Pediatric Perspectives - Cleveland Clinic

INSIde Safer ASd Closure device
Approved 4
Case Study: An Adolescent
with Fibromyalgia 5
Image of the Issue:
Fetal Hydrocephalus 6
Minimizing Pediatric
Radiation Risks 7
enhancing Pediatric
MS Care, Research 8
Pediatric Puzzler 9
Pediatric Perspectives
A Physician’s Newsletter from Cleveland Clinic Children’s Hospital | Spring 2008
Autism:
Significant Language
Delays, Social Skills
Setbacks Warrant
Evaluation Story on page 2

Early Diagnosis of Autism Critical
When a child’s behavior, verbal and nonverbal communication and social skills are not
in line with normal development, pediatricians should include autism in the differential
diagnosis, say Cleveland Clinic Children’s Hospital psychologists.
“The importance of identifying autism early cannot be
stressed enough. Successful treatment and cost savings
are greatly enhanced when the diagnosis is made before
age 3, and preferably by 18 months. The brain is plastic
and receptive during this window of time. The longer
we wait, the more precious learning time we lose,” says
Thomas Frazier, PhD. He and Julie Knapp, PhD, see
patients at the Cleveland Clinic Center for Autism, on
the Shaker Campus of our Children’s Hospital.
Autism is a lifelong brain disorder that begins in early
childhood, affecting critical development in communication,
socialization and behavior. It is four times more
common in boys than girls and crosses all racial, ethnic
and social boundaries.
Because autism varies widely in severity and symptomatology,
it can be extremely difficult to diagnose. Autism
is not necessarily connected to intelligence, and children
with high social interest can have autism, notes Dr.
Frazier. “If children on the autism spectrum are diagnosed
and receive intensive early intervention, up to 30
or 40 percent can be placed in a regular school,” he adds.
What Pediatricians Can do
Pediatricians should pay particular attention to delays
or regression in verbal and nonverbal communication,
self-care and social skills, says Roberta Bauer, MD, a developmental
pediatrician who sees children in the Center
for Autism.
“Pediatricians may identify young children with autism
spectrum concerns during the history and physical.
Children with a narrow range of food preferences,
unusual visual behaviors (looking very closely at toys
during play or out of the corner of their eyes), or hypo- or
hypersensitivity to sound, smell, taste or touch, deserve
further probing.”
She references a report in the March 2008 issue of
Pediatrics in Review recommending clinical probes at
the 12- to 15-month checkups. Pediatricians can ask
parents to describe a typical day, for instance, noting
references to solitary or repetitive play, or to chasing and
“rough-housing” behaviors that reflect sensory motor
stimulation rather than social interaction.
Indications for Referral
Other, age-dependent early warning signs for autism include:
6 months and older: Does not enjoy cuddling, lacks social
smiling, seems aloof (does not share eye contact or notice
when you speak), lacks shared attention with parent
12 months and up: Prefers to play alone, lacks “giving
behavior,” does not show objects to share interest, fails
to follow a point, does not respond to name, seems to live
in “own world,” is unusually attached to objects, does not
babble, point or use other gestures
18 months and up: Plays with toys in functionally inappropriate
ways (lines them up, spins them, views them from corner
of eye), seeks out sensory stimulation, displays unusual fears
or fearlessness, uses repetitive movements (body rocking,
head banging), displays obsessive/fixed behavior
Older ages: Does not attempt to engage caregiver’s attention
in interesting object; has difficulty understanding others’
clear feelings, beliefs or intentions; tends to be aggressive or
self-injurious; resists changes in routine or environment; is
over- or under-sensitive to stimuli; has limited or exaggerated
facial expressions.
Page 2 | Pediatric Perspectives | Spring 08 | Cleveland Clinic’s toll-free physician number is 800.553.5056

Treating Autism at Cleveland Clinic
Dr. Frazier notes that “our program is highly successful in
improving children’s function. Many children come to us
not speaking and with highly impaired social skills. After
a few years of treatment, some show substantial improvement
in their ability to fit in with peers and are placed in
mainstream schools. Others show moderate gains that
are still critical for lifelong function and quality of life.”
The Center’s multifaceted program encompasses
diagnosis, treatment, research, outreach and training.
At its heart is the Lerner School for Autism, which
serves patients from 15 months to 23 years of age using
applied behavioral analysis in individualized education
programs. The student-to-teacher ratio is 1:1 or 1:2,
depending on needs.
“We try to determine which behaviors interfere with a
child’s progress, what the child needs to compensate for
them, and how to get them to be functional communicators
and to learn. Our purpose is to make their lives better
and to improve the family’s quality of life,” says Center
Director Leslie Sinclair.
