A Combinatorial Approach with Cerebellar Tonsil Suspension to Treating Symptomatic Chiari Malformation Type I in Adults: A Retrospective Study

It was found that syringomyelic patients and clinically healthy people out of the families with MRT-features of Chiari malformation, type I, had a confident decrease of depth and square of posterior cranial fossa. Relatives of patients, having no signs of Chiari malformation, type I, had the same changes as well. There was made a conclusion about presence of posterior cranial fossa hypoplasia in all patients with hereditary syryngomyelia and in members of their families, both having MRT-features of Chiari malformation type I, and having no malformation. It was supposed that posterior cranial fossa hypoplasia is a neurovisual reflection of its hereditary phonotype, and the latter in some cases leads to cerebellar tonsil ptosis, and if there are some endogenic and exogenic factors to development of family syringomyelia.

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Spontaneous cerebrospinal fluid leak via foramen rotundum in a non-obese male presented as pseudo-Chiari malformation type I: a case report and literature review

Journal of International Medical Research ◽

10.1177/0300060520924212 ◽

2020 ◽

Vol 48 (6) ◽

pp. 030006052092421

Author(s):

Dang Huu Luong ◽

Yen-Chun Chen ◽

Linh Ngoc Tuong Tran ◽

Shih-Han Hung ◽

Quang Xuan Ly

Keyword(s):

Cerebrospinal Fluid ◽

Case Report ◽

Chiari Malformation ◽

Cerebellar Tonsil ◽

Type I ◽

Transnasal Approach ◽

Chiari Malformation Type I ◽

Patient Reported ◽

Csf Leakage ◽

Foramen Rotundum

Spontaneous cerebrospinal fluid (sCSF) leak from the skull base has been previously reported, but there are few reports of sCSF leak from the foramen rotundum due to its rare occurrence. This case report describes a 15-year-old male patient that presented with left side watery rhinorrhoea that had been present since he was 4 years of age and a history of repeated bouts of meningitis of unknown cause. A discharge sample from the nose tested positive for beta-2 transferrin. Preoperative computed tomography (CT) revealed a fistula between the cerebellopontine angle and the left sphenoid sinus. There was also a pseudo-Chiari malformation type I with ectopia of the cerebellar tonsil. Endoscopic transnasal surgery identified a leak from the foramen rotundum that was repaired using autologous material and a contralateral pedicle nasoseptal flap. At 6 months after surgery, the patient reported no recurrence of the CSF leakage. Postoperative CT imaging revealed that the cerebellar tonsil was back in the normal position, indicating that the preoperative Chiari malformation was possibly due to decreased CSF volume. This current case shows that a rare case of sCSF leak from the foramen rotundum can be effectively repaired using the endoscopic transnasal approach.

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Morphometric and volumetric comparison of 102 children with symptomatic and asymptomatic Chiari malformation Type I

Journal of Neurosurgery Pediatrics ◽

10.3171/2017.8.peds17345 ◽

2018 ◽

Vol 21 (1) ◽

pp. 65-71 ◽

Cited By ~ 13

Author(s):

Siri Sahib S. Khalsa ◽

Ndi Geh ◽

Bryn A. Martin ◽

Philip A. Allen ◽

Jennifer Strahle ◽

...

Keyword(s):

