Ventriculoperitoneal Shunt Complications in Children An Evidence-Based Approach To Emergency Department Management PDF

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Ventriculoperitoneal Shunt Complications February 2016

Volume 13, Number 2
In Children: An Evidence-Based Approach Authors
To Emergency Department Management Jacqueline Bober, DO, FAAP

Pediatric Emergency Medicine Fellow, Pediatric Emergency
Medicine, Kings County Hospital Center and SUNY Downstate
Medical Center, Brooklyn, NY
Abstract Jonathan Rochlin, MD
Clinical Assistant Professor, Division of Pediatric Emergency
Medicine, Department of Emergency Medicine, Kings County
Although much is known about ventriculoperitoneal shunts, there Hospital Center and SUNY Downstate Medical Center, Brooklyn,
are still large gaps in the literature and no evidence-based guidelines NY
on management. To date, there is no general consensus on workup Shashidhar Marneni, MD
Clinical Assistant Professor, Division of Pediatric Emergency
and treatment, and there are many differing diagnostic and therapeu- Medicine, Department of Emergency Medicine, Kings County
tic strategies for management of complications. Ventriculoperitoneal Hospital Center and SUNY Downstate Medical Center, Brooklyn,
shunt complications can be separated into 3 categories: mechanical NY
failure, infection, and functional failure. Knowing the basic anatomy Peer Reviewers
of ventriculoperitoneal shunts, the time of shunt placement, and the Joel M. Clingenpeel, MD, MPH, FAAP, FAAEM
clinical manifestations suggestive of potential complications can help Associate Professor, Pediatrics; Fellowship Director, Pediatric
Emergency Medicine, Eastern Virginia Medical School, Norfolk, VA
with the management of patients with ventriculoperitoneal shunts. Tommy Y. Kim, MD, FAAP, FACEP
This review summarizes the literature on complications of ventricu- Associate Professor, Loma Linda University Medical Center and
Children's Hospital, Department of Emergency Medicine, Division
loperitoneal shunts, examines the literature regarding the workup of Pediatric Emergency Medicine, Loma Linda, CA; Riverside
and management of patients with ventriculoperitoneal shunts, and Community Hospital, CEP, Riverside, CA
makes recommendations for the management of these patients in the
emergency department. Prior to beginning this activity, see “Physician CME Information”
on the back page.
Editor-in-Chief Ilene Claudius, MD Alson S. Inaba, MD, FAAP Robert Luten, MD AAP Sponsor
Associate Professor of Emergency Associate Professor of Pediatrics, Professor, Pediatrics and
Adam E. Vella, MD, FAAP Medicine, Keck School of Medicine University of Hawaii at Mãnoa Emergency Medicine, University of Martin I. Herman, MD, FAAP, FACEP
Associate Professor of Emergency of USC, Los Angeles, CA John A. Burns School of Medicine, Florida, Jacksonville, FL Sacred Heart Children's Hospital,
Medicine, Pediatrics, and Medical Division Head of Pediatric Pensacola, FL; Florida State
Education, Director Of Pediatric Ari Cohen, MD Garth Meckler, MD, MSHS University School of Medicine,
Emergency Medicine, Kapiolani
Emergency Medicine, Icahn School Chief of Pediatric Emergency Medicine Associate Professor of Pediatrics, Pediatric Residency Department,
Medical Center for Women and
of Medicine at Mount Sinai, New Services, Massachusetts General University of British Columbia; Tallahassee, FL
Children, Honolulu, HI
York, NY Hospital; Instructor in Pediatrics, Division Head, Pediatric Emergency
Harvard Medical School, Boston, MA Madeline Matar Joseph, MD, FAAP, Medicine, BC Children's Hospital, International Editor
Associate Editor-in-Chief Marianne Gausche-Hill, MD, FACEP, FACEP Vancouver, BC, Canada Lara Zibners, MD, FAAP, FACEP
Professor of Emergency Medicine
Vincent J. Wang, MD, MHA FAAP Joshua Nagler, MD Honorary Consultant, Paediatric
Associate Professor of Pediatrics, and Pediatrics, Chief and Medical Emergency Medicine St. Mary's
Medical Director, Los Angeles Assistant Professor of Pediatrics,
Keck School of Medicine of USC; Director, Pediatric Emergency Hospital Imperial College Trust,
County EMS Agency; Professor of Harvard Medical School; Fellowship
Associate Division Head, Division Medicine Division, University of London, UK; Nonclinical Instructor
Clinical Medicine and Pediatrics, Director, Division of Emergency
of Emergency Medicine, Children's Florida Medical School-Jacksonville, Emergency Medicine Icahn school
David Geffen School of Medicine at Medicine, Boston Children’s
Hospital Los Angeles, Los Angeles, Jacksonville, FL of medicine at Mount Sinai, New
UCLA, Los Angeles, CA Hospital, Boston, MA
CA Stephanie Kennebeck, MD York, NY
Michael J. Gerardi, MD, FAAP, James Naprawa, MD
Associate Professor, University of
Editorial Board FACEP, President
Cincinnati Department of Pediatrics,
Attending Physician, Emergency Pharmacology Editor
Associate Professor of Emergency Department USCF Benioff
Jeffrey R. Avner, MD, FAAP Cincinnati, OH James Damilini, PharmD, MS, BCPS
Medicine, Icahn School of Medicine Children's Hospital, Oakland, CA
Professor of Clinical Pediatrics at Mount Sinai; Director, Pediatric Anupam Kharbanda, MD, MS Clinical Pharmacy Specialist,
and Chief of Pediatric Emergency Joshua Rocker, MD Emergency Medicine, St. Joseph's
Emergency Medicine, Goryeb Chief, Critical Care Services
Medicine, Albert Einstein College Assistant Professor of Emergency Hospital and Medical Center,
Children's Hospital, Morristown Children's Hospitals and Clinics of
of Medicine, Children’s Hospital at Medicine and Pediatric, Hofstra Phoenix, AZ
Medical Center, Morristown, NJ Minnesota, Minneapolis, MN
Montefiore, Bronx, NY North Shore-LIJ School of Medicine,
Sandip Godambe, MD, PhD Tommy Y. Kim, MD, FAAP, FACEP Hempstead, NY; Associate Director, Quality Editor
Steven Bin, MD Vice President, Quality & Patient Associate Professor, Loma Linda Division of Pediatric Emergency
Associate Clinical Professor Steven Choi, MD
Safety, Professor of Pediatrics and University Medical Center and Medicine, Cohen Children's Medical Medical Director of Quality, The
of Emergency Medicine and Emergency Medicine, Attending Children's Hospital, Department of Center, New Hyde Park, NY
Pediatrics, UCSF School of Children's Hospital at Montefiore;
Physician, Children's Hospital of the Emergency Medicine, Division of Associate Vice President, Montefiore
Medicine; Medical Director, Division Steven Rogers, MD
King's Daughters Health System, Pediatric Emergency Medicine, Loma Network Performance Improvement;
of Pediatric Emergency Medicine, Associate Professor, University of
Norfolk, VA Linda, CA; Riverside Community Assistant Professor of Pediatrics,
UCSF Benioff Children's Hospital, Connecticut School of Medicine,
Hospital, CEP, Riverside, CA Albert Einstein College of Medicine,
San Francisco, CA Ran D. Goldman, MD Attending Emergency Medicine
Professor, Department of Pediatrics, Melissa Langhan, MD, MHS Physician, Connecticut Children's Bronx, NY
Richard M. Cantor, MD, FAAP, University of British Columbia; Associate Professor of Pediatrics, Medical Center, Hartford, CT
FACEP Co-Lead, Division of Translational Fellowship Director, Director of CME Editor
Professor of Emergency Medicine Christopher Strother, MD
Therapeutics; Research Director, Education, Pediatric Emergency Deborah R. Liu, MD
and Pediatrics, Director, Pediatric Assistant Professor, Emergency
Pediatric Emergency Medicine, BC Medicine, Yale School of Medicine, Assistant Professor of Pediatrics,
Emergency Department, Medical Medicine, Pediatrics, and Medical
Children's Hospital, Vancouver, BC, New Haven, CT Keck School of Medicine of USC;
Director, Central New York Poison Education; Director, Undergraduate
Canada Division of Emergency Medicine,
Control Center, Golisano Children's and Emergency Department
Simulation; Icahn School of Medicine Children's Hospital Los Angeles,
Hospital, Syracuse, NY
at Mount Sinai, New York, NY Los Angeles, CA
Case Presentations Introduction
A 7-year-old girl with a history of a VP shunt presents with Ventriculoperitoneal (VP) shunts are the treatment of
a headache for 2 days and worsening fever. The patient’s choice for patients with hydrocephalus, an excessive
mother states that the child had a shunt placed during her accumulation of cerebrospinal fluid (CSF) within the
first year of life for congenital aqueductal stenosis. The pa- brain caused by an imbalance between CSF produc-
tient is febrile in the ED, with a temperature of 38.6°C, but is tion, flow, or absorption. Most commonly, pressure
nontoxic. Her other vital signs are: heart rate, 118 beats/min; builds up proximal to an obstruction, leading to
respiratory rate, 20 breaths/min; blood pressure 98/62 mm ventricular dilatation and raised intracranial pres-
Hg; and oxygen saturation, 100% on room air. Her physical sure (ICP).1 (See Figure 1). The pathophysiology of
examination is unremarkable except for a mildly erythema- hydrocephalus has been known since the 1800s, but
tous throat. What are important points on your history that effective treatment was not available until the 1950s,
should be elicited for a patient with a VP shunt and fever? If when John Holter developed a shunt to allow drain-
you are concerned about a VP shunt infection, what labora- age of excess CSF, in an attempt to treat his own
tory studies should be ordered? Do you need to order any son, who was suffering from hydrocephalus.2 Since
imaging studies to look for a possible shunt malfunction? then, the standard treatment of hydrocephalus has
Should you call this child’s neurosurgeon? been the insertion of a ventricular shunt, and it has
A 3-year-old girl with a VP shunt, who is suffering dramatically reduced the morbidity and mortality of
from altered consciousness and persistent vomiting, is hydrocephalus.3,4
brought to your community ED via EMS. The patient’s A shunt consists of 4 major components: a
mother states the child has been drowsy for the past few proximal catheter, a 1-way valve, a reservoir, and a
weeks. Upon arrival, you place the girl on a monitor distal catheter.5 (See Figure 2, page 3.) Most neuro-
and a nonrebreather mask and obtain the following surgeons place shunts that contain medium-pressure
vital signs: heart rate, 76 beats/min; respiratory rate, valves and drain CSF continuously when the pres-
20 breaths/min; blood pressure, 110/65 mm Hg; and sure in the ventricles is > 10 mm Hg.6 The proximal
oxygen saturation, 100% on the nonrebreather. Upon catheter is normally inserted in the parieto-occipital
primary survey, the patient’s airway is intact, but there
are coarse breath sounds bilaterally over her chest. Her Figure 1. Hydrocephalus: Dilated Ventricles
capillary refill is < 2 seconds. Her GCS score is 9, and Seen On Head Computed Tomography
you note that one pupil seems to be more dilated than
the other and appears to be sluggishly reactive. The
patient is not responding to your commands. What
could be causing this patient’s symptoms? Without a
neurosurgeon in-house, what should your management
of this patient be? Does the patient need imaging prior
to any procedures?
A 5-year-old boy with a history of constipation, mild
developmental delay, and a VP shunt presents with 1
week of vomiting. His mother states he is chronically on
stool softeners. For the past week, the patient has had 2 to
3 episodes of nonbloody, nonbilious vomiting per day. He
has not had diarrhea, but his last stool, which was earlier
today, was watery. The mother states he has not been com-
plaining of headaches, but has been eating and drinking
less. His vital signs are stable upon arrival. The patient’s
examination is normal, including the neurologic examina-
tion, except for mild periumbilical tenderness. There is
no rebound or guarding upon abdominal palpation. You
realize that this could be a typical presentation of consti-
pation, but the patient’s VP shunt makes you consider
possible shunt complications. You begin to wonder if this
could possibly be a shunt obstruction or infection. What
steps should be taken in the management of this patient?
What history is important in this case? What physical
examination findings might help with the diagnosis?
Arrows point to dilated ventricles.