Learning to Look Someone in the eye
Subjects range from language, anatomy, physiology and
algebra, to teaching skills such as how to look someone
in the eye and how to use the Internet to find a job.
Changes to education plans are always evidence-based.
Due to the complexity of learning difficulties across the
autism spectrum, data are gathered and analyzed daily.
The staff also helps families identify resources close to
home and sources of financial assistance.
Contact Information
Dr. Julie Knapp is available to
evaluate children 15 months
of age and older for autism.
Pediatricians may contact
her at 216.448.6421 or at
knappj2@ccf.org.
Autism School Expands On May 8, 2008, the state-of-the-art
Lerner School for Autism opened in the new Debra Ann November
Wing of the Cleveland Clinic Center for Autism. The school on our
Shaker Campus can now serve nearly 100 students, making it one
of the country’s largest autism education and treatment programs.
Visit clevelandclinic.org/childrenshospital for more information.
Visit clevelandclinic.org/childrenshospital
Dear Colleague:
Many things set our Children’s Hospital apart
from other pediatric facilities. We’re an integral
part of a world-renowned organization where
more than 200 pediatricians and specialists
form the foundation of a “virtual” Children’s
Hospital within Cleveland Clinic. Together, we
offer patients and families 24/7 access to worldclass
resources and research expertise.
Our collaborative, multidisciplinary approach
and secure electronic medical records system
gives pediatricians, regardless of location, the
opportunity to partner with our specialists to
provide the best care for patients and families
— whether it’s at our Main Campus Children’s
Hospital, our many community hospitals and
family health centers in Northern Ohio or in
their home town.
Our vision is to use innovation, discovery and
partnership to ensure superior, comprehensive
healthcare for children. For example, in our Fetal
Care Center, high-risk obstetricians, neonatologists
and pediatric specialists offer a wide
range of interventions to yield the best possible
outcome; our pediatric cardiologists have even
improved fetal cardiac blood flow in utero.
Our Children’s Hospital also serves as the only
comprehensive pediatric transplant center in
Northern Ohio, offering heart, lung, liver, pancreas,
bowel and kidney transplantation.
When infants and children are critically ill,
our PICU and NICU staff are standing by at all
times. Our units are staffed 24 hours a day by
on-site, board-certified intensivists and neonatologists
who provide leading-edge interventions,
including ECMO.
We hope you will keep our services in mind if
one of your patients should require acute or
chronic care, and we look forward to partnering
with you in the future. Meanwhile, please enjoy
this issue of Pediatric Perspectives.
Sincerely,
Robert Wyllie, MD
Physician-In-Chief, Cleveland Clinic Children’s
Hospital
Calabrese Chair of Pediatrics, Cleveland Clinic
Chairman, Cleveland Clinic Pediatric Institute
| Pediatric Perspectives | Spring 08 | Page 3

Transcatheter closure of ostium secundum atrial septal defects
(ASDs) is now safer, thanks to a softer, more flexible septal
occluder. Cleveland Clinic pediatric cardiologists regularly utilize
the device — the Helex Septal OccluderTM — to permanently
close holes between the atria in an outpatient procedure.
“The Helex has no sharp points and is unlikely to cause trauma to
the heart,” says pediatric cardiologist Larry Latson, MD, Director
of the Pediatric and Congenital Heart Disease Center’s Cardiac
Catheterization Laboratory in Cleveland Clinic Children’s Hospital.
“The only other device approved in the United States for ASD
closure has a small but not insignificant risk of eroding through
the heart. This has never happened with the Helex.”
The device was approved by the Food and Drug Administration
(FDA) in October 2007 and has been implanted in an estimated
1,000 patients to date. Dr. Latson consulted on the Helex design
and acted as national Principal Investigator for its clinical trials;
he also has a patent on a similar device.
Outpatient Repair
Outpatient repair of ASDs via cardiac catheterization has been
done for nearly two decades. Children generally recover rapidly
and have only a tiny scar in the groin. Most are able to resume
reduced activities the following day and full activity within a week.
The minimally invasive approach is preferred, because ASDs are
often asymptomatic and are discovered accidentally through a
chest X-ray or echocardiogram. The only clue may be a subtle
murmur. Left untreated, ASDs can lead to stroke, right heart failure
or arrhythmia, and reduce the lifespan by 15 to 20 years.
“Parents are usually shocked at the diagnosis, and the concept of
their child undergoing open heart surgery is very frightening. Outpatient
ASD repair is not so scary,” says Dr. Latson.
CME
Safer Minimally Invasive
ASD Closure Device Approved
How the Device Works
The Helex occluder consists of a circular frame made of NitinolTM (nickel titanium), which is covered by a thin membrane of ePTFE.
This form of Gore-TexTM is also used to patch holes in the heart
surgically.
