BOSTON CHILDREN’S HOSPITAL’S

Bladder Exstrophy
Handbook
 Table of Contents
Introduction .............................................................................................................................................................. 3

Meet the Team .......................................................................................................................................................... 4

Overview .................................................................................................................................................................... 5

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Diagnosis .................................................................................................................................................................... 7

Treatment .................................................................................................................................................................. 9

Infancy ..................................................................................................................................................................... 10

Toddlerhood/Preschool ....................................................................................................................................... 15

Elementary and Middle School .......................................................................................................................... 16

High School and Young Adulthood .................................................................................................................. 19

Tests .......................................................................................................................................................................... 21

Tips for Parents ....................................................................................................................................................... 23

Frequently Asked Questions ................................................................................................................................ 25

Resources ................................................................................................................................................................28

ABBREVIATION GUIDE
ART assisted reproductive techniques MSRE modern staged repair of exstrophy
BE bladder exstrophy RNC radionuclide cystogram
BNR bladder neck reconstruction RUS renal ultrasound
CBC complete blood count UDS urodynamic study
CIC clean intermittent catheterization UPJ ureteropelvic junction
CMG cystometrogram UTI urinary tract infection
CPRE complete primary repair of exstrophy UVJ ureterovesical junction
EEC exstrophy-epispadias complex VCUG voiding cystourethrogram
KUB kidney ureters bladder (abdominal x-ray) VUR vesicoureteral reflux

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 Dear families,
The Bladder Exstrophy Handbook has been created by the staff of the Bladder Exstrophy Program of
the Department of Urology at Boston Children’s Hospital to accompany you along your journey of care
with us. The Bladder Exstrophy Program is composed of a dedicated interdisciplinary team of physi-
cians, nurses, nurse practitioners, social workers, child life specialists, public health researchers and

© 2015 Boston Children’s Hospital. All Rights Reserved. For permissions contact the Department of Urology 617-355-7796.
administrative staff. As an established and dedicated program within the Department of Urology, our
interdisciplinary team strives to provide exceptional care for infants, children, adolescents and young
adults with bladder exstrophy or other diagnoses within the exstrophy-epispadias complex (EEC).

This handbook provides easy access to useful information along the continuum of normal develop-
ment, and potential medical and surgical care for your child with exstrophy. It is meant to assist, but not
replace, the critically important partnership between our team and you and your child. Feel free to read
it from cover to cover, or just review the sections that are important to you and to your child’s care.

Our entire team welcomes your partnership as we strive to optimize the care and the experience
for you and your child. Please know that our door is always open for your valuable suggestions
and feedback.

— The Bladder Exstrophy Program

617-355-7796

Joseph G. Borer, MD David A. Diamond, MD

Director, Bladder Exstrophy Program Urologist-in-Chief
Associate Clinical Ethicist
Co-Director, Neurourology and
Senior Associate in Urology
Urodynamics
Co-Director, Urology Trauma Program
Senior Associate in Urology

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Our team
Meet the bladder exstrophy experts

© 2015 Boston Children’s Hospital. All Rights Reserved. For permissions contact the Department of Urology 617-355-7796.
A team of health care professionals that includes physicians, nurses, social workers and child life
specialists will care for your child. Research coordinators and assistants are also important mem-
bers of our team.

Physicians Our bladder exstrophy nurses have Research

The Department of Urology at Boston extensive experience with managing The Bladder Exstrophy Program has
Children’s Hospital is consistently the care of patients of all ages and a dedicated research team in basic
ranked as one of the best pediatric are available to answer questions science and clinical research. In the
urology departments in the country. regarding your child’s condition. Our basic science laboratory, we are
Our physicians have decades of nurses work with the team to plan the investigating the genetics of the blad-
experience performing surgeries and biannual Bladder Exstrophy Support der exstrophy-epispadias complex.
managing the care of patients with Group and annual Teen Picnic. In clinical research, we are conduct-
bladder exstrophy. In addition to ing research studies to improve the
our Urology team, care of patients Social Work quality of life for patients with bladder
with bladder exstrophy is sup- We have a dedicated social worker for exstrophy.
ported by experts in Anesthesiology, the Bladder Exstrophy Program. Our
Radiology, Advanced Fetal Care and social worker helps families navigate Child Life
Orthopedics. any psychosocial issues surrounding Child life specialists help patients and
major pediatric surgical reconstruc- families adjust to and understand
Nursing tion and living with a chronic medical their health care experience while at
In the Department of Urology, our condition. Social workers are available Boston Children’s Hospital. They use
nursing staff includes nurse practi- to help families find housing during developmental interventions such as
tioners (NPs) and registered nurses surgery, work with the child’s educa- play to educate and prepare patients
(RNs). The nursing staff works along- tional institution, provide emotional for tests and procedures. They also
side our physicians to help manage support to the family, and locate both work to enhance patient coping skills
the care of our patients with bladder hospital and community resources during times of potential or realized
exstrophy. Nurses provide patients for the family. Social work staff is also fear, pain and anxiety. This team
and families with education and present at the annual Teen Picnic and helps to plan and facilitate events for
guidance to prepare for all aspects of biannual Support Group and helps the annual Teen Picnic and biannual
care including surgery and homecare. with the planning of these events. Bladder Exstrophy Support Group.

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Overview
What is bladder exstrophy?

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Bladder exstrophy (BE) is a relatively rare combination of complex
anatomical anomalies that occurs during early fetal development.

The disorder usually involves several organ systems in the patients with epispadias, there may be bony pelvis abnor-
body, including the urinary tract, the reproductive tract, malities similar to, but not as severe as, that seen with
the digestive system, the muscles and the skin of the bladder exstrophy.
lower abdominal wall and the muscles and bones of the
Similar challenges with urinary continence and vesicoureteral
pelvis. Some bones of the pelvis are shortened compared
reflux may also be present in epispadias because the inner
to normal pelvic bones. Orthopedic surgery colleagues
lining of the normally closed urethra is exposed on the surface
are routinely involved in care. Bladder exstrophy is seen in
of the penis or between the separated halves of the clitoris. In
approximately 1 in every 40,000 births.
boys, approximately one-half of the urethra (the half closer to
For individuals born with bladder exstrophy: the tip of the penis) is open and exposed. In girls, the exposed
urethra is complete and extends up to the bladder.
• The inner surface of the urinary bladder is open on the
lower abdominal wall.
• The bladder is not closed and not covered as it would
What is cloacal exstrophy?
be normally by muscle and skin, but rather the bladder is The most complex and severe manifestation of the
essentially inside out with the inner surface exposed. The exstrophy-epispadias complex (EEC) is known as cloacal
inner lining of the urethra is exposed on the top surface exstrophy. Individuals with cloacal exstrophy have anatomi-
of the penis in boys or between the separated right and cal findings similar to those found in bladder exstrophy. In
left halves of the clitoris in girls. addition, however, there is an abnormal connection of the
bowel (intestine) to the exposed bladder. This connection
• In the absence of normal closure of the bladder and urethra,
needs to be separated with the help of general surgery
there is no ability for the bladder to store urine, and urine
colleagues, typically on the first or second day of life with,
constantly trickles onto the exposed inner surface of the
in some cases, bladder closure at the same time.
bladder and surrounding skin. Following birth, this may
cause irritation of the nearby skin prior to repair. Similar to bladder exstrophy, in cloacal exstrophy the
umbilical cord exits the body lower than normal on the
What is epispadias? abdominal wall. More often in cloacal exstrophy there may
Epispadias occurs when the urethra fails to close normally, be a large abdominal wall hernia just above the umbili-
and the inner lining of the urethra lays flat and exposed on cal cord, called an omphalocele. This will require repair,
the surface of the penis. Patients with bladder exstrophy will typically on the first or second day of life, or in stages,
also have epispadias, but epispadias may also be present depending on the size of the omphalocele.
on its own. A diagnosis of isolated epispadias is considered In cloacal exstrophy, the pelvic bone abnormalities are
less severe and is also less prevalent than classical bladder often more pronounced relative to those seen in individuals
exstrophy. The prevalence of isolated epispadias is believed with bladder exstrophy. The bones of the vertebral col-
to be 1 in 200,000 to 1 in 400,000 live births. umn (backbone) and the spinal cord are often abnormally
In isolated epispadias, the bladder is closed and covered developed. Individuals with cloacal exstrophy require evalu-
by the lower abdominal wall muscles and skin. In some ation and management by orthopedic and neurosurgery

