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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_1134_Библиотеки_им_академика_М_И_Перельмана.pdf
The Pediatric Patient Cared for in the Adult ICU
Z o ё Maher and Michael L. Nance
3 7
Initial Resuscitation of the Pediatric Intensive Care Patient
Physiology of Shock
There are many similarities between the physiology of
pediatric and adult shock, including the types of circulatory
shock: hypovolemic, cardiogenic, obstructive, and
distributive (Table 37.1 ). Pediatric patients, however, may
demonstrate more subtle manifestations of the shock state,
leading to potential for delayed recognition. Additionally,
the response to states of altered ventricular preload, cardiac
contractility, and vascular resistance is different in pediatric
patients than adults. Cardiac output (CO) is more heavily
dependent on heart rate (HR) than stroke volume (SV) in the
young pediatric population as ventricular myocyte mass is
still developing. Additionally, children are able to mount a
signifi cant and lasting increase in systemic vascular resistance (Fig. 37.1 ). Therefore, in contrast to adults, pediatric
patients in states of shock may manifest tachycardia without
hypotension [ 37 ]. It is critical to recognize shock state before
the development of hypotension. Upon recognition of shock,
volume resuscitation, inotropic support, and vasoactive therapy must be rapidly implemented. Early consideration of
adjunctive support measures such as extracorporeal membrane oxygenation (ECMO), intra-aortic balloon pump
(IABP), and ventricular assist devices (VADs) may improve
outcomes for pediatric patients in refractory shock.
Broselow™ System
The Broselow™ color-coded system is designed for estimation of pediatric weight and endotracheal tube size based
on body length. This color-coded bag provides a number of
resuscitation adjuncts, including the Broselow™ tape
which assists in medication dosage estimation and the
Broselow™ bag which are color-coded, size-based procedural supplies. The Broselow™ system is helpful in the
Table 37.1 Physiologic changes in pediatric shock states
Type of Shock Preload Afterload Contractility
Hypovolemic
Cardiogenic
Obstructive
Distributive or or N
From Wheeler [
140
100
64 ], with permission from Springer
Vascular resistance
N
N
60
Z. Maher , MD (*)
Division of Trauma and Critical Care, Department of Surgery ,
Temple University Hospital , Philadelphia , PA 19140 , USA
zoe.maher@tuhs.temple.edu
e-mail:
M. L. Nance , MD
Department of Surgery , Surgery Perelman School of Medicine,
Pediatric Trauma Program, Children’s Hospital of Philadelphia ,
Philadelphia , PA 19104 , USA
nance@email.chop.edu
e-mail:
© Springer International Publishing Switzerland 2016
N.D. Martin, L.J. Kaplan (eds.), Principles of Adult Surgical Critical Care, DOI 10.1007/978-3-319-33341-0_37
Percent of control
Cardiac output
20
Percent of volume deficit
Fig. 37.1 Physiologic compensation in pediatric hypovolemic shock
(From Wheeler [
64 ], with permission from Springer)
Blood pressure
5025 75
431
432
Z. Maher and M.L. Nance
early resuscitation of the critically ill pediatric patient and
should be maintained at any center with the possibility of
managing pediatric patients. However, Nieman et al. have
recently called into question the accuracy of the tape owing
in part to the challenge of weight-based dosing in the obese
population [ 42 ].
Pediatric Airway Management
The majority of pediatric cardiac arrests result from ventilatory arrest and are only rarely due to a primary cardiac etiology. Therefore, successful management of the pediatric
airway is of critical importance. Of note, up to 10 % of all
pediatric ICU intubations are considered diffi cult, owing in
part to the anatomic challenges of the pediatric airway [ 23 ].
Anatomic Considerations
Anatomic differences between the pediatric and adult airway
persist until the airway has reached anatomic maturity
between the ages of 8 and 14 years. Most signifi cantly the
pediatric airway differs from the adult in terms of (1) relatively larger occipital size increasing risk of supine position
airway obstruction; (2) maximal narrowing of the airway at
the cricoid cartilage due to cylindrical shape of the airway;
(3) relatively larger tongue size; (4) relatively narrow and
short trachea; (5) more acute nasopharyngeal angle; (6)
larger, fl oppy epiglottis; and (7) more cephalad and anterior
larynx (Figs. 37.2 and 37.3 ). These differences must be con-
sidered when managing the pediatric airway.
