Associated disorders of Chiari Type I malformations: a review

Abstract

<jats:p>A single pathophysiological mechanism of Chiari Type I malformations (CM-I) has been a topic of debate. To help better understand CM-I, the authors review disorders known to be associated with CM-I. The primary methodology found among most of them is deformation of the posterior cranial fossa, usually with subsequent decrease in volume. Other mechanisms exist as well, which can be categorized as either congenital or acquired. In understanding the relationship of such disorders with CM-I, we may gain further insight into the process by which cerebellar tonsillar herniation occurs. Some of these pathologies appear to be true associations, but many appear to be spurious.</jats:p>

Beckwith–Wiedemann syndrome and Chiari I malformation—a case-based review of central nervous system involvement in hemihypertrophy syndromes

“…Only one case has been reported in the English literature to the best of our knowledge [14,24].We report a case of a child diagnosed with BWS associated with Type I Chiari malformation. We also review other congenital hypertrophy syndromes and their association with the central nervous system anomalies and in particular Chiari malformation.…”

“…Association of Beckwith-Wiedemann syndrome (BWS) with Type I Chiari malformation is extremely rare [14,24]. Only one case has been reported in the English literature to the best of our knowledge [14,24].We report a case of a child diagnosed with BWS associated with Type I Chiari malformation.…”

Rate of Chiari I Malformation in Children of Mothers with Depression with and without Prenatal SSRI Exposure

“…Unfortunately, there are no studies exploring these factors and risk for CIM per se. CIM has been reported in association with congenital conditions including craniosynostosis, achondroplasia, acromegaly, growth hormone deficiency, hyperostosis, familial vitamin D-resistant rickets, neurofibromatosis type 1, and a variety of spinal defects (Loukas et al, 2011). A genetic contribution to at least a subset of nonsyndromic cases is strongly suspected (Speer et al, 2003).…”

Upward Translation of Cerebellar Tonsils following Surgical Expansion of Supratentorial Cranial Vault: A Unified Biomechanical Explanation of Chiari Type I

“…The near universal treatment of symptomatic Chiari I, in the absence of other identified causative pathology such as mass within the posterior fossa [3,5,8,9,10,11,12,13,14,15,16,17,18,19,58,59] or hydrocephalus [52], is the surgical provision of more space across the cervicomedullary region. When the root cause of Chiari I deformity is supratentorial craniocerebral disproportion or hydrocephalus, cervicomedullary decompression does not address the pathological downward force vector but simply provides additional space for transforaminal herniation and this can be lethal [60].…”