PRESENTATION: RESPIRATORY DISORDER

TOPIC: PULMONARY HYPOPLASIA

COURSE:RESPIRATORY SYSTEM
COURSE CODE:PHS 232
COURSE LECTURER:MISS OGAGAYERE LUCKY
GROUP 3
NAMES OF GROUP MEMBERS
1.Togbeola Oluwayemisi Elizabeth AU21HA4049 Human Anatomy
2.Akinyemi Dupe Rebecca
AU21HS4056 Human Physiology
3.Rufai Ayotomiwa Nimotalahi
Human Anatomy AU21HA4415
4.Adeniyi kadijat Abike
Human Physiology AU21HS4052
5.Richard Oluwafiifunmi Praise
Human Anatomy AU21HA4350
6 Obiekwu Linda Kanayo
AU21HA4044 Human Anatomy
7. Odetunde Adesewa Falilat
AU21HA4045 Human Anatomy
What is Pulmonary Hypoplasia ?

Pulmonary Hypoplasia is a condition in which the lungs are abnormally small and
do not have enough tissue and blood flow to allow the baby to breathe on his or
her own. This can be a life threatening condition. Pulmonary hypoplasia refers to
underdevelopment or incomplete growth of the lungs.If a child has been
diagnosed with pulmonary Hypoplasia ,you can rely on dedicated and
multidisciplinary team of providers to care for the child'severy need. They
coordinate and provide immediate care in theNICU(Neonatal intensive Care Unit)
as well as long time follow up care as the child's lungs continue to develop.

Mechanism of Pulmonary Hypoplasia.

Several Studies have demonstrated that gestation age at rupture of

membranes(15-27weeks of gestation),latency period (duration between rupture
of membranes and birth) and the amniotic fluid (AFI of less than 1cm or 5cm)can
Influence the development of pulmonary Hypoplasia. The mechanism of action of
pulmonary hypoplasia involves disruptions in the normal development of the
lungs, resulting in undergrowth or incomplete formation of lung tissue. This can
occur due to various factors and can be broadly categorized into two main types:
primary lung parenchymal hypoplasia and secondary lung hypoplasia.
1.)Primary lung parenchymal hypoplasia: This type of pulmonary hypoplasia
involves direct defects in the lung tissue development and can result from genetic
abnormalities, intrauterine infections, or certain syndromes. Some of the
underlying mechanisms include:
1.Genetic factors: Mutations or disruptions in genes involved in lung development
can lead to primary lung parenchymal hypoplasia. These genes play crucial roles
in regulating processes such as branching morphogenesis, cell differentiation, and
vascular development in the developing lungs.
2.Intrauterine infections: Certain infections during pregnancy, such as congenital
rubella or cytomegalovirus (CMV) infection, can affect the developing lungs and
interfere with normal lung growth, leading to pulmonary hypoplasia.
3.Syndromes: Certain genetic syndromes, such as Fryns syndrome or Potter
syndrome, are associated with pulmonary hypoplasia. These syndromes often
involve multiple abnormalities and can disrupt lung development.

2.)Secondary lung hypoplasia: This type of pulmonary hypoplasia occurs as a result

of extrinsic factors that limit the space available for lung development r restrict
the movement of the developing lungs. Some of the contributing factors include:

1.Congenital diaphragmatic hernia (CDH): CDH is a condition where there is a hole

in the diaphragm, allowing abdominal organs to herniate into the chest cavity.This
herniation can compress the developing lungs, leading to restricted lung growth
and pulmonary hypoplasia.
2.Oligohydramnios: Insufficient amniotic fluid in the womb can restrict the
movement of the developing fetus, including the lungs. Adequate amniotic fluid is
necessary for normal lung development, and reduced fluid levels can impede lung
growth, resulting in pulmonary hypoplasia.
3.Thoracic deformities: Structural abnormalities of the chest wall, such as severe
scoliosis or skeletal dysplasia, can restrict the expansion of the lungs and limit
their growth.
4.Intrauterine growth restriction (IUGR): Poor fetal growth due to factors such as
placental insufficiency can result in reduced blood flow and nutrient supply to the
developing lungs, affecting their growth and leading to pulmonary hypoplasia.

Overall,the precise mechanisms underlying pulmonary hypoplasia can vary

depending on the specific cause and context. Understanding these mechanisms
helps in diagnosing the condition and guiding appropriate management
strategies.
Causes Of Pulmonary Hypoplasia

1.Congenital diaphragmatic hernia (CDH): This is the most common

cause of pulmonary hypoplasia. It occurs when there is a hole in the
diaphragm, allowing abdominal organs to move into the chest cavity
and compress the developing lungs.
2.Chromosomal abnormalities: Certain chromosomal abnormalities,
such as trisomy 13, trisomy 18, and trisomy 21 (Down syndrome), can
be associated with pulmonary hypoplasia.
3.Intrauterine growth restriction (IUGR): Poor fetal growth resulting
from placental insufficiency or other factors can lead to pulmonary
hypoplasia.
4. Oligohydramnios: Insufficient amniotic fluid in the womb can restrict
lung development and cause pulmonary hypoplasia
5.Thoracic deformities: Structural abnormalities of the chest wall, such
as severe scoliosis or skeletal dysplasia, can restrict lung growth.