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specialists. There may be associated abnormalities of one membrane may prevent appropriate tissue development
or both kidneys such as impaired development, abnormal and ingrowth and the joining together of the supportive
function or position, or a solitary kidney. lower abdominal wall structures. This also results in failure
of bladder and urethral closure, as well as “herniation” of the
What causes bladder exstrophy? open bladder and urethra and the associated abnormalities
described above.
While the exact cause is unknown, the most popular theory
suggests overdevelopment of a normal structure known
as the cloacal membrane, which is present during early Who is affected by exstrophy of the bladder?
fetal development. In normal development, the cloacal Bladder exstrophy is slightly more common in boys than
membrane is present temporarily and eventually dissipates girls. Some reports show a clustering of bladder exstrophy
during normal fetal growth. This allows for normal growth in families, suggesting an inherited (hereditary) factor.
of the bladder, urethra and other pelvic organs, muscles and However, the chance for parents to have another child with
bones, and normal coverage of these structures by lower exstrophy of the bladder is small (one percent or less).
abdominal wall muscle, connective tissue and skin. Over-
development and/or prolonged presence of the cloacal

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Components of the urinary system and their functions
Two kidneys out of the bladder. At the same time, the
A pair of purplish-brown organs located below
the ribs toward the middle of the back. Their
brain signals the sphincter muscles to relax
to let urine exit the bladder through the
How does the
function is to: urethra. When all the signals occur in the urinary tract
• remove liquid waste from the blood in the correct order, normal urination occurs. work?
form of urine Ureteropelvic junction (UPJ)
The body takes nutrients
• keep a stable balance of body fluid volume The transition point from the renal pelvis to
from food and converts
the upper ureter. This is a potential site of con-
• keep a stable balance of salts and other them to energy. After
genital obstruction to urine flow that leads to
substances in the blood the body has taken the
hydronephrosis (dilation of the renal pelvis).
food that it needs, waste
• produce erythropoietin, a hormone that aids
Ureterovesical junction (UVJ) products are left behind
the formation of red blood cells
The transition point from the ureter to the in the bowel and in the
Two ureters bladder. This is another potential site of blood.
Narrow tubes that carry urine from each of the congenital obstruction to urine flow.
The urinary system keeps
kidneys to the bladder. Muscles in the ureter
chemicals such as potas-
walls continually tighten and relax forcing urine
sium, sodium and water
downward, away from the kidneys. About every
in balance by removing
10 to 15 seconds, small amounts of urine are
a type of waste called
emptied into the bladder from the ureters.
urea from the blood.
Bladder Urea is produced when
A spherical shaped, hollow organ located in protein is broken down
the lower abdomen. It is held in place by liga- in the body and is carried
ments that are attached to other organs and in the bloodstream to
the pelvic bones. The bladder’s walls relax and the kidneys, where it is
expand to store urine, and contract and flatten removed.
to empty urine through the urethra.
Other important
Two sphincter muscles functions of the
Circular muscles that help keep urine from Kidneys kidneys include blood
leaking by closing tightly like a rubber band pressure regulation
around the opening of the bladder. and the production of
Nerves in the bladder erythropoietin, which
Alert a person when it is time to urinate or controls red blood cell
Ureters
empty the bladder, and help the bladder productions in the bone
perform its function of storage and emptying. marrow.
Urethra Bladder
The tube that allows urine to pass outside
the body. The brain signals the bladder
Urethra
muscles to tighten, which squeezes urine

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Diagnosis
Detecting bladder exstrophy

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How is it diagnosed?
Bladder exstrophy may be diagnosed either immediately BLADDER EXSTROPHY IN BOYS
at birth based on typical physical findings, or during a fetal
ultrasound or other imaging techniques such as magnetic
resonance imaging (MRI).

In most cases, exstrophy of the bladder is associated with

the following:

• defect of the lower abdominal wall and visibility of the

exposed inner lining (surface) of the bladder, which is
usually bright pink
• shortened penis with the inner lining of the urethra
exposed (epispadias) and open along the top surface of
the penis in boys or in the surface of the body between
the abnormally divided clitoris in girls
• abnormally separated (spread to the side) lower
abdominal wall muscles
• displacement of the umbilical cord, lower than normal BLADDER EXSTROPHY IN GIRLS
on the abdominal wall, usually immediately above the
bladder
• umbilical hernia may be present (section of intestine
protrudes through a weakness in the abdominal muscles),
also called omphalocele
• absence of a normal appearing belly button
• anterior displacement of the anus, which opens on the
skin closer to the scrotum in the boys or vagina in girls
• widened and shortened pubic and other pelvic bones
• outwardly rotated legs and feet in some boys and girls

Illustrations by Sarah Dobbins

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 “ Approach your child’s treatments and path
to health in small steps. Try not to feel totally
overwhelmed by the diagnosis. It will be a
lifelong issue. However, you can overcome

”
the worries as you support your child. And
remember: Attitude is everything.
Parent of a 14-year-old boy with BE

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Prenatal diagnosis delivery. In instances of prenatally diagnosed cloacal
Prenatal care is an important aspect of health for every exstrophy, your health care team will likely prefer a sched-
expectant mother and fetus. For the expectant family, the uled caesarian section for delivery of the baby. The AFCC
prenatal diagnosis of bladder exstrophy may allow time for staff will collaborate with all health care team members to
learning about the condition and preparing for anticipated determine the optimal time for delivery and initial surgical
care. As the pregnancy progresses toward birth/delivery, it is intervention.
helpful for the expectant family to access and use avail- Regardless of the diagnosis, your newborn baby will be
able health care professionals and resources, including this carefully examined and assessed by the neonatology team
booklet and social support. upon delivery. Shortly thereafter, the pediatric urologist
Boston Children’s offers a variety of resources for families, will see your baby to confirm the diagnosis and provide
including the Advanced Fetal Care Center (AFCC). The recommendations for initial care. A more detailed plan for
AFCC provides support through prenatal counseling, management, including the timing and technique for initial
treatment and follow-up for families with a confirmed or surgical repair, will also be determined. Optimally, there will
suspected fetal congenital anomaly. In addition, medical be an opportunity for parents and newborn to begin bond-
staff, social workers, child life specialists and chaplaincy ser- ing, and breastfeeding may also be possible, both before
vices are available to support the expectant mother and her and after initial surgical repair.
family. As part of family preparation and education, a tour Although further surgery beyond the initial repair is often
of the inpatient floors will be offered to promote familiarity required, the majority of individuals with bladder exstro-
with the hospital environment. phy do achieve urinary continence and adequate bladder
Natural vaginal delivery is reasonable for a baby with function, as well as normal sexual function. After your
epispadias or bladder exstrophy but is not ideal for a baby baby’s birth, the pediatric urology team will work with you
with cloacal exstrophy. When there is a prenatal diagnosis throughout the baby’s care.
of bladder exstrophy, your obstetrician may recommend
scheduled induction of labor for a more predictable

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Treatment
Treatment and care for bladder exstrophy

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Treatment for exstrophy of the bladder begins at birth. Most important immediate care involves
tying off the umbilical cord with a soft tie (“umbilical tape”) and covering the bladder and urethra
with soft, transparent material such as a transparent adhesive covering in order to protect the blad-
der and urethral surface from the diaper.
Beyond the immediate concerns for the health and well-being of any newborn, care of a patient with bladder exstrophy
encompasses a range of surgical procedures and tests. Your child’s primary care physician, pediatric urologist and the urology
health care team will work together in order to develop the specific plan of treatment for your child.

The most important goals in the care of a boy or girl with bladder exstrophy are to:
• preserve normal kidney function
• develop adequate bladder function and promote urinary continence
• provide acceptable appearance and function of the external genitalia
• ensure that your child has a typical childhood, supported by physical and psychological health

Possible procedures for infants

There are several different approaches to initial repair for the Technique
boy or girl with bladder exstrophy, and institutions around the There are three basic options for initial repair of bladder
world approach the timing and/or technique for initial and exstrophy. These options can be performed immediately or
definitive repair of bladder exstrophy in different ways. delayed depending on the anatomy and the preference of
the physician and family.
Timing
Initial reconstructive surgery may be performed either Complete Primary Repair of Exstrophy (CPRE)
within the first 2 or 3 days of life (“immediate”) or at approxi- With the CPRE approach, the bladder is closed and the epi-
mately 6 to 12 weeks of age (“delayed”). The “delayed” repair spadias is repaired at the same time. Children who undergo
is preferred at Boston Children’s. Delaying the initial closure CPRE will likely require additional surgery during later years
may be the preference of some pediatric urologists based to manage urinary incontinence and vesicoureteral reflux
on practice or based on the specific anatomy of the baby. (VUR).
For example, some urologists prefer delaying initial surgery
if the anatomy is too small for safe repair, especially if the Modern Staged Repair of Exstrophy (MSRE)
baby is born prematurely. This strategy involves three surgeries for reconstruction of
bladder exstrophy:

