BJA Education, 18(4): 95e101 (2018)

doi: 10.1016/j.bjae.2018.01.001
Advance Access Publication Date: 2 March 2018

Matrix codes: 1A01,

2D01, 3D00

Anaesthetic management of patients with a

congenital diaphragmatic hernia
M. Quinney1 and H. Wellesley2,*
1
University College London Hospitals NHS Foundation Trust, London, UK and 2Great Ormond Street
Hospital for Children NHS Trust, London, UK
*Corresponding author. E-mail: hugo.wellesley@gosh.nhs.uk.

 CDH is not a neonatal surgical emergency.

Learning objectives
By reading this article, you should be able to:
 Recognise a congenital diaphragmatic hernia
(CDH) as a cause of neonatal respiratory distress
and describe the early supportive management
steps
 Identify poor prognostic features for patients
with a CDH
 Define the aims of medical management
 Explain the most likely intraoperative problems
during surgery for CDH.
Key points
 Congenital diaphragmatic hernia (CDH) is a rare
birth defect affecting 1 in 3600 registered births
across Europe.
 Key clinical features are lung hypoplasia and
pulmonary hypertension.
Michelle Quinney FRCA is a specialty trainee in anaesthesia who has
completed 12 months at Great Ormond Street Hospital for Children
and plans to pursue a career in paediatric anaesthesia.
Hugo Wellesley FRCA MA is a Consultant at Great Ormond Street
Hospital for Children and an Honorary Senior Lecturer at the UCH
Institute of Child Health. His clinical interests include general sur-
gery for neonates and children. He is also member of the hospital’s
Clinical Ethics Committee.
 A multidisciplinary approach to optimal timing of
surgery is critical.
 Cardiac abnormalities occur in 14% of cases.
Defect size and presence of cardiac abnormalities
are strong predictors of mortality.
A congenital diaphragmatic hernia (CDH) occurs when a
defect in the diaphragm allows abdominal organs to protrude
into the thoracic cavity (Fig. 1). It affects approximately 1 in
3600 registered births1 and is a potentially life-threatening
condition, the severity of which is primarily related to the
extent of lung hypoplasia and pulmonary hypertension.
Advances in management strategies include protective
ventilation, careful timing of surgery, the judicious use of
extracorporeal membrane oxygenation (ECMO) and the
introduction of both thoracoscopic and fetal intervention, but
it remains a challenging condition to treat successfully with
overall mortality rates still around 30%.2
Aetiology
The exact trigger for development of the diaphragmatic defect
is currently undetermined. Traditionally it was assumed that
herniation of abdominal organs into the chest directly inhibits
normal lung development. However, the occurrence of bilat-
eral pulmonary hypoplasia in unilateral diaphragmatic her-
nias has led to the proposal of other theories.
The ‘dual hit hypothesis’ has evolved from rodent models,
and suggests that pulmonary hypoplasia is the primary
disturbance, occurring as a result of genetic and environment
factors. This then hampers the formation of the diaphragm;

Accepted: February 2, 2018

© 2018 Published by Elsevier Ltd on behalf of British Journal of Anaesthesia.
For Permissions, please email: permissions@elsevier.com

95
Congenital diaphragmatic hernia

Table 2 Classification of CDH according to size of defect

Type Defect

A Small defects entirely surrounded by muscle

B <50% chest wall with absent diaphragmatic tissue
C >50% chest wall with absent diaphragmatic tissue
D Complete absence of hemidiaphragm

(i) birth weight <1.5 kg,

(ii) Apgar score at 5 mins <7,
(iii) presence of chromosomal abnormality,
(iv) presence of major cardiac abnormality, and
(v) suprasystemic pulmonary hypertension on
echocardiography.

