Kelainan

Kongenital
Sistem Respirasi

Meivita Dewi Purnamasari, S.Kep.,Ns.,M.Kep

Departemen Keperawatan Anak
Jurusan Keperawatan Universitas Jenderal Soedirman
Tujuan
Setelah mengikuti sesi kuliah, mahasiswa mampu :
• Menjelaskan etiologi kelainan kongenital pada sistem
respirasi
• Menjelaskan tanda dan gejala
• Menjelaskan manifestasi klinik
• Menjelaskan pemeriksaan diagnosis
• Menjelaskan penatalaksanaan
• Menyusun asuhan keperawatan pada anak dengan
kelainan kongenital pada sistem respirasi
 Choanal Atresia

Pierre Robin
Syndrome

Cleft Lip
Kelainan and/Palate
Kongenital
Sistem Respirasi
Laryngomalacia

Tracheomalacia

Bronchomalacia
 Respiratory conditions are
among the most common
maladies affecting children,
ranging from those that develop
in utero, such as malformations
of the palate and trachea

directly affects children’s stamina

and their ability to engage in
activities in settings that are
conducive to safe air exchange
Congenital abnormalities or
malformations of the respiratory
systems result in respiratory
dysfunction primarily caused by
airway obstructions or collapse

Congenital conditions include

choanal atresia, Pierre Robin
syndrome, and cleft palate,
laryngomalacia (weakness and poor
tone of the larynx), and
tracheomalacia (weakness and
poor tone of the trachea), and
bronchomalacia.
Choanal
Atresia

A unilateral or bilateral bony or

membranous septum between the nose
and pharynx
Nares not paten
A malformed or misshapen nose may occur
in the presence of chromosomal problems
Pierre Robin Syndrome
Abnormally small jaw with upper
airway obstruction [resulting from
tongue prolapse into the pharyngeal
airway]

In the child with Pierre Robin syndrome,

malformation of the mandible makes
the chin appear recessed and restricts
the tongue to a posterior position,
causing easy occlusion of the airway.

Pierre Robin syndrome are associated

with a cleft palate
Cleft Lip and /Palate

• Formation of the lip and/palate occurs at which time

the fetus is sensitive to teratogens that can affect
development of the oral cavity.
• Cleft lip is a result of incomplete fusion of embryonic
structures surrounding the primitive oral cavity, the
maxillary and medial nasal elevations, between the
fifth and eighth weeks of gestation
• The cleft may be unilateral or bilateral and may vary
from a small indentation in the lip to a wide, deep
fissure that extends to the nostril
• Between the seventh and 12th weeks of gestation,
the palate is formed by the migration and fusion of
the lateral palatine processes.
• Cleft palate represents failure of the primary and
secondary palatine plates to fuse.
• The cleft may involve only the soft palate or may
extend into the hard palate. A less obvious type of
cleft palate, a submucous cleft, is the most common
of the posterior palate clefts.
Cause of cleft lip and/palate
• Familial patterns of inheritance (genetic)
• Environmental factors such as alcohol and tobacco use
(resulting in fetal hypoxia).
• Drugs such as phenytoin and retinoids
• Dietary factors such as folic acid and vitamin deficiencies.
Folic acid is recommended as a supplement for women of
childbearing age to prevent neural tube and abdominal
wall defects, and help prevent CLP
• Intrauterine irradiation
• Multifactorial inheritance, which is generally caused by a
combination of genetic and environmental factors.
Problem in child with cleft lip and/palate

• Breastfeeding
– An infant with cleft palate may not be possible breast-
feed because of the inability to produce effective
suction to extract the breast milk.
• Long-term problems
– Impaired facial growth and dental anomalies,
alterations in speech, Eustachian tube dysfunction,
and can increase risk for recurrent otitis media and
associated hearing impairment.
• Mortality
– Related to the severity of associated syndromes.
Assesment (cleft lip and/ palate)

Visual inspection and

palpation of the palate
as part of every
newborn examination

Submucous clefts may

not be identified until
the child is older and
presents with
hypernasal speech
Prognosis (Cleft Lip and/Palate)

