Pediatric Perspectives Spring 2007 - Cleveland Clinic

In This Issue Minimally Invasive Surgery
for Vascular Ring 2
Acute Leukemia Update 4
Vascular Ring:
Minimally Invasive Repair
in Premature Infant
Story on page 2
CT Enterography:
Unparalleled Views 6
Advances in IBD
Research 7
Surface EMG for Motor
Re-education 9
New Surgery for Hip
Impingement 10
Pediatric Perspectives
A Physician’s Newsletter from Cleveland Clinic Children’s Hospital | Spring 2007

Dear Colleague:
This edition of Pediatric Perspectives finds
Cleveland Clinic Children’s Hospital more
active and involved than ever before. We had
more than 5,500 admissions in 2006 and
transported over 1,000 children here for spe-
cialized care from throughout Northeast Ohio
and 13 other states. We also recorded more
than 130,000 outpatient visits on our main
campus, coupled with 240,000 pediatric visits
at our regional Children’s Hospital facilities.
Colleagues in Northeast Ohio and around
the country are taking notice — we were very
pleased to be ranked one of the best pediatric
hospitals in America (U.S.News & World Report
2006), and 53 Children’s Hospital specialists
are recognized in “Best Doctors in America.”
Our staff is growing to meet the demands of
our referring physicians as well as our patients
and families. The Division of Pediatrics and
Children’s Hospital announced four new
department chairs recently: David Magnuson,
M. D., General Surgery; Gerard Boyle, M.D.,
Pediatric Cardiology; Steve Davis, M.D.,
Pediatric Critical Care Medicine; and Elaine
Schulte, M.D., General Pediatrics. More than
20 other subspecialty physicians joined our
staff as well, adding important clinical skills
and research expertise to our institution.
This issue of Pediatric Perspectives contains a
variety of articles we think will be of interest
to our referring physicians. This is just a
sampling of the important work our dedicated
physicians and researchers are engaged in.
We look forward to serving you and your
patients, and we encourage you to contact us
through various channels, phone numbers and
e-mail addresses listed throughout Pediatric
Perspectives. Thank you for your interest in
Cleveland Clinic Children’s Hospital.
Sincerely,
Robert Wyllie, M.D.
Physician-in-Chief, Children’s Hospital
Chairman, Division of Pediatrics
Calabrese Chair of Pediatrics
wyllier@ccf.org
Vascular Ring in Premature Infant
Ethan O’Keefe (pictured on front cover) had typical symptoms
of gastroesophageal reflux (GER) in a newborn — frequent
spitting up, irritability, poor sleep, poor weight gain. However,
after diet and positioning efforts plus trials of histamine-
2 receptor antagonists and proton pump inhibitors failed
to improve his condition, Jon Kannensohn, M.D., Ethan’s
pediatrician at Cleveland Clinic’s Willoughby Hills Family
Health Center, began to suspect something more than GER
was at play.
Dr. Kannensohn referred Ethan, born two months prematurely
in September of last year, for an upper GI series. Pediatric
radiologist Sunny Pitt, M.D., at Cleveland Clinic Children’s
Hospital, imaged Ethan and, in fact, did not find evidence of
GER. Instead, she identified a right aortic arch vascular ring, a
congenital anomaly rarely found in the newborn.
Surgery Needed
Ethan’s parents, Kim Fritz and Dan O’Keefe, of Perry, Ohio,
took their son to Muhammad Ali Mumtaz, M.D., of the
Pediatric and Congenital Heart Surgery Section at Cleveland
Clinic Children’s Hospital. The vascular ring consisted of a
right aortic arch with anomalous left subclavian artery arising
from a Kommerell’s diverticulum. This resulted in constriction
of both the trachea and esophagus. Surgical correction was
needed to treat current symptoms as well as avoid future
functional damage to the esophagus and development of
asthma, and to ensure proper growth and development.
Ethan’s surgery was performed on Dec. 20, 2006, at nearly
3 months of age.
About the Physicians
Sunny Pitt, M.D., a specialist in
pediatric imaging, has appointments
in both Diagnostic Radiology and
General Pediatrics. Contact information:
216.444.4778 or pitts@ccf.org.
Page 2 | Pediatric Perspectives | Spring 07 | Cleveland Clinic’s toll-free physician number is 800.553.5056

Repaired Minimally Invasively
Dr. Mumtaz was able to repair the defect utilizing a musclesparing
thoracotomy, an approach that decreases pain,
shortens recovery time and reduces the risk of growth-related
problems of the chest wall. The ring was divided and the
abnormal vessels were rerouted to their normal positions.
Ethan’s postsurgical course was unremarkable, and recovery
was quicker than expected for a premature neonate, Dr.
Mumtaz reports. Ethan was able to eat normally and was
discharged within 48 hours. His prognosis is excellent.
