Congenital diaphragmatic
hernia
Erica Everett
Resuscitation Officer at CUH
Learning outcomes
• By the end of the session and the associated reading you will be able
to:
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Describe congenital diaphragmatic hernia (CDH)
Describe the difference between the 2 abdominal wall defects
Identify appropriate initial management of an infant with either condition
Discuss support of families of infants with these conditions
What is CDH?
• Congenital diaphragmatic hernia is a failure of the normal
development of the diaphragm during the first trimester. There is a
failure of the muscular component to develop resulting in a
communication between the peritoneal and pleural cavities
• Herniation is usually on the left (80%)
• Bochdalek / posteriolateral defect
• The defect in the diaphragm permits herniation of the abdominal
contents into the thorax
• Causes:
• hypoplasia of the lung on the affected side
• Displacement of the mediastinum on the contralateral side
Antenatal diagnosis and management
• Most cases are diagnosed antenatally at 20 week ultrasound
• Detection rate around 60%
• Referral to fetal medicine centre for multi professional evaluation and
care
• Antenatal counselling for parent(s) includes prognosis depending on
the size of the defect and resultant hypoplasia
Antenatal management
• Multidisciplinary approach
• Antenatal steroids
• Fetal surgery
• Referral to a surgical centre for delivery improves outcomes
Delivery of the infant with known CDH
• LSCS vs SVD
• Timing of delivery
• Induction of labour at 38/40
• Preparation for delivery
• Expect a compromised infant
• Team work and communication
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Consultant led care
Aim is to avoid air insufflation of the herniated bowel
Immediate tracheal intubation following delivery
Resuscitation follows resus council (UK) algorithm
Unexpected CDH
• Signs of CDH are:
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Respiratory distress
Cardiovascular compromise
Scaphoid abdomen
Heart sounds shifted to the right
Unilateral chest movement
BVM resuscitation will often result in deterioration of the infant
• If CDH is suspected the infant should be intubated to avoid BVM
ventilation until x-ray can confirm diagnosis
• Prognosis for undiagnosed compared to antenatally diagnosed CDH is
variable depending on the size of the defect
Postnatal management
• Decompression of the stomach with positioning of a large bore gastric tube
• Aspirate and free drainage
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Chest x-ray to confirm diagnosis
Respiratory management
Venous access and arterial access
Cardiovascular management
• PPHN
• Fluids and nutrition
• NBM
• Full examination to assess for co-morbidities
Postnatal management – nursing
• Care of the ventilated infant
• Adequate sedation +/- muscle relaxant
• Fluid balance
• Minimal handling
• Developmentally supportive care
• Family centred care
Specific pre operative care
• Maintain gastric decompression
• Consent
• Blood products available
• Communication with haematology and surgical staff
• Timing of surgery
• Location of surgery
Repair of CDH
• Delayed surgical correction
• Haemodynamic instability
• Location of surgery
• Small defects are closed with suturing
• Large defects may need a patch
Post operative care
• Maintain stomach decompression
• NBM until paralytic ileus resolves
• IV antibiotics
• Analgesia
• Non pharmacological pain relief
• Pharmacological analgesia
• Developmentally supportive care
• Support of the family
Outcomes
• Mortality rates variable
• May be up to 48%
• Morbidity includes
• Developmental delay
• Nutritional sequlae
• Pulmonary sequlae
• Multidisciplinary follow up required
Families
• http://www.cdhuk.org.uk/about-cdh/what-is-cdh/
Abdominal wall defects
Erica Everett
Neonatal lecturer Anglia Ruskin University
Normal Embryological development
• Midgut grows rapidly week 6-10
• Midgut elongates faster than abdominal wall
• Herniates through umbilical ring
• Rotates and returns to abdominal cavity by 10-12 weeks
• Umbilical ring closes
Abdominal wall defects
• The term abdominal wall defect encompasses gastroschisis and
omphalocele (or exomphalos)
Omphalocele
• Exomphalos occurs in 1:4000 live births
• Failure of the bowel to re-enter the abdomen before 12 weeks gestation
• Extra-abdominal bowel is covered by peritoneum and the umbilical cord is at
the base of the defect
• Associated defects / abnormalities common
Gastroschisis
• Gastroschisis occurs in 1:3800 live births
• Herniation of the abdominal contents through an abdominal wall defect
usually to the right of the umbilical cord
• No peritoneal covering of the extra-abdominal bowel
• Rarely associated with other congenital abnormalities
Comparison
omphalocele
gastroschisis
• Closed
• Cord central
• Normal bowel
• Associated abnormalities 60%
• Open
• Cord left of defect
• Inflamed bowel
• Associated abnormalities 10%
• Cardiac defects
• Neural tube defect
• Trisomies (13/18/21)
• Atresia
Antenatal diagnosis
Gastroschisis
Exomphalous
Antenatal management
• Multidisciplinary approach
• Referral to a surgical centre for delivery improves outcomes
• Avoid exutero transfer
Immediate postnatal management
Gastroschisis
Exomphalous
• Handle bowel carefully
• Cover bowel in plastic
• Maintain midline position of the
bowel
• Observe bowel perfusion
• Gastric tube
• Immediate surgical referral and
early repair
• Sac should be covered
• Maintain midline position of the
defect
• Gastric tube
• Immediate surgical referral
Pre operative care
• Fluid and electrolyte balance
• Temperature instability
• Gastric decompression
• NBM / IV fluid / Nutrition
• Cardiovascular compromise
• Sepsis risk
Surgical repair
Gastroschisis
Exomphalous
• Significant defect may be
difficult to close
• Silo can be utilised to prevent
compromise to bowel perfusion
and respiratory compromise
• Staged reduction of silo until full
closure
Post operative care
• Care of silo
• Daily staged reduction
• Respiratory compromise can be common in this cohort of infants
• Maintain stomach decompression
• NBM until paralytic ileus resolves
• IV antibiotics
• Analgesia
• Non pharmacological pain relief
• Pharmacological analgesia
• Developmentally supportive care
• Support of the family
Outcomes
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Long term NBM and parenteral nutrition
Long hospital admissions
Nutrition
Omphalocele
• Survival >90% in
• Size of defect and exteriorisation of the liver have been found to be factors predictive
of poor outcome
• https://www.npeu.ox.ac.uk/baps-cass/surveillance/exo
• Gastroschisis
• pre-operative mortality 17%
• Post operative survival rate >87%
Families
• Family centred care
• Education
• Parent involvement in surgical pain relief
• Long term expressing of breast milk
• Feeding issues
• Long neonatal journey
• Transfer to paediatric care setting
To recap
• Congenital diaphragmatic hernia and abdominal wall defects are
relatively uncommon in neonatal practice with level II and I units
seeing very few.
• It is our responsibility to keep ourselves updated in all aspects of
neonatal care to ensure best care is achieved.