The device is delivered through a hollow catheter advanced
through the veins to the defect in the atrial septum. It is placed
across the defect, positioned to cover the hole, and released from
the delivery catheter to block shunting of blood between the atria.
Over subsequent weeks, cells infiltrate and grow over the membrane,
making it a permanent part of the atrial septum.
Unlike some previous designs, the Helex can be easily manipulated
to ensure optimal coverage. “If you don’t like the fit after the
device has been deployed, you can pull it back into the catheter,
reposition it and redeploy it,” Dr. Latson advises.
Ideal for Smaller Hearts
Its small size makes it ideal for use in smaller hearts. “You
don’t have to worry about it interfering with other structures in
the heart,” says Lourdes Prieto, MD, another Cleveland Clinic
Children’s Hospital cardiologist who uses the Helex.
“This particular type of occluder is challenging for practitioners to
learn to use well,” notes Dr. Latson. “But the advantages to the
patient are worth it.”
Contact Information
To reach the Cleveland Clinic Center for Continuing Education, call
216.444.5696 or 800.762.8173, or visit clevelandclinicmeded.com.
6th Annual Pediatric Neurology
Update Seminar: 2008
Sept. 5, 2008, Executive Caterers at
Landerhaven, Mayfield Heights, Ohio
14th Annual Pediatric Board
Review Symposium
August 25-29, 2008, InterContinental
Hotel & Bank of America Conference
Center, Cleveland Clinic Campus
To refer patients to Drs. Latson and Prieto or colleagues at
the Center for Pediatric and Congenital Heart Disease, call
216.445.6532.
Photo courtesy of W.L. Gore & Associates, Inc.
Children’s Hospital
Grand Rounds
Community physicians are welcome to earn
CME credit at our Pediatric Grand Rounds.
They are held on Tuesdays from 8 to 9 a.m.,
September through May, in Bunts Auditorium
(TT building, on East 90th Street at our
main campus). To view upcoming speakers
and topics, visit clevelandclinicmeded.com/
pedcme, or call 216.444.5510 or email
kozlowa@ccf.org for details.
Page 4 | Pediatric Perspectives | Spring 08 | Cleveland Clinic’s toll-free physician number is 800.553.5056

Case Study:
Adolescent Learns to
Cope with Fibromyalgia
When chronic pain interferes with a pediatric patient’s daily life,
including school and activities, the Pain Rehabilitation Program at Cleveland
Clinic Children’s Hospital for Rehabilitation serves as a valuable resource.
Interdisciplinary evaluation and treatment take place during a two-week
inpatient and one-week day hospital experience. The core team, a pediatric
physiatrist, psychologist, nurse practitioner, hospitalist, social worker, and
physical and occupational therapists, consults with the appropriate pediatric
specialists. Together, they tailor a treatment plan with individualized goals that
blends intensive behavioral therapy with a rich mix of physical, occupational,
recreational and aquatic therapy. Teachers keep patients current with
schoolwork, and families are taught to cope with their child’s pain.
The following case illustrates how the
Pediatric Pain Rehabilitation Program has
helped to restore one adolescent to a more
normal life.
History prior to admission: A college sophomore
from New York State has a three-year
history of diffuse, disabling pain following a
diagnosis of ankylosing spondylitis. She has
had minimal relief from extensive medical
therapy, physical therapy and acupuncture,
and her course has been complicated by
periodic hospitalizations for pain and intolerance
to multiple medications. During her
freshman year, moderate pain interrupted
her sleep, and 25 mg of subcutaneous etanercept
were prescribed. Six months later,
that dose was increased, nortriptyline was
added and fatigue-induced fibromyalgia
was diagnosed. Due to progressive pain
and sleep difficulties, she dropped out of
college in the fall of sophomore year.
Two-week inpatient course: The patient is
admitted to the Pediatric Pain Rehabilitation
Program. She is evaluated by pediatric
psychologist Gerard Banez, PhD, pediatric
physiatrist Doug Henry, MD, pediatric nurse
practitioner Judy Hall, PNP, pediatric hospitalist
Margarita Neyman, MD, and a social
worker, physical therapist and occupational
therapist. A pediatric rheumatologist and
pain management specialist join the team.
Each team member contributes to a customized
rehabilitation plan. Its success will
depend on the patient’s commitment to
living a normal life with pain and her willingness
to confront pain-specific fears. She
starts a rigorous program of daily physical,
occupational, aquatic and recreational
therapy. Her core muscle strength, endurance
and ability to walk at a normal pace
quickly improve. In individual and group
psychotherapy, she learns self-regulation
strategies for coping independently with
pain, and stress management skills to help
her handle life’s pressures.
Behavioral health specialist Gerard
Banez, PhD, is Director of the Pediatric
Pain Rehabilitation Program.