Image courtesy of John Amodio, MD, FACR, Kings County Hospital
Center, Brooklyn, NY
Copyright © 2016 EB Medicine. All rights reserved. 2 Reprints: www.ebmedicine.net/pempissues

region of the lateral ventricle, and the 1-way valve failure to be independent of valve type.1,7,8 There
and reservoir run behind the ipsilateral ear. The dis- are several risk factors for complications, includ-
tal portion of the catheter is tunneled down through ing: younger age, history of prematurity, number
the neck and chest wall and, most commonly, ends of revisions, and shorter time from insertion to first
in the peritoneal cavity.3,5 Other areas where the revision.4,9-11 Ethnicity also has been linked to shunt
catheter can terminate include the pleural cavity, complications. Specifically, blacks, Hispanics, and
atrium, and gallbladder; however, the peritoneal Native Americans have a higher rate of complica-
cavity is the preferred site of termination, as it is tions compared to Asians and whites.4,12,13 The num-
associated with the fewest complications.7 If placed ber of shunt placements is increasing each year. The
in infancy or childhood, the distal catheter has extra annual malfunction rate is projected to be as high as
length in the abdomen to allow for growth of the 5%.5,14 The mortality rate from a shunt malfunction
patient. Many newer valves allow for the opening can be as high as 1% to 2%.15
pressure to be adjusted externally via a magnetic Both the history and physical examination are
or electromagnetic device; these “programmable" very important when evaluating a patient with a
shunts obviate the need for surgery to change the possible shunt complication, and will guide the
pressure setting.1 emergency clinician in making management deci-
Unfortunately, VP shunts are not long-lasting, sions. This issue will help practitioners identify
and revision may be required 1 to 2 times every possible shunt complications, determine necessary
10 years.3 The Pediatric Shunt Design Trial was a diagnostic and management steps, and decide ap-
randomized controlled trial that compared differ- propriate dispositions.
ent types of shunts in patients with hydrocephalus.
According to this prospective study, approximately Critical Appraisal Of The Literature
one-third of shunts require revision in the first
postoperative year and > 50% fail by the second year A literature search was performed in PubMed using
after insertion.8 This study, as well as others, found the search terms ventriculoperitoneal shunt, VP shunt,
complication, pediatric, paediatric, infant, child, and ado-
Figure 2. Shunt Components Of lescent. For completeness, the terms ventriculoatrial
Ventriculoperitoneal And Ventriculoatrial shunt, VA shunt, and ventriculopleural shunt were
Shunts also searched. More than 1300 articles published
since 1970 were found, and over 250 articles were
Proximal catheter reviewed, based on the clinical relevance of their
abstracts. Supporting articles were gathered from
related articles and the reference lists of reviewed
articles. The search produced mostly case reports,
Reservoir
as well as retrospective and prospective studies
that were mostly chart reviews. There was a limited
1-way valve number of large studies, and very few randomized
controlled trials were found. Textbooks pertaining
to hydrocephalus and VP shunt management were
Distal
used for general information.
catheter
Ventriculoatrial shunt
Pathophysiology

In patients with hydrocephalus, there is a dispar-
ity between CSF production and absorption. CSF is
produced within the choroid plexus of the lateral,
third, and fourth ventricles of the brain. It then flows
through the ventricular system into the subarach-
noid space via the foramen of Luschka and Ma-
gendie of the fourth ventricle and is absorbed by the
venous system via the arachnoid villi and granula-
tions. Any impairment to CSF circulation can lead
to hydrocephalus, increased ICP, and dilation of the
ventricular system. Some causes of hydrocephalus
Ventriculoperitoneal shunt are acquired, such as intraventricular hemorrhage
and brain tumors; others are congenital, such as
http://www.hydro-kids.com/treatment/shunt stenosis of the aqueduct of Sylvius and myelomenin-
Image reprinted with permission of Codman Neuro. gocele.1,5 Hydrocephalus most often occurs within
February 2016 • www.ebmedicine.net 3 Mobile app access: www.ebmedicine.net/app

the first year of life;16 the 3 most common causes are holes or the formation of ventricular loculations.1,5,17
intraventricular hemorrhage (24%), myelomeningo- Proximal catheter obstruction is the most concern-
cele (21%), and brain tumors (9%).5 ing location, as these patients can decompensate
Shunt-related complications can be separated rapidly. Obstruction at the valve is the second most
into 3 categories: mechanical failure, infection, and common site, and obstruction at the distal portion of
functional failure (See Table 1), although these the catheter is the least common site.5 Catheters with
categories can overlap. Mechanical and functional side slits at the distal portion are associated with a
failures often present with symptoms of increased higher incidence of obstruction compared to those
ICP, such as headache, lethargy, vomiting, and be- with a simple distal open-ended tube.23 Although
havioral changes. Clinical clues that point to a shunt uncommon, chronic constipation can cause distal
infection include fever and drowsiness. However, catheter obstructions, especially in children with
these nonspecific symptoms are frequently seen cerebral palsy or bowel and bladder issues. Interest-
in many other conditions (such as viral illnesses); ingly, a single enema can sometimes help cure the
therefore, emergency clinicians must discern be- symptoms of increased ICP.6,24,25
tween a common viral illness and a shunt infection. Fractures, disconnections, migrations, and per-
Although some sources classify shunt complications forations are other causes of mechanical shunt fail-
differently and patients can present with more than ures. Fractures are defined as breaks in the shunt
1 type of complication (ie, an infection leading to an tubing, and are the second most common cause
obstruction), most experts classify shunt complica- of shunt failure in children.5,26 The most likely
tions in a manner similar to that in Table 1. location for a fracture is over the clavicle or lower
ribs.5 Typically, fractures do not present immedi-
Mechanical Failure ately after surgery, but are, instead, a later cause
In the United States, the leading cause of shunt com- of mechanical shunt failure.7,25 Disconnections are
plication is mechanical failure.17 Mechanical failure defined as detachments of the catheter tubing and
occurs due to obstruction, interruption (eg, a break occur mainly in multipiece shunts. Disconnections
or disconnection), or malpositioning of the shunt. typically present shortly after surgery, although
This leads to decreased fluid being shunted away they can present at any time. Factors predisposing
from the brain, causing increased CSF accumulation to disconnection at later stages include increasing
and increased ICP. VP shunt obstruction is the most age of the shunt, restricted mobility, or repeated
common type of mechanical complication.5,18 There trauma.5,17 Migration of the proximal or distal
are many causes of obstruction, including adhe- portion of the catheter may occur.27 For example,
sions, infection, constipation, and even a knot in the normal physical growth of the patient can lead to
catheter itself.3,19,20 An obstruction can develop at the proximal portion of the catheter being drawn
any time after the surgery for shunt placement, but it out from the ventricle and into the brain paren-
occurs more commonly in the first year after inser- chyma, or the distal portion of the catheter being
tion.7,21,22 Shunt obstruction may occur either proxi- drawn out of the distal cavity.5,28 There are some
mally or distally in the catheter that resides in the case reports on migration of the catheter into the
ventricle. The proximal portion is the most common chest cavity, leading to pleural effusion,29,30 and
site of obstruction, due to the growth of the cho- into the scrotum, patent processus vaginalis, lower
roid plexus or ependymal tissue into the catheter’s extremity, and heart.28,31-33 Furthermore, the shunt
may migrate into the abdominal wall or adjacent to
the liver and form local fluid collections or ab-
Table 1. Shunt Complications scesses.17,34 Surgical emergencies may occur due to
perforations from these migrations and have been
Type of Complication Cause of Complication
reported in the intestines, gallbladder, abdominal
Mechanical failure • Obstruction wall, bladder, vagina, breast, diaphragm, bronchial
• Fracture tree, lung, and anus. Perforations occur most com-
• Disconnection monly in the bowel and are reported in < 0.1% to
• Migration
0.7% of VP shunt complications.
• Perforation
Infection • Bacterial Infection
• Parasitic
The current incidence of shunt infection ranges from
• Fungal
5% to 12%.1,3,5 The majority of these are early infec-
Functional failure • Overdrainage tions; approximately 70% occur within the first 2
• Slit ventricle syndrome months after placement and up to 90% occur within
• Pseudocyst
the first 6 months.1,5,35,36
• Ascites
Early shunt infections are usually due to the
• Metastasis
patient’s own bacterial skin flora. Staphylococcus

epidermidis accounts for 50% of infections, while occur during the first 6 months after shunt inser-
Staphylococcus aureus accounts for 20%.37 These tion and are directly related to the amount of CSF
organisms can form biofilms, enabling them to stick drained during shunt placement.3 The overdrainage
to and colonize implanted devices.37 Other organ- of CSF can cause shrinkage of brain volume in the
isms that are found in early shunt infections include cranial vault, shearing the bridging veins, and result
Propionibacterium acnes and enteric bacteria such as in subdural hematomas.
Haemophilus influenzae and Enterococcus species.35
Delayed shunt infections are those that occur 6 Slit Ventricle Syndrome
months or more after surgery. These are uncommon Approximately 40% to 60% of pediatric patients
and often due to the spread of infection from other with CSF shunts develop small, slit-like ventricles
sites. For example, appendicitis or pseudocyst forma- that can be observed on imaging (see Figure 3), but
tion can lead to intra-abdominal pathogens migrating only 10% of these patients develop slit ventricle
up the distal catheter.3,38,39 The causative agent in syndrome (SVS).5 However, some sources state only
delayed shunt infections is often difficult to ascertain, 0.9% to 3.3% of children with shunts have SVS.48
but when an organism is identified, it is most com- This syndrome is likely due to chronic CSF over-
monly Propionibacterium species or S epidermidis.40,41 drainage leading to ventricles that subsequently
Infections caused by fungi or parasites are become extremely small and less compliant.5,7 SVS
rare,42 but fungal infections can be observed up to is characterized by a triad of brief headaches (last-
2 years after the initial shunt placement.43 Candida ing 10-90 minutes), slit-like ventricles noted on a
species are the major culprit; > 75% of Candida head CT scan, and increased time to fill the shunt
infections are due to Candida albicans.43 Infections valve.1,5,48 The most severe form of SVS is termed
from other organisms, including Aspergillus and normal-volume hydrocephalus, whereby patients pres-
Histoplasma, have been noted in the literature.43 ent with severely increased ICP without ventricular
Importantly, infections due to bacteria, fungi, and dilation on imaging, a condition that is extremely
parasites all present similarly.44 difficult to diagnose.48
Shunt infections are harmful in the acute set-
ting and can cause long-term morbidity;35 unrecog- Abdominal Pathology
nized shunt infections can lead to death. Infections In 0.7% to 10% of patients with VP shunts, the
may be associated with the development of multi- peritoneum fails to absorb the drained CSF, leading
loculated hydrocephalus from gram-negative or- to the formation of large cysts within the peritoneal
ganisms, requiring additional surgical procedures cavity that can potentially cause shunt obstruc-
and long-term shunt dependence.35,36 Multiloculat- tion.28 (See Figure 4, page 6.) The largest pseudocyst
ed hydrocephalus is characterized by enlargement documented in the literature was found in a 3-year-
of multiple noncommunicating intraventricular old boy and measured 15 cm in diameter.49 Notably,
and/or periventricular cysts that are difficult to
manage operatively.45 Infection can also travel into Figure 3. Slit-Like Ventricles On A Head
the brain via the proximal catheter, causing subdu- Computed Tomography Scan
ral empyemas.46 Primary shunt infection spreading
to the peritoneum, causing peritonitis or abscesses,
has also been reported in the literature.28 Infections
with more-aggressive organisms (such as gram-
negative bacteria or Candida) can increase morbid-
ity and mortality when compared to indolent skin
bacteria.42 Long-term issues include a higher risk
of future shunt infections and malfunctions. In ad-
dition, shunt infections can lead to reduced IQ and
school performance, as well as lowered cognitive
abilities in children with myelomeningocele.35,47
Functional Failure
Overdrainage
CSF can sometimes be overdrained even though the
shunt may be functioning properly. For example, the
valve pressure may be set too low for a particular
patient, causing excessive drainage of CSF. In addi- Arrows point to slit-like ventricles.
tion, over-drainage may occur during shunt place- Reprinted from Pediatric Emergency Care, Volume 25, Issue 10.
ment itself, leading to extra-axial CSF collections Paulo da Silva, Italo Suriano, and Henrique Neto. Slit-like ventricle
or subdural hematomas.7 Subdural hematomas can syndrome: a life-threatening presentation. Pages 674-676. © 2009,
with permission from Wolters Kluwer Health, Inc.

