CONTENTS -- AUTHORS' APPOINTMENTS -- PREFACE -- ACKNOWLEDGEMENTS -- INTRODUCTION -- 1 CRANIOFACIAL GROWTHAND DEVELOPMENT: A CLINICAL PERSPECTIVE David Dunaway -- Normal craniofacial growth and development -- Disordered craniofacial growth in cranial synostosis -- Summary -- References -- 2 CLASSIFICATION AND CLINICAL DIAGNOSIS D.N.P. Thompson and Jonathan Britto -- Terminology -- Non-syndromic craniosynostosis -- Syndromic craniosynostosis -- References -- 3 SYNDROMIC CRANIOFACIAL DYSOSTOSIS: MOLECULAR AND DEVELOPMENTAL ASPECTS Jonathan Britto and Willie Reardon -- Introduction: FGFR mutations cause certain human skeletal dysplasias -- FGFR signalling mechanisms: relevance to human craniofacial syndromes -- How do FGFR mutations cause disease phenotypes? -- How does FGFR gain of function cause human skeletal prematurity? -- Can FGFR expression in human craniofacial development explainphenotype diversity? -- Why does Apert syndrome display a cleft palate despite a similar midfacemorphology to Crouzon-Pfeiffer syndromes? -- Conclusions -- References -- 4 INCIDENCE AND EPIDEMIOLOGY OF CRANIOSYNOSTOSIS Louise C. Wilson -- Introduction -- Incidence of craniosynostosis -- Syndromic craniosynostosis -- Non-syndromic craniosynostosis -- Inheritance patterns in craniosynostosis syndromes -- The genetic clinic -- References -- 5 PRENATAL DIAGNOSIS OFCRANIOSYNOSTOSIS Olav B. Petersen and Lyn S. Chitty -- Introduction -- Routine scanning -- Prenatal diagnosis in families at increased risk -- Diagnosis in the low-risk population -- Conclusions -- References -- 6 IMAGING THE PATIENT WITH CRANIOSYNOSTOSIS R.I. Aviv, D. Armstrong and W.K. Chong -- Introduction -- Plain films -- Plain film appearances of craniosynostosis -- Computed tomography (CT) -- Assessment of the intracranial structures -- Raised intracranial pressure (ICP): radiological assessment.

Postoperative complications -- Orbital considerations of craniosynostosis -- Imaging of the airway -- References -- 7 PRINCIPLES OF MANAGEMENTOF THE CHILD WITH CRANIOSYNOSTOSIS Richard Hayward and Barry Jones -- Introduction -- Summary of our management philosophy -- Management of syndromic forms of craniosynostosis -- Summary -- References -- 8 MANAGEMENT OF RAISED INTRACRANIAL PRESSURE Richard Hayward and Ken K. Nischal -- An introduction to intracranial pressure -- Incidence of raised ICP in craniosynostosis -- Causes of raised ICP in craniosynostosis -- What clinical problems are associated with raised ICP in childrenwith craniosynostosis? -- How does raised ICP cause harm? -- Management of raised ICP -- Monitoring of children with craniosynostosis for raised ICP -- Conclusions -- References -- 9 AIRWAY MANAGEMENT INSYNDROMIC CRANIOSYNOSTOSIS Susanna Leighton and Roderick Lane -- Introduction -- Sleep and breathing -- Central apnoea and hypopnoea -- Obstructive apnoeas and hypopnoeas -- Clinical features of obstructive sleep apnoea syndrome (OSAS) -- Diagnosis -- Respiratory sleep studies -- International recommendations -- Treatment -- Summary -- References -- 10 OCULAR ASPECTS OF CRANIOSYNOSTOSIS Ken K. Nischal -- Introduction -- The role of the ophthalmologist -- References -- 11 THE DENTAL AND ORTHODONTIC MANAGEMENT OF CRANIOSYNOSTOSIS Robert D. Evans and Rachel Bradford -- Introduction -- Tooth development/eruption and establishment of the occlusion -- Development/eruption of the teeth in craniosynostosis -- General dental care -- Craniofacial morphology and occlusal abnormalities -- Dental and orthodontic complications of craniofacial surgery -- Summary -- References -- 12 THE CHILD WITH CRANIOSYNOSTOSIS: PSYCHOLOGICAL ISSUES Daniela Hearst -- Introduction -- The psychosocial importance of facial appearance.

The early years: birth to 3 years -- Early childhood: 3 to 7 years -- Middle childhood: 7 to 11 years -- Adolescence to early adulthood -- Cognitive development and learning difficulties in children withcraniosynostosis -- References -- 13 NEUROLOGICALPROBLEMS IN THE CHILD WITH CRANIOSYNOSTOSIS AND THE ROLE OF THE PAEDIATRICIAN IN OVERALL MANAGEMENT Lucinda J. Carr -- Introduction -- Neurological problems in craniosynostosis -- The role of the paediatrician -- References -- 14 SPEECH AND LANGUAGE CHARACTERISTICS OF CHILDREN WITH CRANIOSYNOSTOSIS Caroleen Shipster -- Introduction -- Factors influencing the development of speech and language skillsin children with syndromic craniosynostosis -- Speech and language impairment in syndromic craniosynostosis -- Speech and language impairment in children with sagittal synostosis -- Principles of assessment and management -- Assessment -- Conclusion -- References -- 15 HEARING PROBLEMS IN CHILDREN WITH CRANIOSYNOSTOSIS Tony Sirimanna -- Introduction -- Otological manifestations -- Diagnosis of hearing loss -- Effect of hearing loss on the development of the child -- Evidence for early intervention -- Management of OME -- Management of bilateral meatal atresia -- Sensorineural hearing loss -- Conclusion -- Appendix: Classification of degree of hearing loss -- References -- 16 FEEDING PROBLEMS IN SYNDROMIC CRANIOSYNOSTOSIS Valerie Pereira -- Introduction -- The development of feeding skills -- The normal swallow -- The abnormal swallow -- Predisposing risk factors for feeding difficulties in syndromiccraniosynostosis -- The nature of feeding difficulties in syndromic craniosynostosis -- Assessment of feeding in syndromic craniosynostosis -- Management strategies -- Summary and conclusions -- References -- 17 THE ROLE OF THE CLINICAL NURSE SPECIALIST Andrea White -- The clinical nurse specialist.

The clinical nurse specialist and the craniofacial service -- The child with non-syndromic craniosynostosis -- The child with complex/syndromic craniosynostosis -- The future -- Conclusion -- References -- 18 ANAESTHESIA FOR CRANIOSYNOSTOSIS SURGERY S. Mallory and R. Bingham -- Craniofacial syndromes and anaesthesia -- Preoperative preparation -- Intra-operative techniques and agents -- Postoperative care -- Management of blood loss -- Summary and conclusions -- Appendix: Craniofacial unit protocol for postoperative fluid replacement -- References -- 19 SURGERY Barry Jones, David Dunaway and Richard Hayward -- Introduction -- Craniofacial surgery -- The operations most commonly employed in craniosynostosis surgery -- Distraction osteogenesis -- Complications -- References -- AFTERWORD THE EVOLUTION OF A MODERN CRANIOFACIAL UNIT Barry Jones -- INDEX.