Congenital hypertrophic pyloric stenosis

Proven content

Congenital hypertrophic pyloric stenosis is caused by excessive growth of the degenerative circular layer of muscle cells in this part of the stomach. Symptoms occur between 2 and 4 weeks of age as vomiting, which gradually becomes worse with each feeding. Their content is the mother’s curd food with no bile added. Weight loss occurs with good appetite.

