Meningococcemia: The Pediatric Orthopedic Sequelae

CONTINUING EDUCATION
Meningococcemia: The Pediatric
Orthopedic Sequelae
3.6
JANE M. WICK, BSN, RN; IVAN KRAJBICH, MD, FACS; SHANNON KELLY, MPT;
TODD DeWEES, BS, CPO
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Purpose/Goal
To enable the learner to identify meningococcemia early in its
course and understand the multidisciplinary approach to longterm treatment of the orthopedic sequelae of the disease.
Objectives
1.
2.
3.
4.
Discuss the etiology of meningococcemia.
Identify the symptoms of meningococcemia.
Explain how meningococcemia is diagnosed.
Describe orthopedic treatment options for pediatric
patients with meningococcemia.
5. Discuss perioperative nursing care of the pediatric patient
undergoing surgical treatment for meningococcemia.
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This program meets criteria for CNOR and CRNFA recertification, as well as other continuing education requirements.
AORN is provider-approved by the California Board of
Registered Nursing, Provider Number CEP 13019. Check with
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relicensure.
Conflict of Interest Disclosures
Ms Wick, Dr Krajbich, Ms Kelly, and Mr DeWees have no
declared affiliations that could be perceived as posing potential
conflicts of interest in the publication of this article.
The behavioral objectives for this program were created by
Rebecca Holm, MSN, RN, CNOR, clinical editor, with consultation from Susan Bakewell, MS, RN-BC, director, Perioperative Education. Ms Holm and Ms Bakewell have no
declared affiliations that could be perceived as posing potential
conflicts of interest in the publication of this article.
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article.
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AORN recognizes these activities as continuing education for
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http://dx.doi.org/10.1016/j.aorn.2013.03.005
Ó AORN, Inc, 2013
May 2013
Vol 97 No 5 AORN Journal j 559
Meningococcemia: The Pediatric
Orthopedic Sequelae
3.6
JANE M. WICK, BSN, RN; IVAN KRAJBICH, MD, FACS; SHANNON KELLY, MPT;
TODD DeWEES, BS, CPO
www.aorn.org/CE
ABSTRACT
Meningococcal disease affects as many as 3,000 people in the United States per
year, with the highest incidence in children younger than two years of age and
two-thirds of cases occurring in children younger than five years of age. Children
who survive meningococcemia face quality-of-life issues that result from limb
deficiencies. Consultation with an experienced pediatric orthopedic surgeon in
the early stages of the illness is vital for planning surgical approaches for
amputation of the resulting necrotic tissue and for minimizing eventual tissue
loss. Early surgical intervention is rarely indicated in cases of extremity
gangrene unless a secondary infection is present. Allowing time for tissue
demarcation and recovery can be essential for limb length preservation. Maintaining functional joints is important for long-term quality of life and activities of
daily living. AORN J 97 (May 2013) 560-575. Ó AORN, Inc, 2013. http://dx.doi
.org/10.1016/j.aorn.2013.03.005
Key words: meningococcemia, pediatric, orthopedic, purpura fulminans.
M
eningococcal disease is a potentially lifethreatening infection. The annual meningococcal disease rate in the United
States generally ranges from 0.9 to 1.5 cases per
100,000, with the overall and age-specific incidence being markedly cyclical.1,2 There is a higher
incidence of the disease in winter and early spring
related to exposure to viruses that may weaken the
immune system. The disease affects as many as
3,000 people per year, with the highest incidence in
children younger than two years of age.1,2 Children
younger than five years of age account for twothirds of meningococcal cases because of their
immature immune systems and the tendency to
put things in their mouths and to share food and
drinks.3
When children are stricken with meningococcemia, the outcome of the insult they sustain to
their extremities will affect them throughout their
lives. Principles of pediatric amputation and limbsparing options are different than for the adult
population because children have immature immune systems and the majority of their growth and
development is ahead of them. Until a vaccine
becomes available for young children, prevention,
detection, and early emergency intervention are the
keys to their survival.
MENINGOCOCCAL DISEASE
Meningococcal disease is transmitted via the nasopharyngeal secretions of people colonized by
the bacterium Neisseria meningitidis, an aerobic,
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Ó AORN, Inc, 2013
MENINGOCOCCEMIA
gram-negative diplococcus. Whether the organism
continues to colonize the nasopharynx or crosses
the mucosal barrier to enter the bloodstream, central nervous system, or other organs depends on
specific bacterial virulence factors and the host’s
defense mechanisms. After entering the bloodstream, the organism can rapidly produce and release endotoxins.
Five serogroups (ie, A, B, C, Y, W-135) cause
nearly all cases of invasive meningococcal
disease.1,2 In the Pacific Northwest, serogroup B is
the most common cause of meningococcal disease,
accounting for about 60% of reported cases.4,5
Meningococcal vaccines are effective for serogroups A, C, Y, and W-135 but are not effective
against serogroup B.2 Routine meningococcal
vaccination (ie, two doses of MCV4) is recommended for adolescents 11 years through 18 years
of age but is not recommended for younger children unless they fall into an “at risk” category.2-4
Early symptoms of the disease are flu-like in
nature and include fever, severe headache, sore
throat, lack of energy, or muscle and joint aches.
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With small children, who are unable to verbalize
symptoms, high fever and general malaise can
escalate in a matter of hours to lethargy and
septic shock.
As the bacteria multiply in the blood vessels,
toxins are released that damage the vessels and
cause leakage through the tissues underneath the
skin. A late sign of the disease at this stage is
a petechial or purpuric rash (ie, purpura fulminans),
which may appear mild but progresses to a distinctive purple bruising (Figure 1). By the time
these late symptoms appear, immediate medical
treatment is crucial to survival.1,3,6,7
Although this article focuses on meningococcemia, many of the principles presented apply to
septic shock events caused by other organisms (eg,
pneumococcus, gram-negative bacilli, streptococci,
varicella) that occur in the pediatric population
and result in acute infectious purpura fulminans.8
Symptoms of purpura fulminans include symptoms
of septic shock (ie, hypotension, tachycardia, altered levels of consciousness) and a petechial or
purpuric (ie, purple blotch) rash.
Figure 1. Acute phase fulminant meningococcemia and purpura fulminans.
