Congenital Abnormalities of the

Respiratory System
• Congenital abnormalities or malformations of the
respiratory systems are fortunately rare
• Choanal atresia (a unilateral or bilateral bony or
membranous septum between the nose and
pharynx), Pierre Robin syndrome (malformation
of the mandible), laryngomalacia (weakness and
poor tone of the larynx), tracheomalacia
(weakness and poor tone of the trachea), and
broncomalacia (weakness and poor tone of the
bronchous)
• These abnormalities are generally diagnosed at
birth or shortly thereafter, when the child
exhibits varying degrees of respiratory distress
• The presence of cyanosis is a key finding.
• The treatment is largely supportive
• The Primary goal of treatment is to maintain a
patent airway.
• Surgical interventions to relieve the obstruction
(such as for choanal atresia) or structurally
support the airway (such as for severe
tracheomalacia) may be necessary. Adequate
nutritional support to promote optimal weight
gain is a key intervention. These conditions
improve with growth, and in the absence of other
congenital anomalies, the prognosis for normal
respiratory function is good.
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. A tracheostomy is
necessary to maintain an open airway.
 To Family
• Early and ongoing support and education of the
parents is of utmost importance.
• A diagnosis of a congenital anomaly creates a
crisis for the family
• 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.
 To Family
• 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.
 Diagnosa keperawatan
• 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
 Diagnosa keperawatan
• Decreased cardiac output related to
consequences of respiratory distress and
failure
• Imbalanced nutrition: Less than body
requirements related to underlying chronic
lung disease and increased work of breathing
• Risk for infection related to pooling of lung
secretions, ineffective cough and contagious
nature of condition