Overview
Congenital diaphragmatic hernia (CDH) is a birth defect caused by a hole in the diaphragm — the muscle separating the chest and abdomen — that allows abdominal organs, such as the intestines, stomach, and sometimes the liver or spleen, to move into the chest cavity during fetal development. This impedes lung growth and causes serious breathing problems at birth. It affects roughly 1 in every 2,000 to 4,000 births. The most common type is a posterolateral, left-sided defect (Bochdalek hernia), accounting for about 80% of cases, while the anterior Morgagni hernia is less common and generally milder.
Diagnosis
CDH is increasingly diagnosed prenatally via ultrasound at 18-20 weeks of pregnancy. After birth, it presents with distinctive signs: severe respiratory distress, low oxygen levels with the skin turning bluish, rapid breathing sounds, a full chest with a sunken (scaphoid) abdomen, sometimes bowel sounds heard within the chest, and severe constipation from bowel obstruction. A chest X-ray confirms the diagnosis in nearly all cases. Not every case is caught prenatally — some, especially Morgagni or right-sided hernias, may not be discovered until birth or even later in childhood, and these later-discovered cases are generally less severe.
Important note: CDH isn’t a condition requiring emergency surgery right at birth. The priority is medical stabilization in the neonatal intensive care unit (NICU) to control pulmonary hypertension and ensure adequate oxygenation; severe cases may require ECMO. Surgery is usually performed after medical stabilization, often within the first days to two weeks of life.
Pre-Op
The priority after birth is stabilizing the child before surgery is considered. This stage includes:
Prenatal diagnosis (when possible)
An ultrasound at 18-20 weeks of pregnancy. Not every case is caught prenatally, especially Morgagni or right-sided hernias, which tend to be milder.
Immediate stabilization after birth
The child is placed on a ventilator immediately after birth and moved to the NICU. The priority is controlling pulmonary hypertension and ensuring adequate oxygenation, not immediate surgery.
Monitoring and tests
Chest X-rays, blood tests, blood pressure monitoring, and IV nutrition until the child is stable enough for surgery. Severe cases may require ECMO.
Surgical timing
Surgery is usually performed after medical stabilization, often within the first days to two weeks of life.
Post-Op
The surgery aims to return the abdominal organs to their normal position and close the defect in the diaphragm. Below is what the surgery involves and what happens afterward.
Surgical Repair
The surgeon uses either open surgery through a sub-rib incision — for large defects or when the case isn’t suitable for a laparoscopic approach — or thoracoscopic (minimally invasive) repair for selected, stable patients through small incisions in the chest wall. The abdominal organs are returned to their normal position, then the diaphragm defect is closed with sutures if small, or with a synthetic patch if too large to close directly (a patch carries a higher long-term risk of the hernia recurring and needs ongoing monitoring).
Immediately After Surgery
The child remains in the NICU on a ventilator until able to breathe independently.
Recovery Period
Feeding gradually progresses from tubes to oral feeding, along with medications to manage blood pressure and breathing as needed.
Signs of a Successful Surgery
- Stable breathing without needing ventilator support.
- Improving tolerance of feeding.
- Normal weight gain.
- Normal wound healing.
Survival Rates and Possible Complications
For isolated CDH (without other major anomalies) treated at specialized centers, survival rates range between 70% and 90%. Possible complications include: injury to nearby organs such as the digestive tract, lungs, or heart during surgery, internal bleeding, infection at the surgical site, or a reaction to anesthesia. Hernia recurrence is possible but uncommon, and would require another surgery if it happens. CDH survivors need regular, multidisciplinary follow-up (respiratory, surgical, nutritional, and developmental), and most mild-to-moderate cases go on to live normal, active lives.
Symptoms Requiring Immediate Medical Attention
- Difficulty breathing or the skin turning bluish.
- Fever.
- Redness or discharge from the wound.
- Poor feeding or repeated vomiting.
- Unusual lethargy.
Frequently Asked Questions
What is diaphragmatic hernia in children?
Diaphragmatic hernia is a congenital defect caused by incomplete development of the diaphragm muscle (the wall separating the abdomen and chest), which lets abdominal organs like the stomach and intestines move up into the chest cavity and press on the lungs and heart. It affects about 1 in every 2,500 children.
Why is diaphragmatic hernia surgery considered an emergency?
Because abdominal organs protruding into the chest press on the lungs, making breathing difficult, and raise pressure in the pulmonary arteries, straining the heart. Delaying surgery threatens the child's life.
How is the child prepared before surgery?
The child is placed on a ventilator right after birth, moved to intensive care, and undergoes X-rays, blood tests, blood pressure monitoring, and IV nutrition until stable enough for surgery.
How is diaphragmatic hernia surgery performed?
The surgeon typically uses a laparoscopic approach: small incisions in the chest for the camera and instruments, returning the abdominal organs to their normal position, then closing the diaphragm opening with sutures, or with a special mesh if the opening is large.
What happens after diaphragmatic hernia surgery?
The child stays in intensive care on a ventilator until able to breathe independently. Feeding is gradually introduced from feeding tubes to oral feeding, along with medications to manage blood pressure and breathing.
Can diaphragmatic hernia come back after surgery?
Recurrence is possible but rare. If it happens, the child needs another surgery to repair it.
Does the child need long-term follow-up after surgery?
Yes. The child needs regular medical follow-up after surgery to monitor growth and lung function, especially if the hernia was large. Some children need supplemental oxygen or feeding tubes for several months after surgery.
Book Your Consultation Now
Last updated: December 5, 2024