Cerebrospinal fluid shunt malfunction diagnosis

The diagnosis of cerebrospinal fluid shunt malfunction based on a careful clinical history, examination, and investigations such as computed tomography (CT) scanning and plain X-ray shunt series is not always straightforward 1).

For example, ventricular size may not change in cases with a blocked shunt. Pumping a shunt prechamber is notoriously unreliable and potentially dangerous 2).

Admission for observation is expensive and excessive CT scanning carries a radiation burden. Many patients may be admitted and subjected to CT scanning on multiple occasions. There is a need to develop more reliable methods of assessing shunt function and monitoring intracranial pressure (ICP) 3) 4) 5) 6) 7) 8).

Optic nerve sheath diameter may be assessed using ultrasound or magnetic resonance imaging (MRI). Implantable ICP sensors within a shunt system have been blighted by poor long-term stability. Long-term studies of the recently introduced Raumedic NEUROVENT-P-tel and the Miethke SENSOR RESERVOIR are awaited with a keen interest 9).


Several attempts have been made to measure the cerebrospinal fluid flow velocity utilizing different Phase contrast magnetic resonance imaging techniques. In a study, König et al. evaluated 3T (Tesla) MRI scanners for their effectiveness in determining of flow in the parenchymal portion of ventricular shunt systems with adjustable valves containing magnets.

At first, an MRI phantom was used to measure the phase-contrasts at different constant low flow rates. The next step was to measure the CSF flow in patients treated with ventricular shunts without suspected malfunction of the shunt under observation.

The measurements of the phantom showed a linear correlation between the CSF flow and corresponding phase values. Despite many artifacts resulting from the magnetic valves, the ventricular catheter within the parenchymal portion of shunt was not superimposed by artifacts at each PC MRI plane and clearly distinguishable in 9 of 12 patients. Three patients suffering from obstructive hydrocephalus showed a clear flow signal.

Cerebrospinal fluid flow detected within the parenchymal portion of the shunt by phase contrast magnetic resonance imaging may reliably provide information about the functional status of a ventricular shunt. Even in patients whose hydrocephalus was treated with magnetic adjustable valves, the CSF flow was detectable using PC MRI sequences at 3 T field strength 10).


Non-invasive techniques to assess ‘semi-quantitatively’ whether intracranial pressure is raised or not include optic nerve sheath diameter (ultrasound or MRI), tympanic membrane displacement and transcranial Doppler but none have yet been shown to be sufficiently accurate for routine clinical use in patients with potential shunt malfunction. Provision of a separate subcutaneous CSF reservoir is of proven benefit in allowing access to the cerebral ventricles to measure ICP and allow removal of CSF in an emergency


Various invasive diagnostic test procedures for the verification of shunt function have been described:

Invasive CSF pressure and flow measurements

CSF tap test and drip interval test

Infusion tests

By comparison, publications addressing the noninvasive pumping test are rare.

Noninvasive pumping test of all formerly published results are values derived from tests with a variety of reservoirs and valves (at least 2 types).

In a few reports, the shunt/reservoir type used is not even specified, although the technical parameters of such reservoirs and valves are obviously essential.

To judge occlusions distally from the reservoir other authors have had to close the pVC transcutaneously by manual compression.

This is never possible with a sufficient certainty and, if ever undertaken, it usually does provide a source of error.


Rapid cranial MRI was not inferior to CT for diagnosing ventricular shunt malfunction and offers the advantage of sparing a child ionizing radiation exposure 11).

Radionuclide shuntography


1)
Spirig JM, Frank MN, Regli L, Stieglitz LH (2017) Shunt agerelated complications in adult patients with suspected shunt dysfunction . A recommended diagnostic workup. 1421–1428
2)
Bromby A, Czosnyka Z, Allin D, Richards HK, Pickard JD, Czosnyka M (2007) Laboratory study on “intracranial hypotension” created by pumping the chamber of a hydrocephalus shunt. Cerebrospinal Fluid Res 9:1–9
3)
Dupepe EB, Hopson B, Johnston JM, Rozzelle CJ, Oakes WJ, Blount JP, Rocque BG (2016) Rate of shunt revision as a function of age in patients with shunted hydrocephalus due to myelomeningocele. Neurosurg Focus 41(November):1–6
4)
Korinek AM, Fulla-Oller L, Boch AL, Golmard JL, Hadiji B, Puybasset L (2011) Morbidity of ventricular cerebrospinal fluid shunt surgery in adults: an 8-year study. Neurosurgery 68(4):985–994
5)
Paulsen AH, Lundar T, Lindegaard KF (2015) Pediatric hydrocephalus: 40-year outcomes in 128 hydrocephalic patients treated with shunts during childhood. Assessment of surgical outcome, work participation, and health-related quality of life. J NeurosurgeryPediatrics 16(6):633–641
6)
Richards H, Seeley H, Pickard J (2009) Who should perform shunt surgery? Data from the UK Shunt Registry. Cerebrospinal Fluid Res 6:S31
7)
Spiegelman L, Asija R, Da Silva SL, Krieger MD, McComb JG (2014) What is the risk of infecting a cerebrospinal fluid–diverting shunt with percutaneous tapping? J Neurosurg Pediatr 14(4):336– 339
8)
Tamber MS, Klimo P, Mazzola CA, Flannery AM (2014) Pediatric hydrocephalus: systematic literature review and evidence-based guidelines. Part 8: management of cerebrospinal fluid shunt infection. J Neurosurg Pediatr 14(Suppl1):60–71
9)
Antes S, Stadie A, Müller S, Linsler S, Breuskin D, Oertel J (2018) Intracranial Pressure–Guided Shunt Valve Adjustments with the Miethke Sensor Reservoir. World Neurosur 642–650
10)
König RE, Stucht D, Baecke S, Rashidi A, Speck O, Sandalcioglu IE, Luchtmann M. Phase-Contrast MRI Detection of Ventricular Shunt CSF Flow: Proof of Principle. J Neuroimaging. 2020 Nov 4. doi: 10.1111/jon.12794. Epub ahead of print. PMID: 33146931.
11)
Boyle TP, Paldino MJ, Kimia AA, Fitz BM, Madsen JR, Monuteaux MC, Nigrovic LE. Comparison of rapid cranial MRI to CT for ventricular shunt malfunction. Pediatrics. 2014 Jul;134(1):e47-54. doi: 10.1542/peds.2013-3739. Epub 2014 Jun 2. PubMed PMID: 24918222.
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