Basic Airway Management
Basic airway control should begin with the three Ps: position
favorably, prevent aspiration, and promote gas exchange.
Favorable positioning can be accomplished with a jaw thrust
in combination with the head lift-chin tilt maneuver if no cervical spine injury is suspected. The placement of a nasopharyngeal airway, or an oral airway in the obtunded patient with
no gag refl ex, should then be followed by bag-mask ventilation. Aspiration risk can be minimized with initial application
of cricoid pressure in the unresponsive patient and application
of the minimal positive pressure required to generate chest rise
[ 5 , 40 ]. Chest rise indicates adequate volume of ventilation.
Advanced Airway Management
The pediatric advanced airway has classically been
managed with the placement of an endotracheal tube.
However, laryngeal mask airway (LMA) application has
been broadening among the pediatric population in recent
years [
57 ]. The tip of this supraglottic airway device is
designed to oppose the epiglottis providing an airway seal
upon cuff infl ation. However, caution must be exercised to
avoid overfi lling the LMA cuff as this can lead to airway
obstruction or pharyngeal nerve injury [
be used as a temporizing airway or as a conduit for the
placement of an endotracheal tube or other adjuncts to permit intubation. First-generation devices are not designed to
prevent aspiration of gastric contents and should be used
only when endotracheal intubation is not possible. Secondgeneration devices are capable of drainage of gastric contents, though they are not yet widely available [
Methods for approximating pediatric LMA size include
using the combined width of the patient’s second, third, and
fourth digits or the following formula proposed by Ho
et al.: weight (kg) of patient = 2
size and cuff infl ation volume (ml) = 5 × LMA [
43 ]. The LMA can
2×LMA
, where LMA is the
25 ].
26 ].
Endotracheal Intubation Considerations
Length-based estimation and age-based formula estimation
are both acceptable means of choosing endotracheal tube
size, offering comparable accuracy. Additionally, approximation of endotracheal tube diameter by the fi fth digital circumference has been written about by authors such as King
et al. who concluded that this method is inferior to age-based
formulas and should be reserved for situations in which age
is unknown [ 31 ].
The most widely applied age-based estimation formulas
are the Cole and Khine formulas. Cole’s formula predicts
uncuffed endotracheal tube size as equal to (age 4) + 4, whereas
Khine’s formula predicts cuffed endotracheal tube size as
equal to (age/4) + 3 [ 30 , 58 ]. For length-based estimation, the
Broselow™ tape and color-coded system provide guidance for
endotracheal tube sizing. Uncuffed endotracheal tubes are
generally reserved for patients less than 8 years of age, though
recent data has challenged the assertion of cuffed endotracheal
tubes is unsafe for children in this age range [ 58 ]. In addition
to careful consideration of endotracheal tube size, appropriate
selection of type and length of laryngoscope is also critical
(Table 37.2 ).
Fluid Resuscitation
Resuscitation strategy in the critically ill pediatric patient
begins with venous access. Venous access considerations
include size and length of the catheter and available sites
for access. In the adult patient, rapid replacement of volume necessitates the placement of large bore access such as
a 14 gauge peripheral catheter or an 8.5 French central
catheter. However, the same volume replacement can be
37 The Pediatric Patient Cared for in the Adult ICU
Fig. 37.2 Anatomy of the
pediatric ( a ) and adult ( b ) airway
(From Wheeler [
permission from Springer)
64 ], with
Tongue
Epiglottis
(floppier,
u-shaped)
Hyoid bone
Airway
anterior
and higher)
Trachea
(more flexible)
a
433
Funnel
(more
Vocal cords
Thyroid cartilage
Cricoid r ing
(narrowest)
Anterior Posterior
Tongue
Epiglottis
(shorter)
Hyoid bone
Trachea
b
accomplished in a 20 kg 7-year-old using two 20 gauge
catheters or using one 22 gauge catheter with a 10 cc
syringe for fl uid boluses in a 5 kg child [
to clinical goals should be modifi ed based on the etiology
of the shock state. End points should include normal mental
status, less than 2 seconds capillary refi ll, normal central
and peripheral temperature, adequate urine output of
greater than 1 cc/kg/h, and normal age- adjusted pulse and
blood pressure (Table 37.3 ). In the case of hypovolemic
24 ]. Resuscitation
Cylinder
Thyroid cartilage
Vocal cords
(narrowest)
Cricoid ring
Anterior Posterior
shock, a 20 cc/kg bolus of crystalloid should be the initial
choice for fl uid management, followed by a second bolus of
the same in the case of failure to respond. If hemorrhagic
shock is suspected, a 10 cc/kg blood product transfusion
should be considered to replace the second or third crystalloid bolus. Hypotension does not develop in hemorrhagic
shock until up to 50 % of the blood volume has been lost in
the pediatric population. Therefore, early recognition and
intervention are critical.
