be important; however, results have been inconclusive, inconsistent, or not replicated in independent studies. The purpose of this study was to report imaging characteristics for a large cohort of patients with CM-I and identify the imaging findings associated with surgical decompression. METHODS Patients were identified using ICD-9 codes for CM-I for the period from 1996 to 2017. After review of the medical records, patients were excluded if they 1) did not have a diagnosis of CM-I, 2) were not evaluated by a neurosurgeon, or 3) did not have available preoperative MRI. Retrospective chart review was performed to collect demographic and clinical data. Imaging parameters were measured according to the Chiari I Malformation Common Data Elements. RESULTS A total of 731 patients were included for analysis, having a mean follow-up duration of 25.5 months. The mean age at presentation was 8.5 years. The mean tonsil position was 11.4 mm below the foramen magnum, and 62.8% of patients had a pegged tonsil shape. Two hundred patients (27.4%) underwent surgery for life-dominating tussive headache, lower cranial nerve dysfunction, syrinx, and/or brainstem dysfunction. Surgical treatment was associated with a syrinx (OR 20.4, 95% CI 12.3–33.3, p < 0.0001), CM-1.5 (OR 1.797, 95% CI 1.08–2.98, p = 0.023), lower tonsil position (OR 1.130, 95% CI 1.08–1.18, p < 0.0001), and congenital fusion of cervical vertebrae (OR 5.473, 95% CI 1.08–27.8, p = 0.040). Among patients with benign CM-I, tonsil position was statistically significantly associated with future surgery. CONCLUSIONS Comprehensive imaging characteristics for a large cohort of patients with CM-I are reported. Analysis showed that a lower tonsillar position, a syrinx, and CM-1.5 were associated with undergoing posterior fossa decompression. This study demonstrates the importance of considering imaging findings in the context of patient symptomatology.


2006 ◽  
Vol 104 (1) ◽  
pp. 28-32 ◽  
Author(s):  
Brian A. O'Shaughnessy ◽  
Bernard R. Bendok ◽  
Richard J. Parkinson ◽  
Ali Shaibani ◽  
Matthew T. Walker ◽  
...  

Neurosurgery ◽  
2015 ◽  
Vol 77 (1) ◽  
pp. 96-103 ◽  
Author(s):  
Noam Alperin ◽  
James R. Loftus ◽  
Carlos J. Oliu ◽  
Ahmet M. Bagci ◽  
Sang H. Lee ◽  
...  

Abstract BACKGROUND: Suboccipital cough-induced headaches are considered a hallmark symptom of Chiari malformation type I (CMI). However, non--Valsalva-related suboccipital headaches and headaches in other locations are also common in CMI. The diagnostic significance and the underlying factors associated with these different headaches types are not well understood. OBJECTIVE: To compare cranial morphology and hydrodynamics in 3 types of headaches in CMI to better understand the pathophysiological basis for the different headache characteristics. METHODS: Twenty-two cranial physiological and morphological measures were obtained with specialized magnetic resonance imaging scans from 63 symptomatic pretreated CMI patients, 40 with suboccipital headaches induced by Valsalva maneuvers (34 women; age, 36 ± 10 years), 15 with non--Valsalva-related suboccipital headaches (10 women; age, 33 ± 9 years), 8 with nonsuboccipital non--Valsalva-induced headaches (8 women; age, 39 ± 13 years), and 37 control subjects (24 women; age, 36 ± 12 years). Group differences were identified with the use of the 2-tailed Student t test. RESULTS: Posterior cranial fossa markers of CMI were similar among the 3 headache subtypes. However, the Valsalva-related suboccipital headaches cohort demonstrated a significantly lower intracranial compliance index than the non--Valsalva-related suboccipital headaches cohort (7.5 ± 3.4 vs 10.9 ± 4.9), lower intracranial volume change during the cardiac cycle (0.48 ± 0.19 vs 0.61 ± 0.16 mL), and higher magnetic resonance imaging--derived intracranial pressure (11.1 ± 4.3 vs 7.7 ± 2.8 mm Hg; P = .02). The Valsalva-related suboccipital headaches cohort had smaller intracranial and lateral ventricular volumes compared with the healthy cohort. The non--Valsalva-related suboccipital headaches cohort had reduced venous drainage through the jugular veins. CONCLUSION: Valsalva-induced worsening of occipital headaches appears to be related to a small intracranial volume rather than the smaller posterior cranial fossa. This explains the reduced intracranial compliance and corresponding higher pressure measured in CMI patients with headaches affected by Valsalva maneuvers.


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