Craniosynostoses | webob.info
These areas meet at what are called sutures. Sagittal craniosynostosis is the most common type of single suture craniosynostosis When both parents are carriers, there is a 25 percent chance that each child Coronal suture —Skull suture that lies behind the forehead area, across the head from left side to the right side. Coronal - Sagittal - Metopic - Lambdoid - Other The junctions where these bones meet are called sutures. We know that the It is important for parents to realize that ridging on their baby's head does not always mean that a suture is fused. Cranial sutures involved in non-syndromic craniosynostosis include: Metopic Endoscopic approaches are commonly used for sagittal synostosis with excellent results. If parents meet a neurosurgeon who recommends operating without a.
Relevance for Pediatric Psychology Single-suture craniosynostosis and its associated neurobehavioral features represent an important area of study and clinical intervention for pediatric psychologists. Single-suture synostoses are relatively common birth defects that present frequently in hospital-based craniofacial programs and neurodevelopmental centers, either alone or with other developmental disorders. Community pediatric practices are often faced with the differential diagnosis of isolated synostosis versus the relatively frequent presentation of positional plagiocephaly Mulliken et al.
As this review demonstrates, there is growing evidence that single-suture craniosynostosis is associated with neurobehavioral problems, including learning disabilities and behavior problems e.
Although the causal relation between this condition and neurodevelopment is uncertain, it would appear that calvarial abnormality is at the very least a visible and easily diagnosed marker for elevated risk of neurodevelopmental problems. This creates opportunities within systems of pediatric care for early detection of neurobehavioral difficulties and preventative interventions.
Hospital- or clinic-based psychological interventions for parents experiencing extraordinary levels of stress or belonging to certain high-risk categories e. In recognition of the many psychosocial factors affecting the physical and psychological development of infants and children with craniosynostosis and other craniofacial conditions, the practice parameters of the American Cleft Palate-Craniofacial Association require the provision of psychological assessment and intervention services by hospital-based craniofacial teams American Cleft Palate-Craniofacial Association, It is therefore important that pediatric psychologists become familiar with emerging research on this type of craniofacial anomaly and its implications for neurodevelopmental theory and clinical practice.
Before reviewing specific neurobehavioral findings, we first discuss theoretical complexities and measurement issues surrounding the investigation of interrelationships between the brain and cranium, focusing on the potential mechanisms by which calvarial suture fusions might affect neurodevelopment.
After reviewing specific neurobehavioral findings, we provide a methodological critique of this research and offer suggestions for future studies of isolated synostoses. Interrelated Growth of Brain and Cranium Until recently there has been little study or theoretical discussion of the interrelations between the developing brain and cranium Kjaer, Clinical formulations of the relation between calvarial suture fusion and neurodevelopment e.
Two specific pathways, operating alone or in combination, have been proposed in this regard: Another causal pathway is also possible in which primary brain malformation could lead to both synostosis and neurodevelopmental difficulties. We next discuss the details of, and empirical support for, each of these possibilities. Elevated ICP is believed to promote hypovascularity in the approximate region of the fused suture, leading to hypoplasia of underlying brain tissue. Clinical formulations often refer to the reduction or prevention of elevated ICP as one of the primary reasons why cranioplastic surgery may reduce the probability of neurodevelopmental delay or deficits in infants with craniosynostosis e.
What is the empirical evidence for such claims, particularly for children having single-suture synostosis?
Few studies have examined associations between ICP and neurobehavioral status. Renier and Marchac compared the ICP values of children with developmental quotients above and below 90 on standardized cognitive measures.
Among the single-suture anomalies included in these analyses sagittal and unilateral coronalonly those with unilateral coronal synostosis showed the hypothesized inverse relation between cognitive status and ICP; however, it is unclear whether this association was statistically significant no details of these analyses were reported.
Neither Arnaud, Renier, and Marchac nor Gewalli et al. Cohen and Persing have discussed the many problems in interpreting ICP data, including the use of differing measurement techniques and patient selection criteria among investigators; and the complete lack of normative data, which are unlikely to become available until there are reliable methods of noninvasive measurement Mouradian, ICP therefore needs to be measured over an extended period to obtain a stable representation.
The metric most reflective of clinically meaningful intracranial hypertension is still a matter of debate e. Cohen and Persing noted the relative infrequency of clinical indicators of increased ICP in cases of single-suture synostosis e.
The consequences of elevated ICP in the absence of clinical symptoms are unclear. Overall, it would appear that the elevated ICP hypothesis is at best modestly supported by available data, with uncertain implications for the causal relation between synostosis and brain growth.
Secondary Cerebral Deformation Radiographic studies of craniosynostosis have revealed abnormalities in the subarachnoid space beneath the region of suture fusion i. For example, metopic and sagittal synostoses impose midline restrictions that potentially affect frontal lobe development.
In metopic synostosis, the ventral development of the brain may be limited by severely restricted ventral expansion of the anterior cranial fossa and frontal bones. In sagittal synostosis, there are frontal prominences that seem to result from excessive pressure of the growing frontal lobes, presumably due to the inability of the skull to expand sufficiently bi-temporally; in other words, there may be redirection of brain growth toward the frontal lobes because lateral growth is restricted.
Evidence for these hypothesized secondary deformations is just beginning to emerge. Significant differences in subcortical morphology were found. Sagittal patients displayed anteriorly displaced lateral ventricles LV and rostrum of the corpus callosum relative to the unaffected group. In unilateral coronal patients, the anterior LV on the affected side was displaced toward the midline, and the anterior corpus callosum was compressed.
In metopic patients, the posterior LV were truncated, and there was midline constriction in the area between the left and right caudate nuclei. One complication is the lack of one-to-one correspondence between particular sutures and subcortical brain structures.
For example, the sagittal, metopic, and unilateral coronal sutures may all potentially affect frontal lobe growth to some degree. Nevertheless, tentative functional hypotheses can be tendered. If metopic, sagittal, or coronal synostoses have detrimental effects on frontal lobe development, executive functions e.
A syndrome is a disease or disorder that has a pattern of features.
- Author notes
Craniosynostosis at Seattle Children's Most families have never heard of craniosynostosis before their child is born with it. But we treat hundreds of children with this condition each year. Our team has more experience caring for children with craniosynostosis than any other center in the United States.
Endoscopic craniosynostosis repair
Many of our patients are babies who are newly diagnosed. We also care for children who received their initial treatment at another hospital. Please call the Craniofacial Center at for more information, a second opinion or to make an appointment. Each year we care for more than children with craniosynostosis. That includes more than children with complex craniosynostosis conditions. These 50 specialists work together to diagnose and care for our patients.
We will create a personalized plan of care for your child. Our team meets weekly to discuss complex patient cases and decide on the best care for each child. We support your whole family A diagnosis of craniosynostosis can be scary. Our social workers support your child and family through the challenges of this condition.
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