434
Z. Maher and M.L. Nance
a
T
O
P
b
T
O
P
Table 37.2 Pediatric laryngoscope selection
Child’s weight (kg) Laryngoscope
0–3 Miller 0
3–5 Miller 0, 1
5–15 Miller 1
12–20 Macintosh 2
20–30 Macintosh 2, Miller 2
>30 Macintosh 3, Miller 2
From Wheeler [
Table 37.3 Normal age-adjusted vital signs
Age
Premature 120–170 55–75/35–45 40–70
0–3 months 100–150 65–85/45–55 35–55
3–6 months 90–120 70–90/50–65 30–45
6–12 months 80–120 80–100/55–65 25–40
1–3 years 70–110 90–105/55–70 20–30
3–6 years 65–110 95–110/60–75 20–25
6–12 years 60–95 100–120/60–75 14–22
>12 years 55–85 110–135/65–85 12–18
64 ], with permission from Springer
Heart rate
(beats per
minute)
Blood pressure
(mmHg)
Respiratory
rate (breaths
per minute)
c
T
P
O
Fig. 37.3 Positioning for pediatric airway alignment (From Wheeler
64 ], with permission from Springer). Abbreviations: O oral axis, T tra-
[
cheal axis, and P pharyngeal axis
Pediatric Traumatic Brain Injury
For pediatric trauma patients with traumatic brain injury
(TBI), as in adults, prevention of secondary brain injury resulting from hypoxia or hypotension is essential. In addition to
maintenance of cerebral perfusion pressure (CPP) above
40 mmHg, the literature supports avoidance of hypoxemia,
defi ned as PaO 2 less than 60 mmHg [ 32 , 46 , 47 , 61 ].
Conceptually, maintaining CPP and systemic blood pressure
will improve cerebral blood fl ow (CBF), though some data
challenges the notion that these are predictable relationships
[ 45 ]. According to guidelines for the acute management of
pediatric TBI published by the Brain Trauma Foundation [ 32 ],
consideration should be given to:
1. Hypertonic saline infusion for severe TBI with associated
intracranial hypertension:
(a) Dose 3 % normal saline at 0.1–1 cc/kg/h to maintain
ICP <20 mmHg.
(b) Monitor and maintain serum osmolarity 360 mOsm/L.
2. Moderate hypothermia (32–33 °C) for up to 48 h follow-
ing severe TBI
3. Avoidance of prophylactic severe hyperventilation (PCO 2
<30 mmHg) during the fi rst 48 h after injury
Corticosteroids are NOT recommended in the acute management of pediatric TBI, as they have been shown to provide no
benefi t and may increase the risk of inhospital infection [ 18 ].
Pediatric Analgesia and Sedation
General Approach
Pediatric patients in the intensive care unit may experience
pain related to a medical condition, surgical procedure,
37 The Pediatric Patient Cared for in the Adult ICU
435
Table 37.4 Anesthetic agents for pediatric populations
Inhalational anesthetic agents
Benzodiazepines
Opioids
Phenothiazines
Butyrophenones
Antihistamines
Chloral hydrate
Etomidate
Ketamine
Barbiturates
Propofol
Alpha-adrenergic agonists
From Wheeler [
65 ], with permission from Springer
endotracheal intubation, or other procedures. Anxiety and
agitation may compound pain and be precipitated by the
separation from parents and familiar environment, sleep
deprivation, and loss of self-control and the ability to selfsooth [ 54 ]. Attention to analgesia and sedation for the pedi-
atric patient is therefore essential. Tolerance, withdrawal,
and physical dependency on sedative and analgesic medications have long been reported in the adult literature, and
building evidence documents the occurrence in the critically
ill pediatric population as well [ 59 ]. As such, children who
are exposed to long-term infusions of these medications
should be observed for evidence of withdrawal and consideration given to slowly tapering these medications.