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TREATMENT
Intensive care management includes administration
of third-generation cephalosporin antibiotics, ventilatory support, and management of septic shock
and organ failure.6,7 Despite aggressive treatment,
the mortality rate is still 10% for those infected
with meningococcal disease,1-3 so early recognition
and treatment of the disease are vital. Of the 90%
of patients who survive, 20% will have permanent
disabilities,3,7 including
n
n
n
n
n
learning or cognitive difficulties,
limb deficiencies or loss of limbs,
liver or kidney failure,
tissue scarring, or
vision and hearing problems.3,7
The orthopedic sequelae and quality-of-life issues
that result from limb deficiencies and loss of limbs
experienced by a portion of the survivors can be
devastating. Early consultation with a pediatric
orthopedic surgeon, given the high incidence in
children younger than two years of age, is an important consideration.9-11
Tissue Injury
Tissue injury is primarily ischemic in nature and
related to the three stages of meningococcal
disease9-11:
WICK ET AL
which may involve extensive removal of skin,
subcutaneous tissue, muscle, and bone.10,12
n Wound management of extremities with
vacuum-assisted dressings (Figure 2) and devices to promote healthy new granulation
tissue growth and decrease the risk of infection is the next step.12 A vacuum-assisted
wound dressing is a closed system that applies
negative pressure to the wound tissues with
beneficial effects on wound blood flow. The
system acts by removing excessive tissue
fluid from the extravascular space, decreasing
capillary afterload and promoting microcirculation during the early stages of inflammation.
In addition, the mechanical effect of the vacuum
on the tissue at the wound surface appears to
result in a proliferation of healing granulation
tissue. This tissue then covers exposed bone
and may be skin grafted where needed.13
n Amputations or disarticulations (ie, amputation
between the bones of a joint without cutting
bone) occur after tissue demarcation between
necrotic and viable tissue is clearly established;
auto-amputation of fingers or toes is not uncommon (ie, the soft tissue and bone dies and
drops off without needing to be surgically
removed).
n
poor tissue perfusion,
n vascular infarction, and
7,9,10
n gangrene.
Early surgical management of patients may include
the following10,12:
n
The first step is waiting and watching for tissue
demarcation between the healthy tissue and
gangrenous area to occur. This stage may take
several weeks and is the most difficult stage
for surgeons who may want to aggressively
debride what they see as dead or damaged
tissue.10,12
n Surgical debridement of necrotic tissue is the
next step, especially in the presence of a secondary wound infection (eg, wet gangrene),
562 j AORN Journal
Figure 2. A vacuum dressing being applied to both
lower extremities. The legs are incased in a large
wound spongedan option for distal extremity
involvement in small patients.
MENINGOCOCCEMIA
Children with amputations, disarticulations, and
extensive scarring are obvious candidates for continued orthopedic follow-up treatment, but all
children affected by this disease need careful
monitoring. Several retrospective studies have
demonstrated that some patients who display no
signs of skin necrosis later present with growth
plate arrest, which occurs at the time of the insult
but does not become apparent until years after
a purpura fulminans event.9-11,14 Late orthopedic
sequelae that may require surgical treatment
include9-11,14,15
n
growth plate disturbances (Figures 3),
n stump overgrowth at transosseous amputation
sites (Figure 4),
n scar contractures (Figure 5), and
n soft tissue or bone infections.
Growth plate disturbances occur in the early stage
of the disease but may not be recognized for several
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Figure 4. Bone overgrowth on the distal below-theknee amputation stump.
years and may result in angular deformities or
length discrepancies at a later stage.10,11,14,15 In
a number of studies, growth plate disturbances
were found to be more common in lower extremities than in upper extremities.10,11,14,15
EARLY ORTHOPEDIC MANAGEMENT
Usually, the first contact that an orthopedic surgeon
has with a child afflicted with purpura fulminans is
two to three days into the disease process, because
the initial treatment is invariably focused on keeping the morbidly ill child alive. It is only after the
patient survives this critical 24-hour to 48-hour
period and various organ failures have been at least
partially stabilized that the state of the extremities
can be rationally addressed and additional input
Figure 3. An anterior/posterior x-ray of an angular
deformity and growth plate arrest. Note the fused
(prematurely closed) and disrupted growth plates on
the left distal femur and left proximal tibia.
Figure 5. Extensive scarring and contractures.
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sought by the critical care physicians from services
such as orthopedic, plastic, and vascular surgery.
The initial assessment of the extremities and
other areas of skin ischemia is important; however, it is rare that actual surgical intervention is
required. Early surgical intervention invariably
leads to
n
higher amputation levels than eventually would
be needed and
n messy, hard-to-manage wounds that can become
both a site for significant fluid-volume loss and
an easy portal for secondary infection.10,12
Likewise, fasciotomy for the purpose of decompressing compartment syndrome-like situations is
not indicated in the vast majority of these patients
because the ischemic damage, regardless of its
etiology, has already occurred. Compartment syndrome occurs when pressure in a muscle compartment, particularly in patients’ arms and legs,
adversely affects the circulation and threatens the
function and viability of the tissues. Surgical decompression complicates patient care by creating
WICK ET AL
difficult-to-manage wounds and has no discernible
effect on the eventual amputation level.10,12 Hence,
the most therapeutic decision an orthopedic surgeon can make at this point is to wait. This waiting
period may last several weeks, but demarcation
between gangrenous and healthy tissue clearly
emerges over time (Figure 6).10,12
For deep tissues such as bone, a technetium-99
isotope nuclear bone scan can be helpful in determining the level of deep perfusion by identifying
areas where there is unusually active bone formation. These scans are frequently used to pinpoint
stress fracture sites or the presence of arthritis,
infection, or cancer in other patients. Approximately three hours before the scan, the radiologist
or technologist administers a dose of a mildly
radioactive substance called technetium through
the patient’s IV line. The bone scan is performed
approximately three hours later, after the bone
has had time to absorb the technetium. Radiology
technologists use a special beta ray detection
camera to take pictures of the patient’s entire
body in a process that takes 30 to 90 minutes.
Figure 6. Tissue demarcation on the patient’s right hand and arm (a) and both lower extremities (b), and the same
right hand (c) and lower extremities (d) after debridement, demonstrating the viable tissue achieved with the
wait-and-see approach.
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MENINGOCOCCEMIA
Absence of radionucleotide uptake in the part of
the skeleton in question denotes a lack of blood
supply to that part of the bone (ie, dead bone).16
The surgeon uses the scan results to determine
what portion of the bone needs to be amputated.
