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Congenital diaphragmatic hernia (CDH) is a malformation characterized by a defect

in the posterolateral diaphragm, the foramen of Bochdalek, through which the


abdominal viscera migrate into the chest during fetal life.The reported incidence of
CDH varies from 1 in 2200 to 1 in 5000 births.Polyhydramnios is present in 20% of
pregnancies involving an infant with CDH and in 50% of pregnancies associated with
infants with CDH who are stillborn.In most series, 80% of posterolateral
diaphragmatic hernias have been reported to occur on the left side and 20% on the
right side.Bilateral CDH are rare.The size of the defect varies from small (2 or 3cm)
to very large, involving most ofthe hemidiaphragm.A rim ofmuscle is usually present
around the defect which is often covered posteromedially with peritoneum.A hernial
sac, composed of pleura and peritoneum, has been reported in about 20% of
patients. Widespread use ofobstetric sonography has led to an increase in the
frequency ofantenatal diagnosis of CDH, which is established by demonstration of
the abdominal viscera in the chest. Three easily detectable features
polyhydramnios, mediastinal shift and the absence ofan intra-abdominal stomach
bubble should prompt a more careful search for herniated abdominal organs in the
chest.Polyhydramnios is present in about 80% of the pregnancies with fetuses who
have CDH and has also been associated with poor outcome. Postnatally, the most
severely affected babies present with respiratory distress (cyanosis, tachypnoea
and sternal recession) at birth.Other infants develop cyanosis, tachypnoea and
grunting respirations within minutes or hours after birth. Physical examination
reveals a scaphoid abdomen, an increased anteroposterior diameter of the thorax
and mediastinal shift. Breath sounds are absent on the affected side. Associated
congenital anomalies may also be seen or revealed on further examination. CHD
presents beyond the first hours of life in 1020% cases.
Diagnosis of CDH is made postnatally by plain radiography of the chest and
abdomen by demonstration of air-filled loops of the bowel in the chest and a paucity
of gas in the abdomen. The diaphragmatic margin is absent, there is a mediastinal
shift to the opposite side and only a small portion of the lung may be seen on the
ipsilateral side. The mortality rate of infants born with CDH remains high,despite
optimal perinatal care.The high mortality rate in CDH has been attributed to
pulmonary hypoplasia and associated persistent pulmonary hypertension. In recent
years, newer management strategies such as permissive hypercapnia,high
frequency ventilation, extracorporeal membrane oxygenation and delayed surgical
repair have emerged in the care ofhigh-risk CDH patients,which offer some hope of
improving overall survival.
Congenital Eventration of the Diaphragm
Eventration of the diaphragm has been described as an abnormally high or deviated
position ofall or part of the hemidiaphragm.Eventration may be congenital or
acquired as a result of phrenic nerve palsy. Congenital eventration is a
developmental abnormality which results in muscular aplasia of the diaphragm. In
acquired eventration, the diaphragm, which initially had fully developed

musculature,becomes atrophic secondary to phrenic nerve damage and


disuse.Although this section deals with congenital eventration,the clinical features
and principles of management are similar in congenital and acquired forms of
eventration. Clinical features range from being asymptomatic to severe respiratory
distress. Patients may present later in infancy with repeated attacks of pneumonia,
bronchitis or bronchiectasis. Occasionally, patients present later in childhood with
gastrointestinal symptoms of vomiting or epigastric discomfort. In patients with
phrenic nerve palsy,there may be a history of difficult delivery. They may present
with tachypnoea, respiratory distress or cyanosis. Physical examination reveals
decreased breath sounds on the affected side, mediastinal shift during inspiration
and a scaphoid abdomen. The diagnosis of eventration is usually made on a chest
X-ray.Frontal and lateral chest X-rays will show an elevated diaphragm with a
smooth,unbroken outline. Fluoroscopy is a useful investigation for differentiating a
complete eventration from a hernia.Paradoxical movement of the diaphragm is seen
if complete eventration is present. Ultrasonography is the most useful study in the
diagnosis of eventration of the diaphragm and for identification of abdominal organs
underneath the eventration.Other investigation modalities include
pneumoperitonography,contrast peritonography, radioisotope scanning and
computed tomography scans but these are rarely required. Symptomatic patients,
especially those with respiratory distress, need prompt supportive care with
endotracheal intubation and ventilation with humidified oxygen to minimize the
diaphragmatic excursions.A nasogastric tube is passed to decompress the stomach
and intravenous fluids are commenced.Surgery is undertaken once the patients
condition is stabilized