Medication Dosing
Pediatric medication dosages are weight based and should be
calculated and/or confi rmed with the aid of a pediatric dosing
chart or pharmacist. The Broselow™ tape includes a number
of medications utilized in the acute resuscitation of the critically ill child, including sedatives and analgesics. Table 37.4
outlines a number of options for analgesia and sedation.
ICU Procedural Considerations
Central Venous Access
The comparatively small vein size and need for procedural
sedation or analgesia make the placement of a pediatric
central venous catheter (CVC) more challenging than in the
adult patient. Considerations prior to the placement of a
CVC should include the indication for central access, technical factors, and risk and benefi t of chosen placement site.
Indications for central access include inadequate peripheral
venous access, the need to administer noxious medications,
hemodynamic monitoring, and extracorporeal therapies.
Anterior
superior
iliac spine
Inguinal
Femoral
nerve
Femoral
artery
Femoral
vein
Fig. 37.4 Anatomic landmarks for pediatric femoral venipuncture
(From Wheeler [
64 ], with permission from Springer)
ligament
Pubic
tubercle
Indication for CVC insertion, contraindications, including
coagulopathy and risk of sedation administration, and complication profi le should guide site choice [ 12 ]. The pres-
ence of coagulopathy and risk of airway compromise with
sedation should lead the practitioner to consider the femoral venous site. In a study of 121 critically ill pediatric
emergency department patients requiring central venous
access, the majority (83 %) were cannulated via the femoral
vein with the remainder accessed via either the subclavian
or internal jugular approach [ 10 ]. This might refl ect the
presence of a contraindication to other sites or the relative
technical ease with which the anatomic landmarks for a
femoral CVC can be identifi ed (Fig. 37.4 ) [ 1 ]. However, the
mechanical complication rate of femoral access may be
higher than that of the internal jugular vein [ 62 ]. The inter-
nal jugular is often chosen over the subclavian approach
due to the compressibility of the jugular and the improved
success rate for CVC placement with the use of ultrasound
as an adjunct [ 8 , 64 ].
Intraosseous Access
When CVC catheter placement is not possible, intraosseous
(IO) access can safely and effectively provide a route for
administration of fl uid resuscitation, blood products, and
noxious medications [ 2 ]. This route should only be utilized
temporarily, and the practitioner should be familiar with
436
Fig. 37.5 Pediatric intraosseous
line insertion sites (From
Scott-Warren and Morley [
used with permission)
52 ],
Distal femur: The insertion site is the
anterolateral surface approximately
2-3 cm above the lateral condyle
Z. Maher and M.L. Nance
Proximal humerus: Ensure the patient’s
hand is resting on the abdomen and the
elbow is adducted.
The insertion site is the most prominent
aspect of the greater tuberde,
approximately 1cm superior to the
surgical neck.
Proximal tibia: The insertion site is the
flat anteromedial surface of the bone,
approximately 2-3 cm below the tibial
tuberosity.
In children <2 years old the tibial
tuberosity may not yet have
developed in which case the
insertion point is approximately 3 cm
distal and 1cm medial to the lower
aspect of the patella.
insertion technique to avoid complications of IO placement,
including osteomyelitis, bone fracture, and soft tissue infi ltration leading to ischemia or compartment syndrome [ 2 , 41 ].
Technical considerations for the placement of the Arrow
EZ IO ™ are outlined below [ 52 ], (Fig. 37.5 ):
1. Identify anatomic site for the placement: distal femur,
proximal humerus, proximal tibia, and distal tibia.
2. Needle set selection:
A 45 mm needle (yellow hub) should be considered for
proximal humerus insertion in patients 40 kg and greater
and patients with excessive tissue over any insertion site.
A 25 mm needle (blue hub) should be considered for
patients 3 kg and greater.
A 15 mm needle (pink hub) should be considered for
patients approximately 3–39 kg.
3. Insertion: ensure the 5 mm mark is still visible above the
skin to confi rm adequate depth.
4. Insertion completion: removal of the drill apparatus, sterile dressing, and aspiration of the marrow:
If child is responsive to pain: slow infusion of weight-
based IV lidocaine via intraosseous line
If child is unresponsive to pain: prime intraosseous line
with saline
5. Connect fl uid and pressurize up to 300 mmHg.
Insertion should only proceed if
landmarks are clearly appreciated. This is
less likely in younger children as the
greater tubercle is still developing.
