-
BMC Pulmonary Medicine Feb 2020Prolonged and difficult weaning is associated with higher rates of complications and mortality. Therefore, it is important to identify the associated factors.
Arnold-Chiari malformation type I and the posterior dislocation of the odontoid process aggravate prolonged weaning in a patient with severe viral pneumonia: a case report.
BACKGROUND
Prolonged and difficult weaning is associated with higher rates of complications and mortality. Therefore, it is important to identify the associated factors.
CASE PRESENTATION
We describe our experience with a 37-year-old man diagnosed with severe viral pneumonia (influenza A). He presented with acute respiratory failure type I on admission. During intubation, his blood pressure and heart rate decreased, and epinephrine and norepinephrine were administered. Although his clinical condition improved 8 days after intensive care unit (ICU) admission, he experienced difficulty weaning. He remained conscious but had a poor spontaneous cough with sputum production and weak limb muscle strength. His cough reflex was absent during bronchoscopic sputum suction, and he used abdominal breathing during the T-tube test. Magnetic resonance imaging revealed an Arnold-Chiari malformation type I, posterior dislocation of the odontoid process, and syringomyelia, with compression and deformation of the medulla and high cervical cord. The patient was successfully weaned from the ventilator at 20 days after ICU admission.
CONCLUSIONS
Arnold-Chiari malformation type I and posterior dislocation of the odontoid process, which aggravate medullary compression and increase the risk of cervical nerve injury, might be a rare factor affecting prolonged weaning in critical illness.
Topics: Adult; Arnold-Chiari Malformation; Humans; Magnetic Resonance Imaging; Male; Odontoid Process; Pneumonia, Viral; Ventilator Weaning
PubMed: 32046709
DOI: 10.1186/s12890-020-1078-1 -
Acta Neurochirurgica Jul 2020Recently, a novel hypothesis has been proposed concerning the origin of craniovertebral junction (CVJ) abnormalities. Commonly found in patients with these entities,... (Review)
Review
INTRODUCTION
Recently, a novel hypothesis has been proposed concerning the origin of craniovertebral junction (CVJ) abnormalities. Commonly found in patients with these entities, atlantoaxial instability has been suspected to cause both Chiari malformation type I and basilar invagination, which renders the tried and tested surgical decompression strategy ineffective. In turn, C1-2 fusion is proposed as a single solution for all CVJ abnormalities, and a revised definition of atlantoaxial instability sees patients both with and without radiographic evidence of instability undergo fusion, instead relying on the intraoperative assessment of the atlantoaxial joints to confirm instability.
METHODS
The authors conducted a comprehensive narrative review of literature and evidence covering this recently emerged hypothesis. The proposed pathomechanisms are discussed and contextualized with published literature.
CONCLUSION
The existing evidence is evaluated for supporting or opposing sole posterior C1-2 fusion in patients with CVJ abnormalities and compared with reported outcomes for conventional surgical strategies such as posterior fossa decompression, occipitocervical fusion, and anterior decompression. At present, there is insufficient evidence supporting the hypothesis of atlantoaxial instability being the common progenitor for CVJ abnormalities. Abolishing tried and tested surgical procedures in favor of a single universal approach would thus be unwarranted.
Topics: Arnold-Chiari Malformation; Atlanto-Axial Joint; Decompression, Surgical; Humans; Joint Instability; Spinal Fusion
PubMed: 32504118
DOI: 10.1007/s00701-020-04429-z -
Acta Otorrinolaringologica Espanola 2015Chiari malformation is an alteration of the base of the skull with herniation through the foramen magnum of the brain stem and cerebellum. Although the most common... (Review)
Review
INTRODUCTION
Chiari malformation is an alteration of the base of the skull with herniation through the foramen magnum of the brain stem and cerebellum. Although the most common presentation is occipital headache, the association of audio-vestibular symptoms is not rare. The aim of our study was to describe audio-vestibular signs and symptoms in Chiari malformation type i (CM-I).
MATERIALS AND METHODS
We performed a retrospective observational study of patients referred to our unit during the last 5 years. We also carried out a literature review of audio-vestibular signs and symptoms in this disease.