The goal is surgical removal of all necrotic tissue
and preservation of viable deep tissue even if the
tissue is not covered by skin. The surgical treatment
plan should be guided by pediatric, not adult, limb
amputation principles:
n
Preserve limb length whenever possible. This
means preserving not only the length of the
bone but, even more importantly in young
children, preserving the major growth plates.
Loss of the distal femoral growth plate in an
infant will cause the child to have very short
femoral segments as an adult because 70% of
femoral growth comes from the distal femoral
plate.17
n
Preserve the knee joint if possible because the
knee is a vital component of normal gait. Even
a very short segment of the proximal tibia can be
useful, allowing for potential later reconstruction
(eg, lengthening procedures; composite free graft
of bone, muscle, and skin) from the pelvis to
create a more functional limb.17
n
Choose disarticulation versus through-the-bone
amputation whenever possible. For example,
a knee disarticulation is preferable to an abovethe-knee amputation and a Syme disarticulation
(ie, amputation of the foot at the ankle) is
preferable over a transtibial below-the-knee
amputation.17 This avoids overgrowth, provides
a cushion of cartilage that allows end weightbearing on the residual limb, and, in the case
of a through-the-knee amputation, allows for a
more secure prosthetic fitting.
n
A more distal amputation, if the bone is viable,
is better even if the soft tissue coverage is inadequate because soft tissue can be reconstructed later (eg, by wound vacuum dressings,
skin grafts, transpositional flaps, rotational
flaps, free flaps).17
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LATE SURGICAL MANAGEMENT
The initial phase of the disease, with its resulting
insult to the child’s musculoskeletal system, is only
the beginning of the lifelong challenge of dealing
with the long-term sequelae of this condition. The
orthopedic sequelae of this disease are often the
result of amputation, injury to the growth plates,
ischemic injury to the muscles, and scars and
contractures.
Virtually all amputations of the lower limbs and
some upper extremity amputations require prosthetic
replacement. Compared to typical patients undergoing standard amputation, these patients present an
additional challenge in prosthetic fitting because
their residual limbs frequently have poor soft tissue
coverage that contains scars and skin grafts. The
residual limb may also be short and susceptible to
bone overgrowth after transosseous amputations.
Ischemic injury to the remaining growth plate
adds to the challenge of prosthesis fitting because
the growth plate affects normal growth of the
remaining extremity. Growth may stop completely,
resulting in a short residual extremity, or may stop
partially, which leads to angular deformity of the
bone (Figure 7). In a young child, an angular
deformity can be quite pronounced, requiring
multiple surgical procedures to keep the extremity
relatively straight. Growth plate injuries can occur
in either an upper or a lower extremity and can
occur with or without a terminal amputation
having taken place. The proximal growth plate of
an amputated limb can cause an angular deformity
of the residual stump.
Skin and other soft tissue necrosis frequently
leads to scar contractures that affect the range of
motion of joints adjacent to the injury. Contracture
releases, scar resections, and various plastic surgery
procedures may be required to keep the joint
reasonably mobile and functional. Infected wounds
in the affected extremities may be an ongoing
concern. Somewhat surprisingly, these patients are
susceptible to late infections in their extremities
associated both with late surgical or nonsurgical
treatment.10
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WICK ET AL
growth or angle of growth of an extremity) to
correct angular deformity.
Figure 7. Healed limbs with left knee growth arrest
and deformity.
Late surgical procedures that patients may require include
n
n
n
n
n
amputated stump revisions as a result of poor
soft tissue coverage or stump overgrowth;
corrective osteotomies to correct angular malalignment of the extremity;
residual limb lengthening of the short segments
in either amputated or nonamputated limbs;
scar contracture release and other soft tissue
procedures to improve joint motion and functionality (eg, Z-plasty, tissue expanders); and
other procedures to maximize function, such as
n tendon transfers or lengthening to improve
function,
n fibula transfer to the transtibial amputation
stump for improved weight-bearing and to
prevent bone overgrowth, or
n guided growth arrest such as hemiepiphysiodesis (ie, a surgical procedure in which
a bone’s growth plate is altered to affect the
566 j AORN Journal
SPECIAL CONCERNS FOR
PERIOPERATIVE NURSES
In the acute phase of the disease, early surgical
management usually involves resuscitative measures
and line placements, such as endotracheal tubes for
ventilator support, intraosseous needles, peripheral
IV lines if possible, a central venous catheter, an
arterial line, and potentially dialysis access or
a gastrostomy tube.6,7 After the patient is resuscitated and stabilized, the watch-and-wait period
begins as long as the patient’s wounds remain free
from secondary infection (eg, wet gangrene).
After tissue demarcation occurs and the patient
is scheduled for surgical debridement, the surgical
team should prepare for an orthopedic procedure,
which may include amputation and soft tissue
excision of multiple extremities. A plastic surgery
team may work with the orthopedic surgeon if skin
grafts or soft tissue coverage is part of the scheduled procedure. If vacuum-assisted wound dressings are to be used, several devices are available on
the market with a variety of wound sponge sizes.
Most sponges come with occlusive dressings, but
when used on extremities, additional occlusive
dressings may be required to seal the dressing so
that negative pressure can be maintained. When the
dressing is sealed, a hole large enough to allow for
fluid or exudate to pass from the dressing to the
canister is cut in the dressing over the sponge and
an adhesive suction tube disc is applied. This tubing is then connected to the vacuum canister and
the therapy machine is turned on.
All therapy models have leak indicators to verify
an effective seal. If the indicator demonstrates an
ineffective seal, additional occlusive dressings are
applied until a seal is attained. Therapy pressures
are in a targeted range usually between 75 mmHg
and 175 mmHg (ie, therapeutic range is determined
by the surgeon) and the device is set on continuous
mode. Models may differ, so instructions for the
MENINGOCOCCEMIA
device used should be based on manufacturer
specifications. Output to the canister should be
monitored and documented, especially in young
children, to avoid fluid volume imbalances.
The perioperative nurse must understand several
key points concerning the ongoing surgical care of
a patient with meningococcemia:
n
n
n
n
n
n
n
n
n
Thermoregulation as a result of skin impairment
is a vital concern; therefore, careful monitoring
and interventions to maintain normothermia will
be a necessary part of any surgical procedure.
Peripheral line placement may be difficult and
require multiple attempts.