Distal tibia; The insertion site is the
flat aspect of the bone approximately
3 cm proximal to the medial malleolus
Table 37.5 Pediatric arterial catheter sizing
Artery <10 kg 10–40 kg >40 kg
Catheter gauge Catheter gauge Catheter gauge
(French) (French) (French)
Radial, dorsalis
pedis, brachial
Femoral or
axillary
Umbilical (3.5–5.0)
22, 24 22 20, 22
18, 20 16, 18 14, 16, 18
(3.0–4.0) (4.0–5.0) (5.0–6.0)
collateral fl ow can be easily documented using the Allen’s test,
and restraint of the limb is simple to accomplish in the uncooperative patient. Additionally, in the case of pediatric patients
with congenital heart disease, the right radial artery most
closely approximates cerebral perfusion pressure and oxygenation. Other acceptable sites include the dorsalis pedis, femoral, axillary, and brachial arteries. The brachial and femoral
sites increase the risk of malperfusion of the distal extremity,
while the femoral site additionally increases the risk of unrecognized retroperitoneal hematoma and site or blood stream
infection. Ultrasound is a useful adjunct for the placement of
arterial catheters as it has been demonstrated to improve the
fi rst-attempt success in the pediatric population [ 21 ]. Arterial
catheter size selection is critical, as appropriate size selection
will reduce the risk of catheter- associated complications such
as vasospasm, thrombosis, and embolism (Table
37.5 ).
Arterial Access
Indications for arterial access include the need for frequent
arterial blood gases or continuous blood pressure. The radial
artery is the preferred site as it is easily compressible, intact
Intubation
Length-based estimation and age-based formula estimation
are both acceptable means of choosing endotracheal tube
37 The Pediatric Patient Cared for in the Adult ICU
437
size, offering comparable accuracy. Additionally, approximation of endotracheal tube diameter by the fi fth digital circumference has been written about by authors such as King
et al. who concluded that this method is inferior to age-based
formulas and should be reserved for situations in which age
is unknown [ 31 ].
The most widely applied age-based estimation formulas
are the Cole and Khine formulas. Cole’s formula predicts
uncuffed endotracheal tube size as equal to (age 4) + 4,
whereas Khine’s formula predicts cuffed endotracheal tube
size as equal to (age/4) + 3 [
tion, the Broselow™ tape and color-coded system provide
guidance for endotracheal tube sizing. Uncuffed endotracheal tubes are generally reserved for patients less than
8 years of age, though recent data has challenged the assertion cuffed endotracheal tubes are unsafe for children in this
age range [
58 ].
30 , 58 ]. For length-based estima-
Tube Thoracostomy
Drainage of intrapleural air, blood, effusion, or empyema
can be accomplished with the placement of a thoracostomy.
The nature of the effl uent should guide choice of a tube thoracostomy or pigtail thoracostomy. For drainage of pneumothorax alone, pigtail catheters have been shown to be equally
effi cacious with reduced tube site discomfort when compared to tube thoracostomy [ 34 ]. However, in a study by
Petel et al., drainage of empyema by tube thoracostomy was
compared to drainage by pigtail catheter [ 44 ]. Failure rate
was higher among patients treated with pigtail drainage
(43 % vs 14 %, P = 0.045), but duration of illness was shorter
(18.3 ± 1.0 vs 25.6 ± 3.5 days, P = 0.048) [ 44 ]. This difference
may have been related to clogging of the tube and resultant
incomplete drainage of the empyema. Similar concerns have
led many practitioners to choose large bore tube thoracostomy over pigtail drainage of hemothoraces. The placement
of a pigtail catheter is accomplished by sterile Seldinger
technique in the fi fth intercostal space and requires local
anesthetic only. The placement of a tube thoracostomy
begins with local anesthetic and analgesia and may require
sedation depending on patient tolerance. A skin incision is
placed one rib level below the fi fth intercostal space in the
anterior to mid-axillary line. The soft tissue and muscle are
bluntly spread down to the level of the rib, and the pleural
cavity is entered just above the rib. The tube is advanced over
a clamp into the pleural space. The tube should then be connected to a closed drainage system and sutured in place.
