Congenital diaphragmatic hernia

The congenital diaphragmatic hernia is an absence of the closing of the pleuroperitoneal channel between 9 and 10 weeks of pregnancy. Because of the different pressure between the Abdomen and the Thorax, the bodies of the abdomen move in the thorax involving an insufficient development of the lungs with appearance of a pulmonary hypoplasy responsible for the death of half of the children.

Incidence

The incidence of this pathology occurs in a birth on approximately 3.500. In a little more half of the cases, the diaphragmatic hernia is isolated; in 25% of there exist associated malformations; in 20% a chromosomal anomaly is found and in the 5% of the remaining cases a genetic syndrome is highlighted.

the malformations generally associated with a congenital diaphragmatic hernia are: the cardiopathies, the anomalies of the central nervous system, the labio-palatine slits, anomalies renal and of the skeleton.
the chromosomal anomalies most frequent are the Trisomie 18 and the Trisomie 13.
the syndrome generally meeting is the Syndrome of Fryns.

Various types

side Hernia postéro or hernia of Bochdalek

80 to 92% of the congenital diaphragmatic hernias of which 85% of the hernias are on the left, 10% on the right and 5% is bilateral

Hernie of Morgagni-Larrey or hernia of Morgagni
central Hernie

Diagnosis anténatal

The diagnosis of congenital diaphragmatic hernia is generally done during a systematic echography. It is possible that the echography of the second quarters is normal and that is diagnosed only during the third quarters. Indeed, if the hernia is of small size, it is only when the intra-abdominal pressure increases that the internal organs abdominal move into intrathoracic.

The rate of detection of this pathology, in Europe, is of 70% but varies significantly according to the European countries. Indeed, in certain European countries like the Denmark or the Netherlands, the realization of an echography during the pregnancy is not systematic.

Echographic signs

The echographic diagnosis of the left HDC is easier than that of the right HDC. Echographic semiology rests on the description of the bodies hernias in the thorax, often associated with a deviation of the heart and a hydramnios. Majority of discovered in the 2nd quarter. The earliest diagnosis: 15 SA.

1. Left diaphragmatic hernia:

¤ Déviation of the heart on the right, volume sometimes decreased (hypoplasy of the cavities lefts), ¤ In the left hémithorax: - Stomach, anechoic, will intra thoracic, with cardiac movements transmitted to the stomach, - Handles intestinal, echoic, evoked by their peristalsis, - Sometimes echoic images belonging to spleen or the left lobe liver. ¤ On the other hand, direct image of the diaphragmatic opening with difficulty individualisable: solution of continuity of the fine anechoic band (interface), separating the thorax from the abdomen, ¤ Aspect deshabity of the abdomen and weak biometrics. 2. Right diaphragmatic hernia: More difficult diagnosis, improved nevertheless by the coding color which allows to individualize: - A deviation of the systemic axis ombilico Oporto, - rise of the liver (homogeneous and echoic) and of gall bladder (anechoic and pyriforme), - The heart is driven back towards the left hémithorax.

Differential diagnosis

The differential diagnosis of the congenital diaphragmatic fernie is done especially during echography. One can discuss is:

Cyst bronchogenic
Disease adénomatoide of the pulmonary lung
Sequestration
cystic Tératome
neurological Tumor
Tumor pulmonary para oesophagienne
Sterility

Postnatal diagnosis

Etiologies

Chromosomal anomalies

Genetic anomalies

Assumption of responsibility of the pregnancy

The diaphragmatic diagnosis of hernia confirmed by a Center of prenatal diagnosis, it is necessary to evaluate to seek:

Of the anomalies associated in particular cardiac by a fetal echocardiography
the realization of a Chromosomic chart by Amniocentèse or Puncture of fetal blood according to the date of discovered hernia
an evaluation of the risk of pulmonary hypoplasy

Evaluation of the risk of pulmonary hypoplasy

treatment in utero of the congenital diaphragmatic hernia

Assumption of responsibility of the newborn

Sources

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