A pain relapse occurs during her menses
and is refractory to physical therapy.
Pediatric pain management specialist
Samer Narouze, MD, prescribes a
Lidoderm ® 700-mg patch just before
discharge.
One-week day hospital course: At the start
of her day hospital program, the patient
reports that Lidoderm ® has been effective.
She continues to gain strength and
endurance, and demonstrates an ability to
cope with pain while continuing outpatient
therapies.
During this time, pediatric rheumatologist
Steven Spalding, MD, renders a second
opinion. An MRI of her pelvis and hips
is negative for ankylosing spondylitis; he
concurs with the diagnosis of fibromyalgia
(although verification would require discontinuation
of etanercept).
Meanwhile, a discharge plan is developed,
through meetings with her parents and
contact with outpatient providers close to
home. This will facilitate a smooth transition
to home and school and help the
patient continue to progress.
Discharge summary: The patient has
achieved the goals set forth by her team.
She can navigate without gait deviation or
assistive devices, use her upper extremities
functionally throughout the day, complete
daily exercise and sensory programs independently,
and demonstrates the ability to
use relaxation strategies and coping skills.
The patient believes she has the necessary
tools to cope with flares and, optimistic
about her ability to handle stress, plans to
re-enroll in college.
Contact Information
If you have a young patient in chronic
pain who might benefit from the blend
of rehabilitation, subspecialty care
and behavioral therapy available in our
Pediatric Pain Rehabilitation Program,
please contact Kristen Buchannan, LISW,
at 216.448.6158 or at 800.635.2417.
| Pediatric Perspectives | Spring 08 | Page 5

Image
of the Issue
X-Linked Aqueductal
Hydrocephalus
By Neil Friedman, MBChB
Fetal MRI is a useful adjunct to fetal ultrasound
in assessing fetal anatomy and pathology. We
are now able to acquire high-resolution, single
images in less than 1 second, overcoming the
barrier of fetal movement artifact. This is due
to the emergence of higher field-strength 1.5
Tesla MRIs and ultrafast imaging sequence
techniques, such as echo planar imaging and
single-shot, fast-spin echo.
Fetal MRI is increasingly performed for better
definition and characterization of developmental
and acquired fetal central nervous system
anomalies. Detailed, precise structural and
maturational information about the developing
brain allows for significantly better stratification
of “at risk” fetuses with respect to outcome
and potential developmental problems.
Fetal Hydrocephalus: A Case in Point
Fetal hydrocephalus is estimated to occur in
0.05 to 0.3 percent of all pregnancies. Causes
include congenital infections, prior intraventricular
hemorrhage, developmental brain
anomalies such as aqueductal stenosis, genetic
syndromes such as X-linked aqueductal stenosis
and, rarely, congenital brain tumors.
Each type of fetal hydrocephalus carries its
own prognosis and developmental risks. Fiftyfour
to 84 percent of cases are associated with
cranial and extracranial abnormalities. Normal
Axial view shows severe,
dilated ventricles and thin,
compressed brain cortex.
Sagittal true FISP (Fast
Imaging with Steady State
Precession) reveals adducted,
or clasped, thumbs (circled)
and normal fourth ventricle
(arrow), suggesting obstruction
at the aqueduct of Sylvius.
Image of male scrotum
confirms gender.
cognitive outcomes are seen in 16 to 68 percent
of survivors, depending upon the cause.
In this case, fetal MRI allowed us to diagnose
fetal hydrocephalus secondary to X-linked aqueductal
stenosis, due to an L1CAM gene mutation.
Sharing a precise diagnosis and prognosis
with the family allowed them to better prepare
for issues arising at delivery and in the future.
Improved counseling leads to better-informed
decisions by patients about their pregnancies;
occasionally helps to plan the mode, place and
type of delivery; and facilitates perinatal care,
because patients are mentally prepared for
potential risks, problems and interventions in
the newborn period.
Author Contact Information
Dr. Neil Friedman is a Children’s Hospital neurologist
specializing in fetal and neonatal neurology, pediatric
neuromuscular disease, pediatric stroke and neurological
complications of pediatric congenital heart disease.
Pediatricians may reach him at 216.444.6772 or at
friedmn@ccf.org. He collaborates with Janet Reid, MD,
Director of our Pediatric and Fetal MRI Center.
Page 6 | Pediatric Perspectives | Spring 08 | Cleveland Clinic’s toll-free physician number is 800.553.5056
t
t

Minimizing Radiation Dangers
to Pediatric Patients
Cleveland Clinic Children’s Hospital radiologists consider every imaging
request a challenge to find a method of obtaining the optimal image with
the least amount of radiation — or to pursue a radiation-free alternative.
