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Sarah
is a 12-year-old junior high school student who was
screened by the school nurse for scoliosis. She was
found to have a prominent shoulder blade and one shoulder
higher than the other. Her hips are uneven, and when
she bends forward, a rib prominence is noticed. The
nurse sent a note home requesting a scoliosis evaluation.
Sarah was happy that she was finally getting her back
examined because she does not like the way she looks
in a bathing suit or enjoy shopping for fancy dresses
for her school dances because the fit is uneven.
Scoliosis is a lateral (sideward) curvature of the
spine. The spine bends to one side or to both sides
at different levels. The exact cause of idiopathic
scoliosis is unknown. It often runs in families and
is genetic in nature. Kyphosis is an abnormally increased
rounding in the thoracic spine, as viewed from the
side.
Sarah's
parents have a friend who works for Shriners Hospitals
for Children in Philadelphia, so they sought help
from the Shriners Pediatric Spine Center of Excellence.
When Sarah's mom called, she completed an application
over the phone and arranged an appointment for a consultation.
At
the assessment and consultation visit, the care coordinator,
who is a nurse, took a brief medical history of Sarah
before she went for x-rays (AP and lateral thoracolumbar
spine on a 14 x 36" film). Bending films were also
taken to test the spine's flexibility.
FAMILY
DISCUSSIONS
After
consulting with the physicians, it was determined
that Sarah has idiopathic scoliosis with a curve of
60 degrees. Scoliosis treatment methods include bracing
or surgery. However, bracing is usually started when
a spinal curve reaches 25 to 30 degrees, and bracing
cannot "hold" curves greater than 40 degrees.
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Since
bracing was not a feasible option, the physicians suggested
that Sarah needed surgery. The surgical procedures were
discussed, and the care coordinator answered the family's
questions.
SURGICAL
OPTIONS
Several
types of spinal fusion are being performed at Shriners Hospitals
for Children, including posterior and/or anterior spinal
fusion. The posterior spinal fusion (PSF) is performed on
the back of the spine, with an incision down the center
of the patient's back. The anterior spinal fusion (ASF)
is performed on the front half of the spine, which is reached
through an incision on the side of the chest. The ASF can
also be performed by using a thoracoscope. A scope is used
for visualization, with spinal fusion performed through
small holes in the chest similar to knee arthroscopy. It
is determined that Sarah will benefit from a PSF.
CASE
EXAMPLE (a different patient)
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Fig.
1a: This is a 13-year-old with a progressive right
thoracic scoliosis measuring 55°
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Fig
1c: Postoperatively, the curve is reduced to 15°.
Correction was obtained by utilizing screws and hooks
with sublaminar wires to assist with curve translation.
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EDUCATING
THE FAMILY
The
nurse supplied the family with written materials, including
a preoperative spinal fusion information packet; an activity
list, which is a schedule of when Sarah may or may not resume
activities she was involved in prior to her surgery; research
consents, if applicable; and name and phone number of a
family whose child had a similar curve and same procedure.
No
more information is given at the first visit because the
family is usually overwhelmed. It is suggested that they
go home, further discuss the issues, consider a surgery
date, and follow up with the nurse the next week. In Sarah's
case, once the family followed up and set a date, Sarah
began preparing for surgery.
PRESURGICAL
TESTS
Preparation
for surgery began with several tests. Consults were sent
to the family for pulmonary function test (PFT), ECG, and
basic labs (CBC, electrolytes, creatine kinase, sed rate,
PT, PTT, platelets, and urinalysis).
PFTs
evaluate respiratory status, and if the results are abnormal,
the patient will need surgical clearance from a pulmonologist.
Rarely, children with scoliosis show moderate constrictive
disease on their PFTs secondary to asthma. If the results
on the ECG are abnormal, an echocardiogram should be performed
and cardiology clearance obtained.
Occasionally,
the neurological exam by the physician is abnormal and an
MRI is done to ensure that the spine is free of tumors,
intraspinal lesions, or syrinx (a cyst in the spinal cord).
A syrinx sometimes needs to be surgically drained or decompressed
before spinal fusion surgery. A consult with a neurosurgeon
for surgical clearance must also be obtained if MRI findings
are abnormal. SSEP is a noninvasive measurement of conduction
in the central nervous system. Small electrodes are inserted
onto the patient's scalp and extremities and connected to
a monitor.
SSEP
monitoring is done during the surgical procedure to pick
up any neurological problems that occur when spinal instrumentation
is inserted and when the spinal curve is being straightened.
A pre-op baseline SSEP is used as a comparison during surgery.
BLOOD
UNITS NEEDED
Each
patient undergoing this type of spinal surgery needs approximately
two to four units of blood available, an amount specifically
determined by the surgeon. If able, the patient can donate
two units of autologous blood. Close family members can
directly donate other units. If the family is not able to
provide the required amount of blood, the nurse can obtain
blood from a blood bank.
The
Shriners operating room uses a cell-saver machine, and most
patients receive at least one unit of cell-saver. Sarah
needed to start taking iron supplements one week before
donating blood, and then for 7-10 days following surgery.
Because the surgical team prefers not to use frozen blood,
Sarah donated her blood within five weeks before surgery
so the blood would not expire.
INFORMING
THE CHILD'S SCHOOL
Another
presurgical duty of the spine nurse was to contact Sarah's
school gym teacher to inform him that she wouldn't be able
to participate in a full physical education program for
six months after surgery. After that period, Sarah will
be able to participate in limited gym activities.
