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Arch Gynecol Obstet

DOI 10.1007/s00404-013-2858-0

LETTER TO THE EDITOR

Management of acute hydrocephalus due to pregnancy


with ventriculoperitoneal shunt
Xiaoliang Wang Handong Wang Youwu Fan
Zhigang Hu Qun Guan Qin Zhang
Tao Li Chunxi Wang

Received: 17 September 2012 / Accepted: 16 April 2013


Springer-Verlag Berlin Heidelberg 2013

Introduction
The ventriculoperitoneal (VP) shunt is an effective and
mainstay of treatment for hydrocephalus. Although VP
shunt reduces the morbidity and mortality of hydrocephalus considerably, it often malfunctions due to complications such as obstruction, breakage, migration and infection
[1, 2]. In pregnant women, shunt malfunction occurs due to
increasing abdominal pressure associated with enlargement
of the uterus and other organs [3]. We present a case in
which the patient presented an acute VP shunt malfunction
in the third trimester of pregnancy, and the external
drainage of cerebrospinal fluid (CSF) management relieved
her symptoms, so that she was able to successfully deliver a
healthy infant vaginally.

Case report
An 18-year-old female, who was at 30 weeks of gestation,
was admitted to our emergency room due to acute disturbance of consciousness. A computed tomography (CT)
scan showed dilation of the ventricles (Fig. 1d).
Previously, about 2 years ago, the patient had a progressive visual impairment. Magnetic resonance images

X. Wang  H. Wang (&)  Y. Fan  Z. Hu  T. Li  C. Wang


Department of Neurosurgery, Jinling Hospital, School
of Medicine, Nanjing University, Nanjing, Jiangsu Province,
Peoples Republic of China
e-mail: hdwang_nj@yahoo.com.cn
Q. Guan  Q. Zhang
Department of Obstetrics and Gynecology, Jinling Hospital,
School of Medicine, Nanjing University, Nanjing, Jiangsu
Province, Peoples Republic of China

(MRIs) of the head indicated obstructive hydrocephalus


due to the rupture of an epidermoid cyst in the fourth
ventricle (Fig. 1b). Since the content of the epidermoid
cyst had flowed to all ventricles and total resection of the
cyst was impossible (Fig. 1a), a ventriculoperitoneal (VP)
shunt was placed in the right lateral ventricle. Her vision
returned back to normal with rare headaches. A CT scan
after the procedure confirmed the relief of hydrocephalus
(Fig. 1c).
In the emergency room, the patient was not able to open
her eyes spontaneously and showed flexion to pain with no
verbal response (Glasgow Coma Scale, GCS 5 E1V1M3).
A quick but systematic evaluation was made by both
neurosurgeons and obstetricians. The shunt catheter was in
the right lateral ventricle. The pressure of the shunt valve
was high, but the flexibility was good. The heart rate of the
fetus was around 140 beats per minute. The reason for the
VP shunt malfunction was due to increased intra-abdominal
pressure because of the pregnancy. An external drainage in
the left ventricles was done immediately under general
anesthesia.
After the procedure, approximately 60 ml of clear CSF
was drained per day and the CSF analyses were normal.
The patients neurological condition was gradually
improved (GCS8 E1V1M6) with a better flowing ventricle
system that was confirmed by the CT scan (Fig. 1d). Two
days after the external drainage, premature rupture of the
membrane was detected and vaginal delivery was performed successfully without epidural analgesia but under
close surveillance by neurosurgeons and obstetricians. The
infant was healthy with an Apgar score of ten. The external
drainage catheter was removed 2 days later on the condition that VP shunt reverted functioning, confirmed by
another CT scan (Fig. 1f). The patient was discharged
18 days after surgery.

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Arch Gynecol Obstet

Fig. 1 a Axial T1WI magnetic resonance image (MRI) showing the


content of an epidermoid cyst (fat like material) in the lateral
ventricles and the dilation of the lateral ventricles; b Sagittal T1WI
contrast MRI showing the epidermoid cyst located at the fourth
ventricle (arrow); c A computed tomography (CT) scan obtained after
the VP shunting demonstrating the normal size of the ventricle
system; d When the patient had symptoms of acute hydrocephalus,

A CT scan confirmed the enlarged ventricles but the VP shunt


catheter (black arrow) was in the right place. e A CT scan obtained
after the external cerebrospinal fluid (CSF) drainage procedure
showing improved yet not normal lateral ventricles (white arrow:
external CSF drainage catheter, black arrow: VP shunt catheter);
f The ventricular cavity appeared normal when the patient was
discharged

Discussion

disorder (12 %), and severe headaches without increased


intracranial pressure (6 %). However, lower rates of shunt
malfunction and revision were found in pregnant women
with existing VP shunts. Liakos et al. [7] reported that shunt
malfunction and revisions occurred in 18 patients before
delivery out of 138 shunt-dependent pregnancies. Increased
intra-abdominal pressure due to the gravid uterus is considered the principal reason for VP shunt malfunction [8].
Furthermore, acute shunt malfunction was also found in a
patient after Cesarean section delivery.
The standard procedure for shunt malfunction during
pregnancy has not been established yet. However, several
options have been proved effective, such as by increasing
extra CSF drainage. Sasagawa et al. [9] presented a case in
which a 27-year-old female, at 33 weeks of gestation, suffered gait disturbance due to shunt malfunction. A flushing
device of the shunt system was performed and continuously
worked for 3 months. The patients symptoms gradually
improved, and she delivered a healthy infant by vaginal