“We believe it’s our duty to optimize communication
between the clinicians who order the studies and the
pediatric radiologists who perform them. Our patients
are our prime concern, and all of us have their best
interests at heart,” says Janet Reid, MD, Head of the
Section of Pediatric Radiology, pictured above. She also
directs the dedicated Pediatric and Fetal MRI Center at
Cleveland Clinic Children’s Hospital.
Concern about radiation is well-founded, Dr. Reid notes.
Follow-up data from the 1945 atomic bomb survivors
showed an increase in cancer among people living on the
periphery of the explosion. Those people received a uniform
dose of radiation similar to the dose adults receive
during an abdominal CT scan.
Ionizing Radiation’s effect on Children
“Radiation is particularly harmful during the growth
stages, when cells are rapidly dividing. Thus, any ionizing
radiation can be more toxic to children,” she explains.
“When used judiciously, radiation is helpful. Overexposure
is harmful, and underexposure can miss
pathology, requiring tests to be repeated. Repeat tests
expose patients to double the dose of radiation.”
CT: A Potent Form of Radiation
CT scans are particularly problematic because radiation is
delivered 360 degrees around the patient, at doses much
higher than those of chest X-rays. More than 4 million CT
scans were performed on pediatric patients in 2006.
The potential ramifications are so great that an alliance
of radiation professionals initiated a campaign to stress
Visit clevelandclinic.org/childrenshospital
the importance of “child-sizing” the amount of radiation
used (see www.imagegently.org). For five years, Children’s
Hospital radiologists have accomplished this using a
weight-based radiation protocol. Cleveland Clinic pediatric
radiologists avoid performing repeat scans by taking
the time to ensure that optimal images are obtained the
first time. “We try never to scan twice,” Dr. Reid stresses.
‘Child-Size’ Radiation doses
They also work proactively to identify excessive radiation
risks. One example is CT enterography, a study that is
replacing small bowel follow-through in the investigation
of inflammatory bowel disease. The studies produce
superior images of the bowel, but data in adults recently
published in the American Journal of Radiology suggests
they may produce up to five times the radiation exposure
of the traditional small bowel follow-through.
Dr. Reid and colleagues have found that using weightbased
protocols can reduce the amount of radiation to
the levels incurred during a traditional barium study,
making these more accurate studies safer for infants and
children.
Contact Information
Cleveland Clinic Children’s Hospital radiologists are pleased to
consult with pediatricians to ensure that the most appropriate and
safe studies are ordered for young patients Physicians may contact
Dr. Reid directly at 216.445.2999 or at reidj@ccf.org.
| Pediatric Perspectives | Spring 08 | Page 7

Adrenoleukodystrophy
Axial FLAIR MRI shows
hyperintense white
matter lesions in the
periventricular and
posterior deep white
matter region.
Children’s Hospital Link to
Adult MS Center Enhances
Care for Pediatric MS
Childhood multiple sclerosis (MS) is being diagnosed in increasing
numbers of children and adolescents. More than 25,000 individuals
under age 18 in the United States exhibit symptoms that mimic MS.
By Manikum Moodley, MBChB, FCP, FRCP
With its protean clinical manifestations and
lack of biological markers, MS is easy to misdiagnose.
In children, correct diagnosis is an
even greater problem because MS is uncommon
and various genetic and neurometabolic
disorders also produce active neurological impairment
and white matter changes on MRI.
Disorders that may be mistaken for MS in
children include:
• acute disseminated encephalomyelitis
• leukodystrophies
• mitochondrial defects
• organic and amino-acidemia
• obscure vasculopathies, collagen
vascular diseases
diagnostic Implications in Children
Correct diagnosis of metabolic and genetic
diseases is critical in children because of the
many implications not only for their immediate
care but also for determining long-term
prognosis and the need to identify at-risk
family members. In addition, early diagnosis
and treatment may prevent or delay the development
of disability. The potential to treat
MS with disease-modifying agents has also
changed the prognosis of MS significantly.
Our Pediatric White Matter Disorders
Program, within the Department of Pediatric
Neurology at our Children’s Hospital, sets
the highest standard for pediatric MS care. We
are closely aligned with the Mellen Center for
Multiple Sclerosis Treatment and Research,
the well-established center for adults with MS
on our campus. This arrangement makes stateof-the-art
MS care available to young patients,
who can be seen by a multidisciplinary team of
pediatric neurologists, metabolic and genetic
specialists, and pediatric neuroradiologists
with expertise in white matter diseases.
Child-Centered Care, Adult expertise
Most children with MS are cared for either in
adult MS clinics or at general pediatric centers.
