Interdisciplinary Neurosurgery 14 (2018) 183–185

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Interdisciplinary Neurosurgery
journal homepage: www.elsevier.com/locate/inat

Case Reports & Case Series

Cervical compressive myelopathy caused by malfunction of a programmable T

cerebrospinal fluid shunt valve
Kota Satoa, Toru Yamashitaa, Keichiro Tsunodaa, Mami Takemotoa, Nozomi Hishikawaa,
⁎
Jinwei Shanga, Yasuyuki Ohtaa, Ken Kuwaharab, Takao Yasuharab, Isao Dateb, Koji Abea,
a
Department of Neurology, Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama University, 2-5-1 Shikatacho, Kitaku, Okayama 700-8558,
Japan
b
Department of Neurosurgery, Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama University, 2-5-1 Shikatacho, Kitaku, Okayama 700-8558,
Japan

A R T I C LE I N FO A B S T R A C T

Keywords: The primary treatment for hydrocephalus is ventricular shunt placement, and a programmable valve is widely
Hydrocephalus used for ventriculoperitoneal (VP) shunt surgery to reduce over/under drainage of cerebrospinal fluid (CSF).
Ventriculoperitoneal shunt Here, we report a rare case of a patient who developed successive VP shunt malfunction causing spastic muscle
Cervical myelopathy weakness in extremities associated cervical epidural venous distension and compressive myelopathy due to over-
drainage of CSF through a defective VP shunt valve a decade after the initial shunt was placed. One should be
aware and cognizant of this complication and carefully follow the symptoms and potentially utilize brain MRI
with and without contrast to look at over drainage stigmata to avoid the development of neurological compli-
cations.

1. Introduction spontaneously changed from 180 to 160 mm H2O, however, he was

The primary treatment for hydrocephalus is ventricular shunt pla-
cement, and programmable valves are widely used in ventriculoper-
itoneal (VP) shunt surgery to reduce overdrainage and underdrainage
of cerebrospinal fluid (CSF). We herein report a rare case of over-
drainage causing, intracranial hypotension, cervical epidural venous
distension, and cervical compressive myelopathy caused by a defective
programmable valve.
2. Case report
A 28-year-old man with a dull headache visited a local neuro-
surgeon in March 1998 and was diagnosed with idiopathic hydro-
cephalus. In November 1998, a VP shunt operation was performed with
a Codman® Hakim® Programmable Valve system (DePuy Synthes,
Raynham, MA, USA) (valve pressure, 160 mm H2O). The valve pressure
increased to 180 mm H2O 1 month after surgery because of a persistent
dull headache. However, 4 months after surgery, the valve pressure
doing fine.
When the patient turned 30 years of age, he developed intolerable
abdominal pain, and abdominal X-rays revealed twisting of the in-
traperitoneal tube and reversal of the tip end position toward the head.
He underwent abdominal surgery for revision of the distal in-
traperitoneal shunt tubing replacement, and his abdominal pain dis-
appeared. At the age of 34 years, the valve pressure spontaneously
decreased again from 160 to 130 mm H2O (Fig. 1A and B), however,
given that he was doing well no shunt setting changes was pursued. At
the age of 42 years, the patient newly developed bilateral tinnitus, bi-
lateral hand numbness, and weakness in the left leg. At another local
hospital, he underwent brain computed tomography (CT) that showed
slit ventricles (Fig. 1D) compared with the ventricles 2 years before
(Fig. 1C), and without a change in valve pressure. However, the ven-
tricles spontaneously enlarged and recovered again within 1 month
(Fig. 1E). Therefore, he visited our hospital at the age of 46 years.
Upon admission to our hospital, the patient presented slight prox-
imal muscle weakness in the upper and lower extremities (manual

Abbreviations: CSF, cerebrospinal fluid; DTPA, diethylenetriaminepentaacetic acid; EMG, electromyography; FAB, frontal assessment battery; Gd, gadolinium; hr,
hour; M, month; MMSE, mini mental state examination; MMT, manual muscle test; T1WI, T1 weighted imaging; T2WI, T2 weighted imaging; VP, ventriculoper-
itoneal; Y, year
⁎
Corresponding author at: Department of Neurology, Okayama University Graduate School of Medicine, Dentistry and Pharmacy, 2-5-1 Shikata-cho, Okayama
700-8558, Japan.
E-mail address: kosatou@cc.okayama-u.ac.jp (K. Abe).

https://doi.org/10.1016/j.inat.2018.08.009
Received 13 August 2017; Received in revised form 27 August 2018; Accepted 27 August 2018
2214-7519/ © 2018 The Authors. Published by Elsevier B.V. This is an open access article under the CC BY-NC-ND license
(http://creativecommons.org/licenses/BY-NC-ND/4.0/).
K. Sato et al. Interdisciplinary Neurosurgery 14 (2018) 183–185

Fig. 1. Head X-ray showing a spontaneous decrease in valve pressure of the VP shunt valve from (A) 160 mm H2O to (B) 130 mm H2O 6 years after VP shunt surgery.
Brain CT also shows spontaneous changes in the size of the lateral ventricles at (C) 12 years, (D) 14 years, (E) 14 years 1 month, and (F) 17 years after surgery.
Radioisotopic scintigraphy with 111In-DTPA shows (G) accumulation in the abdominal cavity at 6 h (arrows). (H) Brain MRI with gadolinium-enhanced T1WI shows
transverse sinus enlargement (arrow). Cervical MRI with T2WI (I and J, axial; K, sagittal) shows bilateral epidural venous distention (arrows). Iodine-enhanced CT of
the (L) thoracic and (M) lumbar spinal cords shows bilateral epidural venous distention (arrows). (N, O) Cervical MRI 3 months after VP shunt replacement with
T2WI shows improved bilateral epidural venous distention (arrows).

