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Eldon L. Foltz and John D. Loeser

C raniosynostosis is the premature closure of cranial vault sutures. It may involve a varying number of the skull bones and is related to abnormalities in the base of the skull. It is frequently found in association with facial osseous abnormalities and may be part of a more generalized mesenchymal abnormality. 43, 47 Craniosynostosis commonly presents as a deformity of the cranial vault; its pathogenesis remains unclear. Untreated patients with multiple synostoses have been observed to develop mental retardation and cranial nerve dysfunction, 1, 2, 5, 13, 28

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A. Leland Albright and Ryland P. Byrd

transverse fibrous tissue. The suture is believed to prevent fusion of the adjacent calvarial bones. The cause of craniosynostosis in humans is unknown. Synostotic deformities have been produced experimentally by local trauma, hypervitaminosis, and intentional fusion of adjacent cranial bones. 1, 5 Human craniosynostosis has been attributed most often to either a blastemal defect or changes in the skull base. Park and Powers 6 thought that the normal cranial suture was non-osteogenic and that injury to the blastemal suture anlage prevented its differentiation into a

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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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Juan F. Martínez-Lage, Matías Felipe-Murcia, Encarna Guillén Navarro, María-José Almagro, Antonio López López-Guerrero and Miguel A. Pérez-Espejo

molecular basis of KS remains unknown. The reported prevalence of KS in Japan was estimated to be 1 of every 32,000 people. Although initially regarded as a disease exclusive to the Japanese, there have been several reports that support a widespread ethnic distribution of KS. 1 , 2 , 6 , 7 , 12 Skeletal abnormalities constitute a cardinal feature of the syndrome. 10 , 12 Microcephaly has been reported in 65% of affected individuals and craniosynostosis has been documented in approximately 6% of cases. 2 Some reviews of KS do not even mention craniosynostosis as

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M. Michael Cohen Jr.

I n dealing with craniosynostosis, the neurosurgeon's major attention is directed to the specific sutures that are prematurely synostosed and to the overall medical status of the patient. The clinical geneticist's main concern is with the overall pattern of anomalies and which family members are affected. Patients with the same syndrome may have fusion of different sutures. Different classifications of the same phenomena may be equally valid for different purposes. When our concern is with the clinical description and surgical management of craniosynostosis

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Giuseppe Cinalli, Christian Sainte-Rose, Eve Marie Kollar, Michel Zerah, Francis Brunelle, Paul Chumas, Eric Arnaud, Daniel Marchac, Alain Pierre-Kahn and Dominique Renier

B oth abnormal cerebrospinal fluid (CSF) hydrodynamics and craniosynostosis may result in increased intracranial pressure (ICP); however, they may have potentially opposing effects on cranial growth. The aim of this retrospective study was to investigate the interrelationship between these two processes. The association between hydrocephalus and craniosynostosis has been well documented and the incidence of hydrocephalus has been found to be between 4% and 10%. 6–9, 15, 16, 18, 23, 25, 26, 28 A significant increase in hydrodynamic problems among patients with

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Spiros Sgouros, Anthony D. Hockley, J. Henry Goldin, Michael J. C. Wake and Kalyan Natarajan

F or a long time it had been assumed that craniosyn ostosis results in reduced cranial capacity. The dramatically abnormal external appearance of the face and skull of these children contributed toward that view. For craniosynostosis that affects the coronal sutures, considerable debate among surgeons still revolves around identification of the correct age at which cranial expansion procedures should be performed. Views vary widely, with some preferring to perform surgery soon after birth 11, 12, 14, 22, 26, 27, 29, 30, 33, 34, 48 and others deferring surgery

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Alan Siu, Gary F. Rogers, John S. Myseros, Siri S. Khalsa, Robert F. Keating and Suresh N. Magge

C raniosynostosis refers to the premature fusion of cranial sutures. This condition occurs with an incidence of 1:2500 live births and can be present in isolation or as a component of various syndromes. Some forms of syndromic craniosynostosis are caused by discrete genetic mutations (for example, FGFR, Twist, and EFNB) that impact suture patency. Nevertheless, the molecular basis for many forms of syndromic and nonsyndromic craniosynostosis are unknown. Identification of additional associations may offer further clues about the complex biology of bone

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Johnny B. Delashaw, John A. Persing, William C. Broaddus and John A. Jane

D espite many advances in the treatment of craniosynostosis, a normal skull shape is difficult to achieve by surgical measures. Surgical treatment is hampered because of an incomplete understanding of the pathophysiology of craniosynostosis. The modern perception of craniosynostosis began in 1791 with Sömmering's observations 16 that skull growth occurred along calvarial sutures and that failure of growth at a particular suture resulted in cranial deformity. In 1831, Otto 12 described craniosynostosis in man and animals as secondary to microcephaly or

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Federico Di Rocco, Eric Arnaud and Dominique Renier

C raniosynostoses are defined as the result of abnormal calvarial development associated with a premature fusion of ≥ 1 cranial sutures. They are classified according to the sutures involved, according to the associated malformations (syndromic cases), and, currently, according to the genetic anomalies, especially in syndromic cases. The incidence of craniosynostosis is estimated to be in the range of ~ 1 in 2000 to 1 in 2500 live births. 2 , 3 , 6 , 9 , 10 The frequency of the subtype of suture affected varies greatly; the sagittal suture is most