Chiari Malformation Type 1: A Systematic Review of Natural History and Conservative Management.

BACKGROUND: Chiari malformation type 1 (CM-1) is a variation of hindbrain development that can sometimes occur in asymptomatic individuals. Conventional treatment is surgical decompression, but little is known about the natural history of patients who do not undergo surgical management. This information is critical to determine how these patients should be managed. We conducted a systematic literature review to determine the natural history of CM-1, particularly in patients who did not undergo surgery and in asymptomatic individuals, to help patients and physicians determine when surgery is likely to be beneficial. METHODS: The literature search was performed following Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines using the electronic databases PubMed, Scopus, Cochrane Library, and Web of Science. Inclusion and exclusion criteria were predefined. RESULTS: In symptomatic patients who did not undergo surgery, headaches and nausea often improved, whereas ataxia and sensory disturbance tended not to improve spontaneously. Of patients, 27%-47% had an improvement in symptoms after 15 months, and 37%-40% with cough headache and 89% with nausea who were managed nonoperatively improved at follow-up. Most asymptomatic individuals with CM-1 remained asymptomatic (93.3%) even in the presence of syringomyelia. CONCLUSIONS: The natural history of mild symptomatic and asymptomatic CM-1 in adults is relatively benign and nonprogressive; the decision to perform surgical decompression should be based on severity and duration of a patient's symptoms at presentation. It is reasonable to observe a patient with mild or asymptomatic symptoms even in the presence of significant tonsillar descent or syringomyelia.

Prospective analysis of presenting symptoms among 265 patients with radiographic evidence of Chiari malformation type I with or without syringomyelia.

OBJECTIVE: To characterize the presenting symptoms among persons with radiographic evidence of Chiari malformation (CM) type I in an effort to help the primary care provider determine accurate diagnosis and treatment. METHODS: The symptoms of CM can often be vague and ambiguous, leading to misdiagnosis. Patients who had radiographic evidence of CM with or without syringomyelia were prospectively studied. The detailed symptom data were collected from patient reports at initial examination to determine if there was a pattern of symptoms typically attributable to the CM. RESULTS: Analysis of presenting symptoms among 265 patients demonstrated a distinct pattern of symptom constellation. This study reports a prospective analysis of presenting symptoms among 265 patients with radiographic evidence of CM with or without syringomyelia and defines a common presentation pattern. Recognition of typical symptoms attributable to CM can result in appropriate diagnostic workup, accurate diagnosis, and timely treatment, leading to improved patient outcomes.

Update on Chiari malformation: clinical manifestations, diagnosis, and treatments.

The incidence of the Chiari I malformation is unknown; however, with increased diagnostic procedures, the diagnosis is becoming more common. In the past, Chiari I has been diagnosed in adulthood, but the condition is now being observed more frequently in pediatric clients. This article will review the clinical manifestations, diagnostic procedures, and treatment options available for Chiari type I (Chiari I) malformation. The nursing considerations for this condition are also discussed.

Chiari I malformation: clinical presentation and management.

Chiari malformations are cerebellar anomalies. The four types of Chiari malformations, as described more than 100 years ago by Dr. Hans Chiari, have neither anatomic nor embryologic correlation. Their only commonality is that they all involve the cerebellum. Chiari I malformation consists of herniation of the cerebellar tonsils into the foramen magnum, thus crowding the craniocervical junction. Chiari II malformation is almost exclusively associated with myelomeningocele and hydrocephalus. It consists of herniation of not only the tonsils but also all the contents of the posterior fossa into the foramen magnum. This herniation involves the brainstem, fourth ventricle, and cerebellar vermis. Chiari III and IV malformations are rare. Chiari III represents an encephalocele (external sac containing brainstem and posterior fossa contents); thus, the cerebellum and brainstem are descending not only into the spine, but also into an external sac. Chiari IV consists of cerebellar hypoplasia. The Chiari I malformation has the latest mean age of clinical presentation. A Chiari type I anomaly presenting in adulthood is the focus of this article. Surgery is indicated with neurological dysfunction, symptomatic syrinx, or hydrocephalus. Of all Chiari I patients, 15%-20% will have hydrocephalus. For some of them, the hydrocephalus will resolve with ventriculoperitoneal shunting, alleviating the need for a Chiari decompression. Long-term prognosis for patients with symptomatic Chiari type I malformations who undergo surgical treatment is variable, based on the patients' presenting symptoms and spinal cord cyst response.

Arnold-Chiari malformation--a closer look.

Arnold-Chiari malformation (ACM) is a malformation of the cervico-medullary junction characterized by displacement of the cerebellar tonsils, the brainstem and the fourth ventricle into the upper cervical canal. The clinical presentation of this anomaly is largely dependent upon age at onset, associated pathology, and the presence of hydromyelia. Considerable controversy exists within the literature concerning pathogenesis, signs and symptoms, diagnosis, and successful treatment of Arnold-Chiari malformation. This article will examine these issues and discuss the nursing management of patients with this syndrome.