This leaves children and adolescents with
MS underserved. While adult MS clinics have
expertise in managing MS, they often lack experience
in the care of patients with significant
developmental needs. While pediatric centers
have the expertise to distinguish MS from con-
Page 8 | Pediatric Perspectives | Spring 08 | Cleveland Clinic’s toll-free physician number is 800.553.5056

genital and acquired white matter disorders
unique to children, they lack expertise specifically
related to MS because of the limited number
of patients they see. Special programs, expertise
and research in MS are limited as well.
Important Research Considerations
Limitations on research are of particular concern
because the onset of MS in children may
be more proximate to precipitating factors
than in adult MS patients. In addition, because
genetic risks may be greater in pediatric MS,
the need — and opportunities — for research
are greater in the pediatric population than in
the adult setting.
Visit clevelandclinic.org/childrenshospital
Multiple Sclerosis
Axial FLAIR MRI shows
extensive hyperintense
white matter lesions
in the periventricular
region, corpus callosum
and deep white matter.
The etiology and pathogenesis of MS remain
unknown, although it is one of the most common
and most studied neurologic illnesses in
adults. We hope to gather critical data that will
help researchers worldwide better understand
the epidemiology, pathobiology and clinical
outcome of MS in children and adolescents.
Author Contact Information
For more information or to refer a child to the
Pediatric MS and White Matter Disorders Program at
our Children’s Hospital, physicians may contact Dr.
Moodley at 216.444.3135 or at moodlem@ccf.org, or
our scheduler at 216.445.7089.
Pediatric Puzzler
By John DiFiore, MD
Case History: A 15-year-old female with a two-year
history of chronic abdominal pain underwent Ladd’s
procedure for intestinal malrotation at another hospital,
along with hepatic wedge resection for a 3-cm mass
that proved to be focal nodular hyperplasia. After initial
improvement, her pain recurred, and she underwent
surgical lysis of adhesions with no relief. The pain was
determined to be psychiatric in origin and no further
workup was pursued. The patient’s pain became so
severe that she became suicidal. She presented to us
in visible discomfort, describing “sharp, constant and
severe” pain, particularly in the right upper quadrant
and mid-epigastrium. The pain worsened significantly
after eating and with increased physical activity. She
had mild mid-epigastric and right upper-quadrant
tenderness. Turn to page 11 for diagnosis.
Online Services for Physicians
eCleveland Clinic Second Opinions
Request a remote second medical opinion from Children’s
Hospital specialists through the secure eCleveland Clinic
MyConsult Web site. Pediatric cardiologists, hematologists/oncologists,
orthopaedists, and endocrine and
growth disorder specialists will provide detailed second
opinions within five to seven days. We also offer preadoption
healthcare evaluations.
Visit eclevelandclinic.org/myConsult.
Tracking Your Patient’s Care Online
Whether you are referring from near or far, our eCleveland
Clinic service, DrConnect, allows you to track your
patient’s treatment progress online, via a secure website.
Visit eclevelandclinic.org or e-mail drconnect@ccf.org.
Network Serves Referring Physicians
The Pediatric Physician Network enhances service and
communication among Cleveland Clinic Children’s Hospital
staff and referring physicians, offering 24/7 phone and
email access.
Phone: 216.444.DOCS (3627)
Email: pedsnetwork-md@ccf.org (for physician concerns)
pedsnetwork-admin@ccf.org (for administrative questions)
| Pediatric Perspectives | Spring 08 | Page 9

What to Advise Families
Prior to a Child’s
Hospitalization
Bring a complete list of
medications being taken
by the patient, or bring the
medications themselves.
Speak up when you have
questions.
Participate in your child’s daily
visit(s) from clinicians.
Take advantage of all opportunities
for education while you
are here.
Ask about the “handoff” to
the primary care provider at
discharge.
Making Patient Safety a
Top Priority
By Shannon Phillips, MD, MPH
Every day, infants, children and adolescents visit the nation’s hospitals for everything
from a primary care visit to an organ transplant. Patient safety is a top concern for physicians
at every facility. Cleveland Clinic Children’s Hospital has taken steps to ensure
that every aspect of the care we provide is as safe as possible. Many of these ideas may
be useful at other facilities.
Because prepared, educated patients and families are crucial to safety, we identified the
key information every family needs to know when a patient is admitted to the hospital.
An informative eight-minute video is now shown to each patient/family on admission.
Another useful tool implemented at Children’s Hospital is to have executive leaders
routinely walk through patient care areas. They ask the front-line staff, “What might
harm the next patient?” or “What could we do better to keep our patients safer?” Their
answers have led to countless improvements in safety and quality.