Placement of noninvasive monitors may be
challenging because of multiple limb and tissue
involvement.
Blood loss should be carefully monitored for
pediatric patients undergoing extensive tissue
debridement. Collaboration with the anesthesia
professional is necessary to anticipate fluid
replacement and the need for blood products.
Prepping the patient’s skin requires extra diligence because pitting and scar tissue make it
difficult to clean the area or obtain coverage.
Positioning may present challenges if the
patient has contractures.
Secondary infections may be a long-term problem requiring careful monitoring of wound
drainage, swelling, failure to heal, or recurrent skin breakdown related to actions such as
prosthetic use.
Comorbidities (eg, hypertension, hyperkalemia,
anemia) related to organ failure may present
additional challenges. For example, renal failure
may require careful fluid management, consideration for medication metabolism, and potential for dialysis.
Cognitive or developmental delays as a result of
the disease may require special consideration.
Interactions with these patients need to be at
a developmentally appropriate level. Talking
with the child and his or her caregivers is instrumental in recognizing physical and cognitive
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limitations and providing options for care. This
information is an important component in
patient care hand offs between all involved
care providers.
n These patients require multiple procedures
during the course of the disease and often for
years afterward. Because of this, they often
experience high levels of anxiety and fear,
necessitating the involvement of child life
specialists or implementation of other interventions (eg, administering premedication
before procedures, providing distractions such
as listening to music or watching a favorite
DVD, implementing postoperative pain control measures, talking with the patient and his
or her caregivers to identify other potential
therapeutic interventions to alleviate stress).
REHABILITATION AND PROSTHETICS
After surviving the acute course of the disease, the
patient begins the recovery and rehabilitation
stage. A multidisciplinary team sees the patient
in an outpatient clinic setting. The team is
composed of
n
n
n
n
n
n
a care coordination nurse,
a medical social worker,
an occupational therapist,
a pediatric orthopedic surgeon,
a physical therapist, and
a prosthetist.
The initial evaluation is the first step in establishing a trusting and supportive relationship with
the patient and his or her family members. This
relationship has the potential to last for many
years and functions best when the patient and
family members believe in the team’s expertise
and commitment to achieving the best possible
outcome. One important function of the team is
to identify potential barriers to the rehabilitation
and recovery process. Some potential external
barriers to rehabilitation include socioeconomic
concerns, family dynamics, cultural beliefs, and
the family’s proximity to a specialty care center.
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May 2013
When external barriers have been identified, team
members can address these concerns by connecting the parents with available resources specific
to their needs. Additional patient-related medical
barriers to rehabilitation also may exist, such
as scarring, contractures, weakness, intolerance
of treatment, cognitive limitations, and comorbidities (eg, delayed healing, wound infections,
organ failure).
Parental expectations are always a major
topic of conversation at the initial visit, and team
members typically discuss subjects such as the
anticipated length of initial rehabilitation, the time
frame required for their child to take his or her
first steps, frequency of appointments, and other
general expectations that the patient or family
members may have. Another major component of
the first visit is the initial rehabilitation evaluation
and goal setting with assessment and consideration of
n
n
n
n
n
n
WICK ET AL
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the patient’s current range of motion,
the condition of the patient’s residual limbs,
the patient’s developmental level,
the patient’s immediate and long-term mobility
needs,
transitional movement strategies and planning,
and
preprosthetic treatment plans.
Throughout rehabilitation, the team assesses
and addresses the patient’s mobility needs. Part of
this assessment includes an understanding of the
patient’s stage of mobility. Initial mobility needs
are based on a combination of factors, including
the patient’s general condition and the physical
care demands placed on the family. It is important
to remember that most of these children will
continue to need a wheelchair as a mobility device
option, even after prosthetic fitting, in the event of
tissue breakdown or prosthetic malfunction. They
may also choose to use a wheelchair for mobility
because of the energy demands associated with
prosthetic use. Individuals with high-level lower
extremity amputations typically use a wheelchair
568 j AORN Journal
for primary mobility; if the patient has upper extremity involvement and high-level lower extremity amputation, a wheelchair with powered
mobility should be considered. Other reasons to
consider wheelchair mobility include inability to
use crutches because of upper extremity or bilateral
lower extremity involvement or as a temporary
postoperative mobility device or for use during
prosthetic modification.
As rehabilitation progresses, the team’s focus
turns to assessing the patient’s developmental activities, maximizing mobility independence, improving upper and lower extremity strength, and
optimizing range of motion. During preprosthetic
intervention, it is important to protect and prepare
the patient’s limbs for weight-bearing as the child
begins to mobilize. Early mobility focuses on
achieving developmental milestones and ambulation. This may be aided by an age-appropriate
assistive device (eg, crutches, a walker, a push toy)
to give the child balance and support. As the child
progresses in his or her gross motor function, the
team focuses on maximizing function in higherlevel activities (eg, rising from the floor to the
standing position, learning how to fall safely,
climbing stairs, traversing environmental barriers,
potentially running) while continuing to improve
the patient’s strength and range of motion. Mobility
can be further enhanced by using activity-specific
devices, such as
n
an adaptive bicycle;
n upper extremity, task-specific, terminal devices
(eg, a hook, a violin bow holder, a hammer
attachment); and
n specialized prostheses (ie, water legs for beach
or swimming pool access).
The rehabilitation team will individualize components to match the child’s level of function, needs,
and interests (Table 1). Figures 8 through 10 demonstrate use of play incorporated into therapeutic
activities (eg, hopscotch, basketball, climbing).
(View Supplementary Videos 1 through 6 at http://
www.aornjournal.org.)
MENINGOCOCCEMIA
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TABLE 1. Key Physical Therapy Principles for a Pediatric Patient’s Rehabilitation After
Meningococcemia
n
n
n
n
n
n
n
n
n
n
Use an assistive device with a simple design, particularly with a patient who has upper extremity involvement.
Understand that a patient rehabilitating after meningococcemia does not rely on prosthetic use exclusively for function; the
patient will rely on a combination of methods for mobility depending on the task he or she wishes to perform.
Remember that early independence is important; consider keeping the prostheses short and add height and/or joints at
a later time.
Keep the height of the patient low when upper extremities are involved and consider the patient’s arm span when determining
prosthetic height for both function and safety.
Consider growth and delayed effects of the disease.
Remember that changes in growth often necessitate new interventions.
