Clinical Study
Timing of endoscopic third ventriculostomy in pediatric patients with congenital obstructive hydrocephalus: assessment of neurodevelopmental outcome and short-term operative success rate

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Abstract

The purpose of this study was to determine the impact of early (⩽6 months old), midterm (6–12 months old) and late (>12 months old) endoscopic third ventriculostomy (ETV) on the operative success rate and postoperative neurodevelopmental outcome of children with congenital obstructive hydrocephalus. We divided 63 children into three groups according to whether they underwent early, midterm or late ETV. Their preoperative developmental quotient (DQ) was assessed using the Gesell developmental diagnosis schedule (GDDS). Three and 6 months after the initial procedure, GDDS was used to obtain postoperative DQ from two assessments (blinded and non-blinded). Meanwhile, two observers studied the operative success rate of initial ETV. There were no substantial differences between blinded and non-blinded assessments. The success rate of early ETV was only 20.8%. By contrast, this rate was 55% and 73.7% for midterm and late ETV, respectively. Before operation, we observed severe developmental abnormalities in all children (DQ score < 40). However, children in midterm and late ETV groups achieved improvement after the operation, which was particularly remarkable in late ETV group. Six months after the first surgery, 16 (84.2%) children in the late ETV group, nine (45%) in the midterm ETV group and four (16.7%) in the early ETV group had moderate developmental disability. Nevertheless, overall prognosis for the three groups was not optimistic. There were no children with mild neurodevelopmental disability or normal function. Our data confirmed that age is a determinant for ETV effectiveness and overall prognosis.

Introduction

After Jason Mixters performed an endoscopic third ventriculostomy (ETV) for the first time in 1923, the procedure became an option for treating occlusive hydrocephalus of various origins. During the past decades, it has been widely used on pediatric patients with aqueduct stenosis in many centers because of its advantage of providing a natural route for cerebrospinal fluid (CSF) flow within the range of indications. The long-term success rate has been reported to be as high as 80% [1], [2], [3], [4], however, according to some reports on ETV, children younger than 6 months have a lower success rate of 12.5% [6]. Furthermore, several studies have indicated that age at the time of ETV is the most important independent risk factor for surgical effectiveness and long-term functional outcome [5]. More recent evidence from a larger, multicenter series supported the finding that age was the main determinant for outcome in younger children, particularly neonates [20], [24], [25]. Due to the high failure and complication rates of ETV, severe neurodevelopmental sequelae and poor quality of life of these children, ETV has been preformed on a highly selective basis. Therefore, determining suitable candidates for ETV was difficult, but important [7].

Little is known about long term outcome in pediatric patients with hydrocephalus, particularly neurodevelopmental outcomes and health-related quality of life. The effectiveness of ETV should not merely be measured by morbidity rate. In this study, we investigated the success of ETV and the postoperative neurodevelopmental outcome of 63 consecutive children who underwent initial ETV at different ages for the treatment of congenital obstructive hydrocephalus.

Section snippets

Patients

A total of 459 pediatric patients enrolled between January 2010 and January 2014 underwent primary ETV for the treatment of congenital obstructive hydrocephalus (aqueduct stenosis) and 63 fulfilled the highly selective inclusion criteria. The exclusion criteria were previous history of supra- or infratentorial tumor, myelomeningocele, intracranial infection, intracranial cyst, intraventricular hemorrhage, Chiari malformation, Dandy–Walker syndrome, trauma, or bone marrow or blood diseases.

Results

ETV was initially performed in 63 children who were diagnosed for the first time with obstructive hydrocephalus due to congenital aqueduct stenosis. The children were under 18 months of age (median 7.8 months; range: 3.3–18) with no previous history of neurosurgical interventions (external ventricular drainage or VPS). All selected children were categorized into three groups according to whether they underwent early (age  6 months), midterm (6 < age  12 months) or late (age > 12 months) ETV. In addition,

Discussion

In this study, we investigated the success rate of ETV as a primary treatment for congenital obstructive hydrocephalus caused by aqueduct stenosis in pediatric patients with no previous history of ETV or VPS. Health-related quality of life for children with hydrocephalus is of concern for many researchers, however, few studies focus on the impact of ETV treatment on neurodevelopmental outcome in children. Therefore, we assessed pre- and postoperative neurodevelopmental status by means of GDDS.

Conclusion

Our study confirmed that age at the time of surgery was a significant risk factor for both operative success rate and functional outcome. Although children achieved improvement of outcome to some extent after ETV, overall prognosis was not optimistic.

Conflicts of Interest/Disclosures

The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication.

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