The VP shunt is an effective method to provide an improved


neurological outcome for patients with hydrocephalus.
However, the shunt devices have a high propensity to
malfunction. A recent study shows that the overall failure of
VP shunts in adults was 32 % and most of the shunt revisions occurred within the first 6 months. Shunt infection,
obstruction, overdrainage and mechanical complications
are the major contributors for shunt revisions [4]. Thus,
malfunction of shunts in pregnant women remains a challenging medical condition for neurosurgeons and obstetricians. Patients need to be observed regularly to prevent
shunt malfunction. The symptoms of hydrocephalus in
patients with shunt malfunction include headache, nausea,
and vomiting [5]. Wisoff et al. [6] reported that neurological
complications occurred in 76 % of pregnancies in patients
with pre-existing shunts, including symptoms of increased
intracranial pressure (59 %), exacerbation of seizure

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Arch Gynecol Obstet

labor. In another case, Murakami et al. [10] recommended


ventriculoatrial shunt replacement as a treatment option for
the management of VP shunt malfunction during pregnancy. Schiza et al. [11] presented another case in which the
patient received a scheduled Cesarean section delivery at
the 30th week of gestation under epidural anesthesia and her
neurological condition started improving 48 h after delivery
and the symptoms gradually regressed. Riffaud et al. [12]
suggested endoscopic third ventriculostomy as an effective
treatment option for pregnant patients with newly diagnosed obstructive hydrocephalus as well as in cases of
malfunction of a pre-existing shunt. In our case, the patient
encountered acute disturbance of consciousness, and
external CSF drainage was proved to be an effective and
proper treatment. Thus, we suggest that all procedures
should be consulted by neurosurgeons and obstetricians to
achieve better outcomes.
To the best of our knowledge, this is the first reported case
in which the patient encountered acute hydrocephalus due to
pregnancy with pre-existing VP shunt and had spontaneous
vaginal delivery without epidural analgesia while remained
unconscious. Nikolov et al. [13] studied 12 pregnant cases
with pre-existing shunts (nine whit VP shunts and three VA
shunts) and concluded that the presence of a cerebral shunt
does not affect the pregnancy. Vaginal delivery in women
with CSF shunts can be provided successfully with analgesic
medicines that do not increase the intracranial pressure. The
Cesarean section should be performed by obstetrical indications and in cases with increased intracranial pressure. A
larger series of reviews conducted by Bradley et al. [14]
included 34 vaginal deliveries out of 56 patients with preexisting shunts, and a shortened second stage of labor was
suggested to prevent longer periods of increased intracranial
pressure. A Cesarean section may cause shunt malfunction
by increasing the adhesion formation rate around the shunt
tip and opens the possibility to shunt infections. Hwang et al.
[15] reported a case of acute shunt malfunction after a
Cesarean section due to an obstructive blood clot at the shunt
tip. Thus, vaginal delivery should be the first choice in
patients with VP shunts. It should be attempted unless
Cesarean section delivery is inevitably required for obstetrical or neurosurgical reasons [16].
The anesthesia procedure varied from several reports on
deliveries with VP shunt. In a recent study consisting of 77
pregnancies in 37 women, epidural analgesia was used in
38 % of vaginal deliveries and 50 % of Cesarean sections,
while spinal anesthesia was used in 9 % of Cesarean sections and 41 % were performed under general anesthesia
[14]. General anesthesia is preferred to spinal anesthesia by
some anesthesiologists because general anesthesia is relatively safe and could decrease cranial pressure using proper
agents, and without the possibility of CSF infection that
arises with spinal anesthesia [17]. Epidural analgesia was

usually used to manage the rise in cranial pressure during


painful contractions [18]. In our case, the patient was
unconscious during the vaginal delivery and neurological
status were closely monitored by neurosurgeons. Cesarean
section preparation was fully done in case the patient
needed a surgical approach. In a similar case presented by
Littleford et al. [17] a 37-year-old woman, whose VP shunt
failed to function due to pregnancy, successfully gave birth
to a healthy infant by vaginal labor without epidural
analgesia while inhalation and intravenous analgesia
methods were available during the delivery process.
The complications during pregnancy in women with VP
shunt vary with the weeks of gestation [15]. The management must be individualized and multi disciplined according to the neurological condition and gestational age by
neurosurgeons, obstetricians and anesthesiologists. We
suggest a general outline for management as follows: (1)
Women with VP shunts need to be fully evaluated before
pregnancy by both neurosurgeons and obstetricians, and
MRI images should be regularly obtained to assess the
ventricle system. (2) For patients in the second trimester,
shunt revision or VA shunt should be accomplished first and
delivery at full term could be expected. (3) For patients in
the late third trimester, vaginal delivery or Cesarean section
delivery could be achieved with proper interventions.

Conclusion
The management of acute hydrocephalus during pregnancy
with a VP shunt remains a challenging topic for neurosurgeons, obstetricians and anesthesiologists. Each case of
VP shunt malfunction should be individually evaluated,
and applied with proper interventions to achieve better
maternal and infant outcomes. In our case, external CSF
drainage improved the patients neurological outcome
(GCS13 E4V3M6) and spontaneous vaginal delivery was
successfully achieved while the patient remained unconscious. Therefore, these interventions could be an option
for similar case scenarios in the future.
Acknowledgments We would like to thank Juan Pablo de Rivero
Vaccari Ph.D. for critical review of the manuscript.
Conflict of interest The authors certify that no actual or potential
conflict of interest in relation to this article exists.

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