• After surgical repair

• Risk for hearing loss (eustachian tube dysfunction may cause
recurrent otitis media).
• Speech impairments > the repair may affect the function of the
pharyngeal and palatal muscles > requiring long-term speech
therapy.
• Abnormal dental development and malocclusion require the
ongoing involvement of pediatric dentists and orthodontists.
• Maxillofacial surgery and rhinoplasty may be required later in life
if midfacial growth is impaired and the nasal septum is deviated.
• Alterations in appearance and speech impairment
• Negative effect on the child’s selfimage.
• Academic achievement, social adjustment, and behavior
should be monitored, particularly in children with syndromic
cleft conditions.
• The involvement of social workers or other mental health
practitioners is important in dealing with these concerns.
LARYNGOMALACIA

The most common cause of The supraglottic structures

stridor in newborns, collapse into the airway
affecting 45–75% of all during the inspiratory phase
infants with congenital of respiration which
stridor produces inspiratory stridor.

Most infants with

laryngomalacia will have
mild symptoms and a
benign disease course that
resolves by the age of 12 to
24 months
 Clinical Manifestation

• Inspiratory stridor that typically worsens

with feeding, crying, supine positioning, and
agitation.
• The symptoms begin at birth or within the
first few weeks of life, peak at 6 to 8
months, and typically resolve by 12 to 24
months
• Laryngomalacia is usually diagnosed within
the first 4 months of life
Clinical Manifestation (cont’d..)

• Difficult time coordinating the suck

swallow breath sequence needed for
feeding as a result of their airway
obstruction
• The increased metabolic demand of
coordinating eating and breathing against
the obstruction can be so severe that it
results in weight loss and failure to thrive
• Tachypnea, suprasternal and substernal
retractions, cyanosis, pectus excavatum,
and obstructive sleep apnea.
PECTUS EXCAVATUM
PECTUS EXCAVATUM NORMAL
Etiologi Laryngomalacia
• Unknown
• The neurologic theory
• Laryngomalacia may be a consequence of an
underdeveloped or abnormally integrated CNS
system, particularly the peripheral nerves and
brainstem nuclei responsible for breathing and
airway patency.
 TRACHEOMALACIA
Tracheomalacia (softening of
the tracheal cartilage)
compresses the trachea early
It may also occur as a result
of inadequate intratracheal
pressure > abnormal
tracheal development.
May occur as a result of
weakness in the tracheal
wall that exists when a
dilated proximal pouch
in fetal life.
Clinical signs include a
barking cough, stridor,
wheezing, recurrent
respiratory tract infections,
cyanosis, and sometimes
apnea.
BRONCHOMALACIA
• Bronchomalacia is most commonly found in
association with tracheomalacia
• An abnormal weakness in the airway wall and is
found more commonly in premature neonates and
patients with Down syndrome, although it may
occur in full-term infants.
 Congenital Typical clinical
anomalies, or birth About 2% to 3% of history but is
defects, can be all births are confirmed by
identified associated with a flexible
prenatally, at birth, major congenital laryngoscopy in an
or at any point anomaly awake infant
after birth. (laryngomalacia)
Assesment Congenital Abnormalities of the
Respiratory System

Generally diagnosed at birth or shortly

thereafter, when the child exhibits
varying degrees of respiratory distress

The presence of cyanosis (bluish skin

color caused by inadequate oxygenation
of the blood) is a key finding
Intervention Surgical interventions
to relieve the
Supportive: the primary
obstruction (such as for
goal is to maintain a
choanal atresia) or
patent airway. Routine
structurally support the
infant healthcare is
airway (such as for
administered.
severe tracheomalacia)
may be necessary.

These conditions
improve with growth,
Adequate nutritional and in the absence of
support to promote other congenital
optimal weight gain is a anomalies, the
key intervention. prognosis for normal
respiratory function is
good.
 Intervention Pierre Robin
Syndrome

Maintain infant in the prone

position as much as possible
A tracheostomy is necessary
(prevent the tongue from
to maintain an open airway
obstructing the pharynx and
causing respiratory distress)

Improper positioning can

quickly result in complete
obstruction and death
 The infant with a CLEFT
PALATE unable to generate
negative suction pressure in
the oropharynx, and milk is
regurgitated into the nasal
cavity.
Growth failure in infants
with CL/P or CP has been
attributed to preoperative
feeding difficulties.

