Children whose ventricles do not change during shunt malfunction present a diagnostic dilemma. This study was performed to identify risk factors for unchanged ventricular size at shunt malfunction. This retrospective 1:1 age-matched case-control study identified children with shunted hydrocephalus who underwent shunt revision with intraoperative evidence of malfunction at one of the three participating institutions from 1997 to 2019. Cases were defined as patients with a change of < 0.05 in the frontal-occipital horn ratio (FOR) between malfunction and baseline, and controls included patients with FOR changes ≥ 0.05. The presence of infection, abdominal pseudocyst, pseudomeningocele, or wound drainage and lack of baseline cranial imaging at the time of malfunction warranted exclusion. Of 450 included patients, 60% were male, 73% were Caucasian, and 67% had an occipital shunt. The median age was 4.3 (IQR 0.97-9.21) years at malfunction. On univariable analysis, unchanged ventricles at malfunction were associated with a frontal shunt (41% vs 28%, p < 0.001), programmable valve (17% vs 9%, p = 0.011), nonsiphoning shunt (85% vs 66%, p < 0.001), larger baseline FOR (0.44 ± 0.12 vs 0.38 ± 0.11, p < 0.001), no prior shunt infection (87% vs 76%, p = 0.003), and no prior shunt revisions (68% vs 52%, p < 0.001). On multivariable analysis with collinear variables removed, patients with a frontal shunt (OR 1.67, 95% CI 1.08-2.70, p = 0.037), programmable valve (OR 2.63, 95% CI 1.32-5.26, p = 0.007), nonsiphoning shunt at malfunction (OR 2.76, 95% CI 1.63-4.67, p < 0.001), larger baseline FOR (OR 3.13, 95% CI 2.21-4.43, p < 0.001), and no prior shunt infection (OR 2.34, 95% CI 1.27-4.30, p = 0.007) were more likely to have unchanged ventricles at malfunction. In a multicenter cohort of children with shunt malfunction, those with a frontal shunt, programmable valve, nonsiphoning shunt, baseline large ventricles, and no prior shunt infection were more likely than others to have unchanged ventricles at shunt failure.
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