Right: 3-D post-gadolinium
enhanced MR angiogram
image shows the right-sided
aortic arch with its major
branches. The vessel coursing
toward the patient’s left arm is
the anomalous left subclavian
artery.
Below: On this sagittal gradient
echo image, the tracheal air
column (vertically oriented
band of dark signal) is narrowed
in its distal aspect due
to the vascular ring.
Muhammad Ali Mumtaz, M.D., of the
Pediatric and Congenital Heart Surgery
Section at Cleveland Clinic Children’s
Hospital, specializes in neonatal cardiac
surgery, pediatric cardiac transplantation,
adult congenital heart disease,
anomalies of pulmonary veins, minimally
invasive surgery for congenital heart
disease, and valve repair for congenital
valvular lesions. Contact information:
216.444.9125 or mumtazm@ccf.org.
Visit clevelandclinic.org/childrenshospital
| Pediatric Perspectives | Spring 07 | Page 3

Despite the many successes pediatric oncology has seen
in the past two decades, long-term outcomes for acute
myelogenous leukemia (AML), the less common form
of leukemia in children, remain poor. To improve cure
rates for this devastating disease, physicians at Cleveland
Clinic Children’s Hospital and elsewhere are conducting
clinical trials to develop novel therapeutic strategies.
Individualized Therapy Based on Molecular Features
Through the Children’s Oncology Group (COG), collab-
orative studies have refined treatment for both types of
leukemia that are typically seen in children (AML, and
the most common acute lymphoblastic leukemia or ALL)
based on molecular features of the cancer.
“ALL patients are now stratified into risk groups based on
chromosomal features of the leukemia, such as the pres-
ence of translocations, where portions of chromosomes
are broken and then swapped, or the actual number of
chromosomes in the leukemia,” explains Kate Gowans,
M.D., pediatric hematologist/oncologist at Children’s
Hospital.
By MEASURING How MUCH DISEASE REMAINS after early
chemotherapy, the patient’s treatment can be individualized, which
has been shown to improve outcomes.
The State of Acute Leukemia Therapy
Some translocations are highly predictive for a better re-
sponse to chemotherapy, whereas others indicate a need
for more aggressive therapy and possibly bone marrow
transplant, Dr. Gowans notes.
It is also possible to use molecular markers of leukemia
cells that can detect less than one cancer cell among
10,000 normal cells. By measuring how much disease re-
mains after early chemotherapy, the patient’s treatment
can be individualized, which has been shown to improve
outcomes. This “minimal residual disease” concept is
currently applied only to ALL patients, but will likely
make its way into future AML clinical trials.
Castle Connolly Top Doctors
Eleven Children’s Hospital doctors have been profiled in the Castle Connolly
guide, “America’s Top Doctors.” The doctors were selected based on peer nomination
and screening by Castle Connolly’s physician-directed research team.
Targeted Therapy
One of the most exciting new concepts in cancer treat-
ment is targeted therapy using monoclonal antibodies,
Dr. Gowans reports. The most recently completed AML
trial, in which Children’s Hospital participated, examined
the potential effect of adding a monoclonal antibody to
the current AML treatment regimen. This antibody, gem-
tuzumab ozogamicin (Mylotarg), is directed against the
protein CD33, found on the outside of most AML cells.
Some standard chemotherapy would still be necessary to
clear the large number of AML cells present at diagnosis.
However, novel treatments that include targeted therapy
may be able to eliminate minimal residual disease that
may cause relapse in some patients.
Changing Cancer Cells’ Growth Signals
Removal of leukemia-specific growth signals by the drug
STI-571 (Gleevec) has dramatically improved the lives of
many patients with a rare form of chronic myelogenous
leukemia (CML). STI-571 will soon be tested in ALL
patients whose leukemia shows similar growth-signal
abnormalities. Now in early clinical development are
drugs that can target in a similar way a growth signal
called FLT3, which is genetically switched to the “on
position” in about one-third of AML cases. A drug with a
mechanism similar to STI-571 would represent a major
breakthrough in AML therapy.
Cleveland Clinic Children’s Hospital participates in the
Children’s Oncology Group, whose mission is to cure
childhood and adolescent cancer through scientific
discovery and compassionate care. There are currently
approximately 60 clinical trials open for children with
cancer. At Children’s Hospital, we offer comprehensive
services to patients and their families who are diagnosed
with cancer or other blood disorders. Our team of physi-
cians, nurses, clinical research associates, social workers,
pharmacists and child life specialists is specially trained
to care for these patients and their unique needs.
Page 4 | Pediatric Perspectives | Spring 07 | Cleveland Clinic’s toll-free physician number is 800.553.5056

About the Physician
L. Kate Gowans, M.D., has
joint appointments in Pediatric
Hematology & Oncology, the
Brain Tumor and Neuro-Oncology
Center and Taussig Cancer
Center. She is a general clinical
hematologist and oncologist,
with special interest in coagulation
disorders, lymphomas and
sickle cell anemia. She can be
reached at 216.445.3588 or
gowansk@ccf.org.