children typically present with abdominal com- Seizures are relatively common in patients with VP
plaints such as pain, mass, obstruction, and disten- shunts because many children have a concurrent
tion as opposed to adults, who commonly present diagnosis of epilepsy. As a result, a seizure could
with neurologic symptoms.28,49 Prior abdominal be a manifestation of either the underlying seizure
surgeries with adhesions, prior shunt infections, and disorder, or, in conjunction with other signs and
a prior history of sterile inflammatory reactions to symptoms, a shunt malfunction.
VP shunt materials and CSF proteins are all factors
that predispose patients to pseudocyst formation.28 Prehospital Care
CSF malabsorption in the peritoneal cavity also
may lead to a rare complication of fluid accumu- There are limited data on the prehospital care of
lation in the peritoneum and ascites.7 In patients patients with VP shunt complications. Patients with
with central nervous system tumors who have a VP presentations concerning for increased ICP will re-
shunt, metastasis of primary brain tumors to the quire emergent care and transport via appropriately
abdomen via the VP shunt has been reported in the trained emergency medical services (EMS) person-
literature.50,51 nel. Intravenous access may be needed, and intuba-
tion may be necessary. As with any patient that is
Differential Diagnosis brought to the hospital via EMS personnel, Pediatric
Advanced Life Support (PALS) guidelines should be
The differential diagnosis for a shunt complication followed.
is wide, as patients can present with complaints (eg,
fever, headache, and nausea) that are commonly Emergency Department Evaluation
seen in many childhood illnesses. The whole clinical
picture should be taken into account when consider- Focused History
ing the differential diagnosis. For shunt infections, For patients with a VP shunt who present to the
common childhood diagnoses such as otitis media, emergency department (ED), it is imperative to
upper respiratory infection, gastroenteritis, and obtain a focused, yet detailed, history. In addition
urinary tract infection (especially in patients with to the patient’s chief complaint, the questions in
spina bifida) must be considered in the differen- Table 2 should be asked of any patient with a VP
tial.52 Shunt infections can also present similarly to shunt. Information regarding initial shunt placement
meningitis or encephalitis. Shunt malfunctions from and shunt revisions are key points to address, as
functional or mechanical failures can present simi- the risk of blockage and infection is highest within
larly to migraines or viral syndromes, with vague the first few months following shunt insertion and
complaints such as headache, malaise, or fatigue. revision.1 It is also important to ascertain whether
anything has made the symptoms better or worse. In
Figure 4. Cerebrospinal Fluid Pseudocyst On patients with mildly increased ICP, symptoms typi-
A Computed Tomography Scan cally worsen when lying down and improve when
upright. Conversely, in a patient with SVS, head-
aches typically worsen when upright and improve
after lying down.53 It is also imperative to determine
the type of shunt that was implanted. It is useful to
be acquainted with the types of shunts used in the
Table 2. Key Questions For Patients With A

Ventriculoperitoneal Shunt Who Present With
Acute Illness5
• Why was the initial shunt inserted?
• When was the initial shunt placed?
• Is there any history of prior shunt revisions, and, if so, when?
• Is there any history of prior shunt infections, and, if so, when?
• Were there previous shunt malfunctions, and, if so, what were the
reasons?
• What were the symptoms with previous shunt malfunctions, and are
Arrow points to pseudocyst. the current symptoms similar?
BH Wang, L Hasadsri, H Wang, Abdominal cerebrospinal fluid • What type of shunt is implanted?
pseudocyst mimicking full-term pregnancy, Journal of Surgical Case • Is the child acting any differently from prior illnesses (especially in a
Reports, 2012, Volume 2012, Issue 7, page 6, by permission of Ox- developmentally delayed child)?
ford University Press. • Has the child recently had a shunt tap?

patient’s particular hospital/patient population.54 It the early-encounter group included decreased level
is also necessary to obtain information regarding the of consciousness (PPV, 100%), erythema along the
patient’s recent level of activity, as changes in behav- shunt tract from the valve to the abdomen (PPV,
ior or mental status can indicate a shunt complica- 100%), bulging fontanel (PPV, 92%; LR, 33.1), nau-
tion. Parents often know best whether their child is sea and vomiting (PPV, 79%; LR, 10.4), and irrita-
acting normally or not.53 bility (PPV, 78%; LR, 9.8). Only 2 symptoms were
noted to be highly predictive in the late-encounter
Signs And Symptoms group; these included decreased level of conscious-
It can be useful to consider signs and symptoms ness (PPV, 100%) and loss of developmental mile-
according to a patient’s age. For children aged < 1 stones (PPV, 83%; LR, 36.7).56
year, the symptoms of a shunt complication can be In a retrospective study, Kim et al reviewed
nonspecific and can include irritability, vomiting, oral 352 charts of patients with VP shunts with possible
intolerance, and fever.5 Older children more com- shunt malfunctions whose visit entailed a workup
monly present with headache, nausea, vomiting, and (CT scan with or without a shunt series).15 By
decreased level of consciousness. Visual disturbances, univariate analysis and logistic regression, it was
ataxia, and seizures are less common.5,55 Notably, it is determined that lethargy (odds ratio [OR], 1.99; con-
often easier to appreciate abnormal signs and symp- fidence interval [CI], 1.15-3.42) and shunt site swell-
toms in a developmentally age-appropriate child ing (OR, 2.56; CI, 1.08-6.07) were positive predictors
than in a developmentally delayed child. Signs and of shunt malfunction. In contrast to Garton et al,
symptoms also can be divided according to whether vomiting was not predictive of shunt complication;
they are acute or subacute/chronic. (See Table 3.) vomiting was seen in 52% of patients with a shunt
complication and 43% of patients without a shunt
Clinical Signs And Symptoms complication. Furthermore, headache and fever also
Numerous signs and symptoms of shunt complica- were found to not be predictive of shunt complica-
tions have been discussed in the literature, but there tion (80% of patients who presented with fever did
is no consensus regarding which signs and symp- not have a shunt complication). In a prospective
toms predict a shunt complication. Garton et al study, Watkins et al analyzed patients on a neuro-
utilized data from the Pediatric Shunt Design Trial surgical ward in an attempt to identify which signs
and calculated positive predictive values (PPVs) and symptoms were predictive of a shunt complica-
and likelihood ratios (LRs) for shunt complication. Similar to Kim et al, they found that vomit-
tion based on clinical signs and symptoms.56 They ing, headache, and fever were not associated with
divided the patients into early-encounter groups (< an increased risk of shunt complication. However,
5 months after surgery) and late-encounter groups they did note that drowsiness was a significant, but
(> 9 months after surgery). The signs and symp- not definite, predictor.57 Barnes et al found similar
toms that were most predictive of complication in results; their prospective study analyzed 53 patient
referrals to a pediatric neurosurgical center for
presumed shunt block between April and November
Table 3. Signs And Symptoms Of Shunt 1999. The authors concluded that drowsiness (OR,
Complications: Acute Versus Subacute/ 10; CI, 0.69-10.7) was a much better clinical predictor
Chronic56 of shunt complication than vomiting (OR, 0.9; CI,
0.25-3.65), headache (OR, 1.5; CI, 0.27-10.9), or fever
Type of Shunt Signs/Symptoms
(OR, 0.2; CI, 0.03-6.95).58
Complication
In a study published in 2008, Piatt and Garton
Acute • Nausea
reviewed data from the prospective Pediatric Shunt
• Vomiting
Design Trial and the Endoscopic Shunt Insertion
• Irritability
• Seizures
Trial.52 Their study was a secondary analysis of
• Headache prospective data that reviewed the signs and symp-
• Lethargy toms predictive of VP shunt complications. They
• Coma found that the signs or symptoms with the greatest
• Stupor positive LRs for prediction of shunt failure rate were
• Fever (in decreasing order): bulging fontanel (LR, 44.6),
Subacute/chronic • Change in behavior depressed level of consciousness (LR, 26.2), fluid
• Neuropsychological signs collection along the shunt (LR, 20.1), irritability (LR,
• Change in feeding patterns 13.7), abdominal pain (LR, 12.8), nausea/vomit-
• Developmental delay/regression ing (LR, 11.1), accelerated head growth (LR, 6.02),
• Change in school performance and headache (LR, 4.28). The signs and symptoms
• Change in attention span
that were found to be positively predictive of shunt
• Daily headaches
infection were: purulent drainage (LR, ZD [zero in
• Increasing head circumference