This scoring system enables patients to be stratified into

Fig 1 A left-sided hernia. From Langman’s Essential Medical Embryology, 13th
edition.8 Reproduced with kind permission from Wolters Kluwer Health.
the consequent protrusion of abdominal contents into the
thoracic cavity then further hinders lung development on that
sidedthe second ‘hit’.3
Associations
Approximately 10% of patients have associated genetic or
chromosomal abnormalities including trisomy 13, 18 and 21,
Fryns syndrome, Cornelia de Lange syndrome,
BeckwitheWiedemann and CHARGE Syndrome.1 Moreover,
almost a third of babies born with CDH have one or more
structural abnormality. The most common are those affecting
the cardiovascular system (14%; Table 1) although genitouri-
nary (7%), limb (5%), central nervous system (5%) and palatal
(2%) anomalies also occur.1
Severity
The overall severity of a CDH may be described by the clas-
sification in Table 2.4
Accurate prediction of severity and thus survival of infants
with CDH remains a challenge (Table 3). The absence of liver
herniation is the most reliable antenatal predictor of survival.5
Gestational age at diagnosis is also important as it is an early
indicator of defect size e and this has been shown to be one of
the most significant predictors of mortality. Infants with a
near absence of the diaphragm have been found to have a
survival rate of 57% compared with 95% in those who have a
defect small enough for a primary repair.2
A validated scoring system has been proposed based on
various measurements, including:
low (<10%), intermediate (~25%) or high risk (~50%) mortality
groups.6 Cardiac defects have been shown to worsen outcome
regardless of the severity of the hernia: 99% survival compared
with 88% in those with type A defects, and 58% compared with
39% survival in those with type D defects.4 The presence of a
small contralateral lung or a bilateral CDH are also poor prog-
nostic signs. The small number of right-sided herniae makes it
difficult to be sure about their relative prognosis, but they have
recently been associated with an increase in need for a patch
repair and a higher recurrence rate.7
Pathophysiology
Embryology
To understand how these herniae form, it is necessary to
appreciate the embryological development of the diaphragm.
On approximately day 22 of embryonic development, the
septum transversum extends from the anterior aspect of the
body cavity. Pleuroperitoneal folds then originate from the
lateral sides of the body cavity at the beginning of the 5th
week. By the 7th week, the septum transversum and pleuro-
peritoneal folds extend inwards to fuse with each other and
the mesentery of the oesophagus to form the initial complete
diaphragmatic structure and divide the thoracic and abdom-
inal cavities. By the 16th week of gestation, the muscular
component of the diaphragm begins to develop8 (Fig. 2).
The lung parenchyma of neonates with a CDH is also
abnormal. There is abnormal differentiation of type 2 pneu-
mocytes, and thickening of intrapulmonary arteries leading to
pulmonary hypertension. There is also an exaggerated
response to vasoactive substances with endothelin 1 being
Table 3 Antenatal and postnatal prognostic indicators in CDH
Indicators of a poor prognosis

Antenatal
Table 1 The relative frequency of cardiac abnormalities in patients Liver herniation
with CDH1 Early gestational age at diagnosis
Postnatal
Cardiac defect Relative Large defect size
frequency (%) Cardiac abnormalities
Chromosomal abnormalities
Ventricular septal defect 29 Severe pulmonary hypertension
Atrial septal defect 26 Low birth weight
Coarctation of the aorta 8 Low Apgar score at 5 min
Hypoplastic left heart 7 Small contralateral lung
Other 31 Bilateral CDH

