Abhaya V. Kulkarni
Abhaya V. Kulkarni and Iffat Shams
Children with hydrocephalus face several quality of life (QOL) issues that have been poorly studied. The authors' aim was to quantify the QOL for children with hydrocephalus and identify predictors of long-term outcome, using a reliable and validated outcome measure: the Hydrocephalus Outcome Questionnaire (HOQ).
All children (5–18 years old) with treated hydrocephalus attending the neurosurgery outpatient clinic at the Hospital for Sick Children were asked to participate. The patient's QOL was measured by the parent-completed HOQ. Predictor variables were extracted from the medical records. Multivariable linear regression was used to identify those predictor variables that were significantly associated with outcome.
There was an 89% participation rate, with a total of 346 children participating (mean age 11.7 years, mean duration since diagnosis 9.9 years). Their mean HOQ Overall Health score was 0.68 (on a scale of 0 [worst QOL] to 1.0 [best QOL]). On multivariable analysis, the following predictors were associated with a worse overall QOL: increased seizure frequency, increased length of stay (LOS) in the hospital for the initial treatment of hydrocephalus, increased LOS for treatment of shunt infection and shunt overdrainage, increased number of proximal shunt catheters in situ, and increased distance of the family residence from the pediatric neurosurgical center.
For the first time, these results establish baseline QOL values for a typical large group of children many years after their diagnosis of hydrocephalus, by using a validated and reproducible outcome measure. Many of the factors that adversely impact QOL appear to be related to shunt complications and might, therefore, be modifiable.
Shelly Wang, James Drake and Abhaya V. Kulkarni
Spontaneous subaponeurotic fluid collection (SSFC) is an uncommon and newly described entity of unknown etiology, observed in infants less than 1 year of age. The authors report on series of infants who presented to the Hospital for Sick Children (HSC) with SSFC, focusing on the natural history of this condition.
Data from the Hospital for Sick Children were retrospectively reviewed for the period between January 2004 and June 2015. Patient age and sex, birth history, medical history, laboratory findings, and symptoms were reviewed. SSFC location, imaging characteristics, management, and outcome were also analyzed. A MEDLINE and Embase literature search was performed on the condition, yielding previously reported cases of SSFC in the English language.
Nine cases involving patients who presented with SSFC during the study period were identified. The patients were 4 male and 5 female infants (age range 5 weeks to 11 months). All cases of SSFC developed spontaneously over a period of days, and the infants had no history of injuries, trauma, or hair manipulation in the immediate period preceding the development of the subgaleal collections. Six patients underwent remote forceps- or vacuum-assisted instrumented births, although none of the patients developed scalp collections or skin discoloration immediately after birth. All of the cases were managed conservatively on an outpatient (6 cases) or inpatient (3 cases) basis. In 1 case, the size of the fluid collection fluctuated over 4 months, but in all of the cases, the collections resolved spontaneously without structural or infectious complications.
This is the largest series describing SSFC to date and summarizes 9 cases managed at a large academic neurosurgical center. Although the specific pathophysiology of SSFC remains unknown, in some cases the condition may be associated with a remote history of instrumented delivery. SSFC occurs spontaneously without immediate preceding trauma, and an extensive hematology or child abuse workup is not necessary. A conservative approach with outpatient follow-up is advocated.
Abhaya V. Kulkarni
Benjamin C. Warf and Abhaya V. Kulkarni
In the setting of a developing country where preoperative imaging may be limited, the authors wished to determine whether cisternal scarring or aqueduct patency at the time of surgery was sufficiently predictive of the failure of endoscopic third ventriculostomy (ETV) to justify shunt placement at the time of the initial operation.
The status of the prepontine cistern and aqueduct at the time of ventriculoscopy was prospectively recorded in 403 children in whom an ETV had been completed. Kaplan-Meier methods were used to construct survival curves. A Cox proportional hazards model was used to provide estimates of HRs for the time to ETV failure. Several independent variables were tested in a single multivariable model, including those previously shown to be associated with ETV survival, that is, age, hydrocephalus etiology, and extent of choroid plexus cauterization (CPC). In addition, intraoperative variables of particular interest were included in the analysis: status of the aqueduct at surgery (closed vs open) and status of the prepontine cistern at surgery (scarred vs clean/unscarred). Multicollinearity was not a concern since the variance inflation factors for all variables were < 2. The examination of stratified survival curves confirmed the appropriateness of the proportional hazards assumption for each variable.