RESULTS
There were 9 patients (2 males and 7 females), with an average age of 42.8 years. Five patients presented a Ménière-like syndrome; 2 cases, a recurrent vertigo with peripheral features; one patient showed a sudden hearing loss; and one case suffered a sensorineural hearing loss with early childhood onset. The most common audio-vestibular symptom indicated in the literature in patients with CM-I is unsteadiness (49%), followed by dizziness (18%), nystagmus (15%) and hearing loss (15%). Nystagmus is frequently horizontal (74%) or down-beating (18%). Other audio-vestibular signs and symptoms are tinnitus (11%), aural fullness (10%) and hyperacusis (1%). Occipital headache that increases with Valsalva manoeuvres and hand paresthesias are very suggestive symptoms.
CONCLUSIONS
The appearance of audio-vestibular manifestations in CM-I makes it common to refer these patients to neurotologists. Unsteadiness, vertiginous syndromes and sensorineural hearing loss are frequent. Nystagmus, especially horizontal and down-beating, is not rare. It is important for neurotologists to familiarise themselves with CM-I symptoms to be able to consider it in differential diagnosis.
Topics: Adult; Aged; Arnold-Chiari Malformation; Child, Preschool; Female; Headache; Hearing Loss, Sensorineural; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Neuroimaging; Nystagmus, Pathologic; Symptom Assessment; Syringomyelia; Tinnitus; Vertigo
PubMed: 25195076
DOI: 10.1016/j.otorri.2014.05.002 -
Child's Nervous System : ChNS :... Jun 2018Chiari I malformation has been a well-recognized clinical entity; however, its occurrence among infants and toddlers is unusual. Their clinical presentations may be...
OBJECTIVES
Chiari I malformation has been a well-recognized clinical entity; however, its occurrence among infants and toddlers is unusual. Their clinical presentations may be different from other age groups due to their lack of effective verbal communication. The authors analyze their personal series of patients focusing on symptomatology and MRI characteristics. Treatment methods, results, and outcome are analyzed in order to identify appropriate surgical management among infants and toddlers with Chiari I malformation.
METHODS
The authors retrospectively reviewed 16 patients who were diagnosed and surgically treated between 2007 and 2014 during the first 3 years of life with minimum follow-up of 3 years. We focused on the presenting symptoms, magnetic resonance imaging findings, and surgical techniques used for posterior fossa decompression (PFD) and their postoperative outcome.
RESULTS
Twelve patients (75%) presented with signs of headaches such as irritability, inconsolable crying, head grabbing, and/or arching back. Ten patients (62.5%) presented with oropharyngeal and/or respiratory symptoms such as emesis, choking, gagging, snoring, sleep apnea, breathing pause, and/or vocal cord palsy. Only one patient had segmental cervical hydromyelia. At the first surgery, ten patients had PFD with dural scoring (Type 1 procedure), while six others had PFD with duraplasty (Type 2 procedure) with thermal reduction of the cerebellar tonsils in four. Following the first operation, all initially had varying degrees of symptomatic improvement; however, seven patients subsequently had symptomatic recurrence. Persistent crowding at the PFD site on the postoperative imaging indicated greater risk of recurrences in both Type 1 procedure and Type 2 procedure groups. Of seven patients who needed a second operation, fivewere after Type 1 procedure and the two were after Type 2 procedure. The difference of recurrence rates between these two groups is not significant. CSF-related complications occurred in 4 out of 11 patients who had Type 2 procedure (one after primary decompression and three after the second decompression for recurrence).
CONCLUSION
Young patients lacking effective verbal communication often present their Chiari I malformation differently from olderage groups. Behavioral changes indicative of headaches/irritability and oropharyngeal/respiratory symptoms are the primary presenting symptoms. The recurrence rate tends to be higher among the patients after Type 1 procedure (particularly those younger than 18 months) than after Type 2 procedure. We observed that duraplasty at primary or at redo PFD provides for better decompression and long-term outcome. However, one should keep it in mind that there is risk of CSF-related complications following duraplasty, particularly higher tendency after redo PFD.
Topics: Arnold-Chiari Malformation; Child, Preschool; Decompression, Surgical; Female; Humans; Infant; Male; Neurosurgical Procedures; Postoperative Complications; Recurrence; Retrospective Studies
PubMed: 29396718
DOI: 10.1007/s00381-017-3712-7 -
Neurosurgery Clinics of North America Oct 2015This article summarizes the current understanding of the pathophysiology of the Chiari I malformation that is based on observations of the anatomy visualized by modern... (Review)
Review
This article summarizes the current understanding of the pathophysiology of the Chiari I malformation that is based on observations of the anatomy visualized by modern imaging with MRI and prospective studies of the physiology of patients before and after surgery. The pathogenesis of a Chiari I malformation of the cerebellar tonsils is grouped into 4 general mechanisms.
