Pediatric hydrocephalus: 40-year outcomes in 128 hydrocephalic patients treated with shunts during childhood. Assessment of surgical outcome, work participation, and health-related quality of life

Abstract

<jats:sec> <jats:title>OBJECT</jats:title> <jats:p>Treatment for hydrocephalus has not advanced appreciably since the advent of CSF shunts more than 50 years ago. The outcome for pediatric patients with hydrocephalus has been the object for several studies; however, much uncertainty remains regarding the very long term outcome for these patients. Shunting became the standard treatment for hydrocephalus in Norway during the 1960s, and the first cohorts from this era have now reached middle age. Therefore, the objective of this study was to review surgical outcome, mortality, social outcome, and health-related quality of life in middle-aged patients treated for hydrocephalus during childhood.</jats:p></jats:sec> <jats:sec> <jats:title>METHODS</jats:title> <jats:p>Data were collected in all patients, age 14 years or less, who required a CSF shunt during the years 1967–1970. Descriptive statistics were assessed regarding patient characteristics, surgical features, social functioning, and work participation. The time and cause of death, if applicable, were also determined. Kaplan-Meier survival estimates were used to determine the overall survival of patients. Information regarding self-perceived health and functional status was assessed using the 36-Item Short Form Health Survey (SF-36) and the Barthel Index score.</jats:p></jats:sec> <jats:sec> <jats:title>RESULTS</jats:title> <jats:p>A total of 128 patients were included in the study, with no patient lost to follow-up. Of the 128 patients in the study, 61 (47.6%) patients died during the 42–45 years of observation. The patients who died belonged to the tumor group (22 patients) and the myelomeningocele group (13 patients). The mortality rate was lowered to 39% if the patients with tumors were excluded. The overall mortality rates at 1, 2, 10, 20, and 40 years from time of initial shunt insertion were 16%, 24%, 31%, 40%, and 48% respectively. The incidence of shunt-related mortality was 8%. The majority of children graduated from a normal school (67%) or from a school specializing in education for physically handicapped children (20%). Self-perceived health was significantly poorer in 6 out of 8 domains assessed by SF-36 as compared with the background population. Functional status among the survivors varied greatly during the follow-up period, but the majority of patients were self-dependent. A total of 56% of the patients were socially independent, and 42% of the patients were employed.</jats:p></jats:sec> <jats:sec> <jats:title>CONCLUSIONS</jats:title> <jats:p>Approximately half of the patients are still alive. During the 42–45 year follow-up period, the mortality rate was 48%. Two deaths were due to acute shunt failure, and at least 8% of the deaths were shunt related (probable or late onset). The morbidity in middle-aged individuals treated for pediatric hydrocephalus is considerable.</jats:p> <jats:p>The late mortality rate was low, but not negligible. Twelve patients died during the last 2 decades, 1 of whom died because of acute shunt failure. Although the shunt revision rate was decreasing during the study period, many patients required shunt surgery during adulthood. Forty-one revisions in 21 patients were performed during the last decade. Thus, there is an obvious need for life-long follow-up in these patients.</jats:p></jats:sec>

Cerebrospinal fluid (CSF) shunting and ventriculocisternostomy (ETV) in 400 pediatric patients. Shifts in understanding, diagnostics, case-mix, and surgical management during half a century

“…Better nutritional intake, usage of folic acid supplement before and during early pregnancy, amniocentesis, and screening for alpha-fetoprotein may also have been contributing factors [9, 10]. The gradual decreasing number of shunted individuals with NTD in our three cohorts from the 1960s, the 1980s and further, or until today corroborate with their findings [6, 7]. …”

“…At the same time, in the cohort from the twenty-first century, we found a higher proportion of hydrocephalus associated with CNS tumor compared with our previous series [6, 7]. Some studies have found a gradual increase in the incidence of pediatric brain tumors during the last decades [13].…”

“…In the 1960s, NTD was accounting for one third of the children requiring shunt insertion [7]; in the 1980s, the proportion was nearly halved to 17 percentages [6], and in the twenty-first century, the proportion of children was only 8 %. Hemorrhage was the cause of shunting in 7 % in the 1960s, increased to 19 % in the 1980s, and reduced to 12 % in the years 2009–2013 [6, 7]. The most common distinct etiological group in the third cohort was CNS neoplasm (31 %) which accounted for 20 and 17 % in the first and second period.…”

“…Two previous cohorts of children, both including children younger than 15 years of age, initially shunted for hydrocephalus in the same institution during the years between 1967–1970 ( n  = 128) and 1985–1988 ( n  = 138) and formed the basis for our comparison [6, 7]. …”

Surgical Treatment for Neonatal Hydrocephalus: Catheter-Based Cerebrospinal Fluid Diversion or Endoscopic Intervention?

“…Besides the surgical letality (approx-Open Journal of Modern Neurosurgery imately 8%), the mortality rate over 20 years of follow-up has been reduced from 39% in the 1960s to 21% in the 1980s for patients without a tumor as underlying disease [50] [51]. Many patients present with neurologic deficits such as motor, visual or auditory impairment, and a huge number of deaths is due to delayed diagnosis of CSF diversion malfunction, regardless whether a shunt implantation or an endoscopic fenestration have been performed [11] [43].…”