Chiari malformation associated with craniosynostosis

Abstract

<jats:sec> <jats:title>Object</jats:title> <jats:p>Chiari malformation (CM) Type I is frequently associated with craniosynostosis. Optimal management of CM in patients with craniosynostosis is not well-established. The goal of this study was to report on a series of pediatric patients with both craniosynostosis and CM and discuss their management.</jats:p></jats:sec> <jats:sec> <jats:title>Methods</jats:title> <jats:p>The authors searched the medical records of 383 consecutive patients treated for craniosynostosis at a single institution over a 15-year period to identify those with CM. They recorded demographic data as well as surgical treatment and outcomes for these patients. When MR imaging was performed, cerebellar tonsillar descent was recorded and any other associated findings, such as hydrocephalus or spinal syringes, were noted.</jats:p></jats:sec> <jats:sec> <jats:title>Results</jats:title> <jats:p>A total of 29 patients with both CM and craniosynostosis were identified. Of these cases, 28% had associated occipital venous abnormalities, 45% were syndromic, and 52% also had hydrocephalus. Chiari malformation was more likely to be present in those patients with isolated lambdoid synostosis (55%), multisuture synostosis (35%), and pansynostosis (80%), compared with patients with coronal synostosis (6%) or sagittal synostosis (3%). All patients underwent surgical repair of craniosynostosis: 16 had craniosynostosis repair as well as CM decompression, and 13 patients did not undergo CM decompression. Of the 7 patients in whom craniosynostosis repair alone was performed, 5 had decreased tonsillar ectopia postoperatively and 5 had improved CSF flow studies postoperatively. Both patients with a spinal syrinx had imaging-documented syrinx regression after craniosynostosis repair. In 12 patients in whom CM was diagnosed after primary craniosynostosis repair, 5 had multiple cranial vault expansions and evidence of elevated intracranial pressure. In 5 cases, de novo CM development was documented following craniosynostosis repair at a mean of 3.5 years after surgery.</jats:p></jats:sec> <jats:sec> <jats:title>Conclusions</jats:title> <jats:p>Chiari malformation is frequently seen in patients with both multi- and single-suture lambdoid craniosynostosis. Chiari malformation, and even a spinal cord syrinx, will occasionally resolve following craniofacial repair. De novo development of CM after craniosynostosis repair is not unusual.</jats:p></jats:sec>

Progressive postnatal pansynostosis: an insidious and pernicious form of craniosynostosis

“…There are several reports of resolution of craniosynostosis-associated Chiari malformation following cranial vault expansion, which obviated the need for decompression. 3,9,13 Hydrocephalus can also be noted in this patient population and may induce Chiari malformation. 13 Four of our patients underwent VP shunt placement, 1 of whom subsequently underwent posterior fossa decompression.…”

“…3,9,13 Hydrocephalus can also be noted in this patient population and may induce Chiari malformation. 13 Four of our patients underwent VP shunt placement, 1 of whom subsequently underwent posterior fossa decompression. Of the remaining 3 patients, 2 underwent CVR/FOA, and 1 had posterior fossa decompression alone.…”

“…This finding was not unexpected as the anomaly is known to occur at a significantly higher frequency among patients with syndromic forms of craniosynostosis. 13 Nevertheless, craniocerebral disproportion in patients with craniosynostosis has been described as a potential mechanism for the occurrence of Chiari malformation in these patients, 5 and there is some debate regarding the optimal management of this subset of patients. There are several reports of resolution of craniosynostosis-associated Chiari malformation following cranial vault expansion, which obviated the need for decompression.…”

Suboccipital decompression during posterior cranial vault remodeling for selected cases of Chiari malformations associated with craniosynostosis

“…These 2 patients underwent secondary direct decompressions with a duraplasty at a mean of 3.6 years after the initial procedure, a rate that we believe compares favorably with the known natural history of Chiari malformation in this patient population. 13,16 After our review, we remain enthusiastic about a combined posterior cranial vault enlargement and SOD of the foramen magnum. However, we recognize that our retrospective study design does not permit comparisons to 2-stage procedures (vault enlargement and subsequent Chiari decompression).…”

“…In a recent review of 383 consecutive patients treated for craniosynostosis over a 15-year period, Strahle et al noted Chiari malformations in 29 patients. 16 Seventeen of these cases were diagnosed before craniosynostosis repair, and 12 cases were diagnosed after, highlighting the dynamic nature of Chiari malformation in patients with craniosynostosis. Of the 17 patients with Chiari malformation diagnosed prior to craniosynostosis repair, 10 underwent SOD either concurrently (5 patients), prior to craniosynostosis repair (2 patients), or after repair (3 patients).…”