Rely on the expertise of the patient’s parents and community team members; their input is critical for successful management
of the patient.
Impress strongly on the patient and parents that rehabilitation and therapy are ongoing as the child grows and matures.
Anticipate that there will times during rehabilitation that the patient will be unable to use a prosthesis because of multiple
orthopedic needs.
Do not let public opinion prevail over achieving functional objectives and using common sense when determining what is best
suited for the child rehabilitating after meningococcemia.
Practitioners must consider several factors during the first prosthetic fitting, including
n
tissue tolerance and protection of soft tissue and
bone,
n accommodation of contractures and deformities,
and
n keeping the patient close to the ground.
Initially, patients with bilateral transtibial amputations and Syme disarticulations are kept as close to
the ground as practical, and patients with knee
disarticulations and transfemoral amputations are
fitted with stubbies (ie, prosthetic sockets directly
attached to a rocker bottom weight-bearing surface)
(Figure 11).
After the patient progresses and becomes an
independent ambulator using his or her initial
prosthetic devices, the team can increase the height
and complexity of the components. At this point, it
may be necessary for the patient to undergo an
extended period of dynamic alignment to optimize
prosthetic fit. It is not uncommon to have the patient in an unfinished leg for six to eight weeks
as he or she progresses in rehabilitation. Keeping
the prosthesis in an unfinished state allows for
maximum adjustability, which optimizes fit and
alignment before the prosthesis is finished. Over
time, people with Syme disarticulations or transtibial amputations may have their prostheses increased in height to match their peers’ height and
may be provided with higher-functioning prosthetic
feet. Knee disarticulations or transfemoral prostheses will be progressively lengthened; eventually
locking knees will be added and then transitioned
to full-function knees with corresponding changes
in foot components. The complexity of the patients’
prostheses and the patients’ evolving needs as they
grow to adulthood underscore the necessity for
these patients to be treated by a prosthetist who
specializes in pediatrics (Table 2 and Figure 12).
The level of amputation and its effect on the
patient’s ability to function are significant factors
in planning treatment. It is typical to see multiple
levels of amputation on the same patient, potentially
including all four extremities. As a general rule,
higher levels of amputation result in greater energy
expenditure for the patient when he or she is using
a prosthesis or ambulation aid. This requires more
extensive rehabilitation time to allow the patient to
reach maximum function capacity. Studies on pediatric patients show that oxygen consumption is
increased the higher (ie, more proximal) the level of
AORN Journal j 569
May 2013
Vol 97 No 5
Figure 8. Hopscotch is a therapeutic activity that
focuses on single-leg stance, jumping from a surface,
foot placement consistency, and awareness of
dynamic foot placement (ie, the patient is aware of
his or her foot movements in space).
amputation (Table 3).18 Patients learn to balance the
increased energy cost of ambulating, which is
calculated based on standard walking speeds, by
reducing their self-selected walking speeds.
Team members should take upper extremity
involvement, whether it involves loss of a functional hand or reduced limb length, into consideration when determining the height to aspire to for
patients with bilateral lower extremity involvement. The patient’s limited protective extension if
he or she falls and independence with transitional
movements (ie, floor to stand, sit to stand, toileting)
are factors that must be dealt with if the patient has
upper extremity involvement. The patient’s limited
mobility or grasping ability may dictate the need
for lower extremity prosthesis design modification,
or the patient may require use of upper extremity
assistive devices.
570 j AORN Journal
WICK ET AL
Figure 9. Basketball is a therapeutic activity that
focuses on backward balance and upper extremity use.
Upper extremity amputations also may affect the
patient’s ability to perform activities of daily living,
make donning prosthetics more difficult, and hinder
recreational pursuits. Adaptive equipment and
training may be needed to address these issues.
Initially a child may want to try an upper extremity
prosthesis, but the rejection rate is very high.19 A
child with a below-the-elbow amputation may
prefer to use his or her elbow joint rather than
a prosthesis to hold or grip items, while a child with
an above-the-elbow amputation may find a prosthesis too heavy and cumbersome for daily wear. In
many situations, an activity-specific prosthesis may
be beneficial, especially as a child matures and
wants to participate in sports and peer-related
activities. Collaboration with a prosthetist and an
occupational therapist could be helpful in these
circumstances.
MENINGOCOCCEMIA
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Figure 11. Stubbies are prosthetic sockets directly
attached to a rocker bottom, weight-bearing surface
for patients with an above-the-knee amputation.
Figure 10. Climbing stairs is a therapeutic activity
that focuses on uneven surfaces, single-leg stance,
weight shift, and knee flexion and extension.
When the need for intensive rehabilitation
has lessened, the patient’s care can transition to
community-based services and care providers who
have more frequent contact and an increased role
in the child’s everyday care. This often takes the
form of early intervention or school-based physical
therapy. It is important for these therapists to be
able to communicate and collaborate with members
of the specialty team to provide the best possible
care for the patient. Patients may continue to get
their prosthetic care at the specialty care facility
because those providers have the specific skill set
needed to treat this population.
The transition to school can present its own
challenges, which include, but are not limited to,
n
peer education about prosthetic devices,
n teacher education about prosthetic devices,
n peer acceptance, and
n maximizing the patient’s ability to participate in
school-related activities.
It is important to prepare the patient and family
members for situations they may encounter. When
feasible, this transition can be eased by a visit to the
school from one or more of the team members (eg,
child life specialist, social worker, physical therapist, prosthetist) to promote understanding and acceptance of children with differences. As the child
matures, it is important to provide appropriate
support to address potential body image concerns
and other transitional counseling and assistance as
he or she approaches adulthood.
As these patients grow, so does their interest in
recreational activities. Involvement in communitybased programs and appropriate levels of competition and recreation are encouraged. Often, it is
necessary for the parents to consult with the team
so that adaptations to recreational devices or prosthetics can be made to allow for participation in
desired activities, whether through mechanical
innovation or specialized therapy.
CASE REPORT
Patrick contracted meningococcemia in late spring,
just before his third birthday. He was admitted to
a pediatric intensive care unit (PICU) in a Level One
children’s hospital with severe systemic sepsis and
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WICK ET AL
Vol 97 No 5
TABLE 2. Key Prosthetic Principles for a Pediatric Patient’s Rehabilitation After
Meningococcemia
n
n
n
n
n
n
n
n
Be patient with healing; it is not uncommon to have repeated tissue breakdown because of fragile tissue and bony
overgrowth.