The
spine nurse also contacted the school nurse to initiate
homebound instruction for 6 to 8 weeks post-op. When Sarah
returns to school, she should have access to the elevator
and be allowed to leave class 5 to 10 minutes early to avoid
crowded hallways. Because Sarah will only be able to lift
a limited amount of weight, she will need two sets of books--one
for school and the other for home.
ONGOING
SUPPORT
Completing
the necessary testing and blood donation can be overwhelming
and confusing to the patient and family. Sarah and her parents
kept in close contact with the care coordinator for spine
patients at the Philadelphia Shriners Hospital.
To
help Sarah and her family deal with anxiety about the upcoming
surgery and recovery period, the spine nurse strongly suggested
they attend one of the meetings of Curvebusters, a support
group which meets at Shriners once a month. Social Services
would become involved with the case as needed.
PRE-OP
TEACHING
Pre-op
teaching by the spine nurse starts from the initial consult
through the day of surgery. It begins with explaining the
degree of the curve, the surgical procedure, the length
of incision, and duration of hospital stay.
Estimated
blood loss, prevention of paralysis, and the risks and benefits
of surgery are also emphasized. Post-op dressing changes,
bracing needs, pain management, activity levels, and homebound
instruction are included, and the nurse promises to return
calls the same day or before noon the next day.
After
all the tests are completed, Sarah is ready for surgery!
SEDATION
AND TRANSPORT TO OPERATING ROOM
Early
on the morning of surgery, Sarah receives a sedative and
a sip of water. She and her family are escorted to the OR/PACU
(Post Anesthesia Care Unit), where they are greeted by the
nurse and anesthesiologist. She will have a peripheral IV
started in the pre-op holding area and two more IVs and
an A-line inserted after she is asleep.
A wake-up
test, along with SSEP monitoring during surgery, will determine
that no neurological damage has occurred. Toward the end
of the surgery, after the hardware has been placed, the
anesthesiologist will decrease the amount of anesthesia
and ask the patient to move her feet. After successful completion
of the wake-up test, the anesthesia will be resumed and
the surgery will be completed. Patients have no recollection
of this test, nor do they experience pain.
Sarah's
surgery will take 5 hours. A 12- to 16-inch incision is
made down her back and the spine exposed by peeling back
the muscle. Next, small (2-cm) pieces of rib are resected
to reduce the rib deformity. The hooks, screws and rods
are placed and the spine is straightened. For Sarah, the
surgeon chose to use her ribs for bone graft. Some patients
will have small pieces of their pelvis used as bone graft.
Your doctor will decide what is best for you. The rib pieces
are placed against the spine, under the rod, to facilitate
the fusion. The OR/PACU staff is in contact with Sarah's
parents during surgery with updates on progress.
After
surgery, Sarah is taken to the PACU, where she will be monitored
for 2 to 3 hours. Routine PACU monitoring includes vital
signs, intake and output, neurovascular checks, dressing
and skin checks, and labs, including ABGs, hemoglobin, and
hematocrit
. Patients
receive oxygen via face tent at 40% concentration to keep
oxygen saturation above 94%. Most patients have a hemovac
in their back incision, a chest tube or rib protector, and
a Foley catheter along with two peripheral IVs and an A-line.
A
chest x-ray is done to check for lung expansion and atelectasis
(partial collapse of sections of the lung), and the position
of the hardware in the back can also be seen. Pain is controlled
by patient-controlled anesthesia (PCA) using morphine. Immediately
after surgery, most children rate their pain as "5" on a
severity scale of 1-5. By the first post-op day, the usual
rating is 2 to 3. IV antibiotics are given until the patient's
drains are removed. A posterior spinal fusion is a big operation.
The nurses and doctors work very hard to make the patient
as comfortable as possible. Six weeks after surgery, most
kids say that they do not remember much of their first few
days after surgery.
Sarah
is moved to the Extended Post-Anesthesia Care Unit (EPACU)
for two days. The two hemovac drains and one chest tube
are removed once drainage decreases to a minimal amount.
She is taken off the PCA pump and put on oral medication.
Her diet is advanced to clear liquids, and she is allowed
to sit in a chair. By Day 4 Sarah will be transferred out
of EPACU to continue her recovery. Before she is discharged
from the hospital, Sarah will begin to build up her strength
and endurance by walking and performing some nonstrenuous
activities of daily living.
Sarah
and her family are taught how to care for her wounds, and
she is given a prescription for pain medication. Sarah will
return to the outpatient center for a wound check, and her
dressing will be taken off and any remaining steri-strips
on her back will be removed. Her pain level will also be
assessed at this time.
Sarah
will return to the outpatient center two and six months
post-surgery for x-rays and follow-up. After the six-month
check-up, Sarah will come to the outpatient center every
six months three times and then yearly until she is discharged
from the Shriners Hospital system at age 21. The care coordinator
is always available by phone to answer any questions or
handle any issues that may arise.
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Fig
1b: This lateral radiograph shows a straight lateral
thoracic spine, referred to as hypokyphosis.
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Fig
1d: Post-op lateral profile showing a restoration
of the lateral profile to a more normal range.
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HOW
IS SARAH NOW?
Six
to eight weeks post-surgery, Sarah is doing very well. She
has returned to school and is slowly increasing her activity
level. She has discontinued taking pain medication and frequently
attends Curvebusters, the support group for children with
scoliosis. She enjoys counseling other young people who
are about to have surgery. She is happy with the way her
clothes fit--and she has spent a lot of money at the mall
since she looks so great!
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