the denominator]), skin erosion (LR, ZD), meningis- majority of these patients had other signs or symp-
mus (LR, ZD), erythema (LR, ZD), peritonitis (LR, toms indicating a shunt complication.15
75.7), fever (LR, 39.3), abdominal pain (LR, 21.3),
and CSF leakage (LR, 12.2). A decision-tree model Myelodysplasia
with different variables was developed, but it has It should be noted that patients who had a VP
not been validated. This study adds useful informa- shunt placed due to myelodysplasia may have dif-
tion regarding the signs and symptoms predictive of ferent presenting signs and symptoms.53 Lee et al
shunt complication, but further research is needed to found that patients with myelodysplasia who have
validate the decision-tree model. shunt complications presented more frequently
with neck pain and an exacerbation of lower cranial
Seizure nerve palsies or syringomyelia than patients with-
Several studies have addressed the question of out myelodysplasia.55
whether a seizure in a patient with a VP shunt is
indicative of a shunt complication, as many chil- Summary
dren with VP shunts also have epilepsy. Johnson In summary, most articles conclude that the best pre-
et al found that < 3% of patients who presented to dictors of shunt complications are: decreased level of
the ED with a seizure necessitated a shunt revision, consciousness, lethargy, drowsiness, meningismus,
and of the patients who actually required a shunt bulging fontanel and erythema, swelling, fluid col-
revision, only 0.7% had a seizure.59 They concluded lection, purulent drainage, and skin erosion around
that a workup is not required in a patient with a the shunt site. Taken independently, nausea and
VP shunt who has an isolated seizure but is other- vomiting, headaches, and seizures are not strong
wise asymptomatic. In a study published in 1990, predictors of VP shunt complications. Fever alone
Hack et al completed a retrospective chart review does not appear to be a strong predictor of shunt
of 346 pediatric patients with VP shunts who were malfunction, but it can be associated with shunt
admitted for hydrocephalus and myelomeningo- infection. The greater the number of these signs and
cele complications. They noted that a seizure alone symptoms that are present, the higher the likelihood
was not highly predictive of a shunt complication. that a shunt complication exists. While there are no
Indeed, all of the children with actual increased validated clinical decision-making rules regarding
ICP had other signs or symptoms suggestive of a the evaluation of a patient with a potential VP shunt
shunt complication (such as headache, vomiting, complication, knowledge of these signs and symp-
or respiratory compromise).60 Similarly, Kim et al toms can help guide the emergency clinician. Table
found that only 10.7% of patients presenting with 4 summarizes the research regarding clinical predic-
a seizure had a shunt malfunction, and the large tors of shunt complications.
Table 4. Clinical Predictors Of Shunt Complications15,52,56,58-60

Strength of Prediction Predictor Statistical Relevance
Strong Meningismus LR, ZD52 (shunt infection)

Peritonitis LR, 75.752 (shunt infection)
Decreased level of consciousness/lethargy LR, 26.252 (shunt failure); OR, 1.9915; PPV, 100%56
Fluid or erythema around shunt LR, ZD52 (erythema, shunt infection), 20.152 (shunt failure), 12.252 (CSF
leakage, shunt infection); OR, 2.5615; PPV, 100%56
Bulging fontanel LR, 33.156, 44.652; PPV, 92%56
Drowsiness OR, 1058
Medium Loss of developmental milestones LR, 36.756; PPV, 83%56
Abdominal pain LR, 21.352 (shunt failure), 12.852 (shunt infection)
Irritability LR, 13.752, 9.856; PPV, 78%56
Increasing head growth LR, 6.0252 (shunt failure)
Fever (shunt infection) LR, 39.352
Weak Nausea and vomiting LR, 11.152 (shunt failure), 10.456; OR, 0.958; PPV, 79%56
Headache LR, 4.2852 (shunt failure); OR, 1.558
Fever (shunt malfunction) OR, 0.258
Seizure None59,60
Abbreviations: CSF, cerebrospinal fluid; LR, likelihood ratio; OR, odds ratio; PPV, positive predictive value; ZD, zero in the denominator.

Physical Examination Diagnostic Studies
It is important to complete a full physical examina-
tion, including a thorough neurologic examination, Imaging
on every patient with a VP shunt to look for any There is no consensus regarding appropriate imag-
other source of illness. Inspect and palpate the entire ing workup in pediatric patients with potential VP
shunt tract, looking for any erythema, swelling, fluid shunt complications. Neurosurgeons vary widely in
collection, purulent drainage, or skin erosion along their management approach,65 and it is quite com-
the tract. Feel for any disconnection or irregularity.5 mon for patients with possible VP shunt complica-
Follow the shunt to its distal termination and pal- tions to have a noncontrast head computed tomog-
pate the abdomen for any tenderness or mass that raphy (CT) scan and shunt series. These studies
may indicate a pseudocyst. It is possible, although are typically reserved for patients with concern for
not common, to find evidence of a shunt complica- possible mechanical or functional shunt failure.
tion just by palpating the tract.7 Palpating for fluid
around the shunt tract is one of the simplest tests to Noncontrast Head Computed Tomography
diagnose shunt breakage.61 Unfortunately, however, Noncontrast head CT is the most commonly used
the physical examination alone is usually inconclu- test for assessing patients with suspected shunt com-
sive.53 plications. It is widely available and usually does
Bradycardia or hypertension (part of Cushing not require sedation.66 Estimates of sensitivity for
triad) should alert to the possibility of increased head CT recognition of shunt complications range
ICP. Other common signs of increased ICP in infants from 64% to 92%.57,67 Evidence of shunt infection
include bulging fontanel, separation of the cranial can also be seen on a head CT scan; ventriculitis and
sutures, bulging scalp veins, and increasing head meningitis can be seen as enhancement of the epen-
circumference. Less-common signs include loss dymal lining or cortical sulci.17 However, CT scans
of upward gaze (known as sundowning) and sixth have numerous limitations. For example, it has been
cranial nerve palsy.5 Fundoscopic examination shown that in 16% to 24% of cases of shunt compli-
should be considered on all patients for whom there cation, ventricular size (as seen on a head CT scan) is
is a suspicion of a shunt complication. Papilledema, unchanged from prior imaging.15,68 This is likely due
although an uncommon presentation, is concerning to scarring in the ventricles and ventricular walls
for increased ICP.54 that prohibits ventricular expansion.17 It also has
been estimated that approximately one-third of pa-
Pumping The Shunt tients with a shunt failure (mechanical or functional)
Almost all shunts have a reservoir that can be will have no findings on head CT.57,69,70
pumped to check for proximal or distal obstruction, Another concerning issue associated with head
acting as a means to assess shunt patency. Usually, CT scans is radiation exposure. Patients with VP
if the reservoir pumps easily, then the shunt is likely shunts often get multiple head CT scans over their
patent distally. If the reservoir replenishes quickly, the lifetime, compounding the risk of malignancy. There
shunt is likely patent proximally.62 Piatt assessed the are numerous initiatives aimed at decreasing radia-
value of pumping a shunt; the sensitivity for this test tion exposure from CT scans in children, such as the
was low for detecting shunt obstruction (18%-20%) Image Gently campaign (www.imagegently.org/)
and the negative predictive value (NPV) was only and the recommendation to set the radiation dose
65% to 81%.62 Piatt and Garton also reviewed patients to as low as reasonably achievable (ALARA).61 The
in the Pediatric Shunt Design Trial. From their data, Image Gently campaign began in 2007 with the goal
the LR of the shunt pump test as a predictor of shunt of reducing the amount of radiation delivered when
failure was high at 7 (95% CI, 3.00-19.0), but a nega- obtaining a CT scan in children. It recommended
tive test was low at 1.27 (95% CI, 1.13-1.41). These using ALARA CT scans when magnetic resonance
results indicate that a shunt obstruction is still a pos- imaging (MRI) or ultrasound are unavailable.71 Mul-
sibility even if the shunt pumped easily and refilled tiple studies have shown that low-dose head CT is
promptly.52 In summary, it seems that normal results equivalent to normal-dose head CT when looking at
from pumping a shunt do not reliably exclude a shunt the ventricles.72-75 There are no universally adopted
patency issue,52 and that pumping a shunt likely guidelines regarding the dose of radiation for pedi-
does not yield much information when compared atric head CT scans, and there is wide interinstitu-
to imaging studies. In addition, pumping a shunt is tion variability. A head CT scan should be compared
not a benign procedure and has been associated with to a prior normal head CT scan to note any change,
complications including changes in ICP and cerebel- especially regarding ventricular volume and the
lar hemorrhages.63,64 position of the shunt tip.28

Shunt Series 11-32); and specificity 98% (CI, 94-99). Results for a
A shunt series is a series of plain radiographs that positive head CT scan include: LR, 3.5; sensitivity,
capture the entire length of the shunt and usually in- 83% (CI, 71-92) and specificity 76% (CI, 69-82). For a
cludes anteroposterior and lateral views of the skull, CT scan or shunt series, the sensitivity was found to
neck, chest, and abdomen. (See Figure 5.) Shunt se- be 88% (CI, 77-95), and the specificity was 74% (CI,
ries are useful for assessing continuity; the physician 67-81). Notably, 3 patients with an obstruction had
and the radiologist should look for any fractures, an abnormal shunt series and a normal CT scan. The
disconnections, or calcifications. (See Figure 6, page authors concluded that, although it can be costly to
11.) When viewing the shunt series, note that some routinely obtain a shunt series in patients, there are
shunt components may be radiolucent and may be instances where a shunt series can identify a mal-
mistaken for a disconnection.61 Comparison with function that is not seen on a head CT scan.67 Pitetti
prior films can be helpful when there is concern for performed a retrospective chart review of patients
abnormal findings. aged < 18 years with a history of a ventricular shunt
and symptoms of possible obstruction. Of the 291
Diagnostic Accuracy Of Head Computed children reviewed, the rate of malfunction identified
Tomography Versus Shunt Series by a shunt series in the setting of a normal head CT
It has been asked whether a shunt series is neces- scan was 2%.77 Since it is always preferable to reduce
sary when most patients will also get head CT scans. radiation exposure, if a shunt series is performed
Currently, many hospital protocols do not require a after the head CT scan, it seems reasonable that the
shunt series. Desai et al retrospectively reviewed the shunt series does not need to include the 2-view
charts of children who had a shunt series to evaluate skull radiograph and, potentially, the neck view, if
clinically suspected VP shunt failure and compared the CT scout films contain these locations.66
them to patients who had other imaging studies
(eg, head CT). They concluded that the sensitiv- Other Imaging Studies
ity of plain radiography for detection of VP shunt Shuntograms
failure was no higher than 31%, and recommended A shunt series can assess shunt continuity, but not
that patients for whom there are concerns for shunt shunt patency. To test the patency of the proximal
failure should receive CT scans of the head.76 Zorc et and distal portion of the catheter, some centers use
al completed a retrospective study of head CT scans shuntograms.28 Contrast media is injected into the
and shunt series for patients with possible shunt shunt reservoir and serial radiographs are ob-
obstructions. After reviewing 233 images, they were tained.69 This is typically employed when patients
able to calculate sensitivities, specificities, and LRs present with signs and symptoms suggestive of
for both types of imaging studies. Results for a posi- obstruction, but prior imaging is inconclusive. Some
tive shunt series include: LR, 8.6; sensitivity, 20% (CI, centers use shuntograms in lieu of a shunt series.
Figure 5. Radiographic Images From A Shunt Series
PC
R
DC
DCT
Arrows indicate the location of the shunt along the shunt series.
Abbreviations: DC, distal catheter; DCT, distal catheter termination; PC, proximal catheter; R, reservoir.
To view the images online, scan the QR code with a smartphone or tablet or go to:
www.ebmedicine.net/VPshunt_Figures
Images courtesy of John Amodio, MD, FACR, Kings County Hospital Center, Brooklyn, NY.