muscle test: R4+/L4). Muscle tonus of the left extremities was spastic
with hyperreflexia. He had decreased superficial sensations in the left
body and extremities. Laboratory data showed no abnormalities, and
CSF analysis revealed a low opening pressure (70 mm H2O) and an
increase in protein (167 mg/dl) with a normal cell count (3/μl).
Peripheral nerve conduction was normal. Muscle electromyography in
the upper extremities revealed chronic neurogenic change. Brain CT
showed slightly enlarged lateral and third ventricles (Fig. 1F). Radio-
isotopic scintigraphy revealed accumulation of 111In-diethylene-
triaminepentaacetic acid (DTPA) in the abdominal cavity at 6 h
(Fig. 1G, arrows) demonstrating functioning VP shunt.
At that time, the valve pressure was 120 mm H2O. Gadolinium-en-
hanced magnetic resonance imaging (MRI) of the brain showed en-
largement of the transverse sinus without thrombosis (Fig. 1H, arrow)
but showed no pachymeningeal enhancement. Cervical MRI showed
epidural venous distention at the C1 to C5 levels (Fig. 1I–K, arrows),
compressing the cervical spinal cord from both anterior sides (Fig. 1I
and J, arrows). Slight epidural venous distention was also observed in
the thoracic and lumbar cords on iodine-enhanced CT (Fig. 1L and M,
arrows). The enlargement of both the transverse sinus and epidural
veins indicated intracranial hypotension; therefore, the patient was fi-
nally diagnosed with intracranial hypotension due to overdrainage of
CSF due to a defective valve that could not have been reset anymore
and was fixed at 120 mm H2O. Additionally, the spastic weakness in his
extremities was diagnosed as compressive cervical myelopathy due to
epidural venous distention from the bilateral anterior side. We replaced
the VP shunt tube and set the valve pressure at 160 mm H2O, but gross
examination of the removed shunt tube showed no abnormalities. The
patient's neurological symptoms did not improve significantly at the
3 months follow up, but the cervical epidural venous distension im-
proved and the intrathecal space was enlarged 3 months after the op-
eration (Fig. 1N and O).
3. Discussion
VP shunt complications are not rare and are usually caused by
mechanical failures such as spontaneous changes in the pressure set-
ting, shunt obstruction, disconnection, migration, and leakage [1]. In

184
K. Sato et al.
the present case, the alterations to the spontaneous shunt valve (Fig. 1A
and B) and size of the ventricles (Fig. 1C–F) may have indicated the
presence of such valve malfunctions. In particular, the slit ventricle
indicated excessive drainage of CSF (Fig. 1D), which may lead to in-
tracranial hypotension.
V-P shunt trouble is not rare, caused by mechanical failures such as
spontaneous pressure-setting change, shunt obstruction, disconnection,
migration, and leakage [1]. The valve pressure setting may be affected
by mechanical shock or magnetic impulses to the valve device [2].
There are many magnetic impulses in daily life affecting on the valve
setting such as television, cell phone, and toy magnets [2–5]. In a
previous study, more than 40% of the patients required valve pressure
adjustment one to several times during 5 years follow up, and two
thirds of such patients improved their clinical symptoms after the
pressure adjustments [6].
Apart from a magnetic field, intrinsic valve malfunction or blockage
caused spontaneous valve pressure change in 2.1–11.1% of program-
mable V-P shunt cases [6,7]. In the present case, spontaneous shunt
valve alterations were observed at least twice (4 months and 6 years
after the surgery) probably due to spontaneous valve malfunctions.
Spontaneous size changes of ventricles 14 years after the surgery (Fig. 1
C–F) may indicate such valve malfunctions. These valve malfunctions
could make over drainage of CSF to abdominal lumen, and lead in-
tracranial hypotension. In order to find such a shunt trouble, radio-
graphy with contrast agents, CT, and MRI brain with and without
contrast are useful.
Although intracranial hypotension usually presents as an orthostatic
headache [8], our patient did not develop typical orthostatic hypo-
tension. On MRI, intracranial hypotension is usually shown by diffuse
pachymeningeal enhancement [9], transverse sinus enlargement [8],
and spinal epidural venous distention [10], although our patient pre-
sented only the latter two findings (Fig. 1H–M). Replacement of the
shunt tube did not significantly improve his symptoms, and the spinal
cord may have sustained irreversible degeneration.
4. Conclusion
In summary, we have herein reported a rare case of successive VP
shunt difficulty, intracranial hypotension, cervical epidural venous
distension, and cervical compressive myelopathy 14 years after surgery.
185
Interdisciplinary Neurosurgery 14 (2018) 183–185
Cervical myelopathy due to spinal epidural venous distention is an
uncommon but important complication after VP shunt placement
[8,11–13]. This case shows that even after decades of shunting this
complication might occur and it should not be overlooked.
Conflict of interest
The authors state that they have no conflicts of interest. They have
no financial disclosure to make.
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