Ultrasound
Considerable data exists on the benefi ts of ultrasound guidance in the placement of peripheral and central venous
access in the pediatric population, including reduction in
time to the placement and fewer attempts [
guidance for the placement of femoral or internal jugular
central access is now considered standard of care based on
data indicating improved success rates and decreased overall complication rates [
the pediatric patient in assessing for the presence of fl uid in
the pleural space and to guide successful drainage when
present [
35 ].
38 ]. Ultrasound may also be useful in
14 ]. Ultrasound
Indications for ECMO
Indications for consideration of extracorporeal membrane
oxygenation (ECMO) differ between the neonatal and pediatric population. Cases of neonatal severe respiratory failure refractory to maximal medical management, with a
potentially reversible etiology, should prompt consultation
for transfer to an EMCO center. In the pediatric population
(age greater than 30 days to 18 years), consideration for
ECMO is best within the fi rst 7 days of mechanical ventilation at high levels of support. Outcomes after ECMO in the
neonatal and pediatric population are better than those in
their adult counterparts. In 2015, survival to discharge or
transfer among neonatal and pediatric patients treated with
ECMO for respiratory failure was 74 % and 57 %, respectively. In the patient with adequate cardiac performance,
venovenous cannulation is the preferred route. In larger
children, as in adults, access sites include the jugular and
femoral sites.
Psychosocial Considerations in Pediatric Intensive Care
Caring for a critically ill child also necessitates care for the
family of the sick child as well. Excellent communication
with the family requires special attention. An approach to
this communication is outlined in Box
family members during acute resuscitation has been a
debated topic, with evidence that parental presence during
resuscitation efforts is perceived by parents as benefi cial to
both themselves and the patient [ 7 ]. Despite this, acceptance
of parent presence is mixed among providers, with nursing
staff and senior physicians demonstrating higher levels of
acceptance [ 39 ]. Given that up to 25 % of children demon-
strate negative psychological and behavioral outcomes
within the fi rst-year post-discharge from a critical care environment, the psychosocial health of the critically ill patient
also warrants additional attention [ 50 ]. Care should be taken
to minimize pain and anxiety for the child during the ICU
admission.
37.1 . The presence of
438
Z. Maher and M.L. Nance
Box 37.1: Suggestions for Physician Communication
with Families
1. Arrange for a quiet room to sit with the family,
unhurried and away from the demands of the unit.
2. Talk to them in simple terms about what is hap-
pening to their child, what you are attempting to
do, and the chance for and against the child’s
recovery.
3. Ask them for their questions and their input,
respecting cultural and religious perspectives and
recognizing the need for interpreter services.
4. Empathize with the frustration, fears, temptations,
and anxieties with which they struggle.
5. Do not judge them on their thoughts. Instead,
acknowledge and validate feelings.
6. Try to meet with them regularly and more fre-
quently, even for short periods, to keep them
updates on their child’s condition.
7. Designate a specifi c team member to deal with the
family when the stay in the ICU is prolonged.
Families have diffi culty relating to multiple
physicians.
8. Encourage the family’s continued involvement
with the other members of the family.
9. Always remember to bear with them and tolerate
silence as well as their own ways of expressing
their emotions.
10. When the parent has been directly responsible for
what has happened to the child, take whatever
action is required to provide for the immediate and
future safety of the child as well as the other children in the family. Do so, however, without being
judgmental of those involved.
From Wheeler et al. [ 66 ].
failure [
of CF, occurring in greater than 3 % of all CF patients, and
is caused by mucus plugging of the airways with alveolar
air trapping [ 20 ]. Diagnosis is made with chest X-ray
(CXR) or computed tomography (CT). Up to one third will
recur, and failure of conservative management leads to surgical intervention in up to 70 % of cases [
unlike small, asymptomatic pneumothoraces in other populations which are often observed for resolution, standard
treatment is tube thoracotomy drainage irrespective of size
or symptoms. Massive hemoptysis is common in the CF
population owing to the frequency of pulmonary infection
leading to chronic infl ammation and bronchial artery angiogenesis [ 56 ], [ 9 ]. Diagnosis is made by clinical suspicion,
CXR, CTA, and, in select circumstances, bronchoscopy.