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• At the initial surgery, or 1st stage of this staged repair,
the bladder and only the proximal urethra, the part of Developmental Stage
the open urethra nearest the bladder (partial epispadias
repair), are closed/repaired.
• The 2nd stage involves completion of the epispadias
Infant 0–12 months
repair. All infants are unique and develop at their
• The 3rd stage is directed both at improving continence own pace. Talk to your infant’s primary
with bladder neck reconstruction (BNR) and eliminating health care provider if you have any ques-
VUR with bilateral ureteral reimplantation.
tions or concerns.
Urinary Diversion
Typical Development
This strategy involves creating a purposeful and functional
connection between the urinary and intestinal tracts. • Beginning to sit, roll, crawl, stand and walk
Although there are several variations, the primary objective • Beginning to “coo,” babble and use first words
is to divert urine from the urinary bladder into the bowel • Starting to eat breast milk, formula and solids
(large intestine/colon) for temporary storage and then
• Learning from their environment (5 senses: hearing,
allowing for passage of the urine by emptying per rectum

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smelling, seeing, feeling and tasting)
with the stool. Passage, at regular intervals, is under the
control of the child. The bladder and urethra are closed but • Beginning to develop trust with and attachment
are “dry” and are neither exposed to the urine nor necessary to primary caregivers
for handling of the urine.
Impact on Development
• Physical challenges: restriction of motion (traction or
Complete Primary Repair of Exstrophy
spica cast) and/or tubes
(CPRE) approach
• Sensory overload: doctor’s appointments and
The initial surgery for children with bladder exstrophy
hospital stays may be overwhelming (new people,
may combine the first two stages of the modern staged
sounds, lights, smells)
repair of exstrophy: closure of the bladder and the entire
urethra (complete epispadias repair). Called Complete • Trust and attachment challenges: large number of
Primary Repair of Bladder Exstrophy (CPRE), this technique caregivers and interactions associated with pain
combines the goals of the first two stages of the staged (procedures, tests)
reconstruction into a single operation, and may create an
What You Can Do
environment that allows more normal bladder function
(cycling or filling and emptying) to begin earlier, which • Keep routines/schedules as consistent as possible.
could optimize bladder growth and development, and also • Be aware of too much or too little light, music,
result in normal appearance of the external genitalia. touch, smell and encourage “positive touch”.

The operation consists of closure and internalization of the • Promote coping/soothing techniques (swaddling,
bladder, closure of the urethra (epispadias repair), repair of massage, rocking, sucking).
external genitalia and repair/closure of the lower abdomi- • Speak up/advocate as parents/guardians regarding
nal wall muscles and skin. Some newborns may require pain management (use of sucrose, comfort holds)
osteotomy (see Osteotomy section below) to help bring the and infant’s likes/dislikes: YOU know your infant the
pelvic bones closer together. (Some centers prefer oste- best.
otomy in all newborns). Patients may stay in the hospital • Encourage play.
for 1 to 4 weeks of healing and will be carefully observed
• Developmentally appropriate toys include mobiles,
during this time. Antibiotics are given after the surgery
aquariums, rattles, music, mirrors.
to prevent infection, and the urinary tube in the bladder
is usually removed several weeks following surgery. This • Ask about resources and available support, such as
approach has resulted in adequate urinary continence and early intervention.
voiding in some patients, therefore making it unnecessary
to perform formal BNR to treat incontinence. Still, almost all
boys and girls with bladder exstrophy will require surgery for
treatment of vesicoureteral reflux, which is present in nearly
all with bladder exstrophy (See Elementary School section.)
The belly button is also reconstructed at the time of CPRE.

Although the results of complete primary repair have

been promising, parents should expect the possibility of

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 “ Our child’s diagnosis has made us
more aware of people and their differences.
Watching (our son) go through his issues
has made others feel if (he) can go through
his issues and be so wonderful, they can step

”
up and take on and accomplish anything they
set their minds to.

© 2015 Boston Children’s Hospital. All Rights Reserved. For permissions contact the Department of Urology 617-355-7796.
Parent of a 19-year-old boy with BE

further surgery as their child gets older. All of these babies Osteotomy (pelvic)
require temporary immobilization and traction (see below) When a child with bladder exstrophy is born, several
in order to stabilize the initial repair and optimize healing. physical abnormalities typically exist. In nearly all cases, the
In addition, after surgical repair, lifelong follow-up care is pubic bones are widened or spread away from the midline
necessary, with particular attention paid to the child’s devel- (middle of the body). In some cases, the legs and feet may
opment, growth and overall health, particularly bladder and be slightly outwardly rotated. Given these abnormalities, a
kidney function. pediatric orthopedic surgeon who specializes in treating
In some boys and more often in girls, successful complete abnormalities of bone and muscle will evaluate and help to
primary repair will eliminate the need for BNR later in life. care for your child.
(Bladder neck reconstruction is the 3rd stage of the modern An osteotomy is a surgical operation in which a bone is cut
staged repair.) to shorten, lengthen or change its alignment. With bladder
exstrophy, where the pubic bones are spread widely apart in
Complete Primary Repair of Exstrophy (CPRE) the front, just above the genitalia, the osteotomy procedure
approach: Delayed vs. Immediate helps partially correct the abnormalities of the pelvic bones
Delayed Complete Primary Repair of Bladder Exstrophy by bringing these bones closer together. This creates a
(CPRE) is the preferred treatment at Boston Children’s. more normal support of the soft tissues of the pelvis, which
may also help with overall healing and potentially, eventual
We believe that delaying CPRE beyond the first 2 or 3 days urinary continence.
of life may have several advantages for the child and the
family unit: This surgery, performed by an orthopedic surgeon, helps to
decrease the tension placed on the pelvic bones and soft
• Delay presents an opportunity for normal bonding tissues at the time of initial exstrophy closure. In this way, it
between the baby and parents before the initial increases the chances for successful healing of the closure.
reconstructive surgery and lengthy recovery period that Past experience suggests a benefit of improved outcome
follows. and eventual continence for those boys or girls who have
• Time between birth and initial repair allows for growth, osteotomy at the time of bladder exstrophy repair.
development and additional maturation of organs
Newborn babies who undergo immediate repair (2 or 3
(including the bladder and genitalia) and systems prior
days of life), when the pelvis is still malleable (pliable) due to
to the complex surgery, and makes the anesthesia and
the mother’s production of the hormone relaxin, may not
surgery safer.
require osteotomy. If the closure is performed after 2 or 3

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 “ Normal (regular) life is still much more
prominent than the occasional challenge.
The kids will have the same interests
and experiences as any other child. Also,
we learn how remarkably resilient we are.

”
The kids and the parents will be able to handle
the challenges ahead.

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Parent of a 9-year-old girl with BE

days of age, this hormone has decreased, and an osteotomy • Modified Bryant’s traction consists of supporting both
will need to be performed. Osteotomy is also recom- legs by gently supporting the legs straight upward toward
mended for all reoperations for previously failed closures the ceiling, away from the bed, with the legs bent at the
and for almost all babies with cloacal exstrophy. hips as the baby lies on his or her back.
• Buck’s traction is used to immobilize position and align
Umbilicoplasty or “belly button surgery” the legs and hips in a straight line with the child lying flat
In patients born with bladder exstrophy, the belly button on the bed.
(umbilicus), which is the stump of the umbilical cord, is • At many institutions, including Boston Children’s, the
attached to the upper portion of the bladder—an abnor- preferred technique for immobilization is the “spica”
mally low location on the abdominal wall. The umbilical cast. With this technique, a plaster-type cast is applied
stump must be removed during the initial reconstructive to the lower abdomen, pelvis and legs. The spica cast
surgery. is custom-fit and placed by the orthopedic surgery
team immediately after initial repair is completed. The
Research has found that the belly button is an important
baby’s skin and body are protected inside the cast with
aesthetic landmark for most children and adolescents. To
padding. The spica cast allows for close physical contact
create a more normal appearance, umbilicoplasty, or “belly
between you and your baby, and may allow your baby
button surgery,” is often performed. This plastic surgery
to leave the hospital for home earlier while he or she is
is often combined with the initial closure or performed
healing in the cast.
separately, later on in a child’s life. It is usually performed
under general anesthesia and may be combined with other All of these types of traction allow for close physical contact
surgical procedures. In most cases, there is no additional with parents and for breastfeeding if desired.
surgical scar as a result of the procedure, as the primary There are other approaches to osteotomy and postopera-
incision is made at the planned site of the belly button itself. tive immobilization that involve more extensive incisions
in the pelvic bones and more elaborate external fixation
Traction devices. It is possible that alternative approaches may be
favored at your institution or by your child’s orthopedic
After the initial surgery, your child will be partially immo-
surgeon.
bilized, placed in “traction” for several weeks. This helps
stabilize the complex repair and optimizes healing in the
most important period of time. There are several options:

