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Eric L. Rhoton and Albert L. Rhoton Jr.

T he hydromyelic cavity associated with Chiari malformation infrequently extends into the brain stem and rarely extends above the brain stem into the cerebrum. Only three previous reports could be identified in which the syrinx extended rostral to the brain stem. 5, 9, 10 In the two recent cases, the cavity involved the basal ganglia. 5, 10 In the third, an autopsy report published by Spiller 9 in 1906, the syrinx extended into the internal capsule and the head of the caudate nucleus. The term “syringocephaly” has been used to describe the cavity when

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Symptomatic Chiari malformations

An analysis of presentation, management, and long-term outcome

Gregg N. Dyste, Arnold H. Menezes and John C. VanGilder

I n 1891, Chiari 16 described a patient with herniation of the cerebellar tonsils below the plane of the foramen magnum. His subsequent descriptions defined the spectrum which is currently recognized as Types I, II, and III malformations. 15 Arnold later published a similar description of a child with a Type II malformation and myelodysplasia. With a better knowledge of the contributions of the various individuals, it seems more appropriate to label the condition as the Chiari malformation. 49 Currently, three different types of Chiari malformations are

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Benjamin D. Schanker, Brian P. Walcott, Brian V. Nahed, Kristopher T. Kahle, Yan Michael Li and Jean-Valery C. E. Coumans

neuroimaging. 25 , 33 , 35 An estimated 65%–80% of patients with CM present with syringomyelia. 11 , 27 Traditionally, combined surgical decompression and enlargement of the posterior fossa is a common method of treatment, although patients may require additional care for syringomyelia and hydrocephalus. The decision to proceed with surgical intervention often depends on disease severity and/or progression. Chiari malformations have long been considered sporadic conditions, without a heritable etiology. However, there have been a number of case reports identifying

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Jennifer Strahle, Karin M. Muraszko, Steven R. Buchman, Joseph Kapurch, Hugh J. L. Garton and Cormac O. Maher

T he association between CM and craniosynostosis has been recognized for several decades. 36 Chiari malformation occurs in patients with both syndromic and nonsyndromic forms of craniosynostosis. 5 , 7 , 9 , 17 , 18 , 41 , 42 Up to 70% of individuals with Crouzon syndrome and 50%–82% of those with Pfeiffer syndrome have associated CM. 4 , 5 , 7 There are also several reports of CM with nonsyndromic synostosis involving the sagittal, coronal, and even the metopic sutures. 17 , 25 , 41 Cerebellar tonsillar ectopia in patients with craniosynostosis is

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Michael G. Muhonen, Arnold H. Menezes, Paul D. Sawin and Stuart L. Weinstein

T he association between pediatric Chiari malformations and the development of scoliosis has been well documented in the spina bifida population. 17–19, 31, 34, 36–38 Scoliosis occurs in 50% to 70% of these patients, and is therefore a common cause of functional decline. 36 The scoliosis is thought to be secondary to a generalized paresis of the trunk musculature, to congenital structural changes in the vertebrae, and to the effects of abnormal intramedullary pressure with interference of the postural tonic reflexes. 34 The association between scoliosis and

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Amit Mahore, Abhidha Shah, Trimurti Nadkarni and Atul Goel

), autosomal dominant form . J Med Genet 32 : 370 – 374 , 1995 2 Brueton LA , Winter RM : Craniodiaphyseal dysplasia . J Med Genet 27 : 701 – 706 , 1990 3 Cavender RK , Schmidt JH III : Tonsillar ectopia and Chiari malformations: monozygotic triplets. Case report . J Neurosurg 82 : 497 – 500 , 1995 4 Cohen MM Jr : Craniofrontonasal dysplasia . Birth Defects Orig Artic Ser 15 : 5B 85 – 89 , 1979 5 Coria F , Quintana F , Rebollo M , Combarros O , Berciano J : Occipital dysplasia and Chiari type I deformity in a family

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Ilya Laufer, Murray Engel, Neil Feldstein and Mark M. Souweidane

C hiari malformations represent one of the most commonly evaluated and treated entities in pediatric neurosurgery. Chiari malformation Type I most commonly presents with exertional headaches and/or neck pain. Scoliosis and other symptoms referable to syringomyelia are also very common. Less frequent are symptoms or signs of cerebellar dysfunction, brainstem dysfunction, or dissociated sensory loss. 8 , 9 Upper-extremity mononeuropathy has been described as a presenting sign in only 2 instances, whereas focal lower-extremity motor and sensory deficits have

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Jeffrey H. Miller, David D. Limbrick Jr., Matthew Callen and Matthew D. Smyth

spontaneous resolution of syringomyelia with associated CM-I has been previously reported in several studies, 1 , 3 , 5 , 15 and spontaneous resolution of isolated tonsillar ectopia in CM-I has been described only twice before. 4 This is, however, the first report of spontaneous resolution in monozygotic twins. Despite being one of the most commonly evaluated pathological entities in pediatric neurosurgery, the natural history of CM-I is not fully understood. Unlike CM-II or CM-III, there is no relation to neural tube closure defects. 16 Chiari malformation Type I may

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Kiarash Shahlaie, Jonathan Hartman, Garth H. Utter and Rudolph J. Schrot

, del Bigio M : Death from chronic tonsillar herniation in a patient with lumboperitoneal shunt and Crouzon's disease . Br J Neurosurg 6 : 595 – 599 , 1992 6 Couldwell WT , Zhang W , Allen R , Arce D , Stillerman CB : Cerebellar contusion associated with type I Chiari malformation following supratentorial head trauma: case report . Neurol Res 20 : 93 – 96 , 1998 7 Dagnew E , van Loveren HR , Tew JM Jr : Acute foramen magnum syndrome caused by an acquired Chiari malformation after lumbar drainage of cerebrospinal fluid: report

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Michelle Feinberg, Parker Babington, Shawn Sood and Robert Keating

C hiari malformation Type I consists of caudal displacement of the cerebellar tonsils into the upper cervical spinal canal. Syringomyelia is commonly associated with the malformation. Patients with Chiari malformation can present with a multitude of symptoms, including but not limited to headache or neck pain, weakness or numbness in the extremities, ophthalmological or otological disturbances, and lower cranial nerve findings. 15 Trismus, also known as masseter muscle spasm or lockjaw, is restricted mouth opening due to muscular dysfunction. It most