Congenital_Diaphragmatic_Hernia_Flashcards
What is congenital diaphragmatic hernia (CDH)?
CDH is a condition occurring in about 1 in 2,000 newborns, characterized by herniation of abdominal viscera into the chest cavity due to incomplete diaphragm formation, leading to pulmonary issues and respiratory distress.
What causes congenital diaphragmatic hernia?
CDH usually results from the failure of the pleuroperitoneal canal to close completely during fetal development.
What is the most common type of CDH?
The most common type of CDH is the left-sided posterolateral Bochdalek hernia, accounting for about 85% of cases.
What is the survival rate for newborns with CDH?
Despite modern medical intervention, only about 50% of newborns with CDH survive.
summarise
Congenital diaphragmatic hernia
Congenital diaphragmatic hernia (CDH) occurs in around 1 in 2,000 newborns. It is characterised by the herniation of abdominal viscera into the chest cavity due to incomplete formation of the diaphragm. This can result in pulmonary hypoplasia and hypertension which causes respiratory distress shortly after birth.
Pathophysiology
usually represents a failure of the pleuroperitoneal canal to close completely
The most common type of CDH is a left-sided posterolateral Bochdalek hernia which accounts for around 85% of cases.
Only around 50% of newborns with CDH survive despite modern medical intervention.
You are asked to review a neonate with a postnatal diagnosis of congenital diaphragmatic hernia. They are now stable, having had initial medical management. The baby’s parents have done some background reading on the condition and have some questions for you. Which of the following statements regarding congenital diaphragmatic hernia is true?
Once repaired, there is no risk of recurrence.
Congenital diaphragmatic hernias are equally common on the left and right.
A significant consequence of congenital diaphragmatic hernia is systemic hypertension.
The presence of the liver in the thoracic cavity is a poor prognostic factor for CDH
There is no increased risk in a younger sibling if an older sibling had CDH.
The presence of the liver in the thoracic cavity is a poor prognostic factor for CDH
The outcome or prognosis of a patient with CDH is largely dependent on 2 factors
(1) Liver position
(2) Lung-to-head ratio
If the liver has herniated into the chest, the disease is more severe and there is lower chance of survival.
The lung-to-head ratio is a numeric estimate of the size of the foetal lungs, dependent on the amount of lung visible. A ratio >1.0 reflects a better outcome.
Risk of recurrence is possible in CDH, and is dependent on severity of the defect.
CDH is significantly more common on the left hand side. 85% of cases occur on the left, 13% on the right and 2% bilaterally.
Pulmonary hypertension, rather than systemic hypertension, is a risk of CDH.
There is increased risk where there is a positive family history in a sibling.
You are asked to attend a preterm delivery. The neonate is born at 36 weeks gestation via emergency Caesarean section. The neonate has difficulty initiating breathing and requires resuscitation. They are dyspnoeic and tachypnoeic at a rate of 85 breaths/min. On auscultation of the chest, there is reduced breath sounds bilaterally. Heart sounds are displaced medially. The abdominal wall appears concave. What is the most likely diagnosis?
Bronchopulmonary dysplasia
Transient tachypnoea of the newborn
Congenital diaphragmatic hernia
Tetralogy of Fallot
Ventricular septal defect
Congenital diaphragmatic hernia
Congenital diaphragmatic hernia presents with scaphoid abdomen, due to herniation of the abdominal contents into the cleft
Congenital diaphragmatic hernia can present with dyspnoea and tachypnoea at birth. The auscultation findings are due to pulmonary hypoplasia and compression of the lung due to the presence of abdominal contents in the thoracic cavity. Prompt treatment and respiratory support are required.
This classical concave abdominal appearance would not be present in any of the other conditions listed.
A newborn baby is transferred to the neonatal intensive care unit shortly after birth due to respiratory distress. An x-ray taken on arrival is shown below:
What is the diagnosis?
Bronchopulmonary dysplasia
Respiratory distress syndrome
Left-sided neonatal bronchiectasis
Congenital diaphragmatic hernia
Left pneumothorax
Bowel loops can be seen in the left side of the thoracic cavity.