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 “ It made me stronger. Our marriage
benefited from it. It brought my wife

”
and I together to ’team up‘ to support
our daughters.
Parent of a 9-year-old girl with BE

© 2015 Boston Children’s Hospital. All Rights Reserved. For permissions contact the Department of Urology 617-355-7796.
Modern Staged Repair of Exstrophy Stage 2: Epispadias Repair
(MSRE) approach Epispadias is a congenital (present from birth) defect in
MSRE consists of three surgical components: the formation of the urethra that is always associated with
bladder exstrophy.
• the initial repair (bladder closure and partial
epispadias repair) In boys, the proximal part of the urethra close to the
bladder is closed along with the bladder at the first stage of
• completion of the epispadias repair (in boys)
MSRE. The distal (closer to the tip of the penis) open and
• bladder neck reconstruction with bilateral ureteral exposed inner lining of the urethra, on the top surface of
re-implantation the penis, was left untouched at the first stage and is now
closed at the second stage of the MSRE (epispadias repair).
Stage 1: Initial Closure
During this first operation, the bladder and proximal (closest In girls, the urethra is usually closed along its entire length
to the bladder) urethra, and the abdominal wall are closed. at the first stage of MSRE.
The belly button is reconstructed, and an osteotomy is The surgical procedure used to correct epispadias recon-
sometimes performed (the pelvic bones are reformed to aid structs the missing portion of the urethra and restores the
in the repair). Whether or not the infant needs an osteotomy appearance of the external genitalia. The exact surgery
is dependent on how soon after birth the operation is per- used differs according to the complexity of each individual
formed. (With certain physical factors, or if the initial closure case. Because of the varying anatomy of each child with
takes place three days or more after birth, then osteotomy bladder exstrophy, surgical procedures will not only have
is felt to be necessary.) different outcomes but also have different effects on
After surgery, your baby will be placed with the lower abdo- continence. This repair occurs around six months of age,
men, pelvis and legs in traction/ immobilization in order to but the time and extent of the surgery are dependent on
aid the healing of the pelvic bones and the repair. Patients the size of the bladder and the condition of the urethral
may stay in the hospital for three to six weeks of healing tissue (urethral plate).
and will be carefully observed during this time. Antibiotics During this surgery, the urethra is repositioned to its normal
are given after the surgery to prevent infection, and the placement. After the surgery, the dressings will remain in
urinary drainage tube in the bladder is usually removed place for several days, and the diverting urinary catheter
four weeks following surgery. As the size of the bladder (suprapubic cystostomy tube) will typically be removed after
increases over time, your child will be ready for the next several weeks. As with any surgical procedure, postopera-
stage, the epispadias repair. tive care will be individualized as needed.

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 “ Friends, school, sports—you would

”
never know from the outside looking in
that our child was any different.
Parent of a 9-year-old boy with BE

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Stage 3: Bladder Neck Reconstruction (BNR) Hernias (inguinal)
Performance of this stage of the MSRE depends on suf- A hernia occurs when part of the intestine protrudes (pokes
ficient growth of the bladder as judged by the volume of out) through a weakness in the abdominal muscles. A soft
urine it can hold (capacity). Before making the decision to bulge is seen underneath the skin where the hernia has
proceed with this surgery, your child’s bladder anatomy occurred. In children, a hernia usually occurs in either the
and function may be evaluated by one or more of several groin area (an inguinal hernia) or the belly button area (an
studies. These may include a bladder and urethra anatomy umbilical hernia). For children with bladder exstrophy, ingui-
x-ray (voiding cystourethrogram (VCUG)), urodynamic nal hernias are common due to associated lower abdominal
study (UDS) and/or exam under brief general anesthesia; wall muscle and connective tissue abnormalities. Hernias
bladder capacity may be evaluated in this setting as well. develop often within the first few months of life or at some
Bilateral ureteral reimplantation is almost always per- point during infancy.
formed with BNR. This procedure is usually performed
During a hernia operation, your child will be placed under
between the ages of five and 10. (See Elementary School
general anesthesia. A small incision is made in the area of
section for more details.)
the hernia (lower abdominal wall). The intestine, when pres-
When necessary, following initial repair with the CPRE ent in the hernia defect, is replaced back into the abdominal
technique, BNR and bilateral ureteral reimplantation are cavity. The muscles are then stitched back together to
performed in similar fashion for boys and girls in order to strengthen the area.
improve urinary continence and eliminate vesicoureteral
This procedure is usually performed as outpatient surgery,
reflux.
which means that children who have an inguinal hernia
surgically repaired can often go home the same day they
have the operation. Your medical team will provide you with
more detailed post-operative instructions.

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Possible Procedures for Toddler/Preschool Children

Ureteral Reimplantation the kidneys, potentially causing damage to the kidney(s).

Vesicoureteral reflux (VUR) is abnormal backward flow During the procedure, the doctor will reposition the
of urine from the bladder up to the kidney(s). A ureteral ureter so that it’s connected to the bladder correctly.
reimplant is a surgical procedure that corrects VUR by The surgery usually does not exceed a few hours. Your
repositioning the ureters within the bladder so that the child will have one or more drainage tubes after the surgery
urine flows only in the normal direction, from kidney to that will need to stay in place for a few days. In most cases,
bladder. (The ureter is that tube that carries the urine from children are released from the hospital two to four days
the kidneys to the bladder.) If the ureter is connected to the after the surgery.
bladder incorrectly, it can let the urine flow backwards to

Developmental Stage

Toddlerhood/Preschool 1–5 years

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All children are unique and develop at their own pace. Talk to your infant’s primary health
care provider if you have any questions or concerns.

Typical Development
• Jumping and climbing
• Magical thinking
• Short attention spans
• Increasing language skills: receptive language six months ahead of verbal
• Fear of bodily injury
• Temper tantrums and negative behavior are normal
• Establishing routine behaviors
• Increasing independence: getting dressed, etc.
• Using play to learn, express self and work out fears

Impact on Development
• Regression: (e.g., using pacifier during hospitalizations, using diaper after being potty trained)
• Fear of strangers heightened due to large number of caregivers when hospitalized and interactions associated
with pain (procedures, surgery)
• Increased stress and acting out when routines are not followed
• Feeling loss of control (during hospitalizations or tests—can’t eat, etc.)

What You Can Do

• Keep routines and schedules consistent.
• Set limits and offer appropriate choices (e.g., “You have to take your medicine. Do you want to do it by yourself
or do you want mommy/daddy to help?”).
• Promote positive coping and soothing techniques (e.g., taking deep breaths).
• Speak up/advocate as parents/guardians regarding pain management and your child’s likes/dislikes. YOU know
your child the best.
• Give concrete explanations and one-word descriptions for hospital visits and medical procedures.
• Encourage play: developmentally appropriate toys include bubbles, cause-and-effect toys, pop-up books, lights,
etc.; provide opportunities for medical play (play doctor with your child so he/she can “play out” what they have
experienced or fear).

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Possible Procedures for Elementary and Middle School Children

Bladder Neck Reconstruction (BNR)

Developmental Stage
The term “bladder neck reconstruction” refers to a
surgical procedure that reconstructs the anatomy of the
connection between the bladder and the urethra or blad-
der neck. The goal of this procedure is to control urine
Elementary School
leakage and potentially enlarge the bladder as a result. 6–10 years
Typically, in bladder exstrophy, this area is too wide/ All children are unique and develop at their
open and is unable to retain urine within the bladder. The
own pace. Talk to your child’s primary
bladder neck is the lowest part of the bladder, the site
where urine leaves the bladder and enters the urethra on health care provider if you have any ques-
its way to the surface of the body. The primary goal of this tions or concerns.
surgery is continence or dryness and freedom from urine
leakage. This is done by narrowing the channel of urine Typical Development
flow in the area of the bladder neck.

