Treatment analysis of craniosynostosis combined with Chiari malformation in children

Objective To explore the surgical method and outcome of craniosynostosis combined with Chiari malformation in children. Methods A retrospective analysis was conducted on the clinical data of 16 children with craniosynostosis combined with Chiari malformation who were admitted to Department of Neurosurgery, Children′s Hospital of Nanjing Medical University from April to December 2017. All cases were retrospectively analyzed and their imaging results were analyzed. Of the 16 children without hydrocephalus, 12 underwent craniofacial reconstruction of cranial suture or craniofacial reconstruction, of which 1 case underwent posterior fossa reconstruction and suboccipital decompression at 7 months post operation. In 4 children with combined hydrocephalus, 3 underwent routine right ventricle-peritoneal shunting and cranial reconstruction, of which 1 case was treated with posterior fossa reconstruction and suboccipital decompression at 5 months post operation. The other 1 case was Pfeiffer syndrome and underwent right ventricle-peritoneal shunting plus posterior fossa reconstruction as well as suboccipital decompression. At 3, 6 and 12 months post operation, all patients were followed up at the outpatient clinic and underwent the reexamination of head MRI for assessment of brain tissue development, ventricle changes and cerebellum tonsil herniation. Results All 16 children underwent operation successfully without any surgical complications. Revealed by the postoperative skull CT, all the children′s cranial cavity volume was enlarged, and the cranial malformation was improved. The follow-up time of 16 children was (12.1±2.0) months (10-18 months). The review of head MRI showed that brain tissue developed well and the position of the cerebellar tonsils did not decline further. All children were free of neurological deficits, and parents were satisfied with the improvement in the appearance of their skulls and faces. Conclusions The combination of craniosynostosis with Chiari malformation should be fully evaluated before surgery and appropriate individualized treatment program should be selected according to specific conditions. One-stage craniofacial surgery seems an effective treatment method. Key words: Craniosynostosis; Arnold-Chiari malformation; Child; Treatment outcome