gms | German Medical Science

Objective: Chiari malformation type II is a common condition in children with open spina bifida and in this patient group also a frequent cause (up to 80%) of death in early childhood (age 1 to 2 years). There are controversial attitudes how to interpret clinical symptoms and often impressive MRI findings in planning and considering options of care, follow-up and surgical treatment. Aspects and risks of surgical and conservative treatment are reflected reviewing the course of 2 patients in a group of 37 consecutive children with open spina bifida and taking into consideration the results of an inquiry dealing with different questions of care and treatment distributed within a group of 306 meanwhile adult patients with open spina bifida.

Method: 306 adult patients with open spina bifida responded to a questionnaire with several questions about course and treatment including operations performed for Chiari malformation type II. The course of 2 patients (one boy and one girl) operated for this condition in a group of consecutive 37 children was reviewed with special focus on clinical and neurological aspects.

Results: Only 43 of 306 meanwhile adult patients needed treatment for Chiari malformation. 1 child needed early cranio-spinal decompression for rapidly developing and critical hindbrain dysfunction at the age of 1 month. The boy is now 11 months of age and stable with mild vocal cord paresis. With the other child cranio-spinal decompression was performed several times. Follow-up ended at the age of 16 years about 2 years after the last operation performed in another unit with postoperative brain stem ischemia. The young girl is known to have died due to a tragic accident with her wheel chair.

Conclusions: Although impressive narrowing in the cranio-spinal junction is to be found frequently in MRI scans, obviously only a minority of the patients with open spina bifida needs surgical treatment. When Chiari malformation type II becomes symptomatic evaluation of a possible malfunction of an implanted shunt system and cranio-spinal decompression may be necessary but this also carries a significant risk. Systematic clinical and radiological follow-up are of help to find out which child actually needs treatment. The adequate interval for MRI scans possibly requiring sedation or general anesthesia has to be defined individually.