The diaphragm is a thin layer of muscle and tissue that separates the chest

and abdominal cavity. It is the major muscle that the body uses to breathe.
When your child has a congenital diaphragmatic hernia (CDH), it means
that there’s a hole in that layer — or, rarely, that the diaphragm is missing
altogether. CDH affects about one in every 2,500 babies.

Congenital diaphragmatic hernia (CDH) occurs when the diaphragm, the

muscle that separates the chest from the abdomen, fails to close during
prenatal development. This opening allows contents of the abdomen
(stomach, intestines and/or liver) to migrate into the chest, impacting the
growth and development of the lungs. The lungs will be smaller than
expected (pulmonary hypoplasia), and will have less developed blood
vessels. This causes high blood pressure in the lungs (pulmonary
hypertension).

How is a congenital diaphragmatic hernia

diagnosed?
During routine prenatal care an ultrasound may reveal the existence or
suspicion of a diaphragmatic hernia. If a CDH is diagnosed or suspected
during pregnancy, The following tests are performed as part of the
evaluation to confirm the presence of a CDH and to find out how severe it
is. 

If a CDH hasn’t been diagnosed before birth, it is usually diagnosed in the

newborn period when the baby has trouble breathing. Rarely, with small
hernias, and often with Morgagni-type CDH, it is diagnosed later in infancy
or in childhood.

Babies with a Bochdalek type of CDH are more likely to have another birth
defect. Complications of a CDH may include:
 Chronic lung disease: This serious condition can require your child to
have oxygen or medications after being discharged from the hospital.
 Pulmonary hypertension: This is a condition in which the blood
vessels in the lung itself are poorly developed and do not pick up the
oxygen as well from the air delivered to the lungs. This may be
temporary or can be so severe that it is life threatening.
 Gastroesophageal reflux: This is a condition in which acids and fluids
from the stomach move up to the esophagus and can cause
heartburn, vomiting, feeding, or lung problems.
 Failure to thrive: Children with the most serious lung problems are
most likely to have growth problems. Some require special feeding
tubes to give enough calories to grow adequately.
 Developmental delays: These include delays in the ability to roll over,
sit, crawl, stand, or walk. Your baby will almost always reach these
milestones, just usually at an older age than most children.
CAUSE: 
scientists believe that multiple genes from both parents — as well as a
number of environmental factors that we do not yet fully understand — may
contribute.

TREATMENT

N/IICU stay for babies with CDH

Your baby will be settled into the N/IICU where the initial goal is to keep the
baby as stable as possible. Lights will be kept low; noise and handling will
be minimized to optimize functioning of the baby’s small lungs which are
highly reactive to stimulation because of the associated pulmonary
hypertension.
If the baby doesn’t respond well to initial ventilation, the mode of ventilation
can be changed to high-frequency oscillatory ventilation (HFOV) which
delivers breaths and pressure differently than a conventional ventilator.

ECMO
If the baby is unstable, they may move onto extracorporeal membrane
oxygenation (ECMO).
ECMO allows the lungs to rest while a machine takes over the function of
the heart and the lungs, delivering oxygen to the baby while removing
carbon dioxide, the waste product of breathing.

Congenital diaphragmatic hernia repair

Surgical repair of CDH after delivery depends on your individual baby’s
progress in the days following birth.

Recovery after CDH surgery

Your baby will recover from surgery and begin the process of weaning from
the ventilator. Once the baby is ready, feedings of either breast milk or
formula will slowly begin through the NG or OG tube.  When the breathing
tube is removed a speech therapist and lactation consultant will work with
your baby to start feeding by mouth. 
The length of your baby’s hospital stay from birth to discharge is largely
dependent on the severity of your baby’s CDH and their response to
treatment

After your baby is born, they will be taken to and cared for in our Medical
and Surgical Intensive Care Unit (MSICU) at Boston Children’s.
 Your baby will remain on the mechanical ventilator.
 A head ultrasound will be performed to evaluate for any abnormality.
 An echocardiogram will be performed to evaluate the heart function
and assess for any heart defects. Your baby may be placed on
ECMO if needed.
 When your baby's condition has improved, our surgeons will repair
the diaphragmatic hernia with an operation.
  During the operation the abdominal organs will be moved from the
chest cavity back to the abdominal cavity and the hole in the
diaphragm is closed with stitches if the opening is small, or with a
patch if the opening is large or if the diaphragm is absent.

After the surgery

Babies will need to remain in the MSICU for a while after surgery.
 Although the abdominal organs are now in the right place, your
baby’s lungs and the blood vessels in the lungs are still
underdeveloped.
 Your baby will usually need to have breathing support for a period of
time after the operation.
 Even after your baby no longer needs help from a breathing machine,
they may still need oxygen and medications to help with breathing for
weeks, months, or years. 
 Pulmonary hypertension (poorly developed blood vessels in the
lungs) may persist requiring ongoing treatment.
 Nutrition is important for your baby’s lung and overall growth. Your
baby will initially receive nutrition from a special IV, and when your
baby has recovered from surgery feeds will be given through a
feeding tube placed in the nose that goes into the stomach or
intestine.
 Some babies have difficulties with feeding by mouth and require long
term feeding tube for feeding

In CDH, the contents of your child's abdomen, which may include the
stomach, intestines, liver, and/or spleen, may go through the hole and into
the chest. This prevents the normal development of the lung on that side,
and also affects the growth of the other lung. When your child’s lungs don’t
fully develop, they will have trouble breathing after birth.
When the organs from the abdomen prevent the lungs from growing, the
poor lung growth is called pulmonary hypoplasia. Healthy lungs have
millions of small air sacs (alveoli), each of which resembles a balloon filled
with air. With pulmonary hypoplasia:
 There are fewer air sacs than normal.
 The air sacs that are present are only able to partially fill with air.
 The air sacs deflate easily because of problems with a lubricating
fluid called surfactant.
When this happens, your baby is unable to take in enough oxygen to stay
healthy.