Involving Families on Rounds
Putting the patient and family at the very center of care is also crucial. On our Pediatric
Hospital Medicine service, the caregiving team meets at the bedside daily to discuss
physical exam findings, test results and caregiver impressions. The result is a plan of
care for the patient for the day, and for the hospitalization. This keeps families empowered
and involved.
ensuring Accurate Medication Orders
Millions of medication-related errors occur each year. To help reduce these errors, we
developed a medication-ordering form that requires weight-based dosing logic to be
documented. This reinforces a best practice for all of our physicians.
Electronic medical records also optimize safety. In 2008, Children’s Hospital will transition
to electronic order entry and complete the transition to inpatient documentation.
Pediatric-specific drug files are being validated and our physicians are developing order
sets and documentation templates to bring quality and safety measurement to a new level.
Creating Care Pathways
To help standardize care, all of our departments are developing pathways of care for the
most common presentations. The Center for Pediatric Hospital Medicine has treated
asthma using a pathway based on the current best evidence from the National Heart,
Lung and Blood Institute (http://www.nhlbi.nih.gov/guidelines/asthma/asthgdln.pdf).
This process results in reduced length of stay, reduced cost and no increase in readmissions
to the hospital. Working from an order set for these patients also reduces medication-prescribing
errors.
Author Contact Information
Dr. Phillips, a pediatric hospitalist, is Cleveland Clinic’s Patient Safety Officer. Pediatricians may
reach her by email at phillis@ccf.org or at 216.444.4998.
Cleveland Clinic’s toll-free physician number is 800.553.5056

Pediatric Puzzler Continued from page 9
diagnosis and Management: In an extensive
workup, chest and abdominal CT
showed persistent abnormal signal in the
left lateral liver segment at the site of prior
resection. An upper GI series showed no intestinal
obstruction. Upper endoscopy and
colonoscopy were normal. Ultrasound of
the abdomen, pelvis and gallbladder were
normal. No gallstones were evident. Gallbladder-emptying
time was normal. Gastric
emptying time was normal for liquids and
solids. All blood work was normal.
Because her pain worsened after meals
and with activity, intestinal vascular insufficiency
was considered as a source. A CT
angiogram showed severe compression of
her celiac artery with a classic “hooked”
appearance.
The patient underwent surgical decompression
of the celiac artery and division of
the median arcuate ligament. A partial left
lateral segmentectomy was performed for
excision of residual focal nodular hyperplasia,
along with a re-do Ladd’s procedure
and cholecystectomy.
She experienced immediate, complete relief
from abdominal pain. She is eating normally,
is back in school and has returned to
How to Reach a Children’s Hospital Specialist
Call 216.444.DOCS (3627) or
800.553.5056 for
Hospital Transfers and Admissions
(Main Campus)
Critical Care Transport, 24/7
Outpatient Referrals/Consultations (Main
Campus, Fairview or Hillcrest Offices)
Partners in Practice (Pediatric Hospital
Medicine Service)
Pediatric Physician Network, 24/7
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horseback riding, dancing and other favorite
activities. She has completely discontinued
all psychiatric and pain medications.
Celiac artery compression syndrome, or
median arcuate ligament syndrome, is
an unusual cause of chronic intestinal
vascular insufficiency. The celiac artery
is compressed by the lower crura of the
diaphragm, likely due to a congenital, abnormally
low position of the artery or to an
abnormally high position of the ligament.
Clinical Pearl: While patients with chronic
abdominal pain generally require extensive
workup to exclude more common etiologies,
postprandial pain and increasing pain
with activity may be tipoffs to celiac artery
compression syndrome. CT angiography is
diagnostic, and surgery is highly curative.
Author Contact Information
Dr. DiFiore, a pediatric general and thoracic
surgeon, is Director of our Center of Excellence
for the Minimally Invasive Repair of
Pectus Excavatum and Surgical Director
of our Fetal Care Center. He specializes in
congenital malformations, pediatric tumors
and the Nuss procedure. Pediatricians may
contact Dr. DiFiore at difiorj1@ccf.org.
Call 216.448.6035 or
800.635.2417 for
Children’s Rehabilitation Hospital
Admissions (Shaker Campus)
Call 216.448.6179 or
800.635.2417 for
Shaker Campus Outpatient Referrals
Families can call 216.444.KIDS (5437)
for appointments.
Illustrations show normal
celiac artery (left) versus
celiac artery compression by
median arcuate ligament of
the diaphragm (right).
Pediatric Perspectives offers updates on
diagnosis and management from specialists
at the Cleveland Clinic Pediatric Institute
and Children’s Hospital at 9500 Euclid Ave.,
Cleveland, OH 44195.
Chairman Robert Wyllie, MD, welcomes your
feedback at 216.444.2237 or wyllier@ccf.org.
