Presentations

Abstract

Introduction: This study is a comprehensive overview of the pediatric Chiari I Deformity, including the natural history and treatment approaches. Chiari I Deformity is defined as herniation of the cerebellar tonsils through the foramen magnum without caudal displacement of the brainstem (> 3mm in children and >5 mm in adults), which may present with a variety of neurological symptoms. The goal of this research is to discuss the disease course and management plan of this condition, including indications for surgical intervention or for a more conservative approach.

Main Body: A majority of patients diagnosed with the Chiari I Deformity on neuroimaging are asymptomatic or very mildly asymptomatic and 93% of these patients remain clinically stable or improve as they age.1,2 This condition is often an incidental finding with little need for intervention in children. The definitive treatment for Chiari I is the posterior fossa decompression surgical maneuver. This relieves pressure from the herniated cerebellar tonsil and resolves associated symptoms. These symptoms include: occipital-region headache that is worsened by coughing and sneezing (termed a Chiari-associated headache), tinnitus, dizziness, and other symptoms associated with compression of the cerebellar tonsil and/or brainstem. The surgical treatment of Chiari I is often deemed a "cure" as the majority of issues related with the deformity are resolved. The Chiari-associated headache is effectively treated with little to no possibility of recurrence following a posterior fossa decompression.1 Of course, surgery comes with risks. Young children are more susceptible to surgical complications of the standard decompression procedure, including ventral compression and medullary dysfunction1. Additionally, certain complaints show little to no significant improvement following surgical intervention, such as preoperative sensory and cranial nerve deficits. Children also often have a shorter duration of symptoms than the adult form of Chiari I.4 This begs the question of whether or not surgery is necessary or if supportive, conservative care is more appropriate in certain pediatric patients affected by C1D.

Conclusion: Pediatric patients with the Chiari 1 Deformity may present asymptomatically or with a minimal range of symptoms, often discovered incidentally with neuroimaging. Many physicians may quickly jump to surgery to manage this condition, but surgery may not always be the most appropriate course in the pediatric population. Physicians should consider the severity of symptoms along with coexisting issues when deciding whether surgery is necessary in children with Chiari I. Children may outgrow the deformity with age and conservative management is often best, despite how complicated cerebellar tonsillar herniation may seem.