Symptoms Of Pulmonary Hypoplasia

1.Respiratory distress: Newborns with pulmonary hypoplasia often

experience difficulty breathing, rapid breathing (tachypnea), and
retractions (visible inward pulling of the chest wall during inhalation).
2.Cyanosis: Bluish discoloration of the skin, lips, or nail beds due to
inadequate oxygenation of the blood.
3.Poor lung function: Pulmonary hypoplasia can result in decreased
lung volumes and reduced lung compliance, leading to inefficient gas
exchange and respiratory failure.
4.Abnormal chest shape: In severe cases, there may be visible
abnormalities in the chest shape, such as a concave or bell-shaped
chest.
5.Increased risk of infections: Due to compromised lung function,
individuals with pulmonary hypoplasia are more susceptible to
respiratory infections.

It is important to note that the severity of pulmonary hypoplasia can

vary widely, ranging from mild cases that may not require intervention
to severe forms that can be life-threatening. Diagnosis and
management of pulmonary hypoplasia typically involve medical
imaging, respiratory support, and surgical interventions when
necessary.

Diagnosis of Pulmonary Hypoplasia

Diagnosis of pulmonary hypoplasia typically involves a combination of

prenatal and postnatal evaluations. The goal is to assess lung
development, determine the underlying cause, evaluate the severity,
and plan appropriate management strategies. Here are some common
diagnostic approaches:

1.Prenatal diagnosis:
-Ultrasonography: Prenatal ultrasound scans can provide information
about the size and appearance of the fetal lungs. Certain ultrasound
markers, such as an abnormally small lung-to-head ratio, may suggest
pulmonary hypoplasia.
-Fetal magnetic resonance imaging (MRI): In some cases, an MRI may
be performed to obtain more detailed images of the fetal lungs and
assess their development.
-Genetic testing: If there is suspicion of a genetic cause, genetic testing,
such as chromosomal analysis or specific gene sequencing, may be
recommended.

2.Postnatal diagnosis:
-Physical examination: After birth, a physical examination is performed
to assess the newborn's respiratory status, chest shape, and signs of
respiratory distress.
-Chest X-ray: X-ray imaging of the chest can provide information about
lung size, shape, and any associated abnormalities.
-Computed tomography (CT) scan: CT scans can provide more detailed
images of the lungs and help evaluate the extent of pulmonary
hypoplasia and any associated conditions.

Complications of Pulmonary Hypoplasia

1)Mortality due to acute respiratory failure in the neonatal period

2)Chronic respiratory failure or insufficiency
3)Pneumothorax,either spontaneous or as a result of ventilatory
support
4)Persistent pulmonary hypertension caused by a reduced pulmonary
vascular bed and
worsened by hypoxia or a coexisting left-to-right intracardiac
shunt
5)Chronic lung disease of infancy caused by prolonged ventilatory
support
6.)Airway abnormalities, including tracheobronchial compression and
tracheo-malacia caused
by the displaced aorta and enlarged left pulmonary artery.
7.)Restrictive lung disease due to reduced total lung capacity
8.)Recurrent respiratory infections
9.)Recurrent wheezing episodes
10.)Reduced exercise tolerance
11.)Scoliosis in adolescent years due to abnormal thoracic cage
development.
12.)Nutritional,musculoskeletal,neurological,and gastrointestinal
comorbidities
13.)Delayed growth and developments.

Treatment for Pulmonary Hypoplasia

Treatment options for pulmonary hypoplasia depend on the underlying
cause, severity, and the individual's overall health. The primary goals of
treatment are to improve respiratory function and support the
development and growth of the lungs. Here are some common
treatment approaches:
1.Respiratory support:
-Mechanical ventilation: Newborns with severe respiratory distress may
require mechanical ventilation to provide breathing support
-High-frequency oscillatory ventilation (HFOV): HFOV is a specialized
ventilation technique that delivers small, rapid breaths to help maintain
lung inflation and oxygenation.
-Extracorporeal membrane oxygenation (ECMO): In severe cases of
respiratory failure, ECMO may be used as a temporary life support
system to provide oxygenation and circulation support.
2.Surgical interventions:
--Repair of diaphragmatic hernia: In cases where pulmonary hypoplasia
is caused by a diaphragmatic hernia, surgical repair is typically required
to correct the diaphragmatic defect and allow for normal lung
expansion.
-Thoracic or chest wall surgeries: In some cases, corrective surgeries
may be performed to address thoracic deformities or skeletal
abnormalities that restrict lung growth.
3.Supportive care:
-Nutritional support: Adequate nutrition is essential for growth and
development. Nutritional support may be provided through enteral
feeding or intravenous methods.
-Infection prevention: Due to the increased susceptibility to respiratory
infections, appropriate infection prevention measures, such as
vaccination and strict hygiene practices, are important.

The management of pulmonary hypoplasia often requires a

multidisciplinary approach involving neonatologists, pediatric
pulmonologists, pediatric surgeons, and other healthcare professionals.
The specific treatment plan will be tailored to the individual's needs
and may involve a combination of medical interventions and supportive
care