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• entering school and forming relationships with peers
The best candidates for BNR had a successful primary • beginning to develop a sense of control over their
closure and, over time, developed a good-sized bladder environment
with good bladder growth. (If your child’s medical team • understanding relationship between things and ideas,
used the immediate CPRE approach at birth, BNR may not seeking out knowledge
be necessary.) Bilateral ureteral reimplantation is almost
• devising new games and new rules
always necessary at the time of BNR, in order to make
room for bladder neck reconstruction surgery and in • using play to learn, express self and work out fears
order to eliminate VUR.
Impact on Development
Bladder neck reconstruction surgery carries some risks, • regression (e.g., baby talk)
including:
• increased stress and anxiety surrounding painful
• persistence of incontinence procedures, tests and illness (due to their ability to
• inability to pass urine (urinary retention) now ask questions)
• persistence of vesicoureteral reflux (VUR) • anxiety about body alteration, fear of not being well
again
• infection in the urine and/or kidney(s)
• feeling left out/ different due to time away from
In some children, additional surgery may be necessary to
routines, school, family, friends
ensure good kidney function and urinary control. Proce-
dures may include enlarging the bladder so that it may What You Can Do
hold larger volumes of urine to help keep your child dry.
• Keep routines and schedules consistent (school,
This is known as bladder augmentation (described in the
family time, etc.); consider home tutoring if needed.
next section).
• Set limits and offer appropriate choices.
An alternate technique for emptying the bladder is clean
• Promote positive coping and soothing techniques.
intermittent catheterization (CIC)- and/or an alternate route-
continent catheterizable conduit- may also be necessary. • Give concrete explanations and simple descriptions.
• Reassure your child that the condition is not their fault.
The timing of BNR depends to some degree on your child’s
bladder capacity, as well as his or her emotional and devel- • Maintain open communication with your child and
opmental status. With BNR, it is important that both the encourage him/her to ask questions.
child and the family are prepared for urinary continence, as • Encourage your child to take an active role in the
the bladder voiding training before and after the surgery is treatment of their illness (e.g., asking their doctor
rigorous and requires time and effort to be successful. Your questions).
child should be able to participate in a bladder-training • Encourage play by making developmentally-
program before and after surgery. The training begins six appropriate toys, games and crafts available.
months before surgery. Your medical team will review the
• Act out what they are experiencing with medical
specifics of your voiding program. Refusal or difficulties
play.
to follow aspects of the training program, such as timed
voiding, hydration, or medication, could indicate that your
child is not ready for this surgery.

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Bladder Augmentation The operation increases the room inside the bladder (blad-
Bladder augmentation is a surgical procedure that increases der capacity) and decreases the pressure within the bladder
the capacity of the bladder, allowing it to hold more urine. in order to make storage of urine safer. This, in turn, allows
Bladder augmentation may be recommended to help treat the kidneys to drain better.
otherwise irreversible forms of incontinence, protect the Bladder augmentation is chosen as a treatment plan for a
kidneys from high-pressure urine storage or, at times to patient only after a thorough physical exam and appropriate
assist in eliminating vesicoureteral reflux. testing is performed.
With bladder augmentation, the current standard of care is Patients who undergo bladder augmentation must learn to
to use a small portion of the child’s intestine (bowel) as a place a catheter (a small, flexible, plastic tube), or urinary
patch that is placed on the bladder. During the surgery, the tube, into their urethra (self-catheterization). They must also
top of the bladder is opened, and a small patch of stomach, not have any other bowel or urethral disease.
small or large intestine is used to make the bladder larger.

Developmental Stage

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Middle School 11–13 years
All children are unique and develop at their own pace. Talk to your child’s primary health
care provider if you have any questions or concerns.

Typical Development
• Scientific thinking
• Expressing more complex feelings
• Believing in one’s own competence
• Developing sense of self-image, self-development and gender
• Forming peer relationships and social comparisons
• Engaging in school, schedules and routines
• Asserting independence
• Beginning to understand sexual development, puberty

Impact on Development
• Regression (sleep with stuffed animal)
• Challenging the need to rely on parents when it is more normal to want more independence
• Considering long-term effects of illness and impacts on their future
• Feeling increased stress due to time spent away from normal routines at school, as well as friends and family
• Thinking of the impact on adolescent and future adult life (especially in terms of sexuality)

What You Can Do

• Provide appropriate choices.
• Provide opportunities for control.
• Encourage/promote/provide attainable goals and responsibilities.
• Give appropriate jobs.
• Respect reasonable privacy.
• Encourage increased participation in care and independence (e.g., alone time during doctor appointments).
• Encourage continued focus on educational/future goals.
• Give HONEST answers when questions are asked; if you don’t know, say you don’t know.
• Begin age-appropriate sex education.

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 “ It is a life of ups and downs, but the ups
will outweigh the downs. Exstrophy is only
a part of who they are and with the many

”
obstacles they will go through, they are
extremely amazing individuals.
Parent of 14- and 16-year-old boys with
epispadias and bladder exstrophy

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Continent Catheterizable Conduit (urostomy) Around the time of such surgery, after initial healing and
A urostomy is a surgically created alternate opening for during your child’s time in the hospital, the nurses will help
access to the urinary system that is made when long-term perform and teach catheterization and care. However, it is
drainage of urine through the urethra is not possible. With important that you and your child become involved in the
a continent urostomy, the goal is to give your child control care as soon as possible.
over urine flow from their body.
Clean Intermittent Catheterization (CIC)
The Mitrofanoff principle, named after the surgeon who
developed this idea, creates a continent urostomy, or When a child is unable to void (urinate) successfully or
stoma, which is an opening on the skin (typically in the completely, an alternate approach is available: self-cath-
lower abdominal wall) in order to create a connection eterization. Clean intermittent catheterization (CIC) may
between the skin surface and the bladder. The appendix be performed either through the reconstructed urethra or
is often used for this purpose. The urine empties from via an alternate route such as a continent catheterizable
the bladder by inserting a catheter into the small opening conduit.
(stoma), which is usually on the lower abdomen just above If you are interested in hearing more about other children’s
the pubic area. Urine will not flow out of the stoma unless a and parents’ experience with catheterization, please visit our
catheter is inserted. Experience Journal.
You and/or your child will typically need to catheterize
approximately four or more times a day to drain the bladder.
In this manner, your child will have control over their urine
output.

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Possible Procedures for Adolescents and Young Adults

Reconstructive surgery Scar revision

Reconstructive surgery for a child, adolescent or young adult The surgical procedures that children with bladder exstro-
with bladder exstrophy may help revise, smooth or hide the phy undergo may result in scarring on their abdominal
abdominal wall or other surgical scars. Your urology care and genital areas. The degree of improvement that can be
team may seek the advice and expertise of a plastic surgeon achieved with scar revision will depend on the severity of
for the optimal care of your child. Four basic types of surgical scarring and the type, size and location of the scar. If scar
procedures are most commonly used. revision is indicated, either your pediatric urologist or plastic

Developmental Stage

High School 14–18 years

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All adolescents and young adults are unique and develop at their own pace. Talk to your adolescent’s primary health
care provider if you have any questions or concerns.

Typical Development
• Focusing on body image (how they compare to their peers)
• Establishing and maintaining peer relationships
• Concern with what others think
• Wanting/asserting independence
• Feeling “invincible”
• Testing values
• Preparing for adult lifestyle and thinking about future plans
• Developing new relationships with parents
• Developing sexually and possibly becoming interested/engaging in sexual activity

Impact on Development
• Becoming more aware of their illness and impact on the future
• Decreasing adherence to medical regimens (e.g., catheterizing, taking medicines)
• Denying their illness
• Defining self by disease, instead of as a person
• Becoming more “body conscious” in sexual encounters; may delay sexual activity

What You Can Do

• Encourage and provide privacy when possible.
• Promote healthy personal hygiene choices (nutrition, exercise, etc.), independence, mobility and control.
• Encourage normal peer/family interactions.
• Encourage continued focus on educational/future goals.
• Give HONEST answers when questions are asked; if you don’t know, say you don’t know.
• Be an advocate or role model.
• Continue to set limits, yet allow for appropriate control and separation.
• Discuss nuances of sex and address concerns your teen may have.
• Utilize resources available (e.g., clinicians, age-appropriate sex education classes).

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19 BOSTON CHILDREN’S HOSPITAL
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surgeon will recommend the scar revision techniques to
achieve the best results for your child’s specific needs. In Developmental Stage
addition, umbilicoplasty (“belly button surgery”) may be
performed during adolescence. Nowadays, most infants
have this procedure performed during initial closure, but it
Young Adult
can also be performed later in development. These proce- All young adults are unique and develop at their own
dures are optional and may not be necessary for your child; pace. Talk to your young adult’s primary health care
all patients are assessed on a case-by-case basis. provider if you have any questions or concerns.

Monsplasty Typical Development

The mons pubis is the area over the middle of the lower • Transitioning from high school to college/job/
abdominal wall, just above the genitalia, where the pubic workforce
bones meet. This area is composed of soft tissues such • changing environment
as connective tissues and skin. Some bladder exstrophy • Establishing long-lasting relationships and
patients may experience significant scarring and/or depres- commitments
sion of the tissues in the mons pubis area after
• Pursuing the formation of moral values
their surgeries. A monsplasty is a surgical procedure

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that removes scar tissue present, flattens the area of • Becoming sexually active
the mons pubis and joins hair-bearing skin for a more • Taking on more responsibility in their life
normal appearance. • Developing a sense of autonomy

Vaginal Reconstruction Impact on Development

A narrowing at the skin surface, known as vaginal stenosis, • Becoming more accepting of illness
may develop after reconstruction of a girl with bladder • Increasing self-management and independence
exstrophy. This narrowing may occur with changes in
vaginal tissue. If necessary, doctors can address the issue What You Can Do
through a relatively minor surgical procedure called a vagi- • Encourage autonomy.
noplasty. In rare instances, a more extensive reconstructive • Consider advance preparation for independent living
surgical procedure involving reconstruction of the vaginal (e.g., select single dorm room, talk with employer).
canal may be necessary.
• Consider communication with professors and
Vaginoplasty is usually performed during high school years employers regarding conditions.
or later in young adulthood.