About Acute Leukemia
Acute leukemia, the most common type
of childhood malignancy, occurs in two
forms: acute lymphoblastic leukemia (ALL)
and acute myelogenous leukemia (AML).
Although each disease arises from bone
marrow cells, they have dramatically different
characteristics in terms of age, aggressiveness
and curability. Each year, approximately
1,000 children and adolescents are
diagnosed with the rarer type, AML. This
disease represents only 15-20 percent of
childhood leukemia, but accounts for 30
percent of deaths from leukemia. Even
with highly intensive chemotherapy that
requires an almost continuous six-month
hospitalization, blood transfusions and
aggressive supportive care, the long-term
survival rate struggles to exceed 50 percent
in most large series of patients. The main
reasons for treatment failure are chemotherapy
resistance (either at the time of
diagnosis or acquired during treatment)
or treatment-related mortality, particularly
overwhelming infection.
For AML patients, a bone marrow transplant
from a matched sibling offers the best
chance for cure; however, only 25 percent
of patients will have such a donor.
Visit clevelandclinic.org/childrenshospital
Pediatric Puzzler
Teenager with Weight Loss
By Rita M. Pappas, M.D, FAAP
Case History For one 18-year-old man, it started with nausea, vomiting and
abdominal pain that lasted three days. He felt weak, had lost 20 pounds in
the previous six months and suffered from lethargy. He weighed 97 pounds
and was 6 feet tall. He looked pale and in pain. The patient experienced
several episodes of nonbilious and nonbloody emesis. He was hospitalized
for two months for dehydration-related emesis. His constant abdominal
pain was in the epigastrium. For three months before admission, he had
seen a counselor to help him cope with his parents’ divorce. His primary
care physician prescribed escitalopram (Lexapro) and presumed he had
depression. The weight loss could not be explained by an eating disorder.
His body mass index was 13kg/m 2 and skin around his eyes and chest was
hyperpigmented. When he was admitted for treatment, his temperature was
96.4˚ F, his pulse was 107, respiratory rate 20 and blood pressure was
96/41mmHg. Answer on page 11.
Children’s Hospital Speakers Bureau Cleveland Clinic Children’s Hospital
physicians are available for speaking engagements on a wide variety of topics
ranging from fetal diagnosis and treatment of congenital anomalies, childhood
and adolescent cancer, and nonalcoholic fatty liver disease in children, to epilepsy
diagnosis and management, and many more. To request a brochure on
the Children’s Hospital Speakers Bureau or to arrange for a pediatric speaker
at a staff meeting, grand rounds, CME or other event, call 216.445.7767. We
can provide transportation and assist with accommodations as necessary.
Board Review Symposium Cleveland Clinic Children’s Hospital’s 13th Annual
Pediatric Board Review Symposium will be Aug. 27-31 at the InterContinental
Hotel & Bank of America Conference Center.
The symposium is a comprehensive review designed to prepare attendees for
board certification or re-certification. It is also appropriate for those who need
a comprehensive update of basic pediatric clinical information. The symposium
features an audience response system and specialty-specific board simulation
sessions. Attendees receive the new second edition of “The Cleveland
Clinic Intensive Review of Pediatrics.” For more information, contact symposium
director Camille Sabella, M.D., at 216.445.6862 or sabellc@ccf.org, or
visit clevelandclinicmeded.com.
Pediatric Neurology Seminar The 5th Annual Pediatric Neurology Update
Seminar will take place Sept. 7 at Executive Caterers at Landerhaven in
Mayfield Heights, Ohio. This year’s program covers a broad range of topics,
including neuromuscular disease (an update on therapeutic possibilities
in muscular dystrophy and a modern, genetic and clinical approach to the
diagnosis of the floppy infant), cognitive effects of epilepsy and interactive
case-based teaching, mitochondrial disease primer, and social dysfunction
in children with ADHD and its impact on their lives. It will also feature
distinguished guest lecturers. 6.25 CME credits available. Contact course
director, Neil Friedman, M.B., Ch.B., at 216.444.6772 or friedmn@ccf.org,
or visit clevelandclinicmeded.com.
| Pediatric Perspectives | Spring 07 | Page 5

CT Enterography Provides Unparalleled Views
By Stuart Morrison, M.D.
CT enterography, coupled with a new oral
contrast agent, is allowing pediatric radiologists
here to visualize a child’s entire
gastrointestinal system, providing complete
anatomical information unparalleled
in detail and facilitating diagnoses.
CT enterography is already replacing
traditional fluoroscopic upper GI and small
bowel series in many cases. The technique
is especially useful in diagnosing and
following up inflammatory bowel disease.
CT enterography can assess the mucosa
and identify disease activity in Crohn’s
disease, a unique advantage.
