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Purpose: This study examined early language acquisition in children with single suture craniosynostosis (SSC) and in children with deformational posterior plagiocephaly. Our purpose was to determine whether infants with SSC have normal language acquisition at the age of 3 years, and whether infants with deformational posterior plagiocephaly demonstrate parallel development when compared with children with SSC., Methods: The study population includes 61 infants. Twenty of them had synostosis of the sagittal suture, 12 synostosis of other suture and 29 deformational posterior plagiocephaly. Forty-nine of them were operated on a mean age of 10.6 months, and 12 were non-operated children with deformational posterior plagiocephaly. Language skills of participants were prospectively evaluated at the mean age of 3 years 4 months., Results: About one half of the subjects (49%) had normal linguistic development, 30% had slight developmental problems and 21% had severe disorders in speech-language-related skills. These figures showed the prevalence of severe language disorders to be three times higher in our study population when compared with the general population. Children with sagittal synostosis managed better in all language skills compared with other types of SSC. Defective language development was found in deformational posterior plagiocephaly, both operated and non-operated., Conclusions: We found a noticeable developmental risk for specific language impairment in children with nonsyndromic SSC, and that the deviant language development is observable already in early infancy. Contrary to previous beliefs, the developmental risk for defective language development in deformational posterior plagiocephaly was found in both operated and non-operated subjects.

Introduction: This article addresses the relevance of venous hypertension to children born with, in particular, the "syndromic" forms of craniosynostosis. The development of the human cerebral venous system is summarised and the patterns of anomalous venous drainage seen in children with complex forms of craniosynostosis associated with raised intracranial pressure are described., Aetiology: The aetiology of these abnormal patterns is discussed under the general headings of "The constriction theory", "A primary role for FGFR mutations" and "Persistence of the foetal pattern of intracranial venous drainage."Venous hypertension (along with hydrocephalus, cranio-cerebral disproportion and airway obstruction) has an important role in the aetiology of raised intracranial pressure in severely affected children., Treatment: The inter-relationship between these factors is explained and then the treatment of those children whose raised intracranial pressure is considered to be due predominantly to venous hypertension is described.

Introduction: One third of patients with craniofacial dysostosis syndromes and about 15-20% of children with single suture craniostenosis have a documented increase in intracranial pressure (ICP). The early detection of intracranial hypertension is important in order to reduce the risks for brain development and visual function. However, in children with craniosynostosis, the clinical manifestations of abnormally increased ICP are difficult to detect, as the majority of patients may have neither warning signs nor symptoms for a long period of time., Review: Moreover, data from the literature suggest that neither fundoscopic nor radiological findings are necessarily related to intracranial pressure recordings in this type of pathology. In this context prolonged ICP monitoring seems actually to be the most valuable diagnostic tool. Extradural, subdural, and intraparenchymal devices have been used, and different softwares have been employed for PC storage and analysis of the data obtained. Most recent series consider mean ICP combined with plateaux waves as the most sensitive indicators in the final evaluation of the results., Conclusions: The analysis of the literature demonstrates a significant difference in the prevalence of raised ICP preoperatively between non-syndromic and syndromic patients. Among the non-syndromic children, a direct relation between the number of sutures involved and raised ICP is documented. The analysis of plateaux waves seems to be particularly important in children with borderline mean ICP values. Persistent postoperative raised ICP has been described in 6-15% of patients with craniofacial dysostosis. It must be related to the multifactorial etiology of increased ICP in these patients, which includes cerebral venous congestion, upper airway obstruction, and hydrocephalus.

Introduction: Chiari malformation (CM) is a frequent finding in multisutural and syndromic craniosynostosis, occurring in 70% of patients with Crouzon's syndrome, 75% with oxycephaly, 50% with Pfeiffer's syndrome and 100% with the Kleeblattschädel deformity. The pathogenesis of this condition and rationale for treatment are still controversial., Discussion: Since its first description in 1972, several factors have been cited to play a role in inducing CM. In the light of recent publications, the roles of premature fusion of cranial vault and cranial base sutures, of congenital anomalies of the cerebellum and brain stem, of raised intracranial pressure, of venous hypertension and of hydrocephalus are reviewed. Evaluation and management of CM are also discussed., Conclusion: Chiari malformation appears to be an acquired and progressive condition that develops in the first months of life, because of a disproportion between hindbrain growth and an abnormally small posterior fossa, a consequence of the premature fusion of lambdoid and cranial base sutures. Venous hypertension caused by stenosis of the jugular foramen can also be present in these patients, resulting in intracranial hypertension and/or hydrocephalus. Careful MRI evaluation is recommended for the forms of craniosynostosis at a high risk of developing hindbrain herniation. The selection of posterior cranial vault expansion as the first surgical procedure is advocated. In selected cases, treatment of the posterior cranial deformity by occipital vault remodelling and treatment of the Chiari-like deformity by suboccipital decompression can be carried out using the same surgical procedure.

Introduction: Ventriculo-peritoneal (VP) shunt malfunction is usually due to blockage of the ventricular catheter and this is typically apparent as enlarged ventricles on a CT scan of the brain. We describe a less common radiological finding in an infant with a blocked shunt., Case Report: A male infant presenting with hydrocephalus in the neonatal period underwent insertion of a VP shunt. He represented at 17 months of age with the clinical features of raised intracranial pressure. A CT scan of the brain revealed that the ventricles were smaller compared with his previous scan, but X-rays taken as part of the shunt series revealed diastasis of the sagittal, coronal and lambdoid sutures. The shunt was explored and the ventricular catheter was found to be blocked and was replaced. Post-operatively there was complete resolution of the symptoms and the suture diastasis., Conclusions: Suture diastasis with small ventricles on a CT scan of the brain is an unusual radiological finding in an infant with a blocked shunt. Suture diastasis in this patient suggests raised intracranial volume and this may be due to the transependymal absorption of CSF into the white matter.