Topics: Arnold-Chiari Malformation; Cerebrospinal Fluid; Cranial Fossa, Posterior; Humans; Hydrodynamics; Magnetic Resonance Imaging; Skull
PubMed: 26408057
DOI: 10.1016/j.nec.2015.06.003 -
Journal of Clinical Sleep Medicine :... Sep 2022The main aim was to evaluate the prevalence of sleep-disordered breathing (SDB) in patients with Chiari II malformation (CM-II). The secondary objectives were to... (Review)
Review
STUDY OBJECTIVES
The main aim was to evaluate the prevalence of sleep-disordered breathing (SDB) in patients with Chiari II malformation (CM-II). The secondary objectives were to evaluate the association between SDB, morphological abnormalities, and neurological symptoms and to review the literature on patients with SDB and CM-II.
METHODS
The study has a cross-sectional, case-control design. Patients with CM-II (patients) were compared to control patients referred for clinical polysomnography in the Sleep Medicine Unit, matched for age and sex. All patients underwent brain and spinal cord magnetic resonance imaging, and polysomnography was conducted for all participants. A review of the literature about SDB in patients with CM-II was performed.
RESULTS
Forty patients were included (20 patients vs 20 control patients). SDB was identified in 45% of patients, a significantly higher prevalence compared to control patients. Three patients presented with purely obstructive SDB, 3 patients with purely central SDB, and 3 patients with both obstructive and central SDB. Compared with control patients, patients with CM-II showed a higher oxygen desaturation index (median: CM-II, 3.7; interquartile range, 1.6-19.5; control patients: 1.1; interquartile range, 0.3-3.2) and obstructive apnea-hypopnea index (median: CM-II, 1.5; interquartile range, 0.5-5.1; control patients, 0.1; interquartile range, 0.0-0.7). A logistic regression showed that the risk of developing SDB in patients affected by CM-II was 14.7 times higher than in the control population.
CONCLUSIONS
Our study and literature review showed a high prevalence of SDB in patients with CM-II. These patients are often asymptomatic at diagnosis, suggesting that PSG should be routinely provided in this population.
CITATION
Lazzareschi I, Curatola A, Massimi L, et al. Sleep-disordered breathing in patients with Chiari malformation type II: a case-control study and review of the literature. 2022;18(9):2143-2154.
Topics: Arnold-Chiari Malformation; Case-Control Studies; Cross-Sectional Studies; Humans; Polysomnography; Sleep Apnea Syndromes
PubMed: 35645039
DOI: 10.5664/jcsm.10062 -
Neurosurgery Clinics of North America Oct 2015Despite the frequency of Chiari-associated spinal deformities, this disease process remains poorly understood. Syringomyelia is often present; however, this is not... (Review)
Review
Despite the frequency of Chiari-associated spinal deformities, this disease process remains poorly understood. Syringomyelia is often present; however, this is not necessary and scoliosis has been described in the absence of a syrinx. Decompression of the hindbrain is often recommended. In young patients (<10 years old) and/or those with small coronal Cobb measurements (<40°), decompression of the hindbrain may lead to resolution of the spinal deformity. Spinal fusion is reserved for those curves that progress to deformities greater than 50°. Further research is needed to understand the underlying pathophysiology to improve prognostication and treatment of this patient population.
Topics: Arnold-Chiari Malformation; Decompression, Surgical; Humans; Magnetic Resonance Imaging; Scoliosis; Spinal Fusion; Syringomyelia
PubMed: 26408068
DOI: 10.1016/j.nec.2015.06.005 -
Neurosurgery Clinics of North America Jan 2023Anatomic MRI, MRI flow studies, and intraoperative ultrasonography demonstrate that the Chiari I malformation obstructs CSF pathways at the foramen magnum and prevents... (Review)
Review
Anatomic MRI, MRI flow studies, and intraoperative ultrasonography demonstrate that the Chiari I malformation obstructs CSF pathways at the foramen magnum and prevents normal CSF movement through the foramen magnum. Impaired CSF displacement across the foramen magnum during the cardiac cycle increases pulsatile hindbrain motion, pressure transmission to the spinal subarachnoid space, and the amplitude of CSF subarachnoid pressure waves driving CSF into the spinal cord. Central canal septations in adults prevent syrinx formation by CSF directly transmitting its pressure wave from the fourth ventricle to the central canal.