This scan provides a global view of the
entire abdomen not possible with other
imaging modalities. The entire bowel,
from mucosa to surrounding mesentery,
stomach to rectum, can be seen with this
new technique. Surrounding blood vessels
and lymph nodes are discernable as well.
The entire abdominal viscera — liver,
Figure 1 Normal CT enterography.
Coronal reconstruction
showing the normal stomach,
small bowel and large bowel.
pancreas, spleen, kidneys — also can be
visualized as well as the abdominal wall
and retroperitoneum.
The new contrast agent, Volumen E-Z-EM,
uses sorbitol, a non-absorbable sugar alcohol,
which distends the entire small bowel
lumen. This permits excellent images of
the small bowel lumen, mucosa and bowel
wall. In addition, the multi-detector CT
scanner can reformat images in any geometric
plane without losing image quality,
providing spectacular images of the abdomen
and pelvis.
Conventional CT scans of the abdomen
have been difficult to perform in children
for several reasons. Conventional CT
requires the child drink clear liquids or
a positive contrast agent such as dilute
barium. While each of these outlines the
bowel, neither results in optimal images.
Clear liquids are rapidly absorbed in the
small bowel, leaving the majority of the
bowel collapsed and poorly visualized.
Children find positive contrast agents
difficult to drink.
Figure 2 Crohn’s disease
of distal ileum. Coronal
reconstruction showing
bowel wall thickening and
fat wrapping of the distal
ileum [arrow].
The Procedure
An hour before a scheduled CT enterography
scan, the patient drinks Volumen in
three equal doses every 20 minutes. The
scan is performed in less than one minute.
Doses of radiation are low, following national
guidelines, and no sedation is necessary.
Children must not eat or drink liquids
within four hours prior to the scan. The
procedure is not recommended for children
younger than 8 years old.
As in all CT imaging, intravenous contrast
is given during the scan to enhance visualization
of the bowel’s vascular system.
Increased vascularity is present in many
inflammatory bowel diseases. Children
with Crohn’s disease may demonstrate
abnormal enhancement of the bowel mucosa,
which has been shown to correlate
with disease activity. Crohn’s disease can
be identified and very accurately localized
with CT enterography. The new CT can allow
a doctor to make a differential diagnosis
for a child’s thickened bowel wall, often
crucial in guiding therapy and helping plan
surgery. CT enterography also can identify
sinus tracts, fistulae and abscesses.
Cleveland Clinic Children’s Hospital has
performed more than 60 CT enterography
studies with excellent results. We presented
results at the Radiological Society of
North America annual meeting last November.
The study was coauthored by pediatric
radiologist Sunny Pitt, M.D.; Robert Wyllie,
M.D., pediatric gastroenterologist and
Physician-in-Chief of the Children’s Hospital;
and Janet Reid M.D., head of Pediatric
Radiology.
Author Contact Information
Stuart Morrison, M.D., Pediatric Radiology,
can be reached at 216.445.2983 or
morriss@ccf.org.
Best Doctors in America
Fifty-three Children’s Hospital specialists are listed
in “Best Doctors In America 2005-2006,” the latest
version of this peer-reviewed survey by Bostonbased
Best Doctors, Inc. Only 3 percent of boardcertified
doctors in the United States are named to
this annual list.
Page 6 | Pediatric Perspectives | Spring 07 | Cleveland Clinic’s toll-free physician number is 800.553.5056

Giving Back for Medical Advances in IBD
Support provided by generous families, foundations and corporations
in Northeast Ohio and around the United States has enabled Cleveland
Clinic Children’s Hospital specialists to continue cutting-edge research
into inflammatory bowel disease (IBD).
Ulcerative colitis and Crohn’s dis-
ease are estimated to affect as many
as 1 million people in the U.S. alone.
Twenty to 25 percent of these
patients are children. The overall
number of patients with IBD is
rising, and so is the proportion of
children affected.
Despite a remarkable increase in IBD
knowledge over the last few decades,
more detailed information about
IBD’s earliest stages is still needed to
understand what causes the disease
to develop. Unfortunately, the major-
ity of current research utilizes adult
tissue samples to delineate initial
inflammatory events. This misses
the opportunity to study early stages
of gut inflammation. Children are
a unique study population that can
provide more insight into the earliest
immune response, natural history of
the disease, genetic associations and
environmental factors important in
the development of IBD. In children,
events directly leading to IBD, such
as environmental exposure and sub-
clinical inflammation, usually have
been present only for a short time
prior to diagnosis, as opposed to sev-
eral years in adults.
Although the exact mechanism of
IBD remains unknown, studies have
recently identified new cytokines
and pathways that play a key role in
Visit clevelandclinic.org/childrenshospital
mucosal inflammation, known as the
interleukin-23/interleukin-17 axis.
In animal models, administering
IL-23 accelerates the onset of colitis,
whereas its neutralization decreases
inflammation, suggesting that this
pathway is critical in developing in-
testinal inflammation.