Management should include reversal of CF-induced, vitamin K-defi cient coagulopathy and consideration for bronchial artery embolization [ 56 ]. Up to 80 % of CF patients
eventually succumb to respiratory failure resulting from
progression of obstructive airway disease. CF patients with
acute-on-chronic respiratory failure should be managed
with antibiotics, bronchodilators, and aggressive pulmonary toilet, including consideration for bronchoscopy in the
case of larger airway plugging [ 56 ]. Noninvasive positive
pressure ventilation (NIPPV) has been demonstrated to
improve chest symptoms, exertional dyspnea, nocturnal
hypoventilation, and peak exercise capacity in patients with
stable CF [ 63 ]. However, in the CF patient with acute-on-
chronic respiratory failure, NIPPV should be viewed as a
bridge to transplant [ 36 ]. Intubation is associated with poor
outcome in this population, likely related both to overall
disease progression leading to hypercapnia and the inability of conventional ventilation to manage this hypercapnia,
but may be necessary in the case of respiratory fatigue [ 53 ].
For patients with irreversible causes of acute- on- chronic
respiratory failure due to CF, a lung transplant center should
be involved in the initial management decisions.
56 ]. Pneumothorax is a very common complication
19 ]. Therefore,
The Adult ICU Patient with Congenital Disease (Pediatric Disease)
Pulmonary Considerations
Cystic Fibrosis
Many patients with cystic fi brosis (CF) survive to adulthood
and will require critical care at some point. The majority of
the cystic fi brosis-related complications leading to ICU
admission will be pulmonary or gastrointestinal.
Respiratory Complications
The most common adulthood pulmonary complications
include pneumothorax, hemoptysis, and acute respiratory
Gastrointestinal Complications
Pancreatitis and distal intestinal obstruction syndrome
(DIOS) may result in ICU admission of an adult patient
with cystic fi brosis. Pancreatitis in this patient population
is treated similarly to the management in the non-CF
patient, with hydration and analgesia as the cornerstones
[ 29 ]. DIOS occurs in up to 22 % of CF patients and is
more commonly found in patients with concomitant pancreatitis, likely owing to the increased viscosity of the
high-fat stool in these patients [ 15 ], [ 29 ]. Symptoms of
DIOS mimic those of mechanical bowel obstruction with
obstipation, nausea, vomiting, and colicky abdominal
pain as primary manifestations. Treatment of DIOS should
focus on conservative medical management including
enemas or oral treatment with meglumine diatrizoate,
37 The Pediatric Patient Cared for in the Adult ICU
439
laxatives, or N-acetyl-cysteine [ 15 ]. Surgery should be
reserved for those with failure of aggressive medical
therapy.
Cardiac Considerations
Congenital Heart Disease
As a result of tremendous advances in the care of infants
born with congenital heart disease (CHD), over 85 % of these
patients now survive to adulthood [ 60 ]. Admission to the
adult ICU may be unrelated to the primary congenital defect
or may be for reoperation of the primary defect or correction
of a defect recognized in adulthood. Understanding the
pathophysiology of the primary defect should inform
multisystem management decisions. Additionally, patients
with adult congenital heart disease (ACHD) require special
consideration in the ICU due to increased incidence of cardiac, pulmonary, renal, and hepatic dysfunction related to the
primary congenital defect and the increased perioperative
mortality risk in those with thyroid, renal, and hepatic dysfunction [ 48 , 51 ]. These considerations will be the focus of
this section.
Cardiac Arrhythmia
Cardiac arrhythmias are a leading cause of sudden cardiac
death (SCD) in the ACHD population and can be incited by
postoperative state or systemic illness [ 51 ]. Risk factors for
SCD include documented “prior SVTs (predominantly atrial
fl utter or fi brillation), increased QRS duration, QT dispersion, and moderately to severely impaired systolic function
of the systemic and/or subpulmonary ventricle” [ 33 ]. Despite
this association, the most common arrhythmia leading to
SCD is ventricular fi brillation [ 33 ]. Because of this associa-
tion, critically ill patients with ACHD and high-risk features
for SCD, including sustained ventricular tachycardia and
cardiac arrest, should be considered for implantable
cardioverter- defi brillator (ICD) placement [
17 , 51 ].