Please direct correspondence to Editorial
Board Co-Chairs:
Rita M. Pappas, MD
pappasr@ccf.org, 216.444.4998
Jeffrey S. Palmer, MD
palmerjs@ccf.org, 216.445.7504
Editorial Board: John DiFiore, MD, Thomas
Edwards, MD, Rita Pappas, MD, Jeffrey
Palmer, MD, Kathy Whitford, PNP
Editor: Cora M. Liderbach
Art director: Amy Buskey-Wood
Photographer: Tom Merce
Medical illustrator: Beth Halasz
The Pediatric Institute and Children’s Hospital
is one of 26 institutes at Cleveland Clinic that
group multiple specialties together to provide
collaborative, patient-centered care. Backed
by the full resources of Cleveland Clinic, the
institute offers comprehensive medical, surgical
and rehabilitative care for infants, children
and adolescents. More than 200 pediatric
physicians accommodate 500,000 patient
visits annually at our Main Campus, Shaker
Campus, community hospitals and family
health centers. Cleveland Clinic is a nonprofit
multispecialty academic medical center.
Founded in 1921, it is dedicated to providing
quality specialized care and includes an
outpatient clinic, a hospital with more than
1,000 staffed beds, an education institute
and a research institute.
Pediatric Perspectives is written for physicians
and should be relied upon for medical
education purposes only. It does not provide a
complete overview of the topics covered, and
should not replace a physician’s independent
judgment about the appropriateness or risks
of a procedure for a given patient.
© The Cleveland Clinic Foundation 4/08
| Pediatric Perspectives | Spring 08 | Page 11

The Cleveland Clinic Foundation
Pediatric Perspectives
9500 Euclid Avenue/W14
Cleveland, OH 44195
Pediatric Vasculitis Center Offers
Access to International Experts
Because of its rarity in children, vasculitis — a broad
term describing a large group of autoimmune diseases
characterized by blood vessel inflammation — may
persist unrecognized for years.
“Left untreated, the vasculitides can produce significant morbidity
and mortality,” says Steven Spalding, MD, a pediatric rheumatologist
at Cleveland Clinic Children’s Hospital. He and Philip Hashkes,
MD, MSc, Head of the Center for Pediatric Rheumatology,
see children in Cleveland Clinic’s nationally renowned Center for
Vasculitis Care and Research, established in the 1990s.
Over the past five years, more than 80 pediatric patients have
been treated in the Center. The two most common types of vasculitis
affecting children are Kawasaki disease and Henoch-Schönlein
purpura.
“Other types of vasculitis — including Takayasu’s arteritis, Wegener’s
granulomatosis, microscopic polyangiitis, polyarteritis nodosa,
Behçet’s disease, Churg-Strauss syndrome and CNS vasculitis —
are only rarely encountered in children, even by pediatric rheuma-
About the Physicians
Steven Spalding, MD, sees children
with vasculitis, joint pain, juvenile
arthritis and recurrent fevers.
Philip Hashkes, MD, MSc, specializes
in pediatric vasculitis as well
as drug therapy for arthritis and
autoinflammatory (periodic fever)
syndromes.
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tologists,” notes Dr. Hashkes. “Such children are often referred to
Cleveland Clinic so that we can assist with their care.”
Pediatric Signs and Symptoms
Dr. Spalding notes that “manifestations of vasculitis in children
typically include unexplained, persistent fevers; weight loss;
malaise; and signs of gastrointestinal, pulmonary, renal, musculoskeletal
or skin inflammation. However, symptoms and signs
vary by disease.”
To manage these complex problems in children, pediatric rheumatologists
work with a team of international vasculitis experts
to provide the most advanced therapies available. They also collaborate
as needed with other Cleveland Clinic subspecialists,
including:
• otolaryngologists specializing in the treatment
of subglottic stenosis resulting from Wegener’s
granulomatosis,
• vascular surgeons experienced in managing stenotic or
aneurysmal changes in vessels affected by vasculitis,
• neurologists specializing in the diagnosis and treatment
of CNS vasculitis, and
• radiologists with expertise in noninvasive and invasive
vascular imaging techniques.
Access to Clinical Trials
Children seen in the Center for Vasculitis Care and Research have
access to groundbreaking research, ranging from epidemiologic
studies to innovative therapeutic trials. Current studies open to
children include a Pediatric Vasculitis Registry, and longitudinal
biomarker studies, in which clinical data are collected and blood
and urine samples are studied in collaboration with Vasculitis
Clinical Research Consortium members.
Referring pediatricians are notified of all evaluations and recommendations
for their patients. Families are encouraged to sign up
for eCleveland Clinic MyChart, a secure online health management
tool supported by our electronic medical record system, which
gives them online access to their child’s laboratory information.
“We also work closely with the Vasculitis Foundation, which provides
valuable education and family support,” says Dr. Spalding.
For consultations and referrals with our pediatric rheumatologists, please call 216.445.8525.