A cloverleaf skull syndrome probably of Beare-Stevenson type associated with Chiari malformation

Ito, Shuichi; Ohsaki, Etsuro; Goto, Akiko; Takagi, Kimiyo; Koresawa, Mitsuhiko; Ito, Susumu; Sekido, Kenichi; Suzuki, Masami; Torikai, Katsuyukí; Aida, Noriko

doi:10.1016/0387-7604(96)00020-4

Cited by 22 publications

(20 citation statements)

References 10 publications

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“…Of the 9 patients in the entire series within this category, 5 were undergoing reoperations. The association of Chiari malformation and craniosynostosis has been previously reported [8, 9, 10, 11, 12]. This association is probably related not only to mechanical distortion and compression in the posterior fossa by inadequate skull growth, but also to intracranial hypertension caused by chronic elevation of intracranial venous pressure, as proposed by Thompson et al [8], and in some patients to hydrocephalus.…”

Section: Discussionmentioning

confidence: 79%

Reoperation for Chiari Malformations

Sacco¹,

Scott²

2003

Pediatr Neurosurg

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Background: We undertook this study to characterize those patients who required reoperations for Chiari malformation and to determine whether modifications in surgical technique at the initial procedure might have obviated the need for repeat surgery. Methods: We reviewed the hospital records, imaging studies, operative reports, and follow-up data of those patients who were undergoing a second operation as part of a consecutive series of Chiari decompressions carried out by one neurosurgeon over a 14-year period. We wished to determine whether type of Chiari malformation, age at initial surgery, the presence of associated bony anomalies, or initial operative technique contributed to the need for reoperation. Results: Twenty-two (17%) of the surgical procedures represented reoperations (16 of 100 Chiari I and 6 of 33 Chiari II decompressions). Of the patients in the series who were operated on at age 5 years or younger, 7 (16%) were reoperations (5 of 33 Chiari I and 2 of 10 Chiari II decompressions); 15 patients (17%) of those older than 5 years at surgery were reoperations (11 of 67 Chiari I and 4 of 23 Chiari II decompressions). 8 of the 16 patients who underwent reoperations for Chiari I malformation and 4 of the 6 patients who underwent reoperations for Chiari II malformation had their initial decompression surgery at age 5 years or younger. Reoperation was required because of failure to treat adequately an associated syrinx (n = 11), because of persisting neurological symptoms or headache (n = 9), or because of a possible faulty fourth ventricular stent placement (n = 2). 5 of 9 patients in the operative series with Chiari malformations associated with craniosynostosis represented reoperations (55%), and all 3 of the patients whose syrinx persisted after reoperation had craniosynostosis syndromes. Conclusions: Approximately 17% of the patients with Chiari I and Chiari II malformations in this operative series were undergoing a second operation. Factors in this series which were associated with reoperation included a young age at initial surgery, the presence of complex bony anatomy at the foramen magnum, syndromic craniosynostosis, and failure of the surgeon at the initial operation either to assess patency of the foramen of Magendie or correctly place a fourth ventricular stent.

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Section: Discussionmentioning

confidence: 79%

Reoperation for Chiari Malformations

Sacco¹,

Scott²

2003

Pediatr Neurosurg

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“…A summation of all audiological series of Beare Stevenson syndrome reveals hearing loss in 1/26 patients (4%). Of these 26 patients reported to date, 24 have descriptions of the physical examination of the ear [40,61,62,[64][65][66][67][68][69][70][71][72][73][74][75][76][77][78]. Of these 24 patients, only one patient was not described as having ear malformations.…”

Section: Jackson-weiss Syndrome Beare-stevenson Syndrome and Crouzonmentioning

confidence: 99%

Hearing loss in syndromic craniosynostoses: Otologic manifestations and clinical findings

Agochukwu

Solomon

Muenke

2014

International Journal of Pediatric Otorhinolaryngology

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“…The Chiari I malformation has been historically noted in patients with various forms of craniosynostosis [1, 2, 3, 4, 5, 6]. To our knowledge, metopic synostosis has not specifically been described as predisposing patients to cerebellar tonsillar ectopia.…”

Section: Introductionmentioning

confidence: 96%

Preliminary Observations on the Association between Simple Metopic Ridging in Children without Trigonocephaly and the Chiari I Malformation

et al. 2001

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Historically, there have been no accounts of an association between simple metopic ridging and the Chiari I malformation. We prospectively evaluated 50 children with simple metopic ridges and found that 30% of these patients had a Chiari I malformation. During the period in which this screening for simple metopic ridges was performed, only 9% of the total patient population (324) was diagnosed as having a Chiari I malformation. We hypothesize that small decreases in anterior fossa volume in children with a simple metopic ridge and no other clinical findings of trigonocephaly may increase their chance of having a Chiari I malformation that becomes symptomatic. In addition, simple metopic ridging should perhaps be added to the list of bony anomalies associated with the Chiari I malformation, such as atlantal assimilation and retroverted odontoid processes. Future studies aimed at calculating anterior fossa volumes in children with a Chiari I malformation and imaging all children with simple metopic ridges are now necessary to ascertain if there is an increased risk of these patients becoming symptomatic with a hindbrain hernia or having a hindbrain hernia, respectively.

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A cloverleaf skull syndrome probably of Beare-Stevenson type associated with Chiari malformation

Cited by 22 publications

References 10 publications

Reoperation for Chiari Malformations

Reoperation for Chiari Malformations

Hearing loss in syndromic craniosynostoses: Otologic manifestations and clinical findings

Preliminary Observations on the Association between Simple Metopic Ridging in Children without Trigonocephaly and the Chiari I Malformation

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