Craniosynostosis operations in childhood

Abstract

Premature osteosynthesis of one or more cranial bones, either intrauterine or within the first postnatal months, is defined as craniosynostosis. The resulting limitation of intracranial space can cause retardation of cranial growth which, in turn, leads to craniostenosis with increasing intracerebral pressure. Complex forms of craniosynostosis with concomitant malformations (i.e. Apert-, Crouzon-, and Pfeiffer syndromes) must be principally distinguished from simple craniosynostosis. This complex cranio-facial dysostosis is a premature osteosynthesis of cranial and facial bones. In general, as far as Germany is concerned, the incidence of cranio-synostosis amounts to 1/1000 births. If they remain untreated, many of these children will suffer from cortex-associated retardation of intelligence. Surgical management, therefore, is initiated at a very early stage, and should be performed in specialised centres. Recommendations for operation vary from an age of 4 to 36 months. However, an age of 6 months or more is the most frequently preferred age for surgical intervention. Severe respiratory disorders, as well as impossibility of enteral intake of nourishment, are considered absolute indications for surgery, independent of the age; elimination of the stigmatisation regarding environmental contacts of the child is another mandatory indication for operation. The goal of early surgery is reconstruction of physiological, cranial, and facial bone structures ("fronto-orbital" or "fronto-facial advancement"). Correction of craniofacial malformation may be associated with--in part--severe complications for the child. From the anaesthesiologist's point of view, this disease demands highly qualified perioperative management, since a variety of idiosyncrasies and risks must be taken into account: These are, for example, venous air embolism, hypothermia, disorders of water and electrolyte equilibrium, and, extremely vital, difficult intubation and substantial blood loss.