Cleveland Clinic Children’s Hospital
gastroenterologist Franziska Mohr,
M.D., and Claudio Fiocchi, M.D.,
are planning innovative research to
evaluate the immune response in
children with early IBD. They will
compare the immune response in
children with initial manifestations
of IBD with that occurring in chil-
dren with long-standing IBD to learn
when the IL-23/IL-17 axis becomes
involved. Their hypothesis is that this
axis plays a larger role in early stages
of inflammation than in the chronic
disease process.
Clinical Implications
In recent years, the treatment of IBD
has been moving away from broad-
spectrum immunosuppressive drugs,
such as steroids, toward highly spe-
cific biologicals. If early immune
response in IBD is proven to differ
from that in long-standing inflam-
mation, new targets for very specific
disease-altering therapy may be
developed. These highly selective and
Kendall Urban Fund
After Scott and Christine Urban’s
daughter, Kendall, was treated for IBD
at Children’s Hospital, they wanted to
express their gratitude for the care she
received. They decided to give back to
the hospital by providing physicians
and researchers with needed resources
to begin understanding the underlying
causes of IBD. The Urbans created
the Kendall Urban Fund to support the
latest research performed by worldclass
pediatric IBD specialists here.
very early therapies could even alter
the natural progression in IBD and
prevent children from developing the
chronic form of the disease.
“While a majority of IBD researchers
are not yet starting to focus on IL-23
and IL-17 in the pediatric popula-
tion, we hope our efforts will accel-
erate the pace of discovery and the
transfer of new knowledge into novel
therapies that will benefit children
affected by IBD,” says Dr. Fiocchi.
The Kendall Urban Fund is providing
the necessary funding to supplement
this cutting-edge research. Philan-
thropic partners such as the Urbans
will play a key role in funding Cleve-
land Clinic’s research and discoveries
of tomorrow.
About the Physicians
Franziska Mohr, M.D., is a pediatric
gastroenterologist with special expertise
in pediatric IBD. She can be reached at
216.444.3556 or mohrf@ccf.org.
Claudio Fiocchi, M.D., is an adult gastroenterologist
with special interest in pediatric
IBD. He has appointments in the Lerner
Research Institute’s Department of Pathobiology
and in Gastroenterology and Hepatology.
He can be reached at 216.445.0895
or fiocchc@ccf.org.
| Pediatric Perspectives | Spring 07 | Page 7

Recent Recognition and Awards
Leadership Chair
Robert Wyllie, M.D., Children’s Hospital Physician-in-Chief, will be the first holder of the
Leadership Chair for Excellence in Pediatric Care, Research and Education. The chair was
endowed with a $4.6 million donation from Steve and Nancy Calabrese.
Brain Tumor Research Chair
Tanya Tekautz, M.D., Director, Pediatric and Young Adult Brain Tumor Program, is the first
chairholder of the Karen Colina Wilson Endowed Chair in Pediatric Brain Tumor Research at
Cleveland Clinic.
Nightingale Award
Pediatrician Charles Davis, M.D., has won the Nightingale Physician Collaboration Award.
Given by Cleveland Clinic nurses and staff, this award is in recognition of a physician’s ability
to collaborate with nurses.
Teacher of the Year
Manikum Moodley, M.B.,Ch.B., FCP, FRCP, was named 2006 Cleveland Clinic Teacher of the
Year in both Pediatrics and Neurology, the first pediatric neurologist to win the award in two
different areas in the same year.
Top Pediatrician
Rita M. Pappas, M.D., FAAP, Pediatric Hospitalist and Associate Program Director of the
Pediatric Residency Program, is listed in the Consumers’ Research Council of America “Guide
to America’s Top Pediatricians” 2007 Edition.
Editorial Board
Lewis P. Rubin, M.D., Chairman of Neonatology and Co-Director of the Fetal Care Center, was
appointed to the editorial board of Gene Regulation and Systems Biology.
Professional Appointment
Jeffrey S. Palmer, M.D., FACS, FAAP, Director of Minimally Invasive Pediatric Urology (Glickman
Urological Institute), has been elected Secretary of the Ohio Urological Society.
Keynote in Toronto
Elaine Wyllie, M.D., Director of Pediatric Neurology, recently was keynote speaker and a judge
at the Hospital for Sick Children in Toronto’s Division of Neurology resident research competition.
Her talk focused on her recent research and innovations in the surgical treatment of
epilepsy in children.
Hospital Transfers/Admissions
Main Campus/Critical Care Transport (24/7)
216.444.8302 or 800.553.5056
Shaker Campus
216.721.7002 or 800.635.2417
Online
Services for
Physicians
How to Reach a Children’s Hospital Specialist
outpatient Referrals/Consultations
Main Campus, Fairview or Hillcrest Offices
216.444.KIDS (5437) or 800.553.5056
Shaker Campus
216.721.1496 or 800.635.2417
eCleveland Clinic DrConnect
Whether you are referring from near or far, our new
eCleveland Clinic service, DrConnect, can streamline
communication from Cleveland Clinic physicians to your
office. This new online tool offers you secure access to
your patient’s treatment progress at Cleveland Clinic.