Many feeding methods to

Modifications to positioning,
facilitate feeding the infant
bottle selection, and feeder
with cleft palate > use of
supportive techniques can
various nipple designs,
help infants with CP feed
cross-cut nipples, and palatal
efficiently.
obturators
• The Haberman Feeder is used for children with cleft palate and
other orofacial problems that hamper the child’s ability to nurse
• PATIENCE is the key to the feeding process.
 SURGICAL INTERVENTION

Severe airway
obstruction with
significant retractions,
Surgical Management pectus excavatum, cor
pulmonale, pulmonary
Laryngomalacia hypertension, and
hypoxia are all
considered absolute
indications for surgery
Surgical Intervention (Cleft Palate)

Most repairs are

done at age 12 to 24
months, and
preferably prior to The most common
18 months of age, Preoperative techniques to repair
after speech patterns preparation (family) CP include the Veau-
have been focuses on providing Wardill-Kilner V-Y
established. information pushback procedure
regarding the surgical and the Furlow
The insertion of procedure double-opposing Z-
bilateral ventilation plasty.
tubes (in the ears)
has become the
standard of care
Post operative care (cleft palate)
Immediate Risk for airway
postoperative care compromise >
Both in the hospital focuses on AIRWAY laryngeal edema
and at home MANAGEMENT, caused by intubation
HEMOSTASIS, AND during surgery and
PAIN CONTROL incisional edema.

Blood clots may also fall off the incision and

After palate repair, obstruct the airway.
the child must learn Use of a high-humidity oxygen tent may be
to breathe through ordered.
smaller nasal Observe the child carefully for signs of
passages. respiratory distress, bleeding, and excess mucus
in the mouth.

Bleeding may occur at the
suture line. Apply gentle
pressure or ice to the lip
incision, as ordered.
Anticipate the child’s needs and
meet them before the child
becomes distressed.
Medicate for pain relief.
Minimize pain and discomfort
from other sources (e.g.,
hunger, wet diaper, need for
attention or repositioning) to
prevent crying, which results in
tension on the suture line.
Pain is not unusual during the
first 24 to 48 hours
postoperatively. Assess the need
for analgesics and evaluate
their effectiveness after
administration.
The child must be prevented
from putting his or her hands
near the mouth, to avoid
disturbing the incision.
Therefore, elbow immobilizers
are used for the first 10 to 14
days postoperatively
Elbow Immobilizers
Oral feeding is usually delayed
up to 48 hours after cleft
palate repair.
Pureed or soft foods may be
carefully fed to the child by
spoon or cup.

Parents are cautioned against

allowing the child to eat hard
items (e.g., toast, hard
cookies, and potato chips) that
can damage the repaired
palate.
 FAMILY CENTERED CARE

• A diagnosis of a congenital anomaly creates a crisis for

the family.
• Early and ongoing support and education of the
parents. Support the family’s adjustment to a child’s
condition
• Demonstrating an accepting
• Caring attitude toward the child and family,
• Providing the parents with opportunities and
support for normal infant–parent interactions.
• Reinforce the concept that IN MOST CASES THE CAUSE
OF THE DEFECT IS UNKNOWN AND NOT THE FAULT OF
THE PARENTS.
 FAMILY CENTERED CARE

• Educate the family about appropriate positioning and

feeding techniques to ensure adequate intake of
formula or breast milk (either by actual breast-feeding
or breast milk expressed into a bottle) and solid foods
as the infant reaches the appropriate age for
introducing them.
• Parents need to be informed that the infant’s
symptoms may worsen during respiratory illnesses.
• If they have any concern about worsening respiratory
distress, instruct them to seek assistance from their
healthcare professional.
• Parents should also be instructed in CPR.
NURSING DIAGNOSIS

• Ineffective airway clearance related to excess thick

secretions, obstruction, or infection
• Ineffective breathing pattern related to effects of
respiratory disease process
• Impaired gas exchange related to consequences of
underlying respiratory disease process
• Decreased cardiac output related to consequences of
respiratory distress and failure
• Risk for infection related to pooling of lung secretions,
ineffective cough and contagious nature of condition
• Imbalanced nutrition: Less than body requirements
related to underlying chronic lung disease and
increased work of breathing
NURSING DIAGNOSIS

