gms | German Medical Science

Objective: Complex syndromal craniosynostosis represent a group of patients calling for an individual and multidisciplinary approach in diagnostics and treatment concepts.

Methods: 20 newborns with syndromal craniosynostosis such as Crouzon-syndrome, Apert-syndrome or Pfeiffer-syndrome are presented. We present the diverse problems that are associated with these complex forms of syndromic craniosynostosis.

Results: In order to avoid radiation exposure we perform an early MRI to address intracranial abnormalities such as ventricular dilatation, Chiari malformation etc. Additional questions like an altered venous drainage due to stenosed jugular foramen should be addressed by including an additional MR-angiography. The pansynostosis and possible resulting craniostenosis needs early craniectomy in selected cases. The early timepoint of surgery as well as a severe midface hypoplasia might pose anesthesiological risks. Large areas of immature bone-dysplasia bear additional risks of longer surgery, blood-loss and injury of the dural layer or even the brain. Midface hypoplasia bears risk of prolapse of the eye-bulbs and consecutive breathing problems which often result in early tracheotomy. In our own cohort we were able to avoid this by nasopharyngeal tubes. Due to craniostenosis or midface hypoplasia sleeping abnormalities are also frequent in the first months of life. Because of the continuing growth disorder a close surveillance of these children is needed.

Conclusion: Directly after delivery infants with syndromal craniosynostosis present with a range of different problems mainly associated with pansynostosis and midface hypoplasia. An individual multidisciplinary approach in diagnostics and therapy is mandatory.