Heart Failure
Patients with ACHD frequently develop heart failure, and
therefore advanced cardiac monitoring may be necessary in
the ICU. Noninvasive evaluation of cardiac function with
transthoracic echocardiography (TTE) should be done for all
critically ill ACHD patients. Consideration for transesophageal echocardiography (TEE) includes the presence of congenital heart defects, as the imaging quality and
reproducibility of this modality are superior. Ongoing need
for hemodynamic assessment should prompt consideration
for the placement of a miniaturized TEE, with recent data
indicating that brief training in the placement of these probes
is suffi cient to permit accurate collection of hemodynamic
11 ].
data [
Cardiopulmonary
The incidence of right-sided heart dysfunction is higher in
this population than in other groups as is the incidence of
pulmonary vascular disease [
6 ]. Given this, it is very impor-
tant to minimize the cardiac effects of ventilator support. As
such, PEEP should be minimized when possible, and pulmonary vasoconstriction should be avoided by optimizing
PaCo
and preventing hypoxemia [ 51 ]. A recently published
2
scientifi c statement from the American Heart Association on
Congenital Heart Disease in the older adult is an excellent
review and guide on this topic [ 6 ].
Acute Kidney Injury
Up to 50 % of adults with CHD have chronic kidney disease
(CKD), and among those with moderate to severe impairment, baseline mortality is three times higher, and perioperative mortality is signifi cantly increased [
13 , 48 ]. Those with
cyanotic CHD are most likely to develop CKD, the pathogenesis of which is related to hypoxia, activation of the
renin-angiotensin system as a result of marginal systemic
cardiac output, and prior exposure to cardiopulmonary
bypass [
51 ]. Management of critically ill ACHD patients
with CKD must include careful attention to volume status
and early intervention to prevent intravascular volume overload. Consideration should include early continuous venovenous hemodialysis (CVVHD) where appropriate [ 16 ].
Hepatic Dysfunction
Cardiac cirrhosis with portal hypertension and ascites is
common in the ACHD population owing to the physiologic
effects of chronic venous congestion and exposure to hepatotoxic insults [ 51 ]. There are a number of potential contribu-
tors to venous congestion pathogenesis, including right-sided
heart failure, single-ventricle physiology, chronic left-sided
heart failure, and systemic-pulmonary shunting. Many
ACHD patients are additionally exposed to the hepatotoxic
effects of transfusion, cardiopulmonary bypass, and hepatotoxic medications, including anti-arrhythmics [
3 ]. The pat-
tern of hepatic dysfunction may guide diagnosis, with
isolated transaminitis indicating hepatic ischemia and low
fl ow, hyperbilirubinemia and elevated prothrombin time
indicating passive congestion, and cholestatic jaundice indicating ischemic cholangiopathy or obstruction [
3 ].
Additionally, ACHD patients with hepatopathy have an
increased risk of hepatocellular carcinoma and therefore
should be screened regularly with serum AFP levels and
imaging [ 3 ].
Hematologic
Adults with cyanotic CHD are at increased risk for both
thromboembolic and bleeding complications [
22 ]. Chronic
cyanosis leads to secondary erythrocytosis, and more than
one third of patients with cyanotic CHD have iron-defi cient
440
Z. Maher and M.L. Nance
anemia [ 55 ]. These two factors contribute to a state of blood
hyperviscosity, putting these patients at increased risk of
thromboembolic events [
28 ]. Despite this, the same popula-
tion is hypocoagulable due to impaired fi brinogen function
and therefore at risk for bleeding complications [ 27 ]. Given
the competing nature of hematologic derangements in this
patient population, decisions about thromboembolic prophylaxis and modulation of bleeding risk must be
individualized.
Neurologic Considerations
VP Shunt Complications
Ventriculoperitoneal shunt (VPS) placement is the most
common treatment modality for hydrocephalus. Adults with
chronic VPS in place since childhood are at risk for the
development of similar complications to those identifi ed in
any patient with a VPS, including shunt occlusion, disconnection, infection, and abdominal cavity complications, but
with a higher frequency of these complications over a lifetime [ 49 ]. Shunt occlusion should be considered in any
patient with a VPS presenting with headache, depressed
mental status, and/or emesis. Diagnoses can often be made
on CT scan of the head demonstrating hydrocephalus [ 4 ].
Management of this complication nearly always requires
surgical shunt revision. Disconnection of the shunt should be
suspected if focal swelling is noted along the tract of the
shunt or if signs or symptoms of increased intracranial pressure are noted. The site of shunt fracture can often be identifi ed on plain X-ray. Treatment of symptomatic shunt fracture
should include revision, though some controversy surrounds
the management of asymptomatic shunt fracture.
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