scholarly journals Syringomyelia intermittens: highlighting the complex pathophysiology of syringomyelia. Illustrative case

2021 ◽  
Vol 2 (11) ◽  
Author(s):  
Jorn Van Der Veken ◽  
Marguerite Harding ◽  
Saba Hatami ◽  
Marc Agzarian ◽  
Nick Vrodos
Keyword(s):  
Clinical Symptoms ◽  
Incidental Finding ◽  
Foramen Magnum ◽  
Illustrative Case ◽  
Cervical Canal ◽  
Type I ◽  
Natural Evolution ◽  
Chiari Type I Malformation ◽  
Asymptomatic Patients ◽  
History Of

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.

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

2018 ◽  
Vol 21 (1) ◽  
pp. 65-71 ◽  
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.

scholarly journals Functional and morphological changes in hypoplasic posterior fossa

2021 ◽  
Author(s):  
Federico Bianchi ◽  
Alberto Benato ◽  
Paolo Frassanito ◽  
Gianpiero Tamburrini ◽  
Luca Massimi
Keyword(s):  
Morphological Changes ◽  
Cervical Spinal Cord ◽  
Basilar Invagination ◽  
Foramen Magnum ◽  
Type I ◽  
Chiari Type I Malformation ◽  
Upper Cervical ◽  
Hindbrain Herniation ◽  
Cranial Fossa ◽  
2D And 3D

Abstract Background The knowledge of the development and the anatomy of the posterior cranial fossa (PCF) is crucial to define the occurrence and the prognosis of diseases where the surface and/or the volume of PCF is reduced, as several forms of craniosynostosis or Chiari type I malformation (CIM). To understand the functional and morphological changes resulting from such a hypoplasia is mandatory for their correct management. The purpose of this article is to review the pertinent literature to provide an update on this topic. Methods The related and most recent literature addressing the issue of the changes in hypoplasic PCF has been reviewed with particular interest in the studies focusing on the PCF characteristics in craniosynostosis, CIM, and achondroplasia. Results and conclusions In craniosynostoses, namely, the syndromic ones, PCF shows different degrees of hypoplasia, according to the different pattern and timing of early suture fusion. Several factors concur to PCF hypoplasia and contribute to the resulting problems (CIM, hydrocephalus), as the fusion of the major and minor sutures of the lambdoid arch, the involvement of the basal synchondroses, and the occlusion of the jugular foramina. The combination of these factors explains the variety of the clinical and radiological phenotypes. In primary CIM, the matter is complicated by the evidence that, in spite of impaired PCF 2D measurements and theories on the mesodermal defect, the PCF volumetry is often comparable to healthy subjects. CIM is revealed by the overcrowding of the foramen magnum that is the result of a cranio-cerebral disproportion (altered PCF brain volume/PCF total volume). Sometimes, this disproportion is evident and can be demonstrated (basilar invagination, real PCF hypoplasia); sometimes, it is not. Some recent genetic observations would suggest that CIM is the result of an excessive growth of the neural tissue rather than a reduced growth of PCF bones. Finally, in achondroplasia, both macrocephaly and reduced 2D and 3D values of PCF occur. Some aspects of this disease remain partially obscure, as the rare incidence of hydrocephalus and syringomyelia and the common occurrence of asymptomatic upper cervical spinal cord damage. On the other hand, the low rate of CIM could be explained on the basis of the reduced area of the foramen magnum, which would prevent the hindbrain herniation.

The Association of Chiari Type I Malformation and Neurofibromatosis Type 1

1993 ◽  
Vol 32 (3) ◽  
pp. 189-190 ◽  
Author(s):  
Joseph Dooley ◽  
Daniel Vaughan ◽  
Michael Riding ◽  
Peter Camfield
Keyword(s):  
Neurofibromatosis Type 1 ◽  
Chiari Malformation ◽  
Neurofibromatosis Type ◽  
Foramen Magnum ◽  
Type I ◽  
Chiari Type ◽  
Chiari Type I Malformation ◽  
Chiari Type I ◽  
Chiari Malformations

The association of neurofibromatosis type 1 (NF1) with Chiari malformations of the cerebellum and brain stem has been reported on only two previous occasions.1,2 The pathogenesis of both conditions has remained unclear, although the Chiari type I malformation is most likely due to hypoplasia of the posterior fossa with subsequent extension of the cerebellum through the foramen magnum.3 NF1 is also associated with a variety of cerebral dysplasias.4 We present a patient with both of these dysplastic lesions whose Chiari malformation was asymptomatic.

scholarly journals Clinical features of imported cases of coronavirus disease 2019 in Tibetan patients in the Plateau area

2020 ◽  
Author(s):  
Yu Lei ◽  
Xiaobo Huang ◽  
Bamu SiLang ◽  
YunPing Lan ◽  
Jianli Lu ◽  
...  
Keyword(s):  
Clinical Laboratory ◽  
Clinical Symptoms ◽  
Asymptomatic Group ◽  
Asymptomatic Patients ◽  
Abnormal Chest ◽  
Plateau Area ◽  
Imported Cases ◽  
High Altitude Area ◽  
History Of ◽  
The Mean

