What is a congenital diaphragmatic hernia?
Congenital diaphragmatic hernias (CDH) are among the relatively common malformations, with an incidence of 1:2500 births. The baby's thoracic and abdominal cavities are not separated due to the partial absence of the diaphragm. The consequence of this malformation is the displacement of abdominal organs such as the stomach, intestines and in some cases the liver into the thoracic cavity. Compression of the fetal lungs (pulmonary compression) by the organs displaced into the thorax during development in the womb results in underdevelopment of the lungs (pulmonary hypoplasia). Congenital diaphragmatic hernia often co-occurs with other conditions such as infantile heart defects, renal malformations, intestinal atresia, pulmonary sequestration, hydrocephalus, anencephaly, and spina bifida. Chromosomal abnormalities such as trisomy 21, 13 or 18 exist in 10-20%.
How is diaphragmatic hernia diagnosed?
The diagnosis is made by ultrasound. The gastric bladder and heart are often displaced to one side, and part of the liver and/or intestinal loops can be seen in the thoracic region. Fetal MRI (magnetic resonance imaging) helps estimate the remaining lung volume and prognosis after birth.
How is congenital diaphragmatic hernia treated?
Congenital diaphragmatic hernia with lung volume < 25-30% is treated with fetoscopic intratracheal balloon occlusion.
In this procedure, a fetoscope is inserted under local anesthesia and sterile surgical conditions into the uterus and then across the fetal larynx until the trachea is divided (bifurcation). A latex balloon is placed between the larynx and the bifurcation and inflated using saline, after which the instruments are removed under ultrasound guidance. In the 34th-35th SSW, the balloon is removed.
How is the balloon removed?
The balloon is removed by another fetoscopy or by ultrasound-guided puncture of the filled saline. If the child is born before the balloon is removed, the so-called "EXIT procedure" (ex utero intrapartum treatment) is performed immediately during this procedure.
Prof. Dr. Michael Tchirikov has developed a "long-tail balloon" (EU patent) especially for the temporary closure of the fetal trachea for the formation of a proper lung volume during pregnancy, in order to facilitate the removal of the balloon while pulling on its integrated thread.
When can the surgery be performed?
The surgery is performed between the 22nd and 28th SSW.
What are the chances of success?
Survival is increased to 50-60% with successful surgery and formation of sufficient fetal lung volume.
What are the complications?
The most common complication of fetoscopic surgery is premature rupture of the membranes with subsequent premature birth. The complication rate of premature rupture of the membranes with its consequences is significantly reduced by the microinvasive surgical method established by Prof. Dr. Michael Tchirikov, which allows reduction of the diameter of the shaft and flexible optics under 4-D ultrasound control.
Other very rare complications include bleeding (< 1%), possible injury to abdominal organs, and complications of balloon removal after delivery.