96 BJA Education - Volume 18, Number 4, 2018


Fig 2 Embryological development of the diaphragm. From Langman’s Essential Medical Embryology, 13th edition.8 Reproduced with kind permission from Wolters
Kluwer Health.
shown to be dysregulated leading to worsening pulmonary
hypertension. Endothelin 1 concentrations in plasma have
been shown to be higher within the first month of life in ba-
bies that subsequently did not survive or were discharged on
oxygen compared with those discharged on room air.9
Types
The majority of defects are left sided with only 20% of cases
affecting the right side. Bilateral diaphragmatic herniae are
very rare.8 There are two main types of CDH: Bochdalek and
Morgagni.
Bochdalek
Bochdalek herniae occur in 95% of cases. They result from a
failure of fusion of the pleuroperitoneal folds and are conse-
quently posterolateral.
Morgagni
Morgagni hernias make up the remaining 5%. They result
from herniation through the foramen of Morgagnida small
defect within the septum transversum, posterior to the ster-
num. They are therefore retrosternal or parasternal, usually
smaller and right sided in 90% of cases. Morgagni herniae are
associated with a better outcome.8
Antenatal management
Diagnosis
Approximately 50% of cases are diagnosed antenatally as a
result of routine ultrasound screening.10 Sensitivity of
screening improves with advancing gestation, the presence of
associated abnormalities, and with an experienced ultraso-
nographer.10 Right-sided herniae are more difficult to di-
agnose as liver and fetal lung can look very similar on
ultrasound. Fetal magnetic resonance imaging can be used to
confirm a diagnosis. Once a CDH has been identified, high
resolution ultrasound should be used to grade severity in or-
der to aid parental counselling and potentially select those
who may benefit from experimental intrauterine interven-
tion. This usually includes measurements of lung volume and
the position of the liver as well as looking for significant
comorbidity.
Congenital diaphragmatic hernia
Fetal surgery
Fetoscopic endoluminal tracheal occlusion (FETO) is an
experimental procedure that has been undertaken in preg-
nancies with a predicted poor prognosis. Its aim is to pro-
mote lung growth and therefore limit the evolving
pulmonary hypoplasia.
Fetal lungs produce fluid; preventing the efflux of this
fluid by temporarily occluding the trachea with a balloon has
been shown to cause stretching of the lung tissue and
increased production of growth factors and thus accelerated
lung growth. This has been shown to substantially improve
short-term mortality rates in those with a poor prognosis,
but is complicated by the risk of preterm delivery.11 The
TOTAL trial (Tracheal Occlusion To Accelerate Lung
growthdNCT01240057) is currently recruiting patients in
Europe.
Postnatal management
Diagnosis
If not diagnosed antenatally, the initial signs of a diaphrag-
matic hernia will usually occur soon after birth with respira-
tory distress, possibly in the presence of a scaphoid abdomen.
A chest radiograph will typically show abdominal organs
within the thoracic cavity. (Figs 3 and 4).
Medical treatment
Patients born with a congenital diaphragmatic hernia causing
respiratory distress require resuscitation and medical stabi-
lisation before definitive surgical repair.
The vast majority of patients will require tracheal intuba-
tion and artificial ventilation immediately after delivery;
ideally mask ventilation should be avoided to prevent gastric
insufflation. The insertion of a nasogastric tube with contin-
uous or intermittent suction may help to decompress the
bowel and relieve pressure on the lungs. Only those babies
predicted to have good lung development (i.e. with a left-sided
