Arrested Hydrocephalus

Arrested Hydrocephalus in Childhood: Case Series and Review of the Literature

With the advent of shunting systems in the 1950s, hydrocephalus changed from a severely debilitating, and usually lethal, condition to a treatable disease, with greatly improved life expectancy and possibility of near-normal neurological functioning, depending of course on the etiology of the hydrocephalus.[1] This improved further when modern optic instruments enabled endoscopic third ventriculocisternostomy for a subset of patients, thereby eliminating the necessity for implantation of hardware, which always remains prone to malfunctioning. Despite these great improvements in the treatment of hydrocephalus, both shunts and endoscopic procedures are fraught with short- and long-term complications, some of which can be quite devastating. The myriad of shunt systems available nowadays to the neurosurgeon to choose from, clearly illustrates that the “perfect” shunt has not yet been developed, and one may wonder whether the complex dynamics of cerebrospinal fluid (CSF) production, flow, and absorption could ever be substituted by simple mechanical devices. Therefore, the indication to surgically treat any child with a documented hydrocephalus must be very strict to avoid unnecessary treatment and its inherent complications.

In the large majority of children, hydrocephalus will present with clear signs and symptoms of progressively increased intracranial pressure, easily diagnosed by pediatric neurologists, and documented by magnetic resonance imaging.[2] Whenever possible, endoscopic third ventriculocisternostomy will be the first-choice intervention, with ventriculoperitoneal shunting being a good second alternative. However, in some children the clinical and radiological picture is somewhat more difficult, especially when there is no progression or harmful sequelae due to the hydrocephalus, leading to a diagnosis of “arrested hydrocephalus.” Subsequently, serious doubts arise about the necessity, and usefulness, of surgical intervention. Although the literature on arrested hydrocephalus is scant, each pediatric neurosurgery department will recognize this clinical dilemma.

The exact definition of arrested hydrocephalus is not generally agreed upon, and the term “compensated hydrocephalus” is often used interchangeably, and not only in patients never treated for enlarged CSF spaces, but also in those with nonfunctioning shunts. The definition that Hurni et al use, i.e., when a new balance between CSF production and absorption is achieved, and progressive hydrocephalus spontaneously stops, is based on quite old literature.[3] [4] [5] Nevertheless, they are to be commended for listing their experience of five children, seen in two centers within a time frame of 3 years, with long-term follow-up. Their considerations on the difficulty of a definite diagnosis of arrested hydrocephalus are thoughtful, and they openly discuss their doubts. But the decision to shunt a young child entails that child to considerable life-long shunt-related risks, and that fact alone merits every possibility to try and avoid surgery, especially in the absence of clinical signs and symptoms.

*This article is an editorial comment on “Arrested hydrocephalus in childhood: case series and review of the literature” by Hurni et al (Neuropediatrics 2018; doi: 10.1055/s-0038-1660854).

• References

• 1 Drake JM, Sainte-Rose C. The Shunt Book. 1st ed. Cambridge, MA: Wiley-Blackwell; 1995
• 2 Greenberg MS. Handbook of Neurosurgery. 8th ed. New York, NY: Thieme; 2016
• 3 Hurni Y, Poretti A, Schneider J, Guzman R, Ramelli GP. Arrested hydrocephalus in childhood: case series and review of the literature. Neuropediatrics 2018; DOI: 10.1055/s-0038-1660854.
• 4 Johnston I, Teo C. Disorders of CSF hydrodynamics. Childs Nerv Syst 2000; 16 (10-11): 776-799
• 5 Laurence KM, Coates S. The natural history of hydrocephalus. Detailed analysis of 182 unoperated cases. Arch Dis Child 1962; 37: 345-362