Esophageal atresia

Esophageal atresia is a congenital malformation in which an interruption of the esophagus is in the foreground: either the esophagus is not connected to the stomach and empties into the trachea or she has such a strong narrowing ( stenosis) that no food can happen. The incidence of esophageal atresia is approximately 1:3500 births.

Pathogenesis

The esophagus develops during the fetal period from the embryonic foregut, which extends from the pharynx to the stomach of the seedling. From the 20th day of pregnancy a ventral thickening is detectable from the differentiated respiratory epithelium, which from the 26th day of pregnancy a complete separation in the form of a new tube - the later trachea - is formed. Is this separation process is disturbed, a Ösphagusatresie developed.

Classification

The different forms of esophageal atresia are classified according to Vogt as follows:

• Vogt Type I: Ösophagusaplasie ( no accumulation of air in the stomach ), frequency of approximately 1%
• Vogt Type II: atresia without fistula ösophagotracheale ( also no accumulation of air in the stomach ), frequency of about 6 %
• Vogt type IIIa: ösophagotracheale fistula at the upper segment, the lower segment ends in blind pouch, frequency about 1%
• Vogt type IIIb: ösophagotracheale fistula at the lower segment, the upper segment ends in blind pouch, frequency about 85 %
• Vogt type IIIc: ösophagotracheale fistula at the lower and upper segment, frequency about 5 %
• Vogt Type IV: ösophagotracheale fistula without atresia (so-called "H - fistula " ), Frequency: around 2 %.

The data on the frequency vary from author to author.

The extremely rare complete absence of the esophagus is referred to as Ösophagusagenesie.

Symptoms

The newborn patients - often there are premature babies - fall on by coughing, salivation and deterioration of their general condition. Often large amounts of foamy saliva run from the mouth, without that they can be swallowed. Some of the saliva is even vomited, even coughing and Zyanoseanfälle may occur in the child. After a feeding trial developed by Nahrungsaspiration cyanosis. In Vogt IV the affected infants suffer from repeated aspiration pneumonia, show no other symptoms.

Accompanying malformation

In some cases, esophageal atresia is combined with a heart defect. Other typical malformations are those of the extremities, vertebral abnormalities, kidney malformations or intestinal atresias, for example, in the context of VACTERL.

Diagnostics

Prenatally, the sonographic soft markers polyhydramnios out (too much amniotic fluid ) to a possible esophageal atresia.

After the birth, the symptoms point to a esophageal atresia.

To diagnosis the esophagus is probed. A resilient stop is indicative. An x-ray of the thorax shows the air filling of the upper blind sac (so-called medallion characters), and optionally an air filling of the intestine as an indication of a lower fistula. Only in exceptional cases, additional water- soluble contrast agent is added.

Other malformations have to be found by ultrasound. For surgical planning, the location of the aorta must be shown in the echocardiogram.

Therapy

Before an operation, the child's upper torso is slightly raised and sucked the secretion from the non-conducting part constant by a probe.

The surgical correction depends on the distance of the upper to the lower Oesophagusanteils. Is the removal of low, both can be interconnected Oesophagusanteile with an operation which is carried out rapidly. Is the distance great, alternative therapies must be made. There is either an extension of the esophagus treatment over several days or weeks, until the distance is short enough or the lack of the esophagus is replaced by gastric or intestinal Shares to be transferred to the chest. There is a connection to the trachea and lungs, it must be surgically severed and sealed in each case to prevent saliva and food from entering the respiratory tract.

Course and prognosis

Follow-up treatment is usually necessary over several years. Typical problems are tracheomalacia, Gastroesophageal reflux, stenosis at the Ösophagusnahtstelle. The lethality is dependent on associated malformations and birth weight. It is a birth weight greater than 1500 g, without significant heart failure less than 5%.