Maximize pressure distribution with the use of viscoelastic polymer pads and atypical points of contact.
Remember that prosthetic use only becomes a priority when other comorbidities (eg, infection, postoperative skin graft
healing, contracture management) have been resolved.
Be aware that changes in joint alignment as a result of growth plate disturbance often necessitate atypical prosthetic
alignment.
Rely on the expertise of team members; their input is critical for successful management of the patient.
Impress strongly on the patient and parents the importance of regular skin checks; these patients can develop skin
breakdown rapidly.
Before delivering a finished product, leave the prosthesis “in the rough” while the patient with complex problems works with
a therapist.
Do not let public opinion prevail over achieving functional objectives and using common sense when determining what is best
suited for the child rehabilitating after meningococcemia.
multi-organ failure; he spent five weeks on ventilator
support. During his PICU stay, Patrick underwent
a Hickman catheter implantation and gastrostomy
tube placement, after which renal dialysis was
started. He was also diagnosed with adrenal
Figure 12. Fitting new legs for the finished form.
572 j AORN Journal
insufficiency, for which he needed careful monitoring and supplementary corticosteroid treatment.
A pediatric orthopedic surgeon provided consultation about Patrick’s care in the early stage of
his PICU admission and provided care throughout
his hospital stay. After he was discharged from the
PICU, Patrick spent an additional four months on
the general pediatric ward because of ischemic
involvement and gangrene to all four extremities.
Over time, all of the fingers on Patrick’s left hand
auto-amputated. He lost the thumb and little finger
on his right hand, and the three remaining fingers
required surgical tenolysis (ie, surgically releasing
a tendon from adhesions) to regain some range of
motion. Patrick has undergone multiple skin grafts
to his left forearm. Both lower limbs required Syme
disarticulations with skin graft coverage of some of
the necrotic areas. The right Syme disarticulation
healed without incident, but a residual defect
remained on the left stump over the medial malleolus. In addition to the necrotic tissue of the left
lower extremity, an additional small wound was
present on the anterior aspect of the left knee in an
area with previous necrosis and patellar loss.
As a result of the need for ongoing orthopedic
follow-up and rehabilitation, Patrick’s care was
transferred from the university-based acute care
facility to a pediatric orthopedic specialty hospital.
MENINGOCOCCEMIA
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TABLE 3. How Amputation Level Affects Energy Consumption
1
Syme
Transtibial
Transfemoral
disarticulation
amputation
amputation
(disarticulation (amputation
Knee
(amputation
Hip
Walking unilateral at the ankle) below the knee) disarticulation above the knee) disarticulation
Percent of normal
energy consumption
105% 25%
110% 18%
122% 14%
151% 31%
161% 55%
Editor’s note: This table considers only unilateral involvement; it is important to note that many patients in this population have bilateral involvement with
a resulting higher impact on energy expenditure.
1. Jeans K, Browne R, Karol L. Effect of amputation on energy expenditure during overground walking by children with an amputation. J Bone Joint Surg
Am. 2011;93(1):49-56.
His residual lower extremity wounds required six
additional surgical procedures involving irrigation,
debridement, and wound vacuum dressing application. The knee wound healed without incident.
After the sixth surgery, granulation tissue around
the medial malleolus was sufficient to support
a split-thickness skin graft. Manufacturing a prosthetic device for his left lower limb was a challenge
and included creating custom prosthetic liners with
multiple fittings and adjustments. Despite this, Patrick continued to have pressure and skin breakdown
issues on his left stump, so his left Syme disarticulation was revised to a below-the-knee amputation to
improve his prosthetic fit and mobility. Currently,
Patrick’s left stump is healing well, and he was
recently fitted with a shrinker sock (ie, a compressive sock used to reduce limb volume in preparation for his below-the-knee prosthesis).
A 90-degree flexion contracture of his left elbow
required multiple Z-plasty procedures to release
the elbow to a residual 20-degree contracture. A
Z-plasty procedure is a type of transpositional flap
technique that incorporates advancement and rotation by lengthening a contracted scar or rotating
the scar tension line. The surgeon makes the middle line of the Z-shaped incision along the line of
greatest tightness and then raises and transposes the
triangular flaps on opposite sides of the two ends
to relieve tension along the length of the contracture.20 Maintaining mobility of his left arm and hand
has been the focus of Patrick’s occupational therapy.
It is anticipated that his left wrist will eventually
require an arthrodesis (ie, surgical fixation of the
joint designed to accomplish fusion of the joint
surfaces) to improve alignment and function.
To date, although Patrick is only four-and-a-half
years old, he has undergone more than 20 surgical
procedures between the two hospitals. He is seen
every two to three months by a multidisciplinary
team composed of a pediatric orthopedic surgeon,
a care coordinator, a physical therapist, an occupational therapist, and a prosthetist. This team
will follow him through his growth and development, optimizing his mobility and function whenever possible. He may eventually require a renal
transplant for stage 3 renal failure with related
hypertension, hyperkalemia, and anemia.
CONCLUSION
The young survivors of meningococcemia face
a lifetime of challenges, some of which are related
to their limb deficiencies. Regardless of the initial
clinical presentation, early consultation with a
pediatric orthopedist is vital to help patients successfully deal with the orthopedic sequelae associated with the disease. Careful attention to bone
growth and recognition of growth arrests allows for
early detection and timely surgical intervention.
Working with physical and occupational therapists experienced with pediatric development helps
these children maximize their potential. As the
child grows to adulthood, continued access to a
AORN Journal j 573
May 2013
WICK ET AL
Vol 97 No 5
skilled prosthetist for the manufacture and fitting of
prosthetics is also vital. A multidisciplinary team
approach under the supervision of a pediatric orthopedist can optimize and improve the quality of
life for these children.
SUPPLEMENTARY DATA
The supplementary videos associated with this
article can be found in the online version at http://
dx.doi.org/10.1016/j.aorn.2013.03.005.
Acknowledgments: The authors thank the following
people from Shriners Hospital Portland, OR:
Harlan Pine, graphic arts specialist, for obtaining photographs, videotapes, and x-rays; Kelly
Alexander, RN, care coordinator, for providing
background information for the article; and Lisa
McIntyre, OTR, occupational therapist, for providing information concerning occupational therapy for this patient population.
References
1. The changing epidemiology of meningococcal diseases
among U.S. children, adolescents and young adults.