Several studies have demonstrated good diagnostic Ultrasound can also be used to check optic nerve
accuracy (sensitivity of 96% and specificity of 89%) sheath diameter when assessing for increased ICP.
when combining a CT scan with a shuntogram.78 Zaidi et al retrospectively reviewed CT scans of 25 pe-
However, other studies have shown a false nega- diatric patients with confirmed VP shunt obstruction
tive rate of 14%.56,69 When combining a shuntogram requiring intervention and compared them to both
with a head CT, Ouellette et al showed a NPV of self-controls (the same patients in their normal state
96.3% (CI, 85-99.9) and a sensitivity of 97.4% (CI, of health) and age-matched pediatric patients without
79.1-99.8). They also noted that some complications VP shunts. They found that the mean difference in
were detected on the shuntogram when a normal the right optic nerve sheath diameter was 0.89 mm
head CT scan was obtained.79 From this information, (P = .01) when comparing the patients with obstruc-
one can conclude that a shuntogram may be war- tion and the self-controls, and 0.56 mm (P = .002)
ranted if there is a high clinical suspicion for shunt when comparing the patients who had obstruction and
obstruction or other types of mechanical or func- their age-matched controls. They concluded that optic
tional failure, if the head CT scan and shunt series nerve sheath measurements could be used as a means
are inconclusive. to determine VP shunt obstruction.81 Malayeri et al
performed a case-control study of optic nerve sheath
Ultrasound diameter in children with increased ICP and normal
Ultrasound is typically used to evaluate for poten- ICP. They performed ophthalmic ultrasound and
tial abdominal masses, such as pseudocysts.28 In found that the mean diameter in the raised ICP group
lieu of a head CT scan, ultrasound also can be used was 4.6 mm (+/- 0.6 mm). The controls had a mean
in infants with open fontanels.1 In addition, there diameter of 3.3 mm (+/- 0.6 mm). These results are
are case reports in which ultrasound was used statistically significant (P < .001).82 Le et al performed
in place of a shunt series; the ultrasound should a prospective study of pediatric emergency physicians
follow the tract of the shunt catheter and used to who received ultrasound training in ophthalmologic
assess for any disruption.80 studies (ie, optic nerve sheath diameter). They re-
cruited 54 patients who had ultrasounds performed
by the pediatric emergency physician; these were later
Figure 6. Fracture Of A Ventriculoperitoneal reviewed by an ophthalmologist and an ophthalmo-
Shunt logical sonographer. Of the recruited patients, 37% had
a confirmed increase in ICP. They determined that the
sensitivity of optic nerve sheath diameter via ultra-
sound for increased ICP was 83% (95% CI, 0.60-0.94),
specificity was 38% (CI, 0.23-0.54), positive LR was
1.32 (CI, 0.97-1.79), and negative LR was 0.46 (CI, 0.18
-1.23). This study concluded that the trained pediatric
emergency physician had good interrelater reliability
in performing the measurements, but that the operat-
ing characteristics were not significant enough to be
used as the only measurement of ICP.83 In conclu-
sion, because these studies have shown mixed results,
further research is warranted and ultrasound of the
optic nerve sheath diameter alone cannot guide clinical
management.81-83
Advanced Imaging
Multiple studies have demonstrated that MRI is an
adequate substitute for head CT in shunt evaluation,
with less exposure to radiation. Unfortunately, full-
brain MRIs are often time-consuming (taking 30-45
minutes) and are more likely to require sedation
as compared to head CT scans. Furthermore, MRI
can become nondiagnostic with even very minimal
patient movement. Quick-brain or limited-sequence
MRIs (which only last from 8 seconds to 10 minutes)
are as sensitive as CT scans in diagnosing shunt
malfunction in the acute setting.66,84-90 MRIs do have
Arrow indicates a ventriculoperitoneal shunt fracture. limitations, such as high cost and lack of round-the-
Image courtesy of John Amodio, MD, FACR, Kings County Hospital clock scanner and technician availability. In addition,
Center, Brooklyn, NY. it should be noted that certain programmable shunts

Clinical Pathway For The Management Of
Suspected VP Shunt Malfunctions
Patient with a VP shunt presents to the ED
History and physical examination

YES NO
consistent with shunt malfunction?
Consider alternate
Concern for increased ICP
diagnosis
requiring emergent NO
management?
YES
• Monitor ABCs Perform noncontrast head

• Perform emergent CT or rapid MRI and
shunt tap shunt series
• Consult neurosurgery, (Class II)
stat
Results consistent with

NO
acute shunt malfunction?
YES
• Consult with neurosur- Are prior scans

gery unavailable, are scans
• Continue to monitor the unchanged, or is NO
patient there still concern for
malfunction?
YES
• Consult with neurosur- YES Are ventricles slit-like?

gery
• Consider admission for
NO
further monitoring
• Consider alternate
diagnosis
• Consult with
neurosurgery regarding
disposition
Abbreviations: ABC, airway, breathing, circulation; CT, computed tomography; ED, emergency department; ICP, intracranial pressure; MRI, magnetic
resonance imaging; VP, ventriculoperitoneal.
For Class of Evidence definitions, see page 13.

Clinical Pathway For The Management Of Suspected VP Shunt Infections
Patient with a VP shunt

presents to the ED
Shunt placement in the past 6 months

YES NO
or concern for shunt infection?
Does patient have any of the following?:

• Irritability, drowsiness Consider alternate
NO
• Erythema or swelling around shunt infectious diagnosis
site
• Other symptoms or signs suspicious
for infection
(Class II)
YES
Consult neurosurgery for shunt tap
CSF studies consistent

NO Still concern for shunt infection? NO
with shunt infection?
YES YES
• Start antibiotics (broad-spectrum • Consider starting antibiotics in

antibiotics can be given initially until consultation with neurosurgery
specific organisms and sensitivities • Consider lumbar puncture
are identified)
• Admit
(Class II)
Abbreviations: ED, emergency department; CSF, cerebrospinal fluid; VP, ventriculoperitoneal.
Class Of Evidence Definitions

Each action in the clinical pathways section of Pediatric Emergency Medicine Practice receives a score based on the following definitions.
Class I Class II Class III Indeterminate
• Always acceptable, safe • Safe, acceptable • May be acceptable • Continuing area of research
• Definitely useful • Probably useful • Possibly useful • No recommendations until further
• Proven in both efficacy and effectiveness • Considered optional or alternative treat- research
Level of Evidence: ments
Level of Evidence: • Generally higher levels of evidence Level of Evidence:
• One or more large prospective studies • Nonrandomized or retrospective studies: Level of Evidence: • Evidence not available
are present (with rare exceptions) historic, cohort, or case control studies • Generally lower or intermediate levels of • Higher studies in progress
• High-quality meta-analyses • Less robust randomized controlled trials evidence • Results inconsistent, contradictory
• Study results consistently positive and • Results consistently positive • Case series, animal studies, • Results not compelling
compelling consensus panels
• Occasionally positive results
This clinical pathway is intended to supplement, rather than substitute for, professional judgment and may be changed depending upon a patient’s individual
needs. Failure to comply with this pathway does not represent a breach of the standard of care.
Copyright © 2016 EB Medicine. 1-800-249-5770. No part of this publication may be reproduced in any format without written consent of EB Medicine.

are affected by magnetic fields and may malfunction obtained when CSF eosinophilia is noted.94 Periph-
after MRI; these programmable shunts often require eral WBC count is not shown to be useful in the
reprogramming after MRI.66,85 evaluation of most CSF infections.96 Blood cultures
should be considered, as they are positive in 20% to
Summary 25% of patients with confirmed shunt infections.36,53
If there is a concern for possible mechanical or func- Although studies are limited regarding serum
tional failure in a hemodynamically stable patient, C-reactive protein (CRP) in shunt infections, some
a head CT or MRI should be obtained first. When studies have found an elevation in CRP in these pa-
a head CT scout film is obtained, a shunt series can tients.92,97 Using a serum CRP cutoff of > 7 mg/L,
be performed later without imaging the skull and, Schuhmann et al concluded that the sensitivity,
potentially, the neck. In a survey of neurosurgeons, specificity, and NPV of CRP were 97.1%, 73.5%, and
the most important variable in determining pos- 97.3%, respectively.97
sible shunt failure was clinical presentation.65 The Elevated inflammatory cytokines in the CSF, eg,
second most important variable was an increase in TNF alpha, interleukin 1 beta, and interleukin 6, have
ventricular size on neuroimaging studies. The least also been shown to be predictive of a shunt infection.
important variable was an abnormal shuntogram. However, more research is needed to confirm these
The most commonly employed study was a head results and few hospital laboratories are able to quickly
CT scan, followed by a shunt series, and shunt tap, perform these tests in a cost-effective manner.53,98
while the shuntogram was infrequently utilized. Ventriculitis (infection in the ventricles) does not
The Clinical Pathway For The Management Of always accompany meningitis (infection of the me-
Suspected Shunt Malfunctions (page 12) shows a ninges covering of the brain), and there are instances
workup algorithm. when both a shunt tap and a lumbar puncture may
be necessary.55,99 Typically in these cases, a shunt
Laboratory Studies tap is performed first, prior to a lumbar puncture.99
CSF obtained by shunt tap is the mainstay for evalu- However, CSF cultures obtained from a lumbar
ation for potential shunt infections. A shunt tap is puncture are unreliable for detecting shunt infec-
a procedure in which a physician places a needle tions, as cultures can be negative in the setting of an
through the skin layers into the shunt reservoir to col- infection.53
lect CSF that can be used diagnostically and therapeu- One retrospective study showed that increased
tically. In a retrospective review, a causative organism CSF lactate is associated with a higher likelihood of
was identified in 85.7% of shunt taps from patients shunt infection,100 while another retrospective study
with suspected shunt infection.91 revealed normal CSF lactate levels in 20% of patients
Laboratory tests typically sent after a shunt tap with confirmed shunt infections.101
include CSF culture, cell count, glucose, and protein.
In patients with a shunt infection, the CSF may show Summary
an increased white blood cell count (WBC), increased Laboratory results may be normal in patients with a
protein, and decreased glucose when compared to VP shunt infection. There are many reasons for nor-
patients with a shunt malfunction, but no infection.92 mal laboratory values in the setting of an infection,
Most infected shunts have only a modest pleocytosis such as previous antibiotic use or distal catheter
(< 200 WBC/mcL).54 The presence of fever and infection that has yet to migrate proximally. There-
increased CSF WBC count (> 5 cells/mcL) has a high fore, if the clinical scenario is concerning for a shunt
PPV for shunt infection. infection, then the most prudent course is to treat the
McClinton et al reviewed 12 charts of patients patient, even if the laboratory values are normal.
admitted with confirmed VP shunt infections. They
found that a history of fever and CSF neutrophils Treatment
(> 10%) was highly specific for a shunt infection
(specificity, 99%; PPV, 93%; LR, 91). However, this Management depends on the type of VP shunt
study is limited by its small sample size.93 complication that is suspected. Overall, treatment
Although eosinophils in the CSF are commonly of an infection is different from treatment of a
seen in patients with VP shunts, some studies have mechanical or functional failure. As always, assess
found that increased eosinophils in the CSF (eosino- the patient’s vital signs and mental status. If there
philia) correlates with a higher chance of shunt com- is concern for severely increased ICP or hernia-
plications.94,95 Some reported that CSF eosinophilia tion, a shunt tap is indicated.
> 5% has a high PPV for shunt complications (such
as infection),93 while others reported that VP shunt Shunt Tap
infections with P acnes have higher eosinophil Most shunts are tapped by neurosurgeons. Indica-
counts compared to other common organisms. tions for an emergency physician to tap a shunt
Therefore, an anaerobic CSF culture should also be include presentations consistent with severely

