Scoliosis in a child with Chiari I malformation and the absence of syringomyelia: case report and a review of the literature

Abstract

Intriguingly, and scattered throughout the medical literature, many have noted cases of scoliosis in patients with only a Chiari I malformation and no syringomyelia. Moreover, experimental studies have induced scoliosis in animals after compression of the dorsal columns. After a review of the medical literature regarding a potential cause and effect of herniated hindbrain-induced scoliosis in the absence of syringomyelia, this association although rare, does seem plausible.

The association between Chiari malformation Type I, spinal syrinx, and scoliosis

“…First, there have been several reports of patients with CM-I and sco- liosis without syringomyelia; however, most of these cases were drawn from larger case series that included a majority of patients with a syrinx and CM-I. 12,13,22,34 Because both low cerebellar tonsil position and scoliosis are common, it is not surprising that many individuals with both conditions have been identified. 7,23,30,35 Others have cited an improvement in scoliosis after CM-I decompression in patients without an associated syrinx; 12,17,34 however, most patients in these series had a syrinx, making it difficult to support any independent effect of CM-I without a syrinx.…”

“…12,13,22,34 Because both low cerebellar tonsil position and scoliosis are common, it is not surprising that many individuals with both conditions have been identified. 7,23,30,35 Others have cited an improvement in scoliosis after CM-I decompression in patients without an associated syrinx; 12,17,34 however, most patients in these series had a syrinx, making it difficult to support any independent effect of CM-I without a syrinx. 17 Also, the natural history of scoliosis is variable, so it is not clear whether a few examples of postsurgical improvement in patients without a syrinx differs from the natural history of this condition seen in other individuals.…”

“…Some researchers have speculated that asymmetrical compression of the cervicomedullary junction by the cerebellar tonsils can result in scoliosis even in the absence of a spinal syrinx. 5,6,34,37 Nevertheless, there is scant existing evidence for such a causal relationship between CM-I and scoliosis in the absence of a syrinx. Arguing in favor of such a relationship are several case reports of patients with CM-I and scoliosis in the absence of a syrinx.…”

“…Arguing in favor of such a relationship are several case reports of patients with CM-I and scoliosis in the absence of a syrinx. 13,22,34 However, given the high prevaabbreviatioNs CM-I = Chiari malformation Type I; OR = odds ratio. The association between Chiari malformation Type I, spinal syrinx, and scoliosis lence of both CM-I 23,30 and scoliosis, 7,35 both conditions in an individual can occur by chance in many instances.…”

“…In addition, there are case series in which CM-I as a cause of scoliosis has been examined. 11,20,34 Unfortunately, a majority of these patients also have a spinal syrinx, making it impossible to draw any conclusion on the relationship between CM-I and scoliosis in the absence of a syrinx. To examine this relationship, we analyzed a large cohort of children who, over an 11-year span, underwent brain or cervical spine MRI.…”