With one-click convenience, you can track your patient’s
care using the secure DrConnect Web site. To establish
a DrConnect account, visit eclevelandclinic.org or e-mail
drconnect@ccf.org.
Pediatric Perspectives offers updates on
diagnosis and management from specialists
at Cleveland Clinic Children’s Hospital, 9500
Euclid Ave., Cleveland, OH 44195.
Please direct correspondence to Editorial
Board co-chairs,
Rita M. Pappas, M.D., FAAP,
pappasr@ccf.org, 216.444.4998
Jeffrey S. Palmer, M.D., FACS, FAAP,
palmerjs@ccf.org, 216.445.7504
Editorial Board: Thomas Edwards, M.D.;
David Gurd, M.D.; Skyler Kalady, M.D.;
Rocio Moran, M.D.; Oliver Soldes, M.D.;
Kathy Whitford, PNP
Children’s Hospital Physician-in-Chief Robert
Wyllie, M.D., welcomes your feedback at
216.444.2237 or wyllier@ccf.org.
Art director: Amy Buskey-Wood
Photographer: Tom Merce, Don Gerda
Medical illustrator: Joe Kanasz
Cleveland Clinic Children’s Hospital offers
advanced medical care at its Main Campus
and developmental and rehabilitation services
at its Shaker Campus, which is home to
the Cleveland Clinic Children’s Hospital for
Rehabilitation, the Cleveland Clinic Center
for Autism, a pediatric dialysis unit, and
comprehensive therapy services. Pediatric
specialty services are available at Hillcrest
and Fairview hospitals, both of which have
dedicated pediatric emergency departments
as well as neonatal intensive care units.
Pediatric care also is available at Cleveland
Clinic family health centers throughout the
community.
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 5/07
Pediatric Physician Network (24/7)
216.444.DOCS (3627)
General Appointments for Families
216.444.KIDS (5437)
eCleveland Clinic Second opinions
You can also request a remote second opinion from
our Children’s Hospital specialists through the secure
eCleveland Clinic MyConsult Web site. Pediatric
cardiologists, hematologists/oncologists, orthopaedists,
and endocrine and growth disorder specialists provide
detailed second opinions within five to seven days.
We also offer pre-adoption healthcare evaluations. To
request a second opinion for your pediatric patient, visit
eclevelandclinic.org/myConsult.
Page 8 | Pediatric Perspectives | Spring 07 | Cleveland Clinic’s toll-free physician number is 800.553.5056

Surface
Electromyography
Helps Restore
Motor Function
When a car crash severed 3-year-old
Gracie’s spinal cord, it paralyzed her
from the neck down and left her unable
to breathe on her own. Doctors fitted her
with a wheelchair guided by her head
movements. A ventilator attached to her
chair allowed her to breathe. In physical
therapy, Gracie had to master enough head
control to operate her wheelchair. She was
unable to make enough progress, however,
so surface electromyography (SEMG) was
prescribed.
Jeffrey Bolek, Ph.D., Head of the Motor
Control Program at Cleveland Clinic
Children’s Hospital for Rehabilitation,
used SEMG to address Gracie’s functional
goals to perform daily life tasks — not just
to be aware of the muscle activity in her
compromised limbs.
“SEMG for motor re-education typically
involves two channels, one for the active
muscle (the agonist) and one for the muscle
meant to be relaxed (the antagonist),”
Dr. Bolek explains. “In our Motor Control
Program at Children’s Hospital, as many
as 14 muscle sites may be used in a performance-contingent
reward program.” All
of the sites must be working in the proper
way for a video to be activated. If one of
the sites falls above or below threshold,
the video shuts off until the correct motor
movement or posture is regained.
This produces a much more detailed analysis
than from SEMG using only two sites.
The best scenario would target the following
muscles: the bilateral gluteus maximus,
gluteus medius, lower paraspinals, midparaspinals,
lower trapezius, rhomboids,
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Often the patient will exclaim, “I’ve got it!” when he
or she masters a muscle movement — comparable
to a child finally learning to ride a bicycle.
right bicep and right anterior deltoid. This
would promote stable base support and
symmetry in back musculature. Often the
patient will exclaim, “I’ve got it!” when
he or she masters a muscle movement
— comparable to a child finally learning
to ride a bicycle.
Effective intervention in motor dysfunction
involves many facets. Myotatic unit movement
must be addressed, involving multiple
bilateral sites. A good rule is to begin
assuring integrity at the base support, typically
at the pelvis or abdominal muscles,
and targeting other muscles from there.