increased ICP and impending herniation, or acute location.104 Plain films of the skull may be useful if
deteriorations in mental status when neurosurgery is the emergency physician is unsure of the reservoir’s
not immediately available. Tapping the shunt in these location after palpation; a case report noted the util-
scenarios could be life-saving. In addition, the proce- ity of ultrasound in locating the reservoir prior to
dure can be diagnostic, depending upon the flow of shunt tap.105 However, if an emergency physician is
CSF and the results of CSF laboratory studies.1 performing a shunt tap, the patient is likely acutely
VP shunts are composed of 4 components: a deteriorating and there may not be time for any
proximal catheter, a 1-way valve, a reservoir, and imaging studies.104,105
a distal catheter. The shunt is tapped through the
reservoir.35 The procedure requires a sterile solution Management Of Specific Situations
(eg, povidone-iodine solution), a 25-gauge butterfly For clinically unstable patients, PALS algorithms
needle, a 3-way stopcock, a manometer, and a 5-cc or should be followed. Patients with signs of increased
10-cc syringe. Attach the 3-way stopcock to the butter- ICP (eg, hypertension, bradycardia, and irregular
fly needle and manometer.102 After cleaning the over- respirations [Cushing triad] or a fixed and dilated
lying skin with a sterile solution, insert the 25-gauge pupil) require rapid sequence intubation with a
butterfly needle perpendicularly through the skin sedative (eg, etomidate) and a paralytic (eg, ro-
into the center of the reservoir until a “pop” is felt, curonium), as the patient's ability to maintain the
keeping the stopcock open to the manometer. If fluid airway may be compromised. These agents have
starts draining out, a proximal obstruction is unlikely been shown to be relatively safe to use in patients
and fluid will fill into the manometer. At this point, with concerns for increased ICP. Of note, there is
pressures can be measured. Normal ICP is 12 cm H2O increasing research on the safety of ketamine in
(+/- 2 cm).103 Opening pressures ≥ 20 cm H2O indicate patients with intracranial injury.106 However, there
a distal obstruction, whereas low pressures indicate a are very limited data regarding the use of ketamine
proximal obstruction. If the pressure is elevated, within children with impending herniation, and some ex-
draw fluid slowly (approximately 2-3 drops/min) perts prefer not to use ketamine in this population.
until the pressure is < 15 cm H2O.3,102 Withdraw the After securing the airway, it is reasonable to
needle and apply pressure on the site of needle inser- hyperventilate gently with a PCO2 target of 30
tion for 2 minutes. For a video that shows the proper to 35 mm Hg. Peripheral vascular access should
landmarks and technique for performing a shunt tap be obtained; if unsuccessful, an intraosseous line
and obtaining CSF for analysis, go to: http://www. should be placed. The unstable patient may benefit
columbianeurosurgery.org/2010/04/shunt-tap/. from 3% normal saline (3-5 mL/kg IV) or mannitol
When attempting to drain CSF from the reser- (0.25-2 g/kg IV).54,103,107 While the patient’s airway,
voir, a proximal obstruction is likely if fluid cannot breathing, and circulation are being addressed, a
be drained or if the flow ceases quickly. In this case, shunt tap should be strongly considered, as this can
the ICP cannot be relieved by shunt tap. Instead, be life-saving.
the pressure needs to be relieved by a tap through The patient’s neurosurgeon should be contacted
a fontanel, a tap through the cranial sutures (if they as soon as possible. Neurosurgical evaluation is nec-
are split), or a burr hole, all of which would ordinar- essary to determine the need for surgery; specifical-
ily be performed by a neurosurgeon.54 Other meth- ly, for any mechanical or functional failure, includ-
ods for decreasing ICP in the setting of impending ing obstruction. The urgency is greater in patients
herniation include hyperventilation and mannitol. with proximal obstruction than in those with distal
(See the Management Of Specific Situations, in the obstruction, as patients with proximal obstruction
following section.) may decompensate more rapidly. It is also important
A contraindication to a shunt tap is infection to consult neurosurgery in patients with signs and
over the reservoir site. However, if herniation is symptoms concerning for obstruction who have nor-
imminent, it may still be necessary to tap the shunt mal or inconclusive head CT scans and shunt series,
over the infected reservoir, as the patient’s life is in as they can assist in decisions regarding the need for
jeopardy. There are many risks associated with tap- further imaging (ie, shuntogram) or workup.70
ping a shunt, including localized bleeding and infec- Intravenous antibiotics are necessary for shunt
tion, damage to the shunt components, CSF leakage, infections. They should be started if the clinical suspi-
and intraventricular and subdural bleeding due to cion is high, even in the setting of normal diagnostic
the CSF draining too rapidly.5 The risk of infection studies. In general, broad-spectrum antibiotics can
from tapping the shunt has been difficult to quantify, be given until specific organisms and sensitivities are
but has been reported as ranging from negligible to identified. Antibiotics should be chosen to reflect the
as high as 32% in premature infants with reservoirs pathogens prevailing in the patient’s community.37
tapped multiple times.35 It is imperative to ensure A sample empiric antibiotic regimen might include
that the emergency physician is indeed tapping the vancomycin, a broad-spectrum cephalosporin, and a
shunt reservoir, as Martens et al reported a case of third agent for anaerobes.35,37 Once culture results and
intracranial hemorrhage due to tapping the wrong susceptibilities are determined, antibiotic selection
can be modified. Treatment beyond the use of intrave- Ventriculoatrial And Ventriculopleural Shunts
nous antibiotics, such as removal of the shunt, intras- Ventriculoatrial (VA) and ventriculopleural shunts
hunt installation of antibiotics, and externalization of typically are used if VP shunting fails. These shunts
the shunt, are determined by a neurosurgeon.35 have the potential for severe complications. Com-
Tables 5 and 6 summarize the steps used in the plications of VA shunts include severe infection,
workup and management of potential VP shunt venous thrombosis, endocarditis, dysrhythmia,
complications. Table 5 describes all potential work- cardiac tamponade, chronic pulmonary thromboem-
up discussed in this review. Table 6 describes spe- bolization with pulmonary hypertension, and shunt
cific complications with their workup and manage- nephritis.7 Children with VA shunt infections fre-
ment. The Clinical Pathways delineate the workup quently present with fever and leukocytosis; blood
and management of certain patients. cultures are positive in 4.2% of cases.108 Shunt ne-
phritis is typically due to S epidermidis. Patients may
Special Circumstances/Populations present with symptoms of hematuria, proteinuria,
renal insufficiency, and hypertension.5 Obstruction
No Neurosurgeon In-House is a potential complication of VA shunts, although it
Neurosurgeons are not always available in-house or is less common than with other shunts. Keucher and
at community hospitals. In this situation, contact the Mealey demonstrated an overall obstruction rate
patient’s neurosurgeon for an accurate patient history of 4.6% in VA shunts.109 Treatment involves shunt
and for discussion regarding further workup and man- removal and antibiotics.
agement. For emergent situations, such as in patients Complications unique to ventriculopleural shunts
with signs of impending herniation, including hyper- include empyema, noninfectious pleural effusions,
tension, bradycardia, irregular respirations, and/or a and pneumothorax. An empyema can develop in
fixed, dilated pupil, perform an emergency shunt tap response to a shunt infection and usually responds to
as described on page 15. Conversely, if the patient has antibiotics and removal of the shunt. Pleural effusion
a proximal shunt obstruction that cannot be relieved by can develop if the pleural lining is not able to absorb
a shunt tap, consideration should be made for reaching the CSF and this can affect respiratory function.110
out to other in-house surgeons (ie, trauma) for assis-
tance in placing a burr hole, as this may be out of the Controversies And Cutting Edge
scope of the practice of an emergency physician.
A promising development in the prevention of shunt
Table 5. Emergency Department Evaluation infections is antibiotic-impregnated shunts.1 Shunts
Of Shunt Complications impregnated with clindamycin and rifampin have
been introduced in an attempt to reduce shunt-relat-
ed infections. Several reports suggest a benefit from
1. Obtain thorough medical history
these devices,111-113 while others are inconclusive or
• Indication for shunt
• Shunt type
do not show any benefit.114-116 A prospective study
• Date(s) of insertion/revision involving 125 patients noted the potential problem
• Current symptoms of development of antibiotic-resistant bacteria with
• History of prior shunt malfunctions antibiotic-impregnated shunts.117 As these patients
• History of prior shunt infections
2. Perform clinical examination

Table 6. Management Of Specific Shunt
• Complete physical examination, including neurological
Complications
examination
• Valve inspection
• Shunt tract inspection Concern for mechanical or functional failure: imaging
• Low-dose head CT (imperative to compare with prior studies) or
3. Obtain imaginga MRI, if possible
• Head CT (compare with prior scans) • Radiographic shunt series (if CT scout view obtained, exclude head
• Shunt series view from shunt series)
• Radiographic or contrast shuntogramb • Shuntogram
4. Perform shunt tap, CSF analysisa Concern for shunt infection

• Pressure studies • Consider shunt tap
• Laboratory studies (cell count/glucose/protein) • Consider performing LP and sending CSF studies
• Culture and sensitivity • Antibiotics
• Neurosurgery consultation
a
Perform when necessary.
b
Performed by neurosurgery. Abbreviations: CSF, cerebrospinal fluid; CT, computed tomography;
Abbreviations: CSF, cerebrospinal fluid; CT, computed tomography. LP, lumbar puncture; MRI, magnetic resonance imaging.