Bethesda, MD: National Foundation for Infectious
Diseases; 2004.
2. Meningitis (meningococcal disease). National Foundation for Infectious Diseases. http://www.adultvaccination
.com/meningococcal_vaccine_meningitis_vaccine_vacci
nation_adult_immunization.htm. Accessed February 27,
2013.
3. Meningococcal disease (Meningococcal meningitis and
septicaemia). Meningococcal Education. http://www
.meningococcal.org. Accessed February 27, 2013.
4. Meningococcal disease fact sheet. March 2009. Oregon
Health Authority Public Health. http://public.health
.oregon.gov/diseasesconditions/diseasesaz/meningoco
ccaldisease/pages/facts.aspx. Accessed January 14,
2013.
5. Meningitis (meningococcal disease). Washington State
Department of Health. http://www.doh.wa.gov/Youand
YourFamily/Immunization/Diseases/MeningitisMeningo
coccalDisease.aspx. Accessed January 14, 2013.
6. Edlich RF, Bronze MS. Necrotizing fasciitis and purpura
fulminans. Updated September 27, 2012. Medscape
Reference: Drugs, Diseases & Procedures. http://emedici
ne.medscape.com/article/1348047-overview#a30.
Accessed January 14, 2013.
7. Management of invasive meningococcal disease in children and young people: a national clinical guideline. May
2008. Scottish Intercollegiate Guidelines Network. http://
www.sign.ac.uk/pdf/sign102.pdf. Accessed January 14,
2013.
574 j AORN Journal
8. Nadel S, Kroll J. Diagnosis and management of meningococcal disease: the need for centralized care. FEMS
Microbiol Rev. 2007;31(1):71-83. http://onlinelibrary
.wiley.com/doi/10.1111/j.1574-6976.2006.00059.x/pdf.
Accessed January 14, 2013.
9. Davies MS, Nadel S, Habibi P, Levin M, Hunt DM. The
orthopaedic management of peripheral ischaemia in
meningococcal septicaemia in children. J Bone Joint
Surg Am. 2000;82(3):383-386.
10. Canavese F, Krajbich J, LaFleur. Orthopaedic sequelae
of childhood meningococcemia: management considerations and outcome. J Bone Joint Surg Am. 2010;92(12):
2196-2203.
11. Bache C, Torode I. Orthopaedic sequelae of meningococcal septicemia. J Pediatr Orthop. 2006;26(1):
135-139.
12. Canavese F, Krajbich J, Kuang A. Application of the
vacuum-assisted closure in pediatric patients with
orthopedic sequelae of meningococcemia: report of
a case successfully treated. J Pediatr Orthop. 2009;18(6):
388-391.
13. Webb LX, Schmidt U. Wound management with vacuum therapy [article in German]. Unfallchirurg. 2001;
104(10):918-926.
14. Nectoux E, Mezel A, Raux S, Fron D, Klein C, Herbaux B.
Meningococcal purpura fulminans in children. II: Late
orthopedic sequelae management. J Child Orthop. 2010;
4(5):409-416.
15. Buysse CM, Oranje AP, Zuidema E, et al. Long-term skin
scarring and orthopaedic sequelae in survivors of meningococcal septic shock. Arch Dis Child. 2009;94(5):
381-386.
16. Glossary of orthopaedic diagnostic tests. American Academy of Orthopedic Surgeons. http://orthoinfo.aaos.org/
topic.cfm?topic¼A00272. Accessed January 14, 2013.
17. Amputations. POSNA: The Pediatric Orthopaedic
Society of North America. http://www.posna.org/educa
tion/StudyGuide/amputations.asp. Accessed January 14,
2013.
18. Jeans KA, Browne RH, Karol LA. Effect of amputation
on energy expenditure during overground walking by
children with an amputation. J Bone Joint Surg Am.
2011;93(1):49-56.
19. Davids J, Wagner LV, Meyer LC, Blackhurst DW. Prosthetic management of children with unilateral congenital
below-the-elbow deficiency. J Bone Joint Surg Am. 2006;
88(6):1294-1300.
20. Sclafani A. Z-Plasty. Medscape Reference. http://emedici
ne.medscape.com/article/879878-overview. Accessed
February 22, 2013.
Resources
Cohn A, Jackson ML. Meningococcal disease. Centers for
Disease Control and Prevention. http://wwwnc.cdc.gov/
travel/yellowbook/2010/chapter-2/meningococcal-disease
.aspx. Accessed January 14, 2013.
Meningococcal meningitis and septicaemia: guidance notes.
Meningitis Research Foundation. http://www.meningitis
.org/assets/x/50834. Accessed January 14, 2013.
Welch SB, Nadel S. Treatment of meningococcal infection.
Arch Dis Child. 2003;88(7):608-614. http://adc.bmj.com/
content/88/7/608.full. Accessed January 14, 2013.
MENINGOCOCCEMIA
Jane M. Wick, BSN, RN, is an OR nurse at
Shriners Hospital for Children, Portland, OR. Ms
Wick has no declared affiliation that could be
perceived as posing a potential conflict of interest in the publication of this article.
Ivan Krajbich, MD, FACS, is a pediatric orthopedic surgeon in the Department of Pediatric
Orthopedics at Shriners Hospital for Children,
Portland, OR. Dr Krajbich has no declared
affiliation that could be perceived as posing
a potential conflict of interest in the publication
of this article.
www.aornjournal.org
Shannon Kelly, MPT, is a physical therapist
at Shriners Hospital for Children, Portland,
OR. Ms Kelly has no declared affiliation that
could be perceived as posing a potential
conflict of interest in the publication of this
article.
Todd DeWees, BS, CPO, is a certified prosthetist orthotist at Shriners Hospital for Children,
Portland, OR. Mr DeWees has no declared
affiliation that could be perceived as posing
a potential conflict of interest in the publication
of this article.
AORN Journal j 575
EXAMINATION
3.6
CONTINUING EDUCATION PROGRAM
Meningococcemia: The Pediatric
Orthopedic Sequelae
www.aorn.org/CE
PURPOSE/GOAL
To enable the learner to identify meningococcemia early in its course and understand the multidisciplinary approach to long-term treatment of the orthopedic
sequelae of the disease.
OBJECTIVES
1.
2.
3.
4.
Discuss the etiology of meningococcemia.
Identify the symptoms of meningococcemia.