defect, no liver involvement, and good lung growth on ante-
natal scans) should be considered for a trial without
intubation.12
Early echocardiography will reveal the degree of cardiac
impairment, concurrent defects, pulmonary hypertension
BJA Education - Volume 18, Number 4, 2018 97
Congenital diaphragmatic hernia
Fig 3 Chest radiograph of a left CDH, showing significant mediastinal shift. The
nasogastric tube is in the stomach, which has protruded into the left thoracic
cavity.
and presence of right to left shunting and thus guide medical
treatment.
Pharmacological
The mainstays of pharmacological therapy are the optimisa-
tion of adequate gas exchange, minimisation of pulmonary
Fig 4 Chest radiograph of a right CDH with significant liver herniation. An um-
bilical vein catheter is seen projecting into the thoracic cavity on the right side.
ECMO cannulae are also visible.
98 BJA Education - Volume 18, Number 4, 2018
hypertension, and maintenance of organ perfusion in neo-
nates with significant haemodynamic instability.
Pulmonary vasodilators
Nitric oxide. Nitric oxide is commonly used in the manage-
ment of pulmonary hypertension secondary to a variety of
conditions; however when used in intensive care for babies
with a CDH, it does not seem to improve outcome and may be
associated with increased mortality rates.13 When it is used,
response to treatment should be assessed by echocardiogra-
phy and if it has not made a significant difference it should be
stopped.
Other agents
In cases of severe refractory pulmonary hypertension, intra-
venous sildenafil or prostacyclin have been suggested by the
CDH EURO Consortium as additional therapy that may
improve the clinical condition.12
In addition, prostaglandin E1 can be given to maintain
patency of the ductus arteriosus. This encourages a right to
left shunt, thus worsening postductal oxygen saturations, but
in severe cases can help offload the right ventricle and
maintain cardiac output.12
Inotropic/vasopressor drugs. Inotropic or vasopressor drugs
are widely used in the management of CDH to maintain
adequate organ perfusion in the context of systemic vasodi-
latation or impaired cardiac function. They can also decrease
right-to-left shunting, thereby improving post ductal oxygen
saturations, although there is no evidence that this confers
any significant survival benefit. The choice of agent is often
guided by echocardiographic findings and individual prefer-
ence but may include noradrenaline, dopamine, dobutamine
and milrinone.
Neuromuscular blocking agents. Neuromuscular blocking
agents can aid ventilator synchrony and optimise chest wall
compliance but their use should be restricted to situations
where ventilation is increasingly challenging.12
Ventilation
Historically, aggressive ventilation was used to induce hypo-
capnia and alkalosis and thereby reduce pulmonary hyper-
tension; however, protective ventilation strategies that avoid
further injury to damaged lung tissue have reduced mortality
in CDH. The CDH EURO Consortium advocates aiming for the
limitation of peak inspiratory pressures to 25 cm H2O with
PEEP kept at 3e5 cm H2O and allowing permissive
hypercapnia.12
High-frequency oscillatory ventilation
High frequency oscillatory ventilation (HFOV) is classically
used as a rescue strategy when hypoxia and severe hyper-
capnia persist despite maximal conventional ventilation. The
VICI trial (2016) randomised 171 neonates to conventional
ventilation or HFOV as the initial mode of ventilation and
found no significant difference in mortality, but those who
had conventional ventilation were ventilated for shorter pe-
riods, needed less nitric oxide, sildenafil and ECMO, and had
lower requirements for inotropic drugs.14