start presenting to EDs, emergency clinicians should A history from the patient’s caretaker regarding
be aware of their existence and the potential for changes in the mental status from baseline is imper-
these patients to harbor resistant strains of bacteria. ative. An increased number of symptoms increases
Cerebral regional hemoglobin oxygen satura- the likelihood of a possible shunt complication.
tion (rSO2) monitoring is a newer technology that A VP shunt tap can be life-saving in unstable
can help monitor a patient during a shunt tap. With patients with concerns for severely increased ICP.
rSO2 monitoring, probes on a patient's forehead can Consultation with the patient's neurosurgeon is of
help measure perfusion of the brain. During a shunt paramount importance. For more-stable patients with
tap, CSF is released, leading to a decrease in the suspicion for a shunt obstruction, diagnostic studies
ICP, improving tissue perfusion, oxygenation, and (such as a head CT scan and shunt series) are often
metabolism.118-121 Thus, cerebral rSO2 monitoring necessary. CSF studies via a shunt tap are indicated
can help document that the shunt tap has improved for the evaluation of a possible shunt infection, but
cerebral tissue perfusion. Cerebral rSO2 monitor- routine serum laboratory testing is of limited utility.
ing during a shunt tap also has been shown to be a Antibiotics for a presumed shunt infection should be
helpful adjunct in determining the location of shunt started empirically and should reflect the sensitivities
obstruction.118 of the prevailing pathogens in the community. Patient
disposition depends on multiple factors, including the
Disposition nature of the complication, neurosurgical availability,
access for timely follow-up, and family comfort.
Disposition of patients with VP shunt complications
depends upon the nature of the complication, the Case Conclusions
general appearance of the patient, neurosurgical
availability, and the ability of the patient to present You elicited further history about the 7-year-old girl with
for timely follow-up. There are no standard guide- headache and fever. She had a revision a few months ago
lines for situations in which a patient can be safely due to a previous shunt infection. The mother stated that
discharged with a VP shunt complication. All cases the girl appeared similar to how she presented during her
of patients with possible VP shunt complications last shunt infection. Because of this and the fact that no
should be discussed with the patient’s neurosurgeon true focus of infection was found, you initiated a workup
or the in-house neurosurgeon. If the child looks ill that included contacting the pediatric neurosurgeon. The
and there is ongoing concern, the patient should patient’s shunt was tapped and she was started on ceftriax-
be admitted for observation and further in-house one and vancomycin, and admitted to the inpatient ward.
workup. If a complication is diagnosed in a patient The 3-year-old girl who presented with altered mental
with a VP shunt, discussion with the neurosurgeon status and vomiting had signs and symptoms consistent
and admitting team is necessary. If the complication with increased ICP, requiring emergent management. Due
cannot be managed at the current hospital, transfer to her condition and history, you attempted a VP shunt
should be arranged to another institution that has tap without any prior imaging. CSF (10 cc) was drained,
the necessary capabilities, after initial workup and and the patient’s neurosurgeon was contacted. You placed
stabilization. It is never appropriate to discharge a intravenous lines and intubated the patient via rapid
patient with symptoms concerning for shunt in- sequence intubation. The patient began to decompensate,
volvement without discussing the case with a neuro- with bradycardia, hypertension, and abnormal pupils. You
surgeon.53 If a patient is deemed safe for discharge, initiated hyperventilation, gave mannitol, and performed
close follow-up, specifically with the neurosurgeon, another VP shunt tap, which resulted in stabilization of
should be assured. Parental comfort should be en- the patient. The patient’s neurosurgeon arrived and the
sured; consider admission if this is not possible. girl was taken to the operating room for shunt revision.
For the 5-year-old boy who presented with vomit-
Summary ing, his neurosurgeon was contacted for further history.
Kidney, ureter, and bladder x-rays were obtained, showing
VP shunt placement is increasing in the pediatric impacted stool throughout the colon, confirming that the
population, and with this comes the potential for boy was actually constipated. An enema was provided in
increased complications. Complications can be the ED, with good results. The patient tolerated oral intake
grouped into 3 categories: mechanical failure, infec- in the ED without vomiting and his exam remained stable
tion, and functional failure. Signs and symptoms throughout. The patient was discharged home on stool soft-
frequently overlap and are often nonspecific; how- eners and laxatives, with dietary changes, and was given
ever, the best predictors of shunt complications are follow-up with his pediatrician and neurosurgeon.
meningismus, peritonitis, decreased level of con-
sciousness, lethargy, erythema along the shunt tract,
bulging fontanel, and drowsiness. A focused history
and physical examination is needed in each patient.

Risk Management Pitfalls For Patients With VP Shunt Complications
1. “I am concerned that my patient may have 6. “My patient with a VP shunt appears ill, but
a VP shunt obstruction. The neurosurgical there is no history of fever. Therefore, a shunt
resident is at the bedside and pumps the shunt, infection can be excluded.”
which has free flow. Because of this, he wants A patient can have a shunt infection without
to send the patient home.” fever. Other signs and symptoms may be present
Pumping the shunt has been shown not to be that can suggest a possible shunt infection. A
a reliable predictor of shunt patency. If clinical full history and physical examination should be
suspicion for an obstruction is still high, further obtained.
workup and management may be needed.
7. “My patient with a VP shunt presents with
2. “My patient has clinical signs of shunt obstruc- abdominal pain and vomiting; this cannot be
tion, but has a normal head CT scan and shunt related to the shunt.”
series, so his symptoms must not be related to Abdominal pain and vomiting in a patient with
his shunt.” a VP shunt can be a sign of shunt malfunction,
Unfortunately, many patients can have normal or due to common viral or other illnesses.
or unchanged imaging and still have a shunt For example, patients with constipation and
complication. If the suspicion for a shunt a pseudocyst can present similarly. Again, a
complication is high, it is advisable to discuss detailed history and physical examination are
the case with the patient’s neurosurgeon and needed, as well as potential imaging.
consider further imaging or intervention.
8. “Although my patient is stable, I am concerned
3. “My patient has large ventricles on a head CT about a possible shunt infection, so I will per-
scan, so there must be a shunt malfunction.” form a shunt tap.”
Comparing the studies with previous imaging This is an option, if this has been discussed with
should always be done, whenever possible, to the neurosurgeon and you feel comfortable
determine whether there is any change in the performing a tap. Typically, however, a shunt
ventricle size. Also, the clinical picture should be tap is performed by an emergency physician
taken into consideration. only emergently when a patient is acutely
decompensating. There are many risks
4. “My patient is presenting with signs of a pos- associated with shunt taps, and this procedure
sible shunt complication. The head CT scan should be performed by a neurosurgeon, if
is normal, and, therefore, a shunt series is not possible.
necessary.”
Although rare, there are cases of a normal 9. “My patient with a VP shunt has clinical signs
head CT scan with an abnormal shunt series. of increased ICP with impending herniation,
Therefore, a shunt series should be obtained. but I want to wait for neurosurgery to perform
a shunt tap.”
5. “Every patient with a VP shunt and fever As stated in pitfall 8, this is the only
needs laboratory work and diagnostic studies.” circumstance when an emergency physician is
The clinical picture needs to be considered for truly obligated to perform a shunt tap, as it is a
each patient. A focused history and physical potentially life-saving intervention.
examination should be obtained and key
features of shunt infection should be focused 10. “My patient with a history of seizures and
upon, such as bulging fontanel, drowsiness, a VP shunt presented with a breakthrough
lethargy, or erythema around the shunt. seizure; this must be a shunt malfunction or
Laboratory values are not specific. Not all shunt infection.”
infections need imaging—if a shunt infection is Seizures alone are not necessarily a clinical
suspected, a shunt tap is needed. manifestation of a shunt infection or
malfunction. The more concerning signs
and symptoms the patient has, the higher
the likelihood of a VP shunt malfunction or
infection.

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agement of patients with shunted hydrocephalus. Pediatr evaluation of optic nerve diameter in children with raised
Neurosurg. 1999;30(6):288-295. (Survey; 261 respondents) intracranial pressure. J Ultrasound Med. 2005;24(2):143-147.
66. DeFlorio RM, Shah CC. Techniques that decrease or elimi- (Randomized controlled trial; 156 patients)
nate ionizing radiation for evaluation of ventricular shunts 83. Le A, Hoehn ME, Smith ME, et al. Bedside sonographic mea-
in children with hydrocephalus. Semin Ultrasound CT MR. surement of optic nerve sheath diameter as a predictor of
2014;35(4):365-373. (Review article) increased intracranial pressure in children. Ann Emerg Med.
67. Zorc JJ, Krugman SD, Ogborn J, et al. Radiographic evalu- 2009;53(6):785-791. (Prospective study; 64 patients)
ation for suspected cerebrospinal fluid shunt obstruction. 84.* Iskandar BJ, Sansone JM, Medow J, et al. The use of
Pediatr Emerg Care. 2002;18(5):337-340. (Retrospective study; quick-brain magnetic resonance imaging in the evaluation
233 patients) of shunt-treated hydrocephalus. J Neurosurg. 2004;101(2
68. Winston KR, Lopez JA, Freeman J. CSF shunt failure Suppl):147-151. (Retrospective study)
with stable normal ventricular size. Pediatr Neurosurg. 85. Boyle TP, Paldino MJ, Kimia AA, et al. Comparison of rapid
2006;42(3):151-155. (Retrospective study; 12 patients) cranial MRI to CT for ventricular shunt malfunction. Pediat-
69. O’Brien DF, Taylor M, Park TS, et al. A critical analysis of rics. 2014;134(1):e47-e54. (Retrospective study; 298 patients)
“normal” radionucelotide shuntograms in patients subse- 86. Missios S, Quebada PB, Forero JA, et al. Quick-brain mag-
quently requiring surgery. Childs Nerv Syst. 2003;19:337-341. netic resonance imaging for nonhydrocephalus indications.
(Retrospective study; 149 studies) J Neurosurg Pediatr. 2008;2(6):438-444. (Retrospective study;
70. Iskandar BJ, McLaughlin C, Mapstone TB, et al. Pitfalls in 1146 images)
the diagnosis of ventricular shunt dysfunction: radiology 87. O’Neill BR, Pruthi S, Bains H, et al. Rapid sequence magnetic
reports and ventricular size. Pediatrics. 1998;101(6):1031-1036. resonance imaging in the assessment of children with hydro-
(Retrospective study; 100 patients) cephalus. World Neurosurg. 2013;80(6):e307-e312. (Retrospec-
71. Goske MJ, Applegate KE, Boylan J, et al. The Image Gently tive study; 50 patients)
campaign: working together to change practice. AJR Am J 88. Wait SD, Lingo R, Boop FA, et al. Eight-second MRI scan
Roentgenol. 2008;190(2):273-274. (Practice guidelines) for evaluation of shunted hydrocephalus. Childs Nerv Syst.
72. Udayasankar UK, Braithwaite K, Arvaniti M, et al. Low-dose 2012;28(8):1237-1241. (Retrospective study; 44 patients)
nonenhanced head CT protocol for follow-up evaluation 89. Miller JH, Walkiewicz T, Towbin RB, et al. Improved delinea-
of children with ventriculoperitoneal shunt: reduction of tion of ventricular shunt catheters using fast steady-state
radiation and effect on image quality. AJNR Am J Neuroradiol. gradient recalled-echo sequences in a rapid brain MR imag-
2008;29(4):802-806. (Retrospective study; 92 patients) ing protocol in nonsedated pediatric patients. AJNR Am J
73. George KJ, Roy D. A low radiation computed tomography Neuroradiol. 2010;31(3):430-435. (Retrospective study; 179
protocol for monitoring shunted hydrocephalus. Surg Neurol patients)
Int. 2012;3:103. (Pilot study; 10 patients) 90. Ashley WW Jr, McKinstry RC, Leonard JR, et al. Use of
74. Pindrik J, Huisman TA, Mahesh M, et al. Analysis of rapid-sequence magnetic resonance imaging for evalua-
limited-sequence head computed tomography for children tion of hydrocephalus in children. J Neurosurg. 2005;103(2
with shunted hydrocephalus: potential to reduce diagnostic Suppl):124-130. (Retrospective study; 67 scans)
radiation exposure. J Neurosurg Pediatr. 2013;12(5):491-500. 91. Kontny U, Hofling B, Gutjahr P, et al. CSF shunt infections
(Retrospective study; 50 patients) in children. Infection. 1993;21(2):89-92. (Retrospective study;
75. Alhilali LM, Dohatcu AC, Fakhran S. Evaluation of a lim- 350 procedures)
ited three-slice head CT protocol for monitoring patients 92. Lan CC, Wong TT, Chen SJ, et al. Early diagnosis of ventricu-
with ventriculoperitoneal shunts. AJR Am J Roentgenol. loperitoneal shunt infections and malfunctions in children
2013;201(2):400-405. (Retrospective study; 231 studies) with hydrocephalus. J Microbiol Immunol Infect. 2003;36(1):47-
76. Desai KR, Babb JS, Amodio JB. The utility of the plain radio- 50. (Retrospective study; 129 patients)
graph “shunt series” in the evaluation of suspected ventricu- 93. McClinton D, Carraccio C, Englander R. Predictors of
loperitoneal shunt failure in pediatric patients. Pediatr Radiol. ventriculoperitoneal shunt pathology. Pediatr Infect Dis J.
2007;37(5):452-456. (Retrospective study; 238 patients) 2001;20(6):593-597. (Retrospective study; 81 patients)
77.* Pitetti R. Emergency department evaluation of ventricular 94. Fulkerson DH, Boaz JC. Cerebrospinal fluid eosinophilia
shunt malfunction: is the shunt series really necessary? Pedi- in children with ventricular shunts. J Neurosurg Pediatr.
atr Emerg Care. 2007;23(3):137-141. (Retrospective study; 291 2008;1(4):288-295. (Retrospective study; 93 patients)
patients) 95. Bezerra S, Frigeri TM, Severo CM, et al. Cerebrospinal fluid
78. May CH, Aurisch R, Kornrumpf D, et al. Evaluation of shunt eosinophilia associated with intraventricular shunts. Clin
function in hydrocephalic patients with the radionuclide Neurol Neurosurg. 2011;113(5):345-349. (Case report)
99mTc-pertechnetate. Childs Nerv Syst. 1999;15(5):239-244. 96. Fulkerson DH, Sivaganesan A, Hill JD, et al. Progression
(Prospective study; 85 children) of cerebrospinal fluid cell count and differential over a
79. Ouellette D, Lynch T, Bruder E, et al. Additive value of treatment course of shunt infection. J Neurosurg Pediatr.
nuclear medicine shuntograms to computed tomography 2011;8(6):613-619. (Retrospective study; 105 patients)
for suspected cerebrospinal fluid shunt obstruction in 97. Schuhmann MU, Ostrowski KR, Draper EJ, et al. The value
the pediatric emergency department. Pediatr Emerg Care. of C-reactive protein in the management of shunt infections.
2009;25(12):827-830. (Retrospective study; 69 patients) J Neurosurg. 2005;103(3 Suppl):223-230. (Retrospective study;
80. Hamburg LM, Kessler DO. Rapid evaluation of ventriculo- 59 patients)
peritoneal shunt function in a pediatric patient using emer- 98. Asi-Bautista MC, Heidemann SM, Meert KL, et al. Tumor
gency ultrasound. Pediatr Emerg Care. 2012;28(7):726-727. necrosis factor-alpha, interleukin-1 beta, and interleukin-6
(Case report)