Explain how meningococcemia is diagnosed.
Describe orthopedic treatment options for pediatric patients with
meningococcemia.
5. Discuss perioperative nursing care of the pediatric patient undergoing surgical
treatment for meningococcemia.
The Examination and Learner Evaluation are printed here for your convenience. To receive continuing education credit, you must complete the Examination and Learner Evaluation online at http://www.aorn.org/CE.
QUESTIONS
1.
2.
Children younger than five years of age account for
two-thirds of meningococcal cases because they
1. have a tendency to put things in their mouths.
2. have immature immune systems.
3. have well-developed immune systems.
4. tend to share food and drinks.
a. 1 and 3
b. 2 and 4
c. 1, 2, and 4
d. 1, 3, and 4
Meningococcal disease is transmitted via the
nasopharyngeal secretions of people colonized by
the aerobic, gram-negative diplococcus bacterium
a. Acidaminococcus fermentans.
b. Microbacterium neimengense.
576 j AORN Journal
May 2013
Vol 97
No 5
c. Neisseria mengingitidis.
d. Saccharobacter fermentatus.
3.
Early symptoms of the disease are flu-like in
nature and include
1. fever.
2. lack of energy.
3. muscle and joint aches.
4. severe headache.
5. sore throat.
a. 4 and 5
b. 1, 2, and 3
c. 1, 2, 3, and 4
d. 1, 2, 3, 4, and 5
4.
Despite aggressive treatment, the mortality rate
for those infected with meningococcal disease is
a. 10%.
b. 15%.
c. 20%.
d. 25%.
Ó AORN, Inc, 2013
CE EXAMINATION
5.
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Late orthopedic sequelae include
1. growth plate disturbances.
2. scar contractures.
3. soft tissue or bone infections.
4. stump overgrowth at transosseous amputation
sites.
5. purpura fulminans.
a. 1, 2, and 5
b. 1, 2, 3, and 4
c. 2, 3, 4, and 5
d. 1, 2, 3, 4, and 5
2.
3.
4.
5.
6.
6.
Typically, for these patients, fasciotomy to
decompress compartment syndrome-like situations is not indicated.
a. true
b. false
7.
The surgical treatment plan should be guided
by pediatric limb amputation principles, which
include
1. choosing disarticulation versus through-thebone amputation whenever possible.
2. debriding devitalized tissue as soon as
possible.
3. opting for a more distal amputation if the
bone is viable.
4. preserving limb length and the major growth
plates whenever possible.
5. preserving the knee joint if possible.
a. 4 and 5
b. 1, 2, and 3
c. 1, 3, 4, and 5
d. 1, 2, 3, 4, and 5
8.
When planning for a surgical procedure on a child
with meningococcemia, the perioperative nurse
should prepare to
1. help the anesthesia professional monitor
blood loss carefully and anticipate fluid
replacement and the need for blood
products.
assist with peripheral line placement that
may require multiple attempts.
assist with placement of noninvasive monitors, which may be challenging because of
multiple limb and tissue involvement.
encounter positioning difficulties with the
patient who has contractures.
use extra diligence in prepping because the
patient’s skin may be pitted and scarred.
ensure thermoregulation to maintain
normothermia.
a. 1, 3, and 5
b. 2, 4, and 6
c. 2, 3, 5, and 6
d. 1, 2, 3, 4, 5, and 6
9.
During the initial rehabilitation evaluation and goal
setting, team members should assess and consider
1. the patient’s current range of motion.
2. the condition of the patient’s residual limbs.
3. the developmental level and needs of the
patient.
4. transitional movement strategies and planning.
5. preprosthetic treatment plans.
a. 1 and 3
b. 1, 4, and 5
c. 2, 3, and 5
d. 1, 2, 3, 4, and 5
10.
A Z-plasty procedure
1. is a type of transpositional flap technique.
2. lengthens a contracted scar or rotates the scar
tension line.
3. allows for early debridement of devitalized
tissue.
4. involves the middle line of the incision being
made along the line of greatest tightness.
5. relieves tension along the length of the
contracture.
a. 2 and 3
b. 1, 2, 4, and 5
c. 1, 3, 4, and 5
d. 1, 2, 3, 4, and 5
AORN Journal j 577
LEARNER EVALUATION
CONTINUING EDUCATION PROGRAM
Meningococcemia: The Pediatric
Orthopedic Sequelae
T
his evaluation is used to determine the extent
to which this continuing education program
met your learning needs. Rate the items as
described below.
OBJECTIVES
To what extent were the following objectives of this
continuing education program achieved?
1. Discuss the etiology of meningococcemia.
Low 1. 2. 3. 4. 5. High
2. Identify the symptoms of meningococcemia.
Low 1. 2. 3. 4. 5. High
3. Explain how meningococcemia is diagnosed.
Low 1. 2. 3. 4. 5. High
4. Describe orthopedic treatment options for pediatric
patients with meningococcemia.
Low 1. 2. 3. 4. 5. High
5. Discuss perioperative nursing care of the pediatric
patient undergoing surgical treatment for meningococcemia. Low 1. 2. 3. 4. 5. High
CONTENT
6. To what extent did this article increase your
knowledge of the subject matter?
Low 1. 2. 3. 4. 5. High
7. To what extent were your individual objectives met?
Low 1. 2. 3. 4. 5. High
8. Will you be able to use the information from this
article in your work setting? 1. Yes 2. No
578 j AORN Journal
May 2013
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No 5
3.6
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9. Will you change your practice as a result of reading
this article? (If yes, answer question #9A. If no,
answer question #9B.)
9A. How will you change your practice? (Select all that
apply)
1. I will provide education to my team regarding
why change is needed.
2. I will work with management to change/
implement a policy and procedure.
3. I will plan an informational meeting with
physicians to seek their input and acceptance
of the need for change.
4. I will implement change and evaluate the
effect of the change at regular intervals until
the change is incorporated as best practice.
5. Other: _______________________________
9B. If you will not change your practice as a result of
reading this article, why? (Select all that apply)
1. The content of the article is not relevant to my
practice.
2. I do not have enough time to teach others
about the purpose of the needed change.
3. I do not have management support to make
a change.
4. Other: _______________________________
10. Our accrediting body requires that we verify
the time you needed to complete the 3.6 continuing education contact hour (216-minute)
program: ________________________________
Ó AORN, Inc, 2013