“Perhaps of equal importance is presenting
SEMG in a way the patient can understand,
and in a way that maintains motivation
through weeks of therapy,” Dr. Bolek
notes. Rewards need to be tailored to a
patient’s unique motor recruitment pattern
to encourage sustained (slow twitch)
muscle activity. Early treatment uses verbal
cues linked to a desired motor plan, so
external aids like SEMG are less necessary
and internal sense returns to the muscles.
About the Doctor
Jeffrey Bolek, Ph.D.,
is Head of the Motor
Control Program
at Cleveland Clinic
Children’s Hospital
for Rehabilitation. Dr.
Bolek can be reached
at 216.721.5400 or
bolekj@ccf.org.
| Pediatric Perspectives | Spring 07 | Page 9

Surgical Dislocation of the Hip
Reduces Pain, Increases Activity
By David P. Gurd, M.D.
A new hip surgery performed by Children’s Hospital pediatric orthopaedic
surgeons successfully preserves blood supply to the femoral head while
permitting excellent access and exposure to the entire joint.
The two primary indications for this
surgery, known as surgical disloca-
tion of the hip, are hip impingement
that can cause osseous limitation of
joint motion and pain, and unstable
slipped capital femoral epiphysis.
Impingement may be the result of
certain diseases, such as Legg-Calve-
Perthes disease, multiple epiphyseal
dysplasia and other disorders, or it
may be idiopathic. Impingement is
caused by an incongruent femoral
head or neck position, or acetabular
change, where the femur abuts the
acetabulum.
Impingement can be devastating for
active young people. It can cause sig-
nificant pain and severely limit par-
ticipation in normal activities. Early
degenerative changes within the hip
joint are common in children with
this problem. This new operation
reduces pain and improves range
of motion by recreating the femo-
ral neck offset (removing the bone
causing the impingement) and then
Left: Bony lesion limiting hip motion.
Right: Bony impingement removed, now
allowing full and pain-free motion.
permitting repair of impingement-
produced cartilaginous and soft tis-
sue damage.
Intervention has been difficult for
this problem. Medical modalities to
treat the pain do not deal with the
underlying abnormality. Therapy for
improving motion can worsen pain
and soft tissue damage. Operative
intervention has also been quite
difficult. Gaining exposure to the
entire femoral head, acetabulum and
labrum would almost always lead to
complications, the most serious of
which was avascular necrosis.
Standard surgery for unstable
slipped capital femoral epiphysis
involves gentle reduction with in
situ screw fixation. In the literature,
unstable slipped capital femoral
epiphysis has been shown to have a
near 50 percent chance of avascular
necrosis. While this new technique
is not yet used routinely for slipped
capital femoral epiphysis, we expect
it to be used increasingly in hopes
of avoiding avascular necrosis by
maintaining good blood flow to the
femoral head.
The Procedure
This surgery was first described by
Dr. R. Ganz in Bern, Switzerland.
To start the operation, the surgeon
uses a lateral approach to the hip. A
trochanteric osteotomy is then per-
formed and the trochanter (with all
musculature attached) is retracted
anteriorly. This permits access to
the anterior aspect of the hip. A
unique Z-type capsular incision is
performed to preserve the blood sup-
ply to the femoral head. At this point,
the ligamentum teres can be cut and
the femur can be safely dislocated
from the acetabulum. The surgeon
can now fully visualize the femoral
head, acetabulum and labrum. With
the hip relocated, the surgeon is able
to visualize the true hip mechanics.
The hip can be flexed, adducted and
internally rotated to demonstrate
exactly where the hip impingement
is occurring. Visualizing where the
impingement occurs allows the sur-
geon to determine exactly where to
recreate the normal femoral neck
offset. This is a capability we have
never had before. The acetabulum
can be reshaped and the labrum and
cartilage can be repaired.
Patients are usually discharged on
postsurgical day three. Typically
crutches with toe-touch weight bear-
ing are recommended for six weeks.
Children can progressively return to
normal activity levels three months
following surgery.
Page 10 | Pediatric Perspectives | Spring 07 | Cleveland Clinic’s toll-free physician number is 800.553.5056

Physical examination with flexion,
internal rotation and adduction, creating
discomfort to the affected groin.
when Does Hip Pain warrant Attention?
Hip pain in kids is relatively uncom-
mon and always warrants a thorough
assessment by a physician. Physical
findings in children with hip im-
pingement include limited range of
hip motion and pain at the limits of
motion. The pain usually localizes
to the groin. Typically, hip or groin
pain is reproduced when the leg is
flexed, adducted and internally rotat-
ed. Radiographs of the affected hip
may show a change in the femoral
neck offset. MRI can also be very use-
ful to assess for soft tissue damage
and bony edema. Any of these symp-
toms warrant referral to a pediatric
orthopaedist.
Author Contact Information
David Gurd, M.D., 216.445.8001 or
gurdd@ccf.org. Dr. Gurd focuses his
practice on scoliosis, lower extremity
deformity, developmental dysplasia
of the hip, pediatric trauma and gen-
eral pediatric orthopaedics.
