Two Cases of Arnold-Chiari Malformation with Respiratory Failure (original) (raw)
Related papers
Multidisciplinary respiratory medicine, 2013
Arnold Chiari Malformation (ACM) is defined as a condition where part of the cerebellar tissue herniates into the cervical canal toward the medulla and spinal cord resulting in a number of clinical manifestations. Type I ACM consists of variable displacement of the medulla throughout the formamen magnum into the cervical canal, with prominent cerebellar herniation.Type I ACM is characterized by symptoms related to the compression of craniovertebral junction, including ataxia, dysphagia, nistagmus, headache, dizziness, and sleep disordered breathing. We report a case of a life-long non-smoker, 54 years old woman who presented these symptoms associated with bronchiectasis secondary to recurrent inhalation pneumonia, hypercapnic respiratory failure, and central sleep apnea (CSA).CSA was first unsuccessfully treated with nocturnal c-PAP. The subsequent treatment with low flow oxygen led to breathing pattern stabilization with resolution of CSA and related clinical symptoms during sleep....
Neurosurgical Review, 2000
Patients with craniocervical disorders (CCD) show a wide variety of symptoms and signs suggesting cerebellar and/or high cervical lesion. The anatomic localization of respiratory centers and their possible injury may explain the presence of respiratory disturbances in these diseases. The aim of this preliminary study was to evaluate the polysomnographic findings in a group of patients with CCD, most of them with Arnold-Chiari malformation type I, since sleep apnea has been referred to in isolated cases in the literature. Eleven patients (seven females and four males) with CCD diagnosed by magnetic resonance imaging referred from the neurosurgery unit were submitted to clinical history, physical examination with sleep questionnaires, and scored on the Epworth Sleepiness Scale. Full night polysomnography was performed in an Oxford SAC system where EEG, electro-oculography, electrocardiography, chin and leg electromyography, chest and abdominal efforts, airflow, and oximetry were recorded continuously. Nine patients presented with Arnold-Chiari type I malformation, of whom six showed associated syringomyelia. The other two had basilar invagination. Ninety percent of these patients complained of sleep problems (snoring, choking, and witnessed apneas) and 72% presented hypersomnolence (ESS >9). The polysomnographic findings showed sleep fragmentation in 81% of the patients and a reduction of rapid eye movement sleep in 63%. The apnea/hypopnea index was above 5 in 72%, with a predominance of central apnea. Patients with craniocervical disorders present a higher probability of displaying sleep respiratory disturbances. Their sleep complaints should be assessed and patients should be submitted to an overnight sleep recording in order to identify sleep apnea.
patient with Sleep Disordered Breathing (SDB) of central origin secondary to Arnold Chiari type I malformation: a case report. Journal of Surgery and Research 4 (2021): 80-84. Abstract The diagnosis and treatment of Sleep Disordered Breathing (SDB) in pediatric age differs in relation to the adult. Central sleep apnea syndrome (CSAS) is less frequent in childhood, and one of the causes of it is the alterations of the brainstem like the malformation of Chiari type I. The main diagnostic method of SDB is Polysomnography, and there are other methods like brain magnetic resonance imaging (MRI) to investigate its etiology. Here, we share our experience with an 8-year-old female with malformation of Chiari type I and CSAS, and the steps we followed for the diagnosis and successful treatment of such an infrequent pathology in childhood.
Adult Chiari malformation and sleep apnoea
Neurosurgical Review, 2005
Chiari malformation (CM) is primarily characterised by herniation of the cerebellar tonsils through the foramen magnum. Clinically, two main types of CM represent the vast majority of cases: type I (in adults) and type II (in infants). CM may result in neuronal impairment of the brainstem, upper spinal cord and cranial nerves. Part of the afferent and efferet systems and the central respiratory controlling system are located in the cranium-cervical transition and may be damaged in these pathologies, leading to respiratory disorders, such as respiratory failure and death. The ventilatory responses to exogenous and endogenous stimuli, such as responses to hypoxia and hypercapnia, are usually diminished, and apnea may be manifested and detected during sleep, allowing for the diagnosis. This study is a review of the relationship between sleep apnoea and adult CM.