Penile Reconstruction
Boys require extensive reconstruction of the urethra and
penis at the time of initial repair regardless of whether the
initial repair is performed using the CPRE technique or the
MSRE approach. Some boys will require surgical revision
of the penis (penile reconstruction) later in life in order to
repair or revise surgical scars that remain from the original
surgery and/or to straighten or lengthen the penis. This
procedure may be performed at any time, if needed, but
is typically performed in either adolescence or young
adulthood.

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Tests
Diagnostic tests and labs

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Diagnostic tests for bladder exstrophy patients are periodic and may span the entire time your
child receives care at Boston Children’s.

Renal Ultrasound (RUS) Voiding cystourethrogram (VCUG)

In a renal and bladder ultrasound, (RUS), sound waves are A VCUG is a test that uses a special x-ray technology called
passed through the body. When sound waves “bounce off” fluoroscopy to observe the urinary tract and bladder during
the tissues of an organ, the waves can be changed into filling and voiding. Contrast fluid is instilled into the bladder
pictures by a computer. These “snapshots” of the bladder through a catheter. Imaging obtained from the procedure is
and kidneys are used to check for problems and growth. used to assess the presence of residual urine after voiding.
This test is painless and does not require an injection.
DMSA renal scan
Plain abdominal x-ray (KUB) A DMSA (dimercaptosuccininc acid) renal scan is a nuclear
An x-ray of the abdomen, often referred to as a KUB medicine test that allows your child’s doctor to see the size,
(kidneys, ureters and bladder), is needed to investigate these shape, function and position of the kidneys. It can detect
internals structures of the body. Similar to a renal ultra- scarring caused by frequent infections. To perform the scan,
sound, x-rays are painless and require your child to remain a small amount of liquid is injected into your child’s veins.
still while the technician takes a series of photos. The liquid goes to the kidneys where it is processed, and
then images are obtained with a camera.
MRI of pelvis
A pelvic MRI (magnetic resonance imaging) scan is an imag- Radionuclide cystogram (RNC)
ing procedure that uses magnets and radio waves to create A radionuclide cystogram (RNC) is a nuclear medicine test
a photo of the area between the hip bones (the pelvic area). used to check if your child has vesicoureteral reflux. Fluid is
During this procedure, your child/adolescent will need to instilled into the bladder through a catheter and images are
remain motionless in the MRI machine for approximately obtained with a camera.
30 to 60 minutes. In some instances, sedation or general
anesthesia may be necessary.

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 “ They will do everything that other

”
kids do. Bladder exstrophy is just part of
who they are.
Parent of an 8-year-old boy with BE

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Renal MAG3 Scan/MAG 3 with Lasix Renal Scan Lab Work
A renal MAG3 (mercapto-acetyl-triglycine) scan is a nuclear
Complete blood count (CBC)
medicine test that shows how the kidneys are functioning
and how well they are able to drain fluid into the bladder. A complete blood count (CBC) measures the number of
The test can show if there is any obstruction of the kidney. red, white and other blood cells. A CBC can be done for
many reasons, such as to check for infection or a low red
blood cell count (anemia).
Urodynamics study (UDS)
A urodynamics study (UDS) is a test to assess how well a Electrolytes
child’s bladder and urethral sphincter are working at col-
An electrolytes blood test measures the amount of sodium,
lecting, holding and releasing urine. During the test, a thin,
chloride, potassium and bicarbonate found in the blood.
flexible tube (catheter) is placed into the bladder and the
bladder is filled slowly with a warm saltwater (saline) solu-
Creatinine
tion to measure urine flow and bladder pressures.
A creatinine level test is a blood test that measures how well
The study may consist of one or more of these four the kidneys are filtering wastes from the body.
individual parts:

• urinary flow rate (uroflow) Urinalysis

• urethral pressure profile (UPP) Urinalysis is a common diagnostic test that is used to
determine if a patient has a urinary tract infection (UTI). It
• cystometrogram (CMG)
consists of a laboratory examination of urine for various
• electromyogram (EMG) cells and chemicals, such as red blood cells, white blood
The combination of tests used in the urodynamic study will cells, infection, or excessive protein.
depend on your child’s condition.
Urine Culture
Urine culture is a common diagnostic test used to detect
the presence of an infection and identify organisms in a
urine sample that can cause urinary tract infections.

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Tips
Preparing your child

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Talking with your child about upcoming procedures or surgeries can be a source of stress and
anxiety. You may be concerned about how to best approach these topics with your child as well as
when and how to have these discussions.

We understand that every child is a unique individual. Each type of information at his or her own pace and it helps if
child’s age, past hospital experiences, temperament and you are there to answer questions.
coping style can affect how he or she copes with upcoming • If your child asks a question that you cannot answer or do
procedures or surgery. not feel comfortable answering, a good way to respond
Children and adolescents are often present when future is, “That’s a great question. Let’s write it down so we do
procedures or surgeries are discussed with the team. They not forget it. We can ask the doctor or the nurse.”
are often encouraged to participate in these discussions at • If your child is younger and needs concrete reminders
a level appropriate to their age. Therefore, we encourage about the surgery or procedure, consider taking your
you to discuss these plans with your child before return- child to your room and pack your suitcase together. This
ing to the hospital. It will help your child prepare for the is a concrete way to let your child know that you are
experience and give him or her time to process information going to the hospital too.
previously heard and any new information. This will help • You know your child the best. Do not hesitate to contact
build and maintain trust between you and your child. a member of the team if you want to think together about
Here are some tips which you may find helpful: approaches that are best for your child and family.

• When talking with your child, start by using a frame of Age-Specific Tips
reference such as, ”Do you remember when we last went
Parents often ask for information about how and when to
to the hospital and the doctor and nurse spoke with us
talk to their child. There are many ways to help children
about a surgery you need? Well, it’s time we start talking
prepare for a procedure, surgery or inpatient stay in the
together about when this will happen.”
hospital. A child’s personality, language development and
• Remind your child who will be staying with him or her ability to comprehend information can affect understand-
with reassurances that he or she will not be alone during ing of procedures, surgeries and other reasons for coming
this experience. to the clinic or hospital. Previous experiences can also
• Keep explanations simple and wait for your child to ask influence their response. Since children develop at different
questions. This way you will learn what is important to rates and have personalities and coping styles unique to
him or her. them, these guidelines may not describe your child exactly
• Be sure your child is told at a time when you will be or accurately, but may be helpful in guiding you.
available to answer questions. Some families would prefer
to avoid a discussion at bedtime. Others, depending Newborns to 2-years-olds
on their child, find bedtime a time when their child is When your child is very young, concentrate on preparing
comfortable having a sensitive discussion. If so, revisit the yourself for the hospital. If you feel at ease, your child is
topic in the morning. A child needs time to process this usually able to sense this and react in the same way.

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23 BOSTON CHILDREN’S HOSPITAL
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 “ Our child’s diagnosis is just one part of the
family dynamic. When we are going through a

”
surgery, it teaches us how we all need to work
together to support each other.
Parent of a 9 year-old boy with BE

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2- to 3-years-olds 7- to 11-years-olds
At this age, children do not understand time in the same There is great variability in maturity within this age group.
way older children and adults do. Consider telling your child However, elementary-age children are able to understand
in simple language about his or her operation or procedure the reason for an operation or procedure and have typi-
one to two days before going to the hospital. Playing with cally participated in discussions with the team. For younger
your child and incorporating the upcoming hospital events children in this age group, you may want to tell your child
in your play together will be a concrete way to help your of plans a week before going to the hospital if they have not
child at this age. asked questions or discussed their situation since their last
visit with the team. This will give your child plenty of time to
3- to 6-years-olds ask questions and to talk about any worries or concerns.
At this age, children are beginning to learn about the days
of the week and develop a sense of time. However, it is 12-year-olds to adults
difficult for a child to understand why an operation or At this age, it is best to include your child, teen or young
procedure is necessary. Your child may worry that they did adult from the very beginning of any discussions. Encourage
something wrong. Consider reassuring your child that the your child to ask questions and to talk about any worries or
hospital stay is about having something fixed and is never a concerns. Most children are struggling for independence
punishment. Use simple, short explanations. We would rec- from their parents while at the same time seeking their
ommend telling a 3- to 4-year-old child about an operation support. Your child may have valuable suggestions about
or procedure one to two days before going to the hospital what works best for him or her. Consider an approach
and a 5- to 6-year-old child three to five days ahead of which encourages this valuable feedback about how you
time. You may want to remind your child about the last visit can best help them through the hospital stay or procedure.
with the team when these decisions may have been made. This is also a good time to ask your child if they would like
Again, playing with your child to help them understand is to speak to a young adult mentor trained in our Family to
concrete and fun for you both. Family Program, who has experienced a similar surgery or
procedure.

