Congenital diaphragmatic hernia/CDH

Diaphragmatic hernia occurs in about 3–5 per 10,000 pregnancies. Most cases are detected early during pregnancy through ultrasound examination. In Sweden, about 25–30 babies are born with diaphragmatic hernia each year, two thirds of which are boys. 

The diaphragm is a muscular sheet that separates the thoracic cavity from the abdominal cavity. The diaphragm is formed early in fetal development.

A diaphragmatic hernia is a malformation where the muscular sheet does not close properly and an opening appears between the abdominal cavity and the thoracic cavity. This may cause the abdominal organs to slide up through this opening, pushing aside the heart and lungs, which affects the development of the lungs (pulmonary hypoplasia) and the newborn’s respiratory function.

Due to the pulmonary hypoplasia, there is a risk of developing pulmonary hypertension, which is elevated blood pressure in the circulation between the heart and lungs that impairs oxygenation of the blood.  A newborn baby with diaphragmatic hernia requires immediate respiratory support. In cases of severe respiratory failure, so-called ECMO (extracorporeal membrane oxygenation) treatment is administered, with oxygenation outside the body. Surgery to close the hernia and reposition the abdominal organs is performed a few days after birth.

Most infants with congenital diaphragmatic hernia survive due to early intensive care treatment. However, many have persistent symptoms, e.g. from lungs and airways, stomach and intestine, chest and back. Cognitive and motor development may sometimes be affected. Autism and ADHD are more common in children with CDH than in other children. Many children with diaphragmatic hernia have eating difficulties and delayed eating development. Symptoms such as asthma, constipation and severe reflux are common. There is also an increased risk of developing ileus.  Children with congenital diaphragmatic hernia are regularly followed up by a multidisciplinary team throughout their childhood.  

Congenital diaphragmatic hernia, CDH, Bochdalek hernia, Morgagni hernia, congenital diaphragmatic defect

Children with congenital diaphragmatic hernia may require additional dental care and support. Children with respiratory failure often have increased energy needs and must eat more frequently, sometimes even at night, which increases the risk of dental caries. Many have difficulties eating, delayed eating development and oral aversion.

Enamel defects and tooth development disorders are more common than in the population at large. Gastroesophageal reflux is very common and may cause heartburn and vomiting, and lead to dental erosion.

 Many children experience problems with mucus in the airways. Long-term use of respiratory support with CPAP masks may adversely affect bite development. Asthma medication increases the risk of dry mouth and oral fungal infections. Some also have neuropsychiatric disabilities that may affect their ability to participate in dental care. 

• Children with congenital diaphragmatic hernia should be followed up by dental care from an early age, with a focus on information about optimal self-care. They should be offered enhanced preventive measures and dental treatment adapted to their needs. Contact with specialist pediatric dentistry is recommended. 

• It is important to focus on caries prevention, erosion control and early assessment of tooth and bite development. 

• Always consult the responsible physician before sedation with benzodiazepines or nitrous oxide. 

• In case of eating difficulties and oral aversion, contact with a speech therapist is recommended. 

• Coordination between specialists in dentistry, paediatrics and nutrition is important.