ECMO
ECMO is provided as a rescue therapy before surgical correc-
tion in neonates where conventional therapy is failing. It was
first used for CDH in the late 1970s.15 Since then, several trials
have looked at the effect of ECMO on outcome in CDH. This
work, however, has been confounded by advances in ante-
natal diagnosis and counselling, more protective modes of
ventilation, delayed surgery and differences in selection
criteria between centres.15 There is a degree of consensus
however that ECMO does confer a survival advantage in the
most severe cases of CDH (i.e. those with a predicted mortality
of more than 80%), however its value in more moderate dis-
ease is less clear because of the high incidence of ECMO-
related complications.15
Criteria for ECMO in CDH
The CDH EURO Consortium recommends that ECMO is
considered in the following situations:
(i) peak inspiratory pressure >28 cm H2O on conventional
ventilation needed to achieve O2 saturation >85%,
(ii) PaCO2>9.0 kPa (67 mm Hg) despite optimum ventilation,
(iii) pre or post ductal saturations consistently 80% or
70%, respectively,
(iv) pH<7.15 or serum lactate 5 mmol litre 1, and
(v) refractory hypotension despite maximal fluid and
inotropic therapy with urine output <0.5 ml kg 1 h 1 for
at least 12e24 h.12
It is acknowledged however that there is weak evidence to
support these recommendations.
Surgical repair
Surgical correction of a CDH involves returning the abdominal
organs from the chest into the abdominal cavity. The dia-
phragmatic defect is then closed either by a primary repair
(whereby the defect is directly sutured closed) or by inserting a
patch to close larger defects. Surgery can be open or
thoracoscopic.
Open surgery
The open surgical approach is usually via a subcostal incision.
Open surgery is necessary for larger defects, particularly in
the presence of liver herniation, and for sicker patients
requiring higher ventilatory or cardiovascular support.
Occasionally it may not be possible to close the abdominal
wall after open surgery without compromise to ventilatory or
cardiovascular stability and the risk of abdominal compart-
ment syndrome. This is particularly true where the hernia is
large and the abdominal cavity is small. The viscera can
therefore be covered and the patient left with a laparostomy,
with definitive closure occurring at a later date.
Thoracoscopic surgery
This minimally invasive approach has many theoretical ad-
vantages, including less postoperative pain, shorter ventila-
tion time, earlier feeding, shorter hospitalisation, and less
scarring. Thoracoscopic surgery does, however, take longer
and results in higher PaCO2, more severe acidosis, and
decreased cerebral oxygenation saturations,16 the long-term
significance of which are not clear. A requirement for higher
Congenital diaphragmatic hernia
ventilatory pressures may also worsen lung injury and pul-
monary hypertension. In addition, thoracoscopic surgery is
associated with higher rates of recurrence, particularly in the
context of ongoing vasopressor therapy or HFOV,17 and there
is little evidence to suggest an improvement in mortality.18
Careful patient selection is therefore imperative in deter-
mining the appropriate surgical approach for each patient. This
should involve a balanced discussion between surgical, anaes-
thetic, intensive care teams and parents based on both local
experience and the individual risks and benefits of each case.
Timing of surgery
Historically, CDH repair was treated as a surgical emergency.
However, the degree of pulmonary hypoplasia is the major
influence on prognosis and emergency surgery therefore
confers little benefit. There is much debate but little
consensus within the literature regarding the optimal timing
of surgery.
Recommendations from the CDH EURO Consortium state
that the following physiological parameters should be met
before surgery:
(i) mean arterial pressure normal for gestation,
(ii) preductal oxygen saturation consistently 85e95% on
FiO2 <0.5,
(iii) lactate below 3 mmol litre 1, and
(iv) urine output more than 1 ml kg 1 h 1 12.
These recommendations do, however, acknowledge that
repair on ECMO is a viable treatment strategy in the context of
appropriate patient selection.
Surgical repair on ECMO
Surgical repair in patients on ECMO is controversial. The risks
are higher (particularly bleeding) and the CDH Study Group
have reported 49% survival in a cohort of more than 3600
neonates.19 Some centres are now focusing on operating
within the first 72 h of starting ECMO (on average 4.5 days
earlier than the patients in the CDH Study Group cohort) and
have reported better survival rates with less time required on
ECMO after surgery. In one series, 73% of those undergoing
surgical repair within the first 72 h of ECMO survived,
compared with 50% of those repaired after 72 h, and 64% of
those repaired after decannulation.20 Others, however, have
reported 100% survival rates for repair after decannulation
compared with 44% during ECMO, supporting the strategy of
delaying repair.21
Unfortunately, many of the studies have produced con-
flicting results and it does not help that most have small
sample sizes, often around 50e100 patients. The picture is
further complicated by the fact that many of those having a
late repair on ECMO were unable to be weaned off, putting
them in the highest disease severity category and hence less
likely to survive regardless of the timing of surgery.15
Perioperative management
Decision to operate
There is little consensus regarding the best time to operate on
