Investigators from Seattle Children’s Hospital (SCH), Seattle, WA, conducted a retrospective study to evaluate risk of death in children with pediatric-onset nontumor related hydrocephalus, and risk of death specifically related to hydrocephalus rather than comorbidities. The investigators also sought to determine the association of goals-of-care decisions with death from hydrocephalus. For the study, the Clinical Data Repository at SCH was reviewed, and the medical records of children with an ICD-9 or ICD-10 code for hydrocephalus with an encounter between January 2009 and February 2018 were reviewed. Only patients with progressive ventricular dilation, diagnosed before the age of 21 years, were eligible for the study; those with stable ventriculomegaly, or hydrocephalus due to obstruction from a tumor were excluded. Data abstracted on study participants included age at diagnosis, cause of hydrocephalus, associated comorbidities, surgical intervention, and age at death, if applicable. For children who had died, the investigators classified death as related to a comorbidity or due specifically to hydrocephalus, and for those who died from hydrocephalus, the medical record and supplemental materials (eg, supplemental information from providers) were reviewed to assess whether the goals of care were to provide comfort rather than prolong life. A multivariate Cox regression model was used to identify independent demographic and/or clinical predictors of death. Kaplan-Meier curves were used to determine mortality rates.
Data were analyzed on 1,705 patients with nontumor associated hydrocephalus. The most common causes of hydrocephalus were malformation/congenital (443 patients, 26.0%) and posthemorrhagic (425, 24.9%). Mean duration of follow-up of study participants was 9.8 years. Among the study participants, 88 (5.2%) had died at the time of analysis. The mean age of death in these children was 7.1 ±6.9 years. The overall mortality of patients in the cohort was 8.3 deaths per 1,000 person-years. In the multivariate models, significant predictors of death were posthemorrhagic cause of hydrocephalus (compared to spina bifida as the cause; hazard ratio [HR], 2.64; 95% CI, 1.07, 6.51) and any hydrocephalus surgery (HR, 0.48; 95% CI, 0.29, 0.79). Death was related to comorbidities in 65 of the 88 patients who died (76%). Of the 14 children whose death was specifically related to hydrocephalus, 7 died of shunt-related complications. In each of these children, full medical support was provided. For the remaining 7 children who died, death was influenced by prognosis and decisions related to goals of care. Three patients underwent cerebrospinal fluid (CSF) diversion procedures but were subsequently provided comfort care, and in 4 newborns with severe congenital hydrocephalus, CSF diversion was not performed because of goals of care decisions.
The authors conclude that children with hydrocephalus were more likely to die with associated medical conditions than directly because of the hydrocephalus.
Dr Candee has disclosed no financial relationship relevant to this commentary. This commentary does not contain a discussion of an unapproved/investigative...