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24 BOSTON CHILDREN’S HOSPITAL
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FAQs
Frequently asked questions

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Q: How many surgeries will my child need for Q: Will my child have the ability to be
bladder exstrophy? appropriately dry?
It depends on where your child is treated. Here at Boston One of our major goals when treating children with bladder
Children’s Hospital, we use a method called complete exstrophy is to help your child get total control of her
primary repair of exstrophy (CPRE) to treat the condition bladder. Achieving continence may be challenging, but it’s
with a single surgery not long after a child’s birth. generally attainable in all cases.

CPRE allows the bladder to be closed and the epispadias to Many children do well with the practice of good health
be repaired at the same time. The CPRE operation includes habits, such as adequate fluid intake and a regular voiding
closure and internalization of the bladder (moving it inside program. If your child undergoes the CPRE approach to
the body), closure of the urethra (epispadias repair), repair initial surgical management of bladder exstrophy, he or she
of the penis in boys or the external genitalia in girls and may not need bladder neck reconstruction to help attain
repair/closure of the lower abdominal wall muscles and soft continence (as described above).
tissues. (See Infancy section for more details.)
However, some children may need help with emptying
their bladder. These children may use a technique called
Q: Will my child need bladder neck clean intermittent catheterization (CIC). This involves the
reconstruction if she undergoes CPRE? passage of a soft pliable catheter via either the urethra or a
The answer to this question will be based on your child’s catheterizable conduit with a well-hidden lower abdominal
ability to have normal dry periods between voiding/blad- wall stoma (a surgically constructed tube/opening connect-
der emptying (urinary continence). Your child’s bladder ing the bladder to the skin surface). This allows for safe and
capacity and his or her ability to stay dry—referred to as a painless emptying of urine from the bladder.
dry interval—help to determine the need for a bladder neck Although it is uncommon, some children with bladder
reconstruction (BNR). exstrophy may need bladder augmentation in order to
In the modern staged repair of exstrophy (MSRE) approach, increase the bladder’s capacity for holding urine.
which is a three-step surgery, BNR is planned on all
patients. In the single-surgery complete primary repair of Q: Will my child’s genitals look “normal”?
exstrophy (CPRE) approach, however, BNR is performed
In addition to working to provide your child with total
only on patients who have not achieved satisfactory urinary
control over his or her bladder, we also aim to ensure that
continence.
your child’s genital area has a satisfactory cosmetic appear-
So, determining whether or not your child will need BNR ance. Genital repair is performed at different points in care
after CPRE depends on many factors, most important of or at different ages for boys and girls, and may depend on
which is how his or her ability to be dry has developed whether your child’s doctor opts for single surgery (CPRE)
following the initial CPRE surgery. Based on our experience or the three-part surgery (MSRE).
and that of other institutions, about three-quarters of boys
and one-half of girls may eventually need bladder neck
reconstruction following CPRE.

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25 BOSTON CHILDREN’S HOSPITAL
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 “ First thing—breathe. You are in the best
place for your child’s medical needs. It will

”
be hard, but you will get through it with
great support.
Parent of a 18-month-old boy with BE

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Q: If I have another child, will he or she also have Q: Will my child be able to
bladder exstrophy? have children of their own?
The risk of a sibling being born with bladder exstrophy is Almost all boys with exstrophy produce healthy sperm.
very low—less than 1 percent. However, some may have difficulty fathering children
through traditional sexual intercourse. The potential dif-
Q: Will my child be able to play sports? ficulty is in the delivery of the sperm to the egg. Difficulties
may be a result of backward flow of semen during ejacula-
Definitely. In most cases, your child should be able to enjoy
tion (also known as retrograde ejaculation), the inability
a normal, active childhood. The one difference is that he
to ejaculate at all or low semen volume. The good news
or she will potentially have to take special care regarding
is that, if necessary, couples may be able to use Assisted
urinary continence. Even children with bladder exstrophy
Reproductive Techniques (ART), such as intrauterine insemi-
who are normally continent during the day may have “stress
nation and in vitro fertilization.
incontinence” or incontinence while running, jumping,
coughing or engaging in any other activity that puts stress If girls with bladder exstrophy have a problem with sex and
on the bladder. This may cause your child to have small to infertility, it’s most often a result of an anatomical concern.
moderate urinary leakage, and he or she may need to wear In some, the vaginal opening may be too narrow and may
a pad while being active. However, this doesn’t mean that require surgical enlargement. When necessary, this minor
your child cannot play and enjoy sports. procedure should allow for normal sexual intercourse and
achieving pregnancy. 
Q: What are the expectations regarding my Some women with bladder exstrophy may develop uterine
child’s quality of life? prolapse, in which anatomical support for the uterus is
This is a question unique to each family. Our interdisciplin- lacking and the uterus may protrude into the vagina during
ary team welcomes the opportunity to discuss any of your the later stages of pregnancy. If this happens, a woman may
concerns. We expect that your child will be able to partici- need surgery, but she can still have babies by Caesarean
pate in any and all of life’s joyful moments and childhood section (C-section), so that her urinary continence isn’t
activities. Your child’s experience should be much the same affected during vaginal delivery.
as any other child, with the understanding of and apprecia-
tion for the fact that every individual’s life is special and
unique.

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26 BOSTON CHILDREN’S HOSPITAL
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Resources
Information, education & support

© 2015 Boston Children’s Hospital. All Rights Reserved. For permissions contact the Department of Urology 617-355-7796.
At Boston Children’s

Boston Children’s Support Group for Children We provide education and interaction among patients and
with Bladder Exstrophy and their Families families with:
The support group was founded at Boston Children’s in • parent-to-parent networking
1992. The group holds an event where children, adolescents, • guest speakers
young adults and their families can come together, learn
• teen and young adult networking/activities
from each other and receive the kind of peer support that
comes hand-in-hand with those who have shared similar • medical play intervention using real or toy medical
experiences. supplies to help children express feelings and gain
understanding of medical experiences
The Department of Urology at Boston Children’s has pio-
neered efforts in the support and psychosocial development The support group meets twice a year, is free for families
of patients with bladder exstrophy. The support group is a and includes breakfast and lunch. Children have scheduled
combined effort with our surgical programs, nursing, child activities and meet in adjoining rooms relative to where the
life specialists, social workers and hospital volunteers. parents meet.

Support group topics include:

• complete primary repair of exstrophy

• clinical guidelines for care
• the development of the bladder exstrophy clinic
• urodynamics
• bladder neck reconstruction
• strategies for helping children to cope with bladder
exstrophy
• catheterization
• updates on clinical research projects

A typical meeting may also include one or more of the

following:

• guest speaker or panel discussion

• networking and group discussions
• group activities

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27 BOSTON CHILDREN’S HOSPITAL
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During the meetings, children and teens actively participate Child Life Services
in developmentally appropriate expressive and therapeutic Child life specialists possess expertise in child development
activities to encourage peer relationships and normaliza- and are available to help children and families cope with
tion. Opportunities are provided for open discussions about illness and hospitalization.
emotions, illness and hospitalization.

Hale Family Center for Families

Teen Chat
This multi-service resource center offers a variety of pro-
Recognizing the unique concerns and needs of patients grams designed to enhance your family’s experience.
with exstrophy, the center provides a monthly support
dinner group meeting. Past chat topics have included
transitioning to college, maximizing self-care and indepen- Interpreter Services
dence, navigating the challenges of relationships with family Boston Children’s interpreter services arranges for interpret-
and friends, and psychosexual health and education. ers in more than 35 languages to assist you at any point
along the continuum of care.
Teen and Young Adult Picnic
Every year, the center provides an evening for all teens and

© 2015 Boston Children’s Hospital. All Rights Reserved. For permissions contact the Department of Urology 617-355-7796.
young adults to gather together for sharing and activities. As
the summer comes to a close and transitions are antici-
pated for the fall, the picnic provides a wonderful reminder
that teens and young adults are members of a caring and
supportive network.

