Type I Chiari deformity presenting with profound sinus bradycardia: case report and literature review

Selmi, Fahed; Davies, Kath; Weeks, Robert D.

doi:10.1080/02688699550041205

Cited by 26 publications

(8 citation statements)

References 8 publications

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“…43 Rarely, direct brainstem compression may cause autonomic symptoms, such as syncopal episodes, 66 drop attacks, 15 and sinus bradycardia. 51 Central sleep apnea is the most common manifestation of brainstem/ medullary compression in CIM, seen in roughly 13% of pediatric patients at presentation and more common in children younger than 6 years of age. 46,54 Sleep studies can be useful for elucidating evidence of central sleep apnea if patients present with CIM and concern for snoring.…”

Section: Symptoms Clinical Presentation and Patient Selection In Cimmentioning

confidence: 99%

The Chiari I malformation

McClugage¹,

Oakes²

2019

Journal of Neurosurgery: Pediatrics

100

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As with many pathologies, the course of our understanding of the Chiari I malformation (CIM) has developed extensively over time. The early descriptions of the Chiari malformations by Hans Chiari in 1891 opened the door for future classification and research on this topic. However, even over a long timeframe, our understanding of the pathophysiology and, more importantly, treatment, remained in its infancy. As recently as the 1970s, CIM was not discussed in popular neurology textbooks. Syringomyelia is listed as a degenerative disorder with no satisfactory treatment. Radiation therapy was considered an option in treatment, and surgery was thought to play no role. During the last 40 years, equivalent to the duration of a neurosurgical career, our understanding of the pathophysiology and natural history of CIM, coupled with modern MRI, has improved the treatment paradigm for this patient population. More importantly, it has given us evidence confirming that CIM is a disorder responsive to surgical intervention, giving patients once thought to be destined for lifelong disability a comparatively normal life after treatment. The purpose of this article is to offer a review of CIM and its important associated entities. The authors will discuss the evolution in understanding of the Chiari malformation and, importantly, distinguish between symptomatic CIM and asymptomatic tonsillar ectopia, based on imaging and presenting symptomatology. They will discuss techniques for surgical intervention, expected outcomes, and complications after surgery. Proper patient selection for surgery based on appropriate symptomatology is tantamount to achieving good surgical outcomes in this population, separating those who can be helped by surgery from those who are unlikely to improve. While our knowledge of the Chiari malformations continues to improve through the efforts of clinical and basic science researchers, surgeons, and patients, our current understanding of these entities represents a monumental improvement in patient care over a relatively short time period.

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Section: Symptoms Clinical Presentation and Patient Selection In Cimmentioning

confidence: 99%

The Chiari I malformation

McClugage¹,

Oakes²

2019

Journal of Neurosurgery: Pediatrics

100

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“…Speech and swallowing signs also may occur [10, 16, 17]. Brainstem dysfunction may be seen, and sinus bradycardia, hypertension, central sleep apnea, and drop attacks have been reported [10, 20, 21]. Downward herniation of the brainstem can occur in rare circumstances, including with a sudden change in intracranial pressure or a volume shift as might occur from a blow to the occiput or neck [22-26].…”

mentioning

confidence: 99%

Chiari I Malformation in Nephropathic Cystinosis

Rao

Hesselink

Trauner

2015

The Journal of Pediatrics

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Objective To determine the relative incidence of Chiari I malformations in children with cystinosis compared with those in the general population. Study design Magnetic resonance imaging (MRI) scans were performed on 53 patients with nephropathic cystinosis and 120 controls, age range 3-18 years. Results Ten of 53 (18.9%) cystinosis patients had Chiari I or tonsillar ectopia, and only 2 of 120 controls (1.6%) had a similar finding. At least 2 of the patients had symptoms or signs thought to be related to the malformation, and one had surgical decompression. Two had an associated cervical syrinx. Conclusions Children with cystinosis have a 12-fold higher prevalence of Chiari I malformations than the general pediatric population. Chiari I malformations should be high on the differential diagnosis when individuals with cystinosis develop neurologic signs and symptoms, and MRI scans should be performed on children with cystinosis who present with new-onset headache, ataxia, incontinence, or other unexplained neurologic symptoms.

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“…Unlike our case, complication in their case occurred in the intraoperative period as a result of surgical manipulation. Severe sinus bradycardia has been reported following medullary compression in Chiari I malformation, which was managed with a pacemaker implantation [12]. Surgical decompression, in this case, proved curative and the pacemaker was removed.…”

Section: Discussionmentioning

confidence: 90%

Atypical Presentations in Chiari II Malformation

Rath

Bithal²,

Chaturvedi³

2006

Pediatr Neurosurg

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Myelomeningocele with Chiari II malformation and hydrocephalus is a common association seen in infants with a congenital failure of neurulation. Here we report two cases of such an association presenting with different sets of problems. The first patient presented with severe inspiratory stridor due to bilateral abductor vocal cord paralysis, which was relieved completely within 24 h of definitive surgery. The second patient experienced intraoperative cardiac arrest. Definitive surgery was followed after successful cardiopulmonary resuscitation. The cause of the perioperative sequence of events in both cases is attributed to the associated pathologies of Chiari II malformation.

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Type I Chiari deformity presenting with profound sinus bradycardia: case report and literature review

Cited by 26 publications

References 8 publications

The Chiari I malformation

The Chiari I malformation

Chiari I Malformation in Nephropathic Cystinosis

Atypical Presentations in Chiari II Malformation

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