• Ineffective breastfeeding
• Ineffective infant feeding dynamics
• Ineffective infant feeding pattern
• Caregiver role strain
• Risk for impaired attachment
• Parental role conflict
• Risk for aspiration
• Impaired oral mucous membrane integrity
• Risk for delayed development
 Ineffective airway clearance
Child’s airway will be free of secretions, as evidenced by
normal or improved breathing, normal arterial blood gases,
and/or oxygen saturation 92% or greater on pulse oximetry
• Complete respiratory assessment with each routine vital sign
check and as indicated by the child’s condition.
• Encourage child to cough, especially after respiratory
treatments. Teach effective cough techniques
• Provide humidified oxygen as ordered
• Encourage increased fluid intake (clear liquids; avoid dairy
products) if no contraindications, such as cardiac or renal
disease, are present
• Administer prescribed medications and intravenous fluids,
monitor for response and side effects
 Ineffective breathing pattern
Child will demonstrate an effective respiratory rate,
rhythm, and effort, and will experience improved gas
exchange in the lungs, as evidenced by blood gases within
child’s normal parameters.
Child will verbalize ability to breathe comfortably without
sensations of dyspnea and related feelings of fear or
anxiety resulting from shortness of breath
• Complete respiratory assessment with each routine vital
sign check and as indicated by the child’s condition.
• Provide oxygen as needed to the child to maintain
oxygen saturation at more than 92% or within
parameters defined as child’s personal best.
 Ineffective breathing pattern (continued)

• Place child in an upright position to optimize

ventilation.
• Provide reassurance to the child and allay
anxiety. Keep child as calm as possible.
• Administer prescribed medications and
intravenous fluids; monitor for response and
side effects
 Impaired gas exchange

Child will demonstrate improved gas exchange in the

lungs, as evidenced by blood gases within child’s
normal parameters and an alert, responsivemental
status or no further reduction in mental status.
• Complete respiratory assessment
• Provide oxygen as needed to maintain oxygen
saturation at more than 92% or in parameters
defined as child’s personal best.
• Anticipate the need for intubation and mechanical
ventilation. Assist with these procedures as needed.
 Decreased cardiac output

Child will demonstrate adequate cardiac output, as

evidenced by strong peripheral pulses; normal vital
signs; urine output greater than 2 mL/kg/hour; and
warm, pink, dry skin.
• Complete assessment of cardiovascular status
including vital signs, hemodynamic pressures, urine
output, skin temperature, peripheral pulses, and
respiratory effort.
• Administer prescribed medications and intravenous
fluids; monitor for response and side effects.
 Risk for infection
Child will be free from infection, and the parents will
demonstrate adequate knowledge of risk for infection,
signs/symptoms of infection, and measures to reduce infection
risks.
• Complete assessment to monitor for respiratory infection,
including auscultating breath sounds; evaluating for changes
in sputum; and observing for fever, chills, increase in cough,
shortness of breath, nausea, vomiting, diarrhea, and
decreased appetite.
• Evaluate child’s and family’s understanding of techniques to
prevent infection, such as careful hand hygiene, adequate rest
and nutrition, avoiding contact with sick individuals, and so
forth. Provide additional education as needed.
 Risk for infection (continued)

• Follow standard infection control

precautions during all contact with the child.
• Encourage child to cough and expectorate
secretions frequently. Encourage strict
handwashing and infection control practices
for the child, family members, and all care
providers.
 Imbalanced nutrition: Less than body
requirements
Child will demonstrate adequate nutritional intake
for age, and parents will identify steps to achieve
and maintain ideal body weight.
• Assess child’s nutritional status, including height, weight,
body mass index percentile, diet history, child’s ability to eat,
and possible causes for poor appetite.
• Assist child/parents to plan well-balanced meals that
incorporate child’s food preferences and dietary limitations
imposed by disease process (e.g., low-to-moderate fat, high-
protein, high-calorie meals for the child with cystic fibrosis).
Encourage small feedings of nutritious soft foods and liquids.
Add nutritional supplements as ordered.
• Ensure frequent oral care is provided.
Reference

• Bowden, V. R., Dickey, S. B., & Greenberg, S. C.

(2009). Children and their families: the continuum of
care. Philadelphia: W.B. Saunders Company
• Hockenberry, M., & Wilson, D. (2013). Wong’s
essential of pediatric nursing. 9th ed. St. Louis:
Mosby Elsevier
• Landry, A.M., & Thompson, D.M. (2012).
Laryngomalacia: Disease Presentation, Spectrum,
and Management. International Journal of Pediatrics,
1-6.