these patients but it is not a surgical emergency. As such,
these cases should be performed during normal working
hours. Nevertheless, the window of opportunity for these
children may be relatively small. The decision to operate
BJA Education - Volume 18, Number 4, 2018 99
Congenital diaphragmatic hernia
should involve close cooperation between the anaesthetists,
surgeons, intensivists, paediatricians and parents. Cardiore-
spiratory function should be stable with clinical evidence that
pulmonary hypertension is resolving supported by findings on
echocardiography.
Preoperative assessment
A thorough anaesthetic review should involve the standard
neonatal assessment including:
(i)antenatal and perinatal history,
(ii)glucose and fluid management,
(iii)cranial ultrasound results,
(iv) assessment of adequacy of arterial and venous access
(possibly including a long line or central venous
catheter),
(v) endotracheal tube size and position along with the
method used to secure it, and
(vi) blood results and availability of blood products.
Important details pertaining specifically to these patients
include:
(i) previous or ongoing need for HFOV or ECMO (and a his-
tory of any complications of treatment),
(ii) inotrope requirements,
(iii) pulmonary hypertension management,
(iv) current ventilator settings with careful attention to
ongoing trends in support, and
(v) arterial blood gas results.
Finally, relevant surgical factors should be reviewed such
as the side and position of the defect (Morgagni vs Bochdalek),
anticipated size, and degree of liver herniation if present.
Intraoperative challenges
Once a decision has been made to go to theatre, intraoperative
management requires excellent communication amongst the
whole team. It is even more important than usual to be aware
of what the surgeons are doing at all times, as this will directly
impact on the baby’s physiology and therefore anaesthetic
management. Any difficulties with maintaining stability need
to be effectively fed back to the surgical team. Some centres in
the UK routinely have two consultant anaesthetists present;
this can be very helpful but may also bring potential problems
and roles should be clearly established from the start.
The most common intraoperative problem is difficulty
with ventilation, in particular the inability to adequately
control PaCO2despecially during a thoracoscopic repair
where the hemithorax has been insufflated with CO2.
Approximately 30% of exhaled CO2 in these cases comes from
the pneumothorax.16 It is important to keep circuit dead space
to a minimum (e.g. by removing the angle piece and using a
neonatal circuit if available). End-tidal CO2 traces in neonates
are notoriously unreliable because of their small tidal volumes
and transcutaneous CO2 monitoring can be helpful to rapidly
identify trends in PaCO2. In case of difficulty, simple potential
causes should not be forgotten such as kinking of the ETT or a
need for suctioning. Compliance of the lungs can be assessed
with a T-piece and periods of manual hyperventilation may be
required to reduce hypercapnia. It may be necessary to
convert a thoracoscopic repair to an open procedure.
Inhaled nitric oxide should be available in theatre in case of
suspected worsening pulmonary hypertension that does not
100 BJA Education - Volume 18, Number 4, 2018
respond to the usual measures of hyperventilation to reduce
PaCO2, increasing the FiO2, management of acidosis, mainte-
nance of adequate warming, and analgesia.
Haemodynamic stability should also be closely monitored.
Over-hydration should be avoided as it may lead to pulmo-
nary congestion in the postoperative period. In cases of car-
diovascular instability, it may be necessary for vasopressors
or inotropes to be commenced to maintain organ perfusion
and manage intraoperative shunting.
Postoperative management
The patient will need to be transferred to the neonatal
intensive care unit, fully ventilated with ongoing sedation.
Compliance and gas exchange tend to deteriorate in the im-
mediate postoperative period. Pulmonary hypertension may
persist, potentially mandating further ECMO. It is also
important to be vigilant for bleeding, chylothorax, early
recurrence of the hernia or the development of a patch
infection.
Long-term sequelae
The overall survival rate of CDH is 69%2; however, up to 87% of
surviving children will go on to experience long-term
morbidity related to their CDH. The extent of these condi-
tions often relates to initial disease severity.22
(i) Respiratory
Recurrent respiratory tract infections (34%)
Chest deformity (40%)
(ii) Gastrointestinal
Reflux (30%)
Failure to thrive (20%)
(iii) Neurological
Sensorineural hearing loss (50%)
Cognitive impairment (up to 70%)
Long-term management of these conditions requires reg-
ular input of a multidisciplinary team.
Conclusion
CDH remains one of the most challenging conditions for
paediatric anaesthetists, surgeons and intensivists to treat
successfully. Despite advances in ventilation, extracorporeal
organ support and surgical approaches, rates of mortality are
still significant.
CDH is not a neonatal surgical emergency and the clinical
situation must be carefully assessed in order to determine the
optimal timing of corrective surgery for these high-risk
patients.
There is still a high degree of uncertainty as to how best to
manage these patients, especially regarding the optimal
timing of surgery (particularly when ECMO is required) and
the role of thoracoscopic surgery.
Declaration of interest
None declared.

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