Posterior Fossa ◽

Chiari Malformation ◽

Pediatric Patients ◽

Incidental Finding ◽

Foramen Magnum ◽

Cerebellar Tonsil ◽

Type I ◽

Chiari Malformation Type I ◽

Asymptomatic Patients ◽

Volumetric Measurements

OBJECTIVEChiari malformation Type I (CM-I) is typically defined on imaging by a cerebellar tonsil position ≥ 5 mm below the foramen magnum. Low cerebellar tonsil position is a frequent incidental finding on brain or cervical spine imaging, even in asymptomatic individuals. Nonspecific symptoms (e.g., headache and neck pain) are common in those with low tonsil position as well as in those with normal tonsil position, leading to uncertainty regarding appropriate management for many patients with low tonsil position and nonspecific symptoms. Because cerebellar tonsil position is not strictly correlated with the presence of typical CM-I symptoms, the authors sought to determine if other 2D morphometric or 3D volumetric measurements on MRI could distinguish between patients with asymptomatic and symptomatic CM-I.METHODSThe authors retrospectively analyzed records of 102 pediatric patients whose records were in the University of Michigan clinical CM-I database. All patients in this database had cerebellar tonsil position ≥ 5 mm below the foramen magnum. Fifty-one symptomatic and 51 asymptomatic patients were matched for age at diagnosis, sex, tonsil position, and tonsil morphology. National Institutes of Health ImageJ software was used to obtain six 2D anatomical MRI measurements, and a semiautomated segmentation tool was used to obtain four 3D volumetric measurements of the posterior fossa and CSF subvolumes on MRI.RESULTSNo significant differences were observed between patients with symptomatic and asymptomatic CM-I related to tentorium length (50.3 vs 51.0 mm; p = 0.537), supraoccipital length (39.4 vs 42.6 mm; p = 0.055), clivus-tentorium distance (52.0 vs 52.1 mm; p = 0.964), clivus-torcula distance (81.5 vs 83.3 mm; p = 0.257), total posterior fossa volume (PFV; 183.4 vs 190.6 ml; p = 0.250), caudal PFV (152.5 vs 159.8 ml; p = 0.256), fourth ventricle volume to caudal PFV ratio (0.0140 vs 0.0136; p = 0.649), or CSF volume to caudal PFV ratio (0.071 vs 0.061; p = 0.138).CONCLUSIONSNo clinically useful 2D or 3D measurements were identified that could reliably distinguish pediatric patients with symptoms attributable to CM-I from those with asymptomatic CM-I.

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Impact of body mass index on cerebellar tonsil position in healthy subjects and patients with Chiari malformation

Journal of Neurosurgery ◽

10.3171/2014.10.jns141317 ◽

2015 ◽

Vol 123 (1) ◽

pp. 226-231 ◽

Cited By ~ 2

Author(s):

Brandon W. Smith ◽

Jennifer Strahle ◽

Erick Kazarian ◽

Karin M. Muraszko ◽

Hugh J. L. Garton ◽

...

Keyword(s):

Body Mass Index ◽

Posterior Fossa ◽

Body Mass ◽

Chiari Malformation ◽

Age Groups ◽

Foramen Magnum ◽

Cerebellar Tonsil ◽

Posterior Fossa Decompression ◽

Type I ◽

Chiari Malformation Type I

OBJECT It is unclear if there is a relationship between Chiari malformation Type I (CM-I) and body mass index (BMI). The aim of this study was to identify the relationship between BMI and cerebellar tonsil position in a random sample of people. METHODS Cerebellar tonsil position in 2400 subjects from a cohort of patients undergoing MRI was measured. Three hundred patients were randomly selected from each of 8 age groups (from 0 to 80 years). A subject was then excluded if he or she had a posterior fossa mass or previous posterior fossa decompression or if height and weight information within 1 year of MRI was not recorded in the electronic medical record. RESULTS There were 1310 subjects (54.6%) with BMI records from within 1 year of the measured scan. Of these subjects, 534 (40.8%) were male and 776 (59.2%) were female. The average BMI of the group was 26.4 kg/m2, and the average tonsil position was 0.87 mm above the level of the foramen magnum. There were 46 subjects (3.5%) with a tonsil position ≥ 5 mm below the level of the foramen magnum. In the group as a whole, there was no correlation (R2 = 0.004) between BMI and cerebellar tonsil position. CONCLUSIONS In this examination of 1310 subjects undergoing MRI for any reason, there was no relationship between BMI and the level of the cerebellar tonsils or the diagnosis of CM-I on imaging.

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313 Morphometric and Volumetric Comparison of Symptomatic and Asymptomatic Chiari Malformation Type I

Neurosurgery ◽

10.1093/neuros/nyx417.313 ◽

2017 ◽

Vol 64 (CN_suppl_1) ◽

pp. 267-267

Author(s):

Siri Sahib S Khalsa ◽

Ndi Geh ◽

Bryn A Martin ◽

Philip A Allen ◽

Jennifer Strahle ◽

...