Visit clevelandclinic.org/childrenshospital
Pediatric Puzzler
Diagnosis and Management
Addison’s disease, or hypocortisolism, is an endocrine or hormonal disorder
that can occur at any age when adrenal glands produce inadequate hormones
such as cortisol or aldosterone. Symptoms include weight loss, muscle weakness,
fatigue, low blood pressure and darkening of the skin. Half the time,
nausea, vomiting and diarrhea will occur. Already low blood pressure may fall
further when standing, causing dizziness or possible fainting. Irritability and
depression are possible. Hypoglycemia can be severe in children. Menstruation
may become irregular or stop.
Early stages of adrenal insufficiency can be difficult to diagnose, although
medical history, especially skin darkening, will lead a doctor to suspect it.
Diagnosis is made by laboratory tests to determine if cortisol levels are insufficient.
The best diagnostic test is ACTH stimulation (adrenocorticotropic hormone
test). When short ACTH test results are abnormal, a longer corticotropin-releasing
hormone (CRH) stimulation test is used to confirm the diagnosis.
X-rays of the adrenal and pituitary glands are useful in determining the cause.
Addison’s disease affects about one in 100,000. Most often the cause is gradual
destruction of the adrenal cortex, the outer layer of the adrenal gland. Adrenal
insufficiency occurs when at least 90 percent of the adrenal cortex has
been destroyed. About 70 percent of reported cases are autoimmune related.
Symptoms generally progress slowly, but usually become severe enough to
prompt patients to seek medical treatment. In 25 percent of patients, symptoms
first become apparent during an Addisonian crisis, or acute adrenal
insufficiency, prompted by a stressful event such as illness or an accident.
Crisis symptoms could include sudden penetrating pain in the lower back, abdomen
or legs, severe vomiting and diarrhea, dehydration, low blood pressure
and loss of consciousness. An Addisonian crisis can be fatal if left untreated,
and therefore requires immediate injections of salt, fluids and glucocorticoid
hormones.
Once symptoms are controlled and medication stopped, further testing should
be delayed for up to a month for an accurate diagnosis. Treatment involves
oral hydrocortisone or fludrocortisone acetate.
Author Contact Information
Continued from page 5.
Rita Pappas, M.D., General Pediatrics: 216.444.4998 or pappasr@ccf.org.
Cleveland Clinic Children’s Hospital has established the Pediatric
Physician Network to improve service and communication with referring
physicians.
“We have been listening,” says pediatric urologist Jeffrey S. Palmer, M.D.,
FACS, FAAP, who directs the network. “We are here for referring physicians.
We want to know how we can improve the care we provide for
their patients.”
The network offers a 24-hour/seven-day-a-week phone number and email
access. When a physician calls, he or she will speak to Dr. Palmer.
We welcome referring physician’s feedback and are happy to meet to
discuss any issues. Office-related or other issues will be handled by our
network administrator.
Phone: 216.444-DoCS (3627)
Pediatric Physician Network
E-mail: pedsnetwork-md@ccf.org (physician issues) or
pedsnetwork-admin@ccf.org (administrative issues)
| Pediatric Perspectives | Spring 07 | Page 11

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Images of the Issue
1 Right hip, dislocated
Developmental dysplasia of the hip (DDH)
is one of the most common musculoskeletal
disorders in newborns.
DDH is the result of abnormal mechanical stresses either prenatally
(e.g., oligohydramnios, multiple gestation) or postnatally and in
children with an underlying neuromuscular dysfunction. Current
recommendations for newborn hip assessment include clinical
examination at birth and at well-baby visits (medical history
and clinical examination using Ortolani and Barlow maneuvers),
ultrasound exams of infants with abnormal examinations, ultrasound
screening of all high-risk infants and AP pelvic radiographs for
infants older than four to six months. Older children with DDH may
have asymmetric thigh and gluteal skin folds or a waddling gait.
Evaluating the infant hip with realtime ultrasound should address
femoral head position, stability during dynamic testing (analogous
to the Ortolani and Barlow tests) and acetabular morphology. Most
cases are treated conservatively, with a 95 percent success rate
if diagnosed early. Treatment includes a flexible or rigid harness
on both hips to maintain abduction and external rotation. Surgical
open or closed reduction is necessary in some refractory cases and
missed cases. Only a small number of DDH cases goes undetected
until later in infancy, childhood or early adulthood.
Zahra Karimloo, M.D., Pediatrics Radiology Fellow
Pinar Karakas, M.D., Pediatrics Radiology Staff
2 Left hip, normal
Image 1 Coronal image from the
right hip demonstrates right femoral
head is dislocated and now lies
supero-lateral to the acetabulum.
Image 2 Coronal image through
the left hip demonstrates normally
located left femoral head within the
acetabulum.