Chiari Malformations

Author(s):  
Marco Maurtua ◽  
Mathew Lyons ◽  
Nicholas DaPrano

Chiari malformations are structural defects in the base of the skull and cerebellum. These conditions are characterized by the abnormal displacement of part of the cerebellum and brainstem through the foramen magnum into the upper spinal canal causing autonomic dysfunction, neurologic deficits, and hydrocephalus. Chiari malformations are classified into several types based on their severity and the parts of the brain affected by the herniation. In neonates, Chiari malformations are commonly associated with spina bifida and myelomeningocele. Anesthesia for surgical correction of Chiari malformations presents a unique set of challenges and demands that requires knowledge of both the neurophysiology of the condition and the surgical process.

Author(s):  
Simon Rinaldi

This chapter covers four congenital neurological disorders which may be encountered in adult medicine: cerebral palsy, Chiari malformations, spina bifida, and tethered cord syndromes. Cerebral palsy is a disturbance of motor function arising from damage to the developing fetal or infant brain. It usually refers to a disorder resulting from a non-progressive insult which occurred at less than 3 years of age. Chiari malformations are congenital abnormalities of the anatomy and structural relationships of the cerebellum, the brainstem, and the foramen magnum. Dysraphism is a failure of opposition of anatomical structures which are normally fused. Spinal dysraphism is synonymous with spina bifida, a failure of embryological fusion of the neural tube. In all types, the vertebral arch fails to completely form. The tethered cord syndromes involve a restriction of the normal cephalad migration of the conus during life. This can occur both with and without spina bifida.


2018 ◽  
Vol 96 (2) ◽  
pp. 88-97 ◽  
Author(s):  
Yohaan Fernandes ◽  
Desire M. Buckley ◽  
Johann K. Eberhart

The term fetal alcohol spectrum disorder (FASD) refers to the entire suite of deleterious outcomes resulting from embryonic exposure to alcohol. Along with other reviews in this special issue, we provide insight into how animal models, specifically the zebrafish, have informed our understanding of FASD. We first provide a brief introduction to FASD. We discuss the zebrafish as a model organism and its strengths for alcohol research. We detail how zebrafish has been used to model some of the major defects present in FASD. These include behavioral defects, such as social behavior as well as learning and memory, and structural defects, disrupting organs such as the brain, sensory organs, heart, and craniofacial skeleton. We provide insights into how zebrafish research has aided in our understanding of the mechanisms of ethanol teratogenesis. We end by providing some relatively recent advances that zebrafish has provided in characterizing gene-ethanol interactions that may underlie FASD.


2019 ◽  
Vol 17 (07) ◽  
pp. 1950029 ◽  
Author(s):  
Lihai Ren ◽  
Dangdang Wang ◽  
Chengyue Jiang ◽  
Yuanzhi Hu

The biofidelity is an essential requirement of the application of human head finite element (FE) models to investigate head injuries under mechanical loadings. However, the influence of the foramen magnum boundary condition (FMBC) on intracranial dynamic responses under head impacts has yet to be fully identified until now. This study aimed to investigate the effect of different modeling methods of the FMBC on intracranial dynamic responses induced by forehead impact, especially the axonal injury associated dynamic responses. The total human model for safety (THUMS) was applied in this study. Two FE models with different FMBC modeling methods were developed from the THUMS model. Then, three forehead impact FE models were established respectively, including the original THUMS model. Further FE simulations were conducted to investigate the influence of FMBC modeling methods on intracranial dynamic responses. Though, difference between the intracranial dynamic responses (relative skull-brain motion and strain responses) at areas far from the foramen magnum were slightly, the corresponding difference at the brain stem area were distinctly. Meanwhile, the predicted axonal injury risk of the brain stem white matter was varying among each other. Different modeling methods of FMBC could result in different intracranial dynamic responses of the brain stem, and affect the axonal injury prediction. Therefore, the modeling of the FMBC should be further evaluated for the study of brain stem injury using human head FE models.


1993 ◽  
Vol 32 (3) ◽  
pp. 189-190 ◽  
Author(s):  
Joseph Dooley ◽  
Daniel Vaughan ◽  
Michael Riding ◽  
Peter Camfield

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.


