CDH

Congenital Diaphragmatic Hernia

(NOTE:  Jason Lee was born with CDH and at 3 years old developed scar adhesion’s that herniated his stomach and poisoned him. What is sad is that we kept telling the doctors something was wrong and they wouldn’t listen. And NO ONE told us about the possibility of scar adhesion’s despite the fact we asked them from the beginning what to look out for during his life.  It wasn’t until after his passing they told us scar adhesion’s are common. Too late!)

Congenital Diaphragmatic Hernias (CDH) is a rare condition that is present at birth and characterized by the protrusion of organs from the abdomen into the chest through an abnormal opening (hernia) in the muscle that divides the chest from the abdominal cavity (diaphragm). This could cause the lungs to be overcrowded and not grow enough to support the baby even if the diaphragm is surgically repaired.

1 in 3,000 Babies Affected
Survival Lower with Multiple Defects
43% Have Additional Birth Defects
RECURRENCE RISK LOW
CLUES ABOUT CAUSES
Scar Adhesions

Parents and doctors face difficult, urgent decisions when a fetus or newborn is diagnosed with diaphragmatic hernia. Although the defect can be surgically repaired (sometimes even with experimental prenatal surgery), many babies die because their lungs are too small or because they have additional birth defects. The California Birth Defects Monitoring Program reviewed 237 diaphragmatic hernia cases from over 700,000 births to provide insight based on California’s experience.

1 in 3,000 Babies Affected

Statewide, 3.3 per 10,000 babies were born with diaphragmatic hernia, about 200 babies each year. Rates were similar in all years studied (1983-1987), and were slightly higher in rural counties. The mother’s age, race, and number of previous births did not alter risks. Twins were more likely than single births to have diaphragmatic hernia.

Survival Lower with Multiple Defects

55% of live born babies with isolated diaphragmatic hernia were alive at age 1, but only 29% of those with additional birth defects survived. None of the 15 infants with chromosome defects lived. 10 cases were stillborn –all had multiple birth defects.

The survival rate was similar in babies of all races. Among those with additional birth defects, males were 3 times more likely to survive than females –girls tended to have more serious associated defects than boys.

A baby with diaphragmatic hernia alone was unlikely to have low birth weight or be born prematurely, but infants with additional birth defects were born slightly earlier and smaller. Higher birth weight improved survival chances.

43% Have Additional Birth Defects

96% of affected infants had posterolateral diaphragmatic defects, where the muscular sheet separating the chest and abdominal cavities failed to close normally by 6-7 weeks after conception. 84% of defects were on the left side and 13% were right-sided.

43% of the 227 babies with posterolateral defects had other structural birth defects. Heart defects were the most common, found in 26% of all infants. There were 12 infants with chromosome abnormalities. Several others had recognized birth defects patterns such as the Cornelia de Lange syndrome, Fryns syndrome, and CHARGE association.

4% of infants had other types of diaphragmatic hernias. All had multiple birth defects; none survived.

RECURRENCE RISK LOW

Brothers and sisters of affected infants were no more likely than other children to have birth defects. Only 1 family had a previous child with a diaphragmatic hernia.

CLUES ABOUT CAUSES

Do the different forms of diaphragmatic hernia have the same underlying cause? Isolated and non isolated diaphragmatic hernia have different seasonal occurrence patterns, urban rural distributions, and risks in boys and girls suggesting they are different defects.

Diaphragmatic hernia can be triggered in animals by dietary deficiencies and prenatal exposure to certain chemicals, including the herbicide nitrogen. Except for its association with chromosome abnormalities and certain birth defects syndromes, there is no known cause for diaphragmatic hernia in humans. Identifying subtypes with different risk patterns is the first step in uncovering causes.

SCAR ADHESIONS

This section is being researched further. Our son Jason passed away because scar adhesions, from his surgery at birth, had built up and herniated his stomach which in turn poisoned his own body. The fact that scar adhesions were possible was not relayed to us by his pediatric surgeon or his pediatrician. If your infant has had any type of surgery, please be aware of scar adhesions. Have routine x-rays done. I will be looking into this further to give specific information as I am able. This subject is still very hard for me, but important to get out.

REFERENCE: Torfs CP, Curry CJR, Bateson TF, Honore LH. A population-based study of congenital diaphragmatic hernia. Teratology 1992; 46:555-565.

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