Controversies in the diagnosis and management of craniosynostosis: a panel discussion.
This introductory article summarizes and comments on a group of four papers based in part on a panel discussion of craniosynostosis (CS) held at the 53rd annual meeting of the American Cleft Palate-Craniofacial Association. The purpose of this panel was to review the differential diagnosis of CS and the evidence for increased intracranial pressure (ICP) and developmental problems in CS patients. First, a correct diagnosis must be made, with true synostosis being differentiated from positional deformities and other normal variants. Second, medical indications must be balanced against the risks of operating on CS patients. Release of fused sutures is commonly undertaken to avoid increased ICP, although studies of ICP in infants and children with CS are hampered by a lack of normative data and by difficulties with measurement techniques. A subgroup of children with isolated craniosynostosis may have increased ICP, which can be deleterious to brain function. Longitudinal studies of children with isolated CS suggest an increased risk of mental retardation and learning disorders; surgical release of the suture may not diminish this risk. Patients with metopic suture fusion appear to be particularly at risk. These findings must be confirmed with a larger sample size. These uncertainties raise ethical issues and complicate medical decision-making for the infant with CS. A trusting and truthful relationship between the parents and the professional is necessary for a balanced discussion of the best interests of the child. All patients with confirmed synostosis should be followed for evidence of progressive deformity, intracranial hypertension, and neurodevelopmental problems.
Mouradian
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Craniofacial Program, Children's Hospital Medical Center, University of Washington, Seattle 98105-0371, USA. wmoura@chmc.org
D001921:Brain / Q000254:growth & development / Q000503:physiopathology; D002648:Child; D003393:Cranial Sutures / Q000601:surgery; D003398:Craniosynostoses / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology / Q000601:surgery; D003657:Decision Making; D002658:Developmental Disabilities / Q000209:etiology; D003937:Diagnosis, Differential; D004992:Ethics, Medical; D005624:Frontal Bone / Q000002:abnormalities / Q000601:surgery; D006801:Humans; D007223:Infant; D008607:Intellectual Disability / Q000209:etiology; D019586:Intracranial Hypertension / Q000209:etiology; D007427:Intracranial Pressure; D007859:Learning Disabilities / Q000209:etiology; D010347:Patient Care Planning; D011368:Professional-Family Relations; D012307:Risk Factors; D012886:Skull / Q000002:abnormalities; D016896:Treatment Outcome