Revision Chiari Surgery in Young Children: Predictors and Outcomes

Children aged 0-6 years with Chiari malformation (CM) often present with atypical symptoms and require revision surgery more often than older children. We studied characteristics and outcomes of CM patients in this age-group who underwent one or more revision surgeries to assess how often revision surgery is necessary and successful in this age-group.

We retrospectively reviewed patients who were diagnosed with CM 1 or CM 1.5 and surgically treated with posterior fossa decompression (PFD) with or without duraplasty before their 7th birthday. Basic demographics, preoperative presentation, operative details, and postoperative outcomes were analyzed.

Forty patients (mean age 3.2 ± 1.7 years, 35% female) were reviewed. The most common presenting symptoms were headache, dysphagia, and respiratory problems. Eight patients required one or more revision surgeries 11.6 ± 7.6 months on average after their initial surgery. Comparing the revision and no revision groups, dysautonomia was significantly more common prior to initial surgery in the children requiring revision (37.5 vs. 3.1%, p = 0.02). The revision group also trended toward more dysphagia (75.0 vs. 46.9%, p = 0.24) and respiratory problems (75.0 vs. 40.6%, p = 0.12). The most frequent reasons for reoperation were symptom recurrence (6/8), residual posterior fossa compression (3/8), significant scar tissue formation (2/8), ventral brainstem compression (1/8), and suspected craniocervical instability (1/8). Of the 8 children undergoing reoperation, surgery achieved symptom resolution or improvement in 5, while 3 had unchanged symptoms. Two patients underwent a third surgery, after which 1 showed improvement and the other did not. This last patient showed short-term improvement after a fourth surgery but had symptom recurrence 12 months later.

Oropharyngeal and respiratory problems are particularly common in children aged 0-6 years with CM. Presentation with dysautonomia or other signs of brainstem compression will often predict an additional surgery will be needed after an initial PFD. Symptom recurrence is the most frequent reason for reoperation, and revision surgeries lead to improved clinical outcomes in the majority but not all of these young patients. Surgery in very young children is successful, but reoperation should be integrated into an up-front discussion algorithm particularly in children with severe symptoms.