Artikel
Treatment of Chiari malformations in children
Behandlung von Chiari Malformationen im Kindesalter
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Autoren
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J. Klekamp
- Zentrum Neurochirurgie, Christliches Krankenhaus Quakenbrück
| Veröffentlicht: | 30. Mai 2008 |
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Gliederung
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Objective: Whereas Chiari I malformation (CMI) is caused by a small posterior fossa, well treatable by decompression and does not represent a primary CNS abnormality, the pathophysiology of Chiari II malformation (CMII) and justification of its surgical treatment are discussed controversially. This paper demonstrates surgical results for children with CMI and CMII to provide treatment guidelines.
Methods: Hospital files, neuroradiological examinations, intraoperative documentations and follow-up examinations were analyzed. Rates of postoperative improvements and clinical recurrence rates according to Kaplan-Meier statistics were determined.
Results: 37 of 492 CMI-patients and 15 of 25 CMII-patients presented in the pediatric age group. Children with CMI were significantly older than those with CMII (12±4 years and 6±6 years, respectively). For both groups symptom patterns changed with age: in newborns, respiratory problems and caudal cranial nerve dysfunctions were observed, whereas in elder children scoliosis, gait ataxia and occipital pain predominated. Clinical history was significantly longer in CMI and more dramatic in CMII. All children underwent decompression either at the foramen magnum (CMI) or the cervical canal (CMII), provided no hydrocephalus or functional shunt was present. Postoperatively, 69% of children with CMI were improved compared to 33% with CMII, whereas 27% and 50%, respectively, remained unchanged. Clinical recurrence rates were 13% after 1 and 5 years for CMI and 16% and 44%, respectively, for CMII. Late deteriorations in CMII were related to postoperative cervical instability.
Conclusions: Children with CMI and CMII benefit from decompressive surgery. In CMII, indication for surgery should be considered early as further neurological progression can be prevented if measures are taken to avoid or treat postoperative instability. This study does not support views, that neurological deteriorations in sufficiently shunted children with CMII indicate an untreatable developmental defect of the CNS.