Keyword(s):

Posterior Fossa ◽

Chiari Malformation ◽

Pediatric Patients ◽

Foramen Magnum ◽

Cerebellar Tonsil ◽

Type I ◽

Chiari Malformation Type I ◽

Asymptomatic Patients ◽

Specific Symptoms ◽

Volumetric Measurements

Abstract INTRODUCTION Chiari malformation type I (CMI) is typically defined by a cerebellar tonsil position 5 mm or more below foramen magnum. Low cerebellar tonsil position is a frequent incidental finding, even in asymptomatic individuals. Non-specific symptoms such as headache and neck pain are common in those with low tonsil position and in those with normal tonsil position, leading to uncertainty regarding appropriate management for many patients with low tonsil position and non-specific symptoms. Since cerebellar tonsil position is not strictly correlated with typical CMI symptoms, we sought to determine if other 2D morphometric or 3D volumetric measurements on MRI could distinguish asymptomatic from symptomatic CMI patients. METHODS We retrospectively analyzed 102 pediatric patients from the University of Michigan clinical CMI database. All patients in this database have cerebellar tonsil position at least 5 mm below foramen magnum. Fifty-one symptomatic and 51 asymptomatic patients were matched for age at diagnosis, sex, tonsil position, and tonsil morphology. Five 2D anatomic MRI measurements and four 3D volumetric measurements of posterior fossa and CSF sub-volumes on MRI were measured. RESULTS >There were no significant differences observed between symptomatic and asymptomatic CMI patients related to basilar impression (−5.9 mm vs. −7.0 mm; P = 0.026), tentorium length (50.3 mm vs. 51.0 mm; P = 0.537), supraoccipital length (39.4 mm vs. 42.6 mm; P = 0.055), clivus-tentorium distance (52.0 mm vs. 52.1 mm; P = 0.964), clivus-torcula distance (81.5 mm vs. 83.3 mm; P = 0.257), total posterior fossa volume (PFV; 183.4 mL vs. 190.6 mL; P = 0.250), caudal PFV (152.5 mL vs. 159.8 mL; P = 0.256), fourth ventricle volume to caudal PFV ratio (0.0140 vs. 0.0136; P = 0.649), or CSF volume to caudal PFV ratio (0.071 vs. 0.061; P = 0.138). CONCLUSION We did not identify any 2D or 3D measurements that could reliably distinguish pediatric patients with symptoms attributable to CMI from those with asymptomatic CMI.

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Syrinx location and size according to etiology: identification of Chiari-associated syrinx

Journal of Neurosurgery Pediatrics ◽

10.3171/2014.12.peds14463 ◽

2015 ◽

Vol 16 (1) ◽

pp. 21-29 ◽

Cited By ~ 19

Author(s):

Jennifer Strahle ◽

Karin M. Muraszko ◽

Hugh J. L. Garton ◽

Brandon W. Smith ◽

Jordan Starr ◽

...

Keyword(s):

Spinal Cord ◽

Cervical Spine ◽

Chiari Malformation ◽

Spinal Dysraphism ◽

Tethered Cord ◽

Spinal Tumors ◽

Cerebellar Tonsil ◽

Type I ◽

Chiari Malformation Type I ◽

The Brain

OBJECT Syrinx size and location within the spinal cord may differ based on etiology or associated conditions of the brain and spine. These differences have not been clearly defined. METHODS All patients with a syrinx were identified from 14,118 patients undergoing brain or cervical spine imaging at a single institution over an 11-year interval. Syrinx width, length, and location in the spinal cord were recorded. Patients were grouped according to associated brain and spine conditions including Chiari malformation Type I (CM-I), secondary CM (2°CM), Chiari malformation Type 0 (CM-0), tethered cord, other closed dysraphism, and spinal tumors. Syringes not associated with any known brain or spinal cord condition were considered idiopathic. Syrinx characteristics were compared between groups. RESULTS A total of 271 patients with a syrinx were identified. The most common associated condition was CM-I (occurring in 117 patients [43.2%]), followed by spinal dysraphism (20 [7.4%]), tumor (15 [5.5%]), and tethered cord (13 [4.8%]). Eighty-three patients (30.6%) did not have any associated condition of the brain or spinal cord and their syringes were considered idiopathic. Syringes in patients with CM-I were wide (7.8 ± 3.9 mm) compared with idiopathic syringes (3.9 ± 1.0, p < 0.0001) and those associated with tethered cord (4.2 ± 0.9, p < 0.01). When considering CM-I–associated and idiopathic syringes, the authors found that CM-I–associated syringes were more likely to have their cranial extent in the cervical spine (88%), compared with idiopathic syringes (43%; p < 0.0001). The combination of syrinx width greater than 5 mm and cranial extent in the cervical spine had 99% specificity (95% CI 0.92–0.99) for CM-I–associated syrinx. CONCLUSIONS Syrinx morphology differs according to syrinx etiology. The combination of width greater than 5 mm and cranial extent in the cervical spine is highly specific for CM-I–associated syringes. This may have relevance when determining the clinical significance of syringes in patients with low cerebellar tonsil position.

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