Overall actuarial 3-year success was 57%. Consistent with previous results, age, hydrocephalus etiology, and extent of CPC were significantly associated with ETV success. A closed aqueduct and an unscarred cistern were each independently associated with significantly better ETV success (HRs of 0.66 and 0.44, respectively). The presence of cisternal scarring more than doubled the risk of ETV failure, and an open aqueduct increased the risk of failure by 50%.
Intraoperative observations of the aqueduct and prepontine cistern are independent predictors of the risk of ETV failure and can be used to further refine outcome predictions based on age, hydrocephalus etiology, and extent of CPC. Further studies will test validity in several African centers and determine what threshold of failure risk should prompt shunt placement at the initial operation.
Abhaya V. Kulkarni, Ruth Donnelly and Iffat Shams
The Hydrocephalus Outcome Questionnaire (HOQ) is an established means of measuring quality of life, but the cognitive component of this questionnaire has never been formally compared with gold-standard neuropsychological test scores. The authors hypothesized that the HOQ Cognitive Health score would demonstrate a relatively strong correlation with neuropsychological test scores, whereas much weaker correlations would be seen for HOQ Physical and Social-Emotional Health scores.
A cross-sectional study of children with long-standing hydrocephalus presenting to The Hospital for Sick Children's Neurosurgery Clinic was performed between July 2006 and September 2008. Participating children and families completed the HOQ and a battery of 21 standard neuropsychological tests and questionnaires. Pearson correlation analysis was then performed.
A total of 83 patients (81% participation) was accrued; the mean age was 11.5 ± 3.4 years (mean ± SD) at the time of assessment. The mean age at hydrocephalus treatment was 1.3 ± 2.6 years. The mean overall HOQ score was 0.69 ± 0.21. The HOQ Cognitive score had a moderate or strong correlation with 19 (90%) of 21 neuropsychological test scores, much more so than the HOQ Social-Emotional score (5 moderate or strong correlations, 24%) and the HOQ Physical score (1 moderate correlation, 5%). For 19 neuropsychological tests (90%), the HOQ Cognitive score had a stronger correlation than the other scores. The HOQ Cognitive score had particularly strong correlations with the Verbal IQ, List Learning, Behavior Problems, and Metacognitive Abilities components.
Data from a wide-ranging representative sample of children with long-standing hydrocephalus provide added evidence of the validity of the HOQ Cognitive score and the overall domain structure of the HOQ itself.
Abhaya V. Kulkarni, James M. Drake and Maria Lamberti-Pasculli
Object. Hydrocephalus is a common condition of childhood that usually requires insertion of a cerebrospinal fluid (CSF) shunt. Infection is one of the most devastating complications that may arise from the presence of CSF shunts. In this study, the authors prospectively analyzed perioperative risk factors for CSF shunt infection in a cohort of children.
Methods. Between 1996 and 1999, 299 eligible patients underwent CSF shunt operations (insertions and revisions) that were observed by a research nurse at a tertiary care pediatric hospital. Several perioperative variables were recorded. All cases were followed postoperatively for 6 months to note any development of CSF shunt infection. A Cox proportional hazards model was used to analyze the relationship between the variables and the development of shunt infection.
Thirty-one patients (10.4%) experienced shunt infection. Three perioperative variables were significantly associated with an increased risk of shunt infection: 1) the presence of a postoperative CSF leak (hazard ratio [HR] 19.16, 95% confidence interval [CI] 6.96–52.91); 2) patient prematurity (< 40 weeks' gestation at the time of shunt surgery: HR 4.72, 95% CI 1.71–13.06); and 3) the number of times the shunt system was inadvertently exposed to breached surgical gloves (HR 1.07, 95% CI 1.02–1.12).
Conclusions. Three variables associated with an increased incidence of shunt infection have been identified. Changes in clinical practice should address these variables, as follows. 1) Great care should be taken intraoperatively to avoid a postoperative CSF leak. 2) Alternatives to placement of a CSF shunt in premature infants should be studied. 3) Surgeons should minimize manual contact with the shunt system and consider the use of double gloves.