Family-to-Family Program
Families and patients benefit from speaking to others
receiving care in the Bladder Exstrophy Program. The
nursing coordinator and social worker are able to match
you with a family who is eager to listen and to answer any
of your questions, which may include how they managed
challenges associated with hospitalizations, surgeries,
separation from family and navigating school.

Beyond our world-class bladder exstrophy care, Boston

Children’s Hospital offers numerous services that touch the
lives of our patients and families. These services include:

Case Management Center

The case management team provides care coordination
services, including discharge planning for post-hospitaliza- Clown Care
tion services. Our utilization management case managers The Big Apple Circus Clown Care is a community
support appropriate use of and access to health care outreach program of the Big Apple Circus that brings
resources at the hospital. This includes work with insurance the joy and excitement of classical circus clowning to
companies to facilitate coverage of medically necessary the bedsides of hospitalized children five days a week,
care. The center’s dedicated group of nurses and support 50 weeks a year.
staff has advanced degrees in specialty areas such as public
health, nursing and business.

Chaplaincy
Our chaplaincy offers spiritual companions to children
and families of all traditions and to those with no religious
affiliation.

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28 BOSTON CHILDREN’S HOSPITAL
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 Social Work
At Boston Children’s, clinical social workers and resource
specialists are available in every area of the hospital to help
you and your child or adolescent face a broad range of
psychosocial issues and challenges.

For a comprehensive list or for more detailed informa-

tion about these resources, visit bostonchildrens.org/
familyresources.

Learn more about Boston Children’s

services and resources at
bostonchildrens.org/familyresources

Pawprints

© 2015 Boston Children’s Hospital. All Rights Reserved. For permissions contact the Department of Urology 617-355-7796.
Pawprints is our therapy dog visitation program,
which provides hospitalized children and their
families a healthy diversion from the usual hospital
routine and an opportunity for social interaction.

Additional Resources

National Resources Maine Department of Health and Human Services

Division of Population Health/Children with Special Health
Association for Bladder Exstrophy Community Needs
bladderexstrophy.com maine.gov
207-287-5357
Youth Rally
rally4youth.org New Hampshire Department of Health and Human Services
Special Medical Services
United Ostomy Associations of America, Inc.
dhhs.nh.gov/DCBCS/BDS/sms/index.htm
ostomy.org
603-271-4488
The Bedwetting Store New York Department of Health
bedwettingstore.com Children and Youth with Special Health Care Needs
(CYSHCN) Program/Community, Family, and Minority
Health/Special Health Care Needs
Massachusetts and Regional Resources *
health.ny.gov
Massachusetts Department of Public Health 518-473-7016 or your local county health department
Division for Children and Youth with Special Health Needs Vermont Department of Health
mass.gov/dph/specialhealthneeds Children with Special Health Needs
800-882-1435, 617-624-6060 healthvermont.gov/family/cshn/cshn.aspx
800-660-4427, 802-863-7338
Connecticut Department of Public Health
Children and Youth with Special Health Care Needs *Please check your state public health department for resources
ct.gov/dph should your family reside in other regions of the country or world.

800-505-7000
(Information Line to connect to appropriate state region)

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29 BOSTON CHILDREN’S HOSPITAL
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A patient’s story

Thomas Vincent
Overcoming bladder exstrophy

O
n a warm June morning, a District of Columbia
tour guide stops in front of the Korean War Memo-
rial. Pointing out the 19 statues erected in tribute
to soldiers who gave their lives in the conflict, he explains
that the impressive seven-foot sculptures are meant to
represent the 38th parallel, the demilitarized zone separat-
ing North and South Korea. When he turns to ask the group
why a reference to the 38th parallel would only contain 19

© 2015 Boston Children’s Hospital. All Rights Reserved. For permissions contact the Department of Urology 617-355-7796.
statues, 16-year-old Thomas Vincent immediately offered a
theory.

Thomas, who at the age of 16, was in the nation’s capital

to meet his senators and congressional representative as
a patient representative of Boston Children’s Hospital, has
always been the portrait of an over-achieving student.
When not busy with homework, chances are you could find
Thomas practicing for any of the three sports he played for
his school. If he’s not on the field or the court, you might
find him tutoring other students or at Spanish club. At first,
second and even third glance, he is healthy and vital—the
last person you might believe required extensive surgery
only hours after he was born.

Dreary start to a bright future

When Thomas was born early Thanksgiving morning in
1996, the obstetrician and pediatrician-on-call at the Thomas 2013 (photo by Matt Parker)
Vincent’s local hospital immediately knew something was
wrong. “I just heard them say, ‘We have a problem here’ and The procedure—developed by former Boston Children’s
they took him away,” Thomas’ mother Jean explains. “It was physician Michael Mitchell, MD, in 1989—not only placed
terrifying.” Thomas’s bladder back inside his abdomen, but also
Looking at the newborn Thomas, doctors could see part of repaired anomalies to his urethra and reset his hips. By
his bladder was exposed, and they soon diagnosed him with performing CPRE, Thomas’ surgeons, W. Hardy Hendren,
bladder exstrophy, a rare congenital condition where part MD, and Joseph G. Borer, MD, were able to address all of
of a newborn’s bladder protrudes out of the body. (Or, as these issues in a single procedure, rather than spreading the
Thomas has gotten used to explaining, “it basically means operation across three separate surgeries as other treat-
my insides were on the outside.”) ment methods require.

Although bladder exstrophy can sometimes be diagnosed in It took Hendren and Borer more than 12 hours to complete
utero through an ultrasound, not all technicians know what the CPRE, lasting from Thanksgiving afternoon to 4 a.m. the
to look for. So, when Thomas was delivered with his bladder next morning. In the end, the procedure was a success. A
exposed, his doctors were as surprised as his parents. day later, Jean was able to join her husband in Boston and
set eyes on her son for the very first time. To give his organs
Within hours, a transport team whisked the newborn to time to heal, Thomas’ doctors placed the newborn in trac-
Boston Children’s—with his father following in his car— tion, and it would be another three-and a-half weeks before
where Thomas would become only the second patient at Jean was able to hold her son.
Boston Children’s to undergo a complete primary repair of
exstrophy (CPRE).

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30 BOSTON CHILDREN’S HOSPITAL
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 “ Having a condition like this doesn’t mean

”
a child can’t lead a full, active life. Thomas is
living proof of that.

© 2015 Boston Children’s Hospital. All Rights Reserved. For permissions contact the Department of Urology 617-355-7796.
No sign of slowing
Fast-forward to 2015, Thomas’ senior year in high school:
you would never know he is managing with a congenital
bladder condition. Like most bladder exstrophy patients,
he’s had other operations, but none have slowed his drive
or spirit. Some have been planned, including surgery to
enlarge his bladder and install a stoma to help drain it. Oth-
ers were unanticipated, including surgery to repair a bladder
rupture from a sports injury.

Regardless of the time he’s spent in hospitals, Thomas

has barely skipped a beat in his busy life. At school, he is
a member of the National Honor Society, has played for
his school’s basketball, soccer and tennis teams since his
freshman year and was named captain of the soccer team
last fall. He is also an intern/research assistant at a science
and technology firm in Hampton, NH.

“When you throw in homework, it’s a lot,” he admits.

Thomas also isn’t shy about speaking up and sharing his seriousness of thought not normally associated with boys
experiences with other patients. When he first attended his age. Watching him in this scenario, it was easy to see
Youth Rally, a weeklong summer camp for teenagers why Boston Children’s invited Thomas and his parents to
with bladder and bowel conditions, he was so engaged D.C. to represent the hospital at Family Advocacy Day, an
with other campers he was mistaken for a counselor. This annual event that brings families and legislators together to
summer, Thomas will officially take on that leadership role. discuss health care reform.
“Growing up, everyone says this is a rare condition, and a lot
of kids think no one knows what they’re going through,” he Looking to the future
says. “But Youth Rally’s motto is ‘you are not alone’, which Thomas will soon graduate from high school and will
is something that really resonates with me. I take it to heart.” attend Duke University where he plans to study biomedical
engineering and pursue a 5-year master’s degree or apply
The missing 19 statues to medical school, or both, he says.
Back in Washington, D.C., Thomas was sure that the answer Though he may face more surgery in the future, given his
had something to do with the soldiers’ reflections being track record, it’s unlikely to slow him down. As his father
visible in the memorial’s reflective wall, and when he voiced Maurice says, “There are different levels of severity for blad-
his answer, the tour guide confirmed his theory. Later der exstrophy, but having a condition like this doesn’t mean
that afternoon when Thomas spoke with Senators Kelly a child can’t lead a full, active life. Thomas is living proof of
Ayotte and Jeanne Shaheen and Congresswoman Carol that.”
Shea-Porter, he was enthusiastic and engaged, displayed a

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