AbstractCoronavirus disease 2019 (COVID-19), caused by SARS-CoV-2, has rapidly spread throughout China, but the clinical characteristics of Tibetan patients living in the Qinghai-Tibetan plateau are unknown. We aimed to investigate the epidemiological, clinical, laboratory and radiological characteristics of these patients. We included 67 Tibetan patients with confirmed SARS-CoV-2 infection. The patients were divided into two groups based on the presence of clinical symptoms at admission, with 31 and 36 patients in the symptomatic and asymptomatic groups, respectively. The epidemiological, clinical, laboratory and radiological characteristics were extracted and analysed. No patient had a history of exposure to COVID-19 patients from Wuhan or had travelled to Wuhan. The mean age of Tibetan patients was 39.3 years and 59% of the patients were male. Seven patients presented with fever on admission and lymphocytopenia was present in 20 patients. 47 patients had abnormal chest CTs at admission instead of stating that 20 were unchanged. Lactate dehydrogenase levels were increased in 31 patients. Seven patients progressed to severe COVID-19; however, after treatment, their condition was stable. No patients died. Of the 36 asymptomatic patients, the mean age was younger than the symptomatic group (34.4±17.3vs 44.9±18.1 years, P=0.02). Lymphocyte count and prealbumin levels were higher in the asymptomatic group than the group with clinical symptoms (1.6±0.5 vs 1.3±0.6 and 241.8±68.2 vs 191.9±60.3, respectively; P<0.05). Imported cases of COVID-19 in Tibetan patients were generally mild in this high-altitude area. Absence of fever or radiologic abnormalities on initial presentation were common.

Trzyipółletni chłopiec z malformacją Chiariego typu I – opis przypadku

2017 ◽  
Vol 21 (3) ◽  
Author(s):  
Anna Worch ◽  
Małgorzata Wielopolska
Keyword(s):  
Chiari Malformation ◽  
Foramen Magnum ◽  
Common Type ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Chiari Type I Malformation ◽  
Large Head ◽  
Exacerbation Of Symptoms ◽  
Chiari Type I ◽  
Cerebellar Tonsils

Chiari malformation is the most common structural defect of the posterior fossa and cerebellum, consisting of displacements of hindbrain inclination through the foramen magnum, often associated with syringomyelia. In the most common type I of this disease, only the cerebellar tonsils are descended. Chiari type I malformation may remain asymptomatic in early childhood, however a number of unspecific symptoms, such as: neck pains and headaches, vertigos, balance and sight disorders, may strengthen with age. Because of unspecificity and various exacerbation of symptoms the disease is often misdiagnosed or undiagnosed. We describe a case of a 3.5-year old boy with whom his mother visited a pediatrician because of a chronic cough. During the examination mother of the patient informed that all infections start with vomiting and the patient is not able to stand up from horizontal position without turning first to the side and then to the abdomen. During the examination, a large head circumference was noticed (56 cm, > 97 percentile). MRI showed elongated cerebellar tonsils descended below the foramen magnum (left 14 mm, right 11 mm). Chiari malformation type I with associated syringomyelia was diagnosed. The patient was reffered to neurosurgery consultation.

Cough headache

2020 ◽  
pp. 220-224
Author(s):  
Julio Pascual ◽  
Peter van den Berg
Keyword(s):  
Posterior Fossa ◽  
Foramen Magnum ◽  
Type I ◽  
Chiari Type I Malformation ◽  
Cough Headache ◽  
Suboccipital Craniectomy ◽  
Secondary Form ◽  
Structural Lesion ◽  
Chiari Type I ◽  
Tonsillar Descent

Cough headache exists in a primary and secondary form. The latter is due to tonsillar descent or, more rarely, to other space-occupying lesions in the posterior fossa/foramen magnum. Up to 40% of patients have an underlying structural lesion. Most patients with primary cough headache respond to indomethacin and suboccipital craniectomy with posterior fossa reconstruction can relieve cough headache in Chiari type I malformation.

scholarly journals Traumatic Transient Herniation Concomitant with Tonsillar Hemorrhagic Contusion in a Child

2017 ◽  
Vol 5 (6) ◽  
pp. 771-773 ◽  
Author(s):  
Ahmet Öğrenci ◽  
Orkun Koban ◽  
Murat Ekşi ◽  
Onur Yaman ◽  
Sedat Dalbayrak
Keyword(s):  
Foramen Magnum ◽  
Type I ◽  
Tonsillar Ectopia ◽  
Chiari Type ◽  
Chiari Type I Malformation ◽  
Pediatric Age Group ◽  
Downward Displacement ◽  
The Relationship

Downward displacement of cerebellar tonsils more than 5 mm below the foramen magnum is named as Chiari type I malformation and named benign tonsillar ectopia if herniation is less than 3 mm. It does not just depend on congenital causes. There are also some reasons for acquired Chiari Type 1 and benign tonsillar ectopia/herniation. Trauma is one of them. Trauma may increase tonsillar ectopia or may be the cause of new-onset Chiari type 1. The relationship between the tonsil contusion and its position is unclear. We present a case of pediatric age group with tonsillar herniation with a hemorrhagic contusion. Only 1 case has been presented so far in the literature. A case with unilateral tonsil contusion has not been presented to date. We will discuss the possible reasons for taking the place of the tonsils to the above level of the foramen magnum in the follow-up period, by looking at the literature.

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