Dr. Nelson of Tulane University School of Medicine received honorariums from Sunovian and LivaNova and consulting fees from Supernus and BioMarin.)
Justine Ker of Tulane University School of Medicine has no relevant financial relationships to disclose.)
Dr. Lorincz of the University of Michigan received honorariums from Alexion for advisory board service.)
The mainstay treatment for hydrocephalus remains the placement of a ventricular shunt with a terminus most commonly in the peritoneum, atrium, or pleural cavity. Although this is an effective treatment, there are numerous complications that can occur in the operative placement and management of neurosurgical shunts. The latest thoughts on the placement and management of shunts are highlighted in this overview. The importance of minimizing radiation exposure in the evaluation of the shunted patient is discussed, including the use of rapid sequence MRI scans for surveillance and emergent evaluation. The potential impact of tablet computers on programmable shunts is also reviewed.
• Shunt malfunction is a life-threatening condition and must be treated urgently.
• It is imperative when evaluating for a potential shunt malfunction, the “baseline” image is known to have been taken at a time of shunt function to avoid the misinterpretation of “no change” as the incorrect conclusion of “no evidence of shunt malfunction.”
• Image gently in shunted patients as their lifetime exposure to unnecessary radiation puts them at increased risk of developing leukemia, brain tumors, and other cancers.
• Attention to detail in the operating room (reduced traffic, preoperative antibiotics, patient preparation, and efficient operating) is key to reducing shunt infections.
Historical note and terminology
Hydrocephalus is one of the most commonly encountered pediatric neurosurgical diseases.Hanak et al 2017). More importantly, the possibility of malfunction impacts significantly on the quality of life of patients with shunted hydrocephalus. Because shunt failure is often progressive and symptomatic, recognition of early signs of shunt failure is imperative for earlier operative exploration. This article concentrates on the nature of shunt malfunction, its diagnosis, and management.
.nez-Lage et al 2008). Occlusion of the ventricular catheter is the cause of approximately half of mechanical obstructions of shunts (Jorgensen et al 2016). The most common type of mechanical obstruction is blockage of the ventricular catheter. This is typically thought to occur by the growth of choroid plexus into the ventricular catheter, although more recent evidence suggests that the type of cellular infiltrate is dependent on the timing of shunt obstruction (Sarkiss et al 2014). For instances of choroid plexus obstruction, there is an increased risk of hemorrhage during a shunt revision as the removal of the ventricular catheter can cause bleeding from the highly vascular choroid plexus, resulting in intraventricular and parenchymal hemorrhage. This hemorrhage increases the risk of subsequent shunt malfunction and can also result in more severe neurologic injury. The shunt system can occlude anywhere along its course, including the proximal catheter, reservoirs, the valve, and the distal tubing.
Disconnection of a shunt rarely occurs shortly after placement of a new shunt unless there was a technical error, such as not attaching the catheter effectively. In some shunt systems, the ventricular catheter snaps on to a reservoir, and if this is not done adequately the ventricular catheter can disconnect from the reservoir, resulting in malfunction.Yamamoto et al 2002). The most extensive calcification was seen in the neck, presumably due to the increased mechanical stress in this region. pain in the neck or chest wall region, limitation of neck movement due to tethering of the shunt, and cutaneous manifestations such as irritation of the skin overlying the shunt (Salim et al 2015). Most surgeons will revise a shunt when a fracture or disconnection is demonstrated because the fibrous tract along a fractured shunt can drain CSF for an indeterminate length of time but will ultimately fail. Deposition of sodium chloride and other crystals involving the valve has also been demonstrated as a contributing factor in shunt malfunction (Sgouros 2004).
Distal obstruction is far less common than proximal obstruction, and the occurrence is more likely to be delayed (Hanak et al 2017). In particular, premature infants with a high protein count in their CSF may present with a distal obstruction without infection. In premature infants with certain types of shunts, the obstruction can often be cleared by irrigating the shunt, obviating the need for an operation. Distal obstructions can be associated with an infection. When distal obstruction is suspected, an abdominal ultrasound study can be useful to look for a pseudocyst, ascites, or constipation. CSF ascites can be due to infection, shunt disseminated metastases, or poor absorption of CSF into the peritoneum. Infection must be ruled out in cases of ascites. It is more common for a small pseudocyst to be infected than a large one. Treatment of infected pseudocysts involves administration of antibiotics and externalization of the shunt for a brief period of time (Dzongowski et al 2017).
The evaluation of a shunt malfunction is first and foremost a complete history and physical examination including a funduscopic examination. The Image Gently campaign has emphasized the importance of reconsidering the need for radiation as well as altering the type of radiation performed, particularly in children (Goske et al 2008). Children with ventriculoperitoneal shunts are at risk of developing malignancies at a later date if the exposure to diagnostic radiation is not carefully considered with every evaluation (Pearce et al 2012). There are studies to support not performing a routine shunt series in every child with a possible shunt malfunction unless there is clinical evidence to suggest a break. In a retrospective longitudinal cohort analysis performed by Antonucci and colleagues, most CT scans obtained in the cohort were not followed by surgical intervention (Antonucci et al 2017). If available, rapid sequence MRI can be used to assess the ventricular size. The advantage of rsMRI is that there is no radiation and it does not require sedation as it can be performed in less than 1 minute. If that modality is not available, consideration of a low-dose CT would be another option for minimizing radiation exposure. In the child with an open fontanelle, ultrasound is effective. In any event, careful consideration should be given to ordering diagnostic radiation in these patients. The routine of ordering a shunt series and a CT scan on all cases, often even prior to notifying the neurosurgeon, must be stopped.
One publication describes the use of cerebral regional oxygen saturation monitoring in the assessment of pediatric shunt patients. This technology has been used most commonly for the intraoperative monitoring of children undergoing repair of congenital heart disease. The advantage is that it is a noninvasive, easy-to-apply technology that gives instantaneous readings. The researchers found that in the setting of malfunctioning shunts, there was an asymmetrical hemispheric cerebral regional oxygen saturation, and greater changes in regional oxygen saturation occur for distal versus proximal shunt malfunction (Abramo et al 2015). Although this methodology requires more study, it potentially provides another noninvasive and safe technique for the evaluation of shunt malfunction.
In shunted adult patients with a diagnosis of normal pressure hydrocephalus, a technique of using noninvasive auditory tests (as currently used for universal neonatal hearing screenings) for the diagnosis of cerebrospinal fluid shunt malfunction was described by Sakka and colleagues (Sakka et al 2016). Additionally, ultrasound-based measurements to characterize the movement of flow through the shunt-valve interface in response to CSF flow has been shown to be another potential mode of noninvasive testing for shunt malfunction (Aralar et al 2018).
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