General anaesthesia with muscle relaxation is used. The baby is positioned supine
on a warm blanket. The most commonly preferred approach is abdominal.This offers
good exposure,easy reduction of the abdominal viscera and recognition and
correction of associated gastrointestinal anomalies. A subcostal transverse muscle
cutting incision is made on the side of the hernia.
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Figure 13.1
The contents of the hernia are gently reduced in the abdomen. On the right side,
the small intestine and colon are first reduced and the liver is withdrawn last.After
the hernia is reduced,an attempt is made to visualize the ipsilateral lung.This is
usually done by retracting the anterior rim of the diaphragm.Often,a hypoplastic
lung can be observed at the apex. A hernial sac, composed of pleura and
peritoneum, is present in about 20% of patients. The sac, if present,is excised to
avoid leaving a loculated spaceoccupying lesion in the chest.
Figure 13.2

examination reveals decreased breath sounds on the affected side, mediastinal shift
during inspiration and a scaphoid abdomen. The diagnosis of eventration is usually
made on a chest X-ray.Frontal and lateral chest X-rays will show an elevated
diaphragm with a smooth,unbroken outline. Fluoroscopy is a useful investigation for
differentiating a complete eventration from a hernia.Paradoxical movement of the
diaphragm is seen if complete eventration is present. Ultrasonography is the most
useful study in the diagnosis of eventration of the diaphragm and for identification
of abdominal organs underneath the eventration.Other investigation modalities
include pneumoperitonography,contrast peritonography, radioisotope scanning and
computed tomography scans but these are rarely required. Symptomatic patients,
especially those with respiratory distress, need prompt supportive care with
endotracheal intubation and ventilation with humidified oxygen to minimize the
diaphragmatic excursions.A nasogastric tube is passed to decompress the stomach
and intravenous fluids are commenced.Surgery is undertaken once the patients
condition is stabilized.
Chapter 13

Congenital Diaphragmatic Hernia and Eventration

117
Figure 13.1
Liver
Stomach
Colon
Figure 13.2
Prem Puri
118
Figure 13.3
Most diaphragmatic defects can be sutured by direct sutures of the edges of the
defect.Usually the anterior rim of the diaphragm is quite evident. However, the
posterior rim may not be immediately apparent
and may require dissection for delineation.The posterior rim of the diaphragm is
mobilized by incising the overlying peritoneum.
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Figure 13.4,13.5
The defect is closed by interrupted non-absorbable sutures.Occasionally,the
posterior rim is absent altogether, in which case the anterior rim of the dia

phragm is sutured to the lower ribs with either periostial or pericostal sutures.
Chapter 13

Congenital Diaphragmatic Hernia and Eventration

119
Figure 13.3
Figure 13.4 Figure 13.5
Prem Puri
120
Figure 13.6
Ifthe defect is large,it may not be possible to repair it by direct suture. Various
techniques have been described and include the use of prerenal fascia, rib
structures, the latissimus dorsi muscle, rotational muscle flaps from the thoracoabdominal wall and prosthetic patches.The operations involving muscle flaps are too
long and complex for critically ill patients and can lead to unsightly chest
deformities. Prosthetic materials, including Marlex mesh, reinforced silicone
elastomer,preserved pericardial heterografts, preserved dura and the
polytetrafluoroethylene patch (PTFE),have been advocated.The most
commonly used prosthetic material presently is Surgisis soft tissue graft,which is
incorporated into adjacent tissue,and this tends to lessen the risk,extension or
displacement,with a decreased risk of infection. Abdomen is closed in layers.Ifthe
abdominal cavity is small, gentle stretching of the abdominal wall will enable safe
closure in most of the patients.Chest drain should be avoided. The argument
against the use ofa chest drain is in avoidance ofbarotraumas as it increases the
transpulmonary pressure gradient.
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Figure 13.7
Plication of the diaphragm has been used for many years to treat
eventration.Plication increases both tidal volume and maximal breathing capacity
and has been successful in many clinical series.An abdominal approach through a
subcostal incision is preferred for left-sided eventration but a thoracic ap
proach through a posterolateral incision via the sixth space may be used for rightsided lesions.The transabdominal approach allows good visualization ofthe entire
diaphragm from front to back and easier mobilization of abdominal contents.
Chapter 13
121

Congenital Diaphragmatic Hernia and Eventration

Figure 13.6
Figure 13.7
Prem Puri
122
Figure 13.8,13.9
Plication of the diaphragm is carried out using nonabsorbable sutures and avoiding
injury to the phrenic nerve. In cases of complete eventration, the diaphragm may be
strengthened by a muscle flap or prosthetic patch.
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Chapter 13

Congenital Diaphragmatic Hernia and Eventration

123
Figure 13.8
Right phrenic nerve
Figure 13.9
Prem Puri
124
CONCLUSION
After transfer to the intensive care unit,the infant is kept warm,given maintenance
requirements of intravenous fluids and has vital signs monitored closely with regular
blood gas analyses and monitoring of preductal and postductal oxygenation.
Ventilatory support is continued postoperatively with the aim of maintaining
preductal PO2 around 80100 mmHg, PCO2 up to 60 mmHg,and pH greater than
7.25 with hyperventilation (rates up to 150 per min) and the
lowest possible pressures and low tidal volumes.The intrathoracic air pocket will
usually reabsorb but evidence of increasing air and fluid with mediastinal shift
requires insertion of a chest drain. Weaning from ventilation should be meticulous
and slow as small variations in pH,PO2and PCO2will lead to persistent pulmonary
hypertension. Weaning should commence with lowering of FiO2, then peak
pressures and finally respiratory rate.