Revision Chiari Surgery in Young Children: Predictors and Outcomes

Abstract

<b><i>Introduction:</i></b> 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. <b><i>Methods:</i></b> 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. <b><i>Results:</i></b> 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%, <i>p</i> = 0.02). The revision group also trended toward more dysphagia (75.0 vs. 46.9%, <i>p</i> = 0.24) and respiratory problems (75.0 vs. 40.6%, <i>p</i> = 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. <b><i>Discussion/Conclusion:</i></b> 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.

Reoperation in Chiari-1 Malformations

“…Basaran and Colleagues [27] reported a higher rate of complications in CM-1.5 than in CM-1. This finding was not confirmed by Chae and Greenfield [24], who reported no significant differences in the rate of reoperations. Patients with CM-1.5 would have more severe syndromes and shorter clinical histories [27] and may require different treatment modalities [14].…”

“…Reoperation due to failed treatment is a major problem in the management of CM-1 syndrome. All series show rates of treatment failures that are not negligible [7,11,[17][18][19][20][21][22][23][24][25][26]. However, the skillfulness of the surgeon may play a significant role: in the series of Walker-Palmer et al [26], the complication rate ranged from 11 to 20% for extradural, and 10.5 to 40% for intradural surgeries depending on the experience of the surgeons.…”

Natural History of Chiari 1 Deformity

“…They concluded that recurrence of symptoms is the most common reason for reoperation and that there were improved outcomes clinically in most if not all of these young patients. 22 Case 5 of our series demonstrates a scenario where multiple surgeries were performed with apparent initial improvement, but currently with MRI appearances demonstrating a worsening scenario once again.…”

“…Chae and Greenfield 22 reported that of 45 patients who underwent decompressive surgery for Ch1, 8 (18%) required revision surgeries (1 or more) and 5 of these showed improvement or resolution after reoperation with no change seen in 3 (of which 2 underwent a third surgery with improvement in one case and none in the other, with the last patient being operated for the fourth time, and recurrence of symptoms a year later). They concluded that recurrence of symptoms is the most common reason for reoperation and that there were improved outcomes clinically in most if not all of these young patients 22 . Case 5 of our series demonstrates a scenario where multiple surgeries were performed with apparent initial improvement, but currently with MRI appearances demonstrating a worsening scenario once again.…”

A gene network implicated in the joint-muscle pain, brain fog, chronic fatigue, and bowel irregularity of Ehlers-Danlos and “long” COVID19 syndromes

“…Expanded studies of EDS and long COVID19 symptoms and outcomes-Systematic ascertainment of patient findings via the findings of S1 Table included few laboratory and no medical measures like the results of echocardiography-vascular screening [7], tilt-table [30,31], nerve conduction [13], intestinal motility [36], or imaging for Chiari [134], median arcuate ligament [135], or nutcracker changes [136] that lead to immediate therapies. Evaluation protocols adding the latter results could improve symptom descriptions [129] and outcome measures [130] for EDS and long COVID19 as done for COVID19 infection in patients with rheumatic diseases [131].…”