Resolution of syndromic craniosynostosis-associated Chiari malformation Type I without suboccipital decompression after posterior cranial vault release

Levitt, Michael R.; Niazi, Toba; Hopper, Richard A.; Ellenbogen, Richard G.; Ojemann, Jeffrey G.

doi:10.3171/2011.11.peds11268

Cited by 32 publications

(20 citation statements)

References 51 publications

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“…Summarizing these data, in their series, the Chiari malformation was surgically treated in 16 of the 29 patients: 5 during a combined procedure and 11 at a separate operative setting. It was their observation, and has been that of others, 8 that it is possible for the radiographic appearance of the Chiari malformation to improve after craniosynostosis repair alone. Interestingly, this has been our experience as well.…”

Section: Discussionmentioning

confidence: 99%

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

Scott

Fearon

Swift

et al. 2013

PED

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Object The optimal management of Chiari malformations in the setting of craniosynostosis is not well established. In this report the authors describe their outcomes with the combined technique of simultaneous suboccipital decompression (SOD) during posterior cranial vault remodeling (PCVR). Methods A retrospective review was performed of all patients undergoing PCVR and simultaneous SOD. Demographic data, diagnosis, imaging studies, operative intervention, and clinical follow-up were evaluated. Results Thirty-four patients were identified as having undergone a simultaneous PCVR/SOD for Chiari malformation associated with craniosynostosis. Eighty-eight percent of these patients had syndromic, multisutural craniosynostosis, and the remaining patients had unilateral lambdoid craniosynostosis. There were no postoperative complications as a direct result from this combined procedure. Two patients required a subsequent direct approach for decompression of the Chiari malformation. The interval between these subsequent surgeries was 3 years and 19 months. Conclusions Chiari malformations are commonly associated with syndromic, complex craniosynostosis and isolated lambdoid craniosynostosis. In appropriately selected patients, a combined posterior cranial vault enlargement and SOD of the foramen magnum is associated with a low complication rate and appears to be an effective procedure.

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Section: Discussionmentioning

confidence: 99%

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

Scott

Fearon

Swift

et al. 2013

PED

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“…The specific pathogenesis of the various hindbrain herniations is still unknown, and different theories have been proposed, including: the caudal traction theory, hindbrain dysgenesis and developmental arrest theory, hydrocephalic and hydrodynamic theory of Gardner, small posterior fossa/hindbrain overgrowth theory, craniosynostosis theory (Levitt et al, 2012), and the lack of embryological ventricular distention theory (McLone and Knepper, 1989). The specific pathogenesis of the various hindbrain herniations is still unknown, and different theories have been proposed, including: the caudal traction theory, hindbrain dysgenesis and developmental arrest theory, hydrocephalic and hydrodynamic theory of Gardner, small posterior fossa/hindbrain overgrowth theory, craniosynostosis theory (Levitt et al, 2012), and the lack of embryological ventricular distention theory (McLone and Knepper, 1989).…”

Section: Discussion Proposed Mechanisms Of Chiari Malformationsmentioning

confidence: 99%

Relationship between pharyngitis and peri‐odontoid pannus: A new etiology for someChiariImalformations?

et al. 2015

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The pathophysiology underlying Chiari I malformations (CIMs) provides room for debate with several theories attempting to address this issue. We retrospectively reviewed many of our past patients with pediatric CIMs (specifically, those with peri-odontoid pannus), and present a hypothesis for the development of the malformation in some of said patients. Our experience with the pediatric CIM has shown that almost 1 in 20 patients who present with symptoms is found to have a peri-odontoid pannus. These masses ranged in size from 4 to 11 mm in diameter. Forty percent had a history of clinically significant pharyngitis or pharyngeal abscess. Pannus formation around the dens (odontoid) resulted in ventral compression of the craniocervical junction in each of these patients. Highlighting the hypermobility that causes such lesions, following fusion, the pannus and symptoms in several patients were diminished. Impairment of normal cerebrospinal fluid circulation out of the fourth ventricle and across the craniocervical junction appears to be a plausible endpoint in this discussion and a suitable explanation for some patients with CIM. Still, the mechanisms by which cerebrospinal fluid circulation is compromised may be variable and are not well understood. This is the first study dedicated to the evaluation of pannus formation in the CIM population. We hypothesize that pharyngeal inflammatory conditions contribute to the formation and progression of hindbrain herniation in a small subset of patients with CIMs.

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“…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.…”

Section: Discussionmentioning

confidence: 99%

Progressive postnatal pansynostosis: an insidious and pernicious form of craniosynostosis

Wood¹,

Oh²,

Keating

et al. 2015

PED

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OBJECT Progressive postnatal pansynostosis (PPP) is a rare form of craniosynostosis that is characterized by a normal head shape, insidious decrease in percentile head circumference, and high rates of elevated intracranial pressure (ICP). This investigation describes the clinical, radiographic, and genetic features of this entity. METHODS The authors’ craniofacial database for the period 1997–2013 was retrospectively culled to identify patients who had a normal or near-normal head shape and CT-confirmed multiple-suture synostosis. Patients with kleeblatt-schädel or previous craniofacial surgery were excluded. All demographic information was collected and analyzed. RESULTS Seventeen patients fit the inclusion criteria. Nine patients had a syndromic diagnosis: Crouzon syndrome (n = 4), Pfeiffer syndrome (n = 2), Saethre-Chotzen syndrome (n = 1), Apert syndrome (n = 1), and achondroplasia (n = 1). With the exception of 3 patients with mild turricephaly, all patients had a relatively normal head shape. Patients were diagnosed at an average age of 62.9 months. Nearly all patients had some combination of clinical, radiographic, or ophthalmological evidence of increased ICP. CONCLUSIONS PPP is insidious; diagnosis is typically delayed because the clinical signs are subtle and appear gradually. All normocephalic infants or children with a known or suspected craniosynostotic disorder should be carefully monitored; any decrease in percentile head circumference or signs/symptoms of increased ICP should prompt CT evaluation.

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Resolution of syndromic craniosynostosis-associated Chiari malformation Type I without suboccipital decompression after posterior cranial vault release

Cited by 32 publications

References 51 publications

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

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

Relationship between pharyngitis and peri‐odontoid pannus: A new etiology for someChiariImalformations?

Progressive postnatal pansynostosis: an insidious and pernicious form of craniosynostosis

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