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

Abstract

ObjectiveTo characterize shifts from the 1960s to the first decade in the 21st century as to diagnostics, case-mix, and surgical management of pediatric patients undergoing permanent CSF diversion procedures.MethodsOne hundred and thirty-four patients below 15 years of age were the first time treated with CSF shunt or ETV for hydrocephalus or idiopathic intracranial hypertension (IIH) in 2009–2013. This represents our current practice. Our previously reported cohorts of shunted children 1967–1970 (n = 128) and 1985–1988 (n = 138) served as backgrounds for comparison.ResultsIn the 1960s, ventriculography and head circumference measurements were the main diagnostic tools; ventriculoatrial shunt was the preferred procedure (94 %), neural tube defect (NTD) was the leading etiology (33 %), and overall 2-year survival rate was 76 % (non-tumor survival 84 %). In the 1980s, computerized tomography (CT) was the preferred diagnostic imaging tool; ventriculoperitoneal shunt (VPS) had become standard (91 %), the proportion of NTD children declined to 17 %, and the 2-year survival rate was 91 % (non-tumor survival 95 %). Hydrocephalus caused by intracranial hemorrhage had, on the other hand, increased from 7 to 19 %. In the years 2009–2013, when MRI and endoscopic third ventriculocisternostomy (ETV) were matured technologies, 73 % underwent VPS, and 23 % ETV as their initial surgical procedure. The most prevalent etiology was CNS tumor (31 %). The proportion of NTD patients was yet again halved to 8 %, while intracranial hemorrhage was also reduced to 12 %. In this last period, six children were treated with VPS for Idiopathic Intracranial Hypertension (IIH) due to unsatisfactory response to medical treatment. They all had headache, papilledema, and visual disturbances and responded favorably to treatment. The 2 years of survival was 92 % (non-tumor survival 99 %). In contrast to the previous periods, there was no early shunt related mortality (2 years). Aqueductal stenosis was a small but distinctive group in all cohorts with 5, 6 and 3 % respectively.ConclusionsThe case-mix in pediatric patients treated with permanent CSF diversion has changed over the last half-century. With the higher proportion of children with CNS tumor patients and inclusion of the IIH children, the median age at initial surgery has shifted substantially from 3.2 to 14 months. Between the 1960s and the current cohort, 2 years of all-cause mortality fell from 24 to 8 %. Prolonged asymptomatic periods, extending 15 years, were relatively common. Nevertheless, 18 patients experienced shunt failure more than 15 years after last revision, and first-time shunt failure has been observed 29 years after initial treatment. This underscores the importance of life-long follow-up.

Post-haemorrhagic hydrocephalus is associated with poorer surgical and neurodevelopmental sequelae than other causes of infant hydrocephalus

“…In this study, shunt survival was poorest in the PHH group, with revision rates and number of revisions beyond a year and within 5 years significantly higher in PHH than in congenital or “other” hydrocephalus. This is consistent with Paulsen et al who demonstrated more frequent revisions in the PHH group than other aetiologies, and Notarianni et al who similarly showed higher 5-year shunt failure rates in PHH compared to congenital and “other” hydrocephalus, with no significant differences between PHH and myelomeningoceles [ 6 , 20 ].…”

“…The congenital and "other" hydrocephalus groups had significantly more "0 s" (normal outcome) than PHH at every time point (except 10 years for "other") (p < 0.01), and significantly fewer "3 s" than PHH at 5 and 10 years (p < 0.01). PHH, post-haemorrhagic hydrocephalus; NTDs, neural tube defects; NDO, neurodevelopmental outcome other aetiologies, and Notarianni et al who similarly showed higher 5-year shunt failure rates in PHH compared to congenital and "other" hydrocephalus, with no significant differences between PHH and myelomeningoceles [6,20].…”

Neuroendoscopic surgery in neonates — indication and results over a 10-year practice

“…Furthermore, the demonstrated effect of NEL to lower CSF protein concentration has been used prior shunt implantation [24]. The overall shunt revision-free survival rate of 66.7% and a patient rate of 28.8% from 72 infants who needed revision within the first year is acceptable for this high-risk cohort of neonates [25][26][27]. On contrary to the rather effective treatment of hydrocephalus by CSF diverting shunt, the failure of all performed ETVs (n = 6) to permanently control a hydrocephalic situation reflect the previously published experiences after this operative procedure in very young children [4].…”

Outcomes in adulthood after neurosurgical treatment of brain tumors in the first 3 years of life: long-term follow-up of a single consecutive institutional series of 97 patients

“…If treated today, these patients might have been candidates for modern radiotherapy at tumor progression. The latter late death was caused by an acute shunt failure which is exceedingly rare, but the risk of such failure is impossible to fully eliminate [ 12 ]. The fact that 72% of long-term survivors in this LG tumor group had full-time working capacity is important and very interesting.…”