concentrations in cerebrospinal fluid predict ventriculoperi- 2007;7:38. (Retrospective study; 258 patients)
toneal shunt infection. Crit Care Med. 1997;25(10):1713-1716. 116. Steinbok P, Milner R, Agrawal D, et al. A multicenter multi-
(Prospective observational study; 64 patients) national registry for assessing ventriculoperitoneal shunt in-
99. Scribano PV, Pool S, Smally AJ. Comparison of ventriculo- fections for hydrocephalus. Neurosurgery. 2010;67(5):1303-46.
peritoneal shunt tap and lumbar puncture in a child with (Prospective multicenter noncontrolled open-label registry;
meningitis. Pediatr Emerg Care. 2002;18(4):E1-E3. (Case 440 patients)
report) 117. Demetriades AK, Bassi S. Antibiotic resistant infections with
100. Leib SL, Boscacci R, Gratzl O, et al. Predictive value of cere- antibiotic-impregnated Bactiseal catheters for ventriculoperi-
brospinal fluid (CSF) lactate level versus CSF/blood glucose toneal shunts. Br J Neurosurg. 2011;25(6):671-673. (Prospec-
ratio for the diagnosis of bacterial meningitis following tive study; 125 patients)
neurosurgery. Clin Infect Dis. 1999;29(1):69-74. (Retrospective 118. Abramo TJ, Zhou C, Estrada C, et al. Innovative application
study; 73 patients) of cerebral rSO2 monitoring during shunt tap in pediatric
101. Conen A, Walti LN, Merlo A, et al. Characteristics and ventricular malfunctioning shunts. Pediatr Emerg Care. 2014.
treatment outcome of cerebrospinal fluid shunt-associated (Prospective case series; 94 patients)
infections in adults: a retrospective analysis over an 11-year 119. Rocque BG, Lapsiwala S, Iskandar BJ. Ventricular shunt tap
period. Clin Infect Dis. 2008;47(1):73-82. (Retrospective as a predictor of proximal shunt malfunction in children:
study; 78 episodes) a prospective study. J Neurosurg Pediatr. 2008;1(6):439-443.
102. Roberts JR. Robert’s and Hedges’ Clinical Procedures in Emer- (Prospective study; 51 patients)
gency Medicine. 6th ed. Philadelphia, PA: Elsevier Saunders; 120. Miller JP, Fulop SC, Dashti SR, et al. Rethinking the indica-
2014. (Textbook) tions for the ventriculoperitoneal shunt tap. J Neurosurg
103. Ladde JG. Central nervous system procedures and devices. Pediatr. 2008;1(6):435-438. (Retrospective study; 155 patients)
In: Tintinalli JE, Stapczynski JS, Ma OJ, et al, eds. Tintinalli's 121. Petrella G, Czosnyka M, Keong N, et al. How does CSF
Emergency Medicine: A Comprehensive Study Guide. 7th ed. dynamics change after shunting? Acta Neurol Scand.
China: The McGraw-Hill Companies, Inc; 2011. (Textbook) 2008;118(3):182-188. (Retrospective study; 25 patients)
104. Maartens NF, Aurora P, Richards PG. An unusual complica-
tion of tapping a ventriculoperitoneal shunt. Eur J Paediatr
Neurol. 2000;4(3):125-129. (Case report) CME Questions
105. Vega RA, Buscher MG, Gonzalez MS, et al. Sonographic
localization of a nonpalpable shunt: Ultrasound-assisted
ventricular shunt tap. Surg Neurol Int. 2013;4:101. (Case
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impregnated shunt catheters in decreasing the incidence of
shunt infection in the treatment of hydrocephalus. J Neuro-
surg. 2005;103(2 Suppl):131-136. (Retrospective study; 211 1. Which of the following statements regarding
patients) VP shunt mechanical and functional failures is
112. Aryan HE, Meltzer HS, Park MS, et al. Initial experience TRUE?
with antibiotic-impregnated silicone catheters for shunt-
a. Slit ventricle syndrome is relatively
ing of cerebrospinal fluid in children. Childs Nerv Syst.
2005;21(1):56-61. (Prospective study; 31 patients) rare and can present with headaches and
113. Govender ST, Nathoo N, van Dellen JR. Evaluation of an large ventricles on a CT scan.
antibiotic-impregnated shunt system for the treatment of b. Disconnections and knots in VP shunts
hydrocephalus. J Neurosurg. 2003;99(5):831-839. (Prospective cannot be detected on a shunt series.
randomized controlled trial; 110 patients) c. Changes in ventricle size are always seen
114. Kan P, Kestle J. Lack of efficacy of antibiotic-impregnated on a head CT in patients with a VP shunt
shunt systems in preventing shunt infections in children.
malfunction.
Childs Nerv Syst. 2007;23(7):773-777. (Retrospective study;
160 procedures) d. Patients with a shunt obstruction can
115. Ritz R, Roser F, Morgalla M, et al. Do antibiotic-impregnated present with headaches and vomiting.
shunts in hydrocephalus therapy reduce the risk of infec-
tion? An observational study in 258 patients. BMC Infect Dis.

2. Which of the following statements regarding a 7. Which of the following regarding the imaging
VP shunt obstruction is TRUE? of a patient with a potential shunt malfunction
a. Among VP shunt complications, they are the is TRUE?
least common VP shunt complication. a. A shunt series includes images of the head,
b. They typically occur at the reservoir. neck, chest, and abdomen.
c. There are acute and subacute signs and b. Limited-sequence MRI of the brain is not
symptoms of shunt obstruction. useful.
d. Ventricular size must be enlarged on CT to c. Head CT is useless unless there are prior
confirm obstruction. images available for comparison.
d. Shuntograms are widely used as first-line
3. A 4-year-old who had a VP shunt revision 2 imaging.
months ago presents with fever. Which of the
following physical examination findings, if 8. A patient with a VP shunt and a recent shunt
present, increases the likelihood that the pa- revision is suspected of having an infection.
tient has a shunt infection? Which of the following is the best test to con-
a. Erythema around the shunt firm this diagnosis?
b. Rapid heart rate a. Peripheral WBC count
c. Abdominal tenderness b. Shunt fluid for gram stain and cell count
d. A maculopapular rash on the extremities c. Blood culture
d. CSF eosinophils
4. A 2-year-old patient with a VP shunt presents
with fever, abdominal pain, and vomiting. You 9. A patient with a VP shunt presents with hy-
must palpate the abdomen to check for pos- pertension, bradycardia, and a decreased level
sible: of consciousness. The patient's neurosurgeon
a. Masses, such as a pseudocyst is on the way, but will arrive in about 1 hour.
b. Suprapubic tenderness due to a urinary tract What is the next best step in management?
infection a. Immediately perform a shunt tap.
c. Tenderness in the right lower quadrant from b. Do nothing until a neurosurgeon evaluates
appendicitis the patient.
d. All of the above c. Obtain peripheral access and give mannitol
5g/kg IV.
5. Which of the following is a sign or symp- d. Obtain peripheral access and start a normal
tom of a potential VP shunt obstruction in a saline bolus.
10-month-old child?
a. Bulging fontanel 10. A clinically stable patient has a VP shunt ob-
b. Decreased level of consciousness struction requiring surgery. You are an emer-
c. Increasing head circumference gency physician practicing in a local communi-
d. All of the above ty hospital ED with no in-house neurosurgeon.
What should you do?
6. You are concerned that your patient with a VP a. Admit the patient to the local community
shunt has a shunt obstruction. Clinically, he is hospital.
stable. What is the first test to order? b. Contact the child’s neurosurgeon and
a. Head CT with contrast arrange for transfer to another hospital.
b. Head CT without contrast c. Discharge the child and have him follow-up
c. Shuntogram with his neurosurgeon.
d. Shunt tap d. Perform a shunt tap to help relieve the shunt
obstruction.

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Ventriculoperitoneal Shunt Complications in Children An Evidence-Based Approach To Emergency Department Management PDF

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Ventriculoperitoneal Shunt Complications February 2016

To Emergency Department Management Jacqueline Bober, DO, FAAP

Arrows point to dilated ventricles.

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Table 2. Key Questions For Patients With A

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Table 4. Clinical Predictors Of Shunt Complications15,52,56,58-60

Strong Meningismus LR, ZD52 (shunt infection)

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Figure 5. Radiographic Images From A Shunt Series

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Patient with a VP shunt presents to the ED

History and physical examination

• Monitor ABCs Perform noncontrast head

Results consistent with

• Consult with neurosur- Are prior scans

• Consult with neurosur- YES Are ventricles slit-like?

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Patient with a VP shunt

Shunt placement in the past 6 months

Does patient have any of the following?:

Consult neurosurgery for shunt tap

CSF studies consistent

• Start antibiotics (broad-spectrum • Consider starting antibiotics in

Abbreviations: ED, emergency department; CSF, cerebrospinal fluid; VP, ventriculoperitoneal.

Class Of Evidence Definitions

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2. Perform clinical examination

4. Perform shunt tap, CSF analysisa Concern for shunt infection

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