Topics: Adult; Humans; Syringomyelia; Hydrodynamics; Arnold-Chiari Malformation; Subarachnoid Space; Cerebrospinal Fluid Pressure
PubMed: 36424067
DOI: 10.1016/j.nec.2022.08.007 -
Journal of Integrative Neuroscience May 2022Chiari I malformation is defined as caudal displacement of the cerebellar tonsils into the foramen magnum. The most commonly associated finding is cervical...
INTRODUCTION
Chiari I malformation is defined as caudal displacement of the cerebellar tonsils into the foramen magnum. The most commonly associated finding is cervical syringomyelia. The most common presenting symptom is occipital and upper cervical pain. The incidence of syringomyelia is between 30% and 70%. Surgery is recommended for symptomatic patients. The main purpose of the study is to present the data of children with Chiari type I malformation that were treated surgically in low-volume center and to describe some atypical presentations in children.
MATERIALS AND METHODS
At University Medical Centre Maribor, Slovenia, we performed a retrospective study of children with Chiari type I malformation that were treated surgically in the period from 2012 until 2021. The indication for surgery was symptomatic Chiari type I malformation. Suboccipital decompression with laminectomy of at least C1 (in one case also C2) with splitting of dura or duraplasty was performed. In a few cases coagulation of both tonsillar tips was necessary to achieve sufficient decompression and restoration of adequate cerebrospinal fluid (CSF) flow.
RESULTS
Ten children (under 18 years of age) underwent surgery for Chiari I malformation. Four patients had atypical presentation. In nine patients there was improvement after surgery. Complication rate was zero with no revision surgery. In one case suboccipital headache persisted. In all cases with preoperative syringomyelia it improved after surgery. Scoliosis improved in two out of three cases. There was no mortality after the procedure.
DISCUSSION
When patients with Chiari I malformation become symptomatic, it is recommended to perform surgery as soon as possible. Comparing the techniques; dura-splitting technique has advantages of lesser operation duration, lesser intraoperative bleeding and lower complication rates than duraplasty.
CONCLUSIONS
The majority of patients with Chiari I malformation improve after surgery. Surgical procedure is safe with very low morbidity and mortality. Surgical technique must be an individualized patient tailored choice.
Topics: Adolescent; Arnold-Chiari Malformation; Child; Decompression, Surgical; Dura Mater; Humans; Magnetic Resonance Imaging; Retrospective Studies; Syringomyelia; Treatment Outcome
PubMed: 35864752
DOI: 10.31083/j.jin2104100 -
The Bone & Joint Journal Jun 2017In the United Kingdom, lower incidences of intraspinal abnormalities in patients with early onset idiopathic scoliosis have been observed than in studies in other... (Review)
Review
AIMS
In the United Kingdom, lower incidences of intraspinal abnormalities in patients with early onset idiopathic scoliosis have been observed than in studies in other countries. We aimed to determine the rates of these abnormalities in United Kingdom patients diagnosed with idiopathic scoliosis before the age of 11 years.
PATIENTS AND METHODS
This retrospective study of patients attending an urban scoliosis clinic identified 71 patients satisfying a criteria of: clinical diagnosis of idiopathic scoliosis; age of onset ten years and 11 months or less; MRI screening for intraspinal abnormalities. United Kingdom census data combined with patient referral data was used to calculate incidence.
RESULTS
Mean age at diagnosis was six years with 39 right-sided and 32 left-sided curves. Four patients (5.6%) were found to have intraspinal abnormalities on MRI. These consisted of: two combined Arnold-Chiari type 1 malformations with syrinx; one syrinx with a low lying conus; and one isolated syrinx. Overall annual incidence of early onset idiopathic scoliosis was one out of 182 000 (0.0006%).
CONCLUSION
This study reports the lowest rates to date of intraspinal anomalies in patients with early onset idiopathic scoliosis, adding to knowledge regarding current incidences of these abnormalities as well as any geographical variation in the nature of the disease. Cite this article: 2017;99-B:829-33.
Topics: Age Distribution; Age of Onset; Arnold-Chiari Malformation; Child; Child, Preschool; Female; Humans; Incidence; Infant; Magnetic Resonance Imaging; Male; Nervous System Malformations; Retrospective Studies; Scoliosis; Syringomyelia
PubMed: 28566405
DOI: 10.1302/0301-620X.99B6.BJJ-2016-1159.R1