Clinical and radiological findings in Arnold Chiari malformation
Journal of Ayub Medical College Abbottabad Jamc, 2010
BACKGROUND: The Chiari Malformation I (CMI) is a disorder of uncertain origin that has been traditionally defined as downward herniation of the cerebellar tonsils through the foramen magnum. The anomaly is a leading cause of syringomyelia and occurs inassociation with osseus abnormalities at the craniovertebral junction. In contrast to other Chiari malformations, CMI tends to present in the second or third decade of life and is sometimes referred to as the 'adult-type' Chiari malformation. The objective was to document clinical and radiological findings in Arnold Chiari Malformation-I.METHOD: This was a descriptive study carried out in Ayub Teaching Hospital Abbottabad at Neurosurgery Department during July 2008-July 2010. We examined a prospective cohort of 60 symptomatic patients. All patients underwent magnetic resonance imaging of the head and spine.RESULTS: There were 40 female and 20 male patients. The age of onset was 24.9 +/- 15.8 years. Common associated radiological problems included syringomyelia (60%), scoliosis (25%), and basilarinvagination (12%), increased cervical lordosis 5 (8.5%), and Klippel Feil syndrome 2 (3.3%). The most consistent magnetic resonance imaging findings were obliteration of the retrocerebellar cerebrospinal fluid spaces (70% patients), tonsillar herniation of at least 5 mm (100% patients), and varying degrees of post fossa anomalies. Linical manifestations were headaches, pseudotumor-like episodes, a Meniere's disease-like syndrome, lower cranial nerve signs, and spinal cord disturbances in the absence of syringomyelia.CONCLUSION: These data support accumulating evidence that CMI is a disorder of the para-axial mesoderm that is characterised by underdevelopment of the posterior cranial fossa and overcrowding of the normally developed hindbrain. Tonsillar herniation of less than 5 mm does not exclude the diagnosis. Clinical manifestations of CMI seem to be related to cerebrospinal fluid disturbances (which are responsible for headaches, pseudotumor-like episodes, endolymphatic hydrops, syringomyelia, and hydrocephalus) and direct compression of nervous tissue.
Symptomatic Arnold-Chiari malformation: review of experience with 22 cases
Journal of Neurosurgery, 1987
✓ Twenty-two patients with closed myelomeningoceles, shunted hydrocephalus, and symptomatic Arnold-Chiari malformations were studied retrospectively. Seventeen of the 22 patients were aged 6 months or younger; five patients were aged 3 to 23 years. Patients in the younger group presented with stridor, apnea, and/or feeding difficulty; those in the older group presented with hemiparesis, quadriparesis, oscillopsia, nystagmus, or opisthotonos. Fourteen of the 17 younger patients underwent surgical decompression of the Arnold-Chiari malformation: 10 within 18 days following the onset of symptoms and four on Day 19 or later. Of the 10 infants with early treatment, five eventually died secondary to continued symptoms and five survived. Of those surviving, three were asymptomatic and two had continuing symptoms but were improved over their preoperative state. Among the four patients undergoing surgery later, two died, one had lessening of stridor, and one had complete relief of symptoms. ...
Type I Chiari malformation presenting central sleep apnea
Auris Nasus Larynx, 2014
Type I Chiari malformation (CM-I) is a neuroanatomical abnormality of the cranial posterior fossa resulting in cerebellar tonsillar herniation through the foramen magnum into the cervical spinal canal. CM-I is the most common of the 5 known subtypes of the Chiari malformation [1]. The estimated prevalence of CM-I is 0.1-0.5% [2,3], although the exact prevalence is unknown because some CM-I patients may be asymptomatic [4]. Sleep apnea is one clinical manifestation of CM-I. The anatomical site of the respiratory centers and its possible injury may explain the presence of sleep-disordered breathing in CM-I patients. All types of apnea, including central, obstructive, and mixed, have been described in CM-I patients. Central apnea, a rare condition in the general population, is common among CM-I patients, especially in pediatric patients [5], although it rarely presents without other neurological symptoms [2].
Journal of Neurosurgery, 2010
Object. One of the feared consequences of craniovertebral junction diseases is apnea. Although several cases of patients with central apnea have been described, obstructive sleep apnea has been identified as the most frequent manifestation of sleep respiratory disorder. Neuronal involvement may be responsible for both central and obstructive apneas. The objective of this work was to study the effect of posterior fossa decompressive surgery on respiratory parameters during sleep in patients with craniovertebral junction malformations and breathing-related sleep disorders.
Sleep-disordered breathing as presenting manifestation of Chiari type I malformation: a case report
Tanaffos, 2014
Chiari Type I malformation (CM-I) is a rare disorder with displaced cerebellar tonsils through foramen magnum. Here we present a 30-year-old man with severe central and obstructive sleep apneas as presenting manifestations of CM-I. The patient underwent neurosurgery and the follow-up polysomnography revealed the resolution of central apnea while obstructive apnea remained unchanged. Central sleep apnea (CSA) could be associated with an underlying pathology; thus, further investigation is recommended in affected subjects.