1997 ◽  
Vol 10 (1) ◽  
pp. 63-102 ◽  
Author(s):  
N. Colombo ◽  
C. Maccagnano ◽  
C. Corona ◽  
A. Beltramello ◽  
G. Scialfa

Injury to the cervical spinal cord is a major health problem owing to its frequency and to the often devastating sequelae of serious trauma with respect to long-term disability for the patient. Cervical injuries are often reported in association with head trauma and cervical spinal cord injury appears to be a major contributing factor in acute death secondary to traffic accidents producing severe head injuries. A high incidence of neurological deficits is reported in cervical spinal trauma, but cervical injuries can escape detection in the acute phase if clinically silent or in patients unconscious from to head trauma. The most important predisposing factor in the concomitant occurrence of head and neck trauma is transmission of forces through the cranial vault to the cervical spine. Other underlying cervical spine diseases, either congenital or developmental, may also predispose to the development of cervical injuries. The spine includes bony-ligamentous structures and nervous structures. The bony-ligamentous involucre is anatomically predisposed to perform three major tasks: 1) maintenance of spinal statics; 2) mobilization in the three anatomic planes and 3) protection of nervous and vascular structures inside the spinal canal. The cervical spine is subjected to varying forces of flexion, flexion-rotation, extension and vertical compression which result in damage to the different components of the spine when they are applied beyond physiological limits. Biomechanical considerations of the different motion patterns that occur in the cervical spine are essential to understand the contribution of mechanical stresses to the development of specific spinal injuries. This chapter tackles the problem of a logical management of cervical spinal trauma based on clinical presentation to: a) identify the preferential diagnostic modality to investigate that type of injury (conventional X-Ray, Computed Tomography, Magnetic Resonance); b) interpret images, indipendently from the diagnostic modality utilized, considering the cause-effect relation between the traumatic force and the anatomic-functional structures involved by the trauma. The clinical picture may include pain, movement limitations and/or radiculo-myelopathy. Cerebral neurologic deficits can be the consequence of traumatic damage to the carotid and vertebral artery system in the neck. Evaluation of injury instability is one of the main goals of radiographic investigation. One classifies bony instability which is temporary, as opposed to disco-ligamentous instability which is permanent and usually requires surgical stabilization, and mixed instability. Conventional lateral and antero-posterior radiographs should be initially performed in patients with cervical trauma and in polytrauma and comatous patients who are difficult to assess clinically. They effectively screen vertebral fractures, vertebral body and facet dislocations and pre-vertebral soft tissue swelling. However, ligament disruption and instability can be underestimated by a normal disco-vertebral alignment. Dynamic flexion-extension views, useful to reveal such an instability, should never be performed in the acute phase particularly if fractures and neurologic deficits are present. CT scan, in addition, has several advantages: the axial plane provides an optimal view of the size and shape of the spinal canal, bony fragments and foreign bodies within the canal are very well depicted, posterior element fractures are better visualized. A preexsisting spondylotic narrow canal is well evaluated by CT as are post-traumatic disc herniations. Widening of the apophyseal joints, suggesting disruption of facet capsules and spinal instability, is best demonstrated by CT. However, CT has some limitations in evaluating ligament instability since it is performed in the neutral position and, in addition, it cannot visualize the medulla and its potential traumatic lesions. After the introduction of MRI, myelography and CT-myelography are no longer used to investigate cervical spine lesions involving cord and nerve roots. MRI should be performed in every patient presenting with neurologic deficits. The usefulness of MR is in detecting extradural compressive lesions like disc herniation and haematomas that need to be decompressed surgically. MRI can also evaluate ligamentous integrity and disk rupture. Bony fractures are revealed by MRI either by signal or morphologic alterations of vertebral bodies, but thin, linear fractures are less well identified than with CT. One of the main advantages of MRI is the direct identification of intrinsic cord pathology such as cord contusion and haemorrhage. Cord haemorrhage seems to be predictive of a complete lesion and of poor outcome. Therefore MRI is proposed to assess the prognosis of traumatic cord lesions, the best time for imaging ranging between 24 and 72 hours after injury.


2021 ◽  
Vol 27 (4) ◽  
pp. 4004-4009
Author(s):  
Ivan Maslarski ◽  
◽  
Lyudmila Belenska-Todorova ◽  

Purpose: Emissary foramens in the skull and emissary veins (EV), respectively, have been known for a long time, but their importance is often disregarded. In the present study, we introduce variants of occipital emissary foramens (OEF) unilaterally located on the left, close to оccipital condylе in a formation of three apertures that open together in a sinus near clivus. Material/Methods: Corpses of 30 dеad people were dissected in the Department of Anatomy, and standard techniques for soft tissue separation or maceration of the skull were used. After the removal of the calvaria, fixed bones of the skull were disarticulated. Foramens were cleaned using a double-ended probe, and depth and diameter measurements were performed using an atomical caliper. Results: We found variants of mastoid emissary foramens (MEF) situated into two groups, each of a couple of foramens. Their clinical significance is discussed, compared with existing experience and analysis of their phylogeny and embryogenesis. Conclusion: We introduce OEF and MEF to be important markers for detection of dural venous sinuses (DVS). The latter, together with EV, provide an important mechanism for decreasing intracranial pressure. This happens due to the absence of a valve apparatus in the veins of the brain, and the lack, or small amount, of muscle tissue. There is a possibility of existing varicose veins, such as those caused by arterio-venous fistulas and the pathology associated with it. We suggest the application of EV in imaging as an important study before surgery by lateral and transcondylar approach to the anterior foramen magnum.


2021 ◽  
Vol 1 (12) ◽  
pp. 896-903
Author(s):  
Genta Faesal Atsani ◽  
Zanetha Mauly Ilawanda ◽  
Ilma Fahira Basyir

Neural tube defects (NTD) are one of the birth defects or congenital abnormalities that occur in the brain and spine, and commonly find in newborns worldwide. Anencephaly and spina bifida are the two prevalent forms of NTD. The incidence of spina bifida happen on average 1 in 1000 cases of birth worldwide and there are 140,000 cases per year worldwide. Source searches were carried out on the online portal of journal publications as many as 20 sources from MedScape, Google Scholar and the Nation Center for Biotechnology Information / NCBI with the keywords “Neural tube defects (NTD), prevention, and spina bifida”. Spina bifida is a congenital abnormality that occurs in the womb due to a failure of closing process the neural tube during the first few weeks of embryonic development which causes the spine not completely close around the developing spinal cord nerves. NTD can ensue multifactorial conditions such as genetic, environmental, and folate deficiency. The use of folic acid supplementation starting at least 3 months before pregnancy, those are 400 mcg (0.4 mg) per day and 800 mcg per day during pregnancy can reduce the risk of developing neural tube defects such as spina bifida. Generally, spina bifida is undertaking by surgery and the regulation of patients comorbid. Public can find out prevention to avoid or reduce the risk of spina bifida so that the incidence of spina bifida can decrease along with the increasing awareness of the community regarding this disease.


Radiology ◽  
1950 ◽  
Vol 54 (4) ◽  
pp. 591-594 ◽  
Author(s):  
Julian R. Lewin ◽  
Henry T. Wycis ◽  
Barton R. Young

2018 ◽  
Vol 30 (3) ◽  
pp. 392-399 ◽  
Author(s):  
Daniel R. Rissi

Feline infectious peritonitis (FIP) is one of the most important viral diseases of cats worldwide. Our study describes the neuropathology and the diagnostic features of 26 cases of FIP in domestic cats. The average age of affected individuals was 11.8 mo, and there was no sex or breed predisposition. Clinical neurologic signs were noted in 22 cases, and rabies was clinically suspected in 11 cases. Twenty cats had lesions in multiple organs, and 6 cats had lesions only in the brain. Gross neuropathologic changes occurred in 15 cases and consisted of hydrocephalus (10 cases), cerebellar herniation through the foramen magnum (6 cases), cerebral swelling with flattening of gyri (2 cases), and accumulation of fibrin within ventricles (2 cases) or leptomeninges (1 case). Histologically, 3 main distinct distributions of neuropathologic changes were observed, namely periventricular encephalitis (12 cases), rhombencephalitis (8 cases), and diffuse leptomeningitis with superficial encephalitis (6 cases). Fresh tissue samples were submitted for fluorescent antibody testing (FAT) after autopsy in 17 cases, and positive results were found in only 7 cases. Immunohistochemistry (IHC) for feline coronavirus confirmed the diagnosis in all 26 cases. IHC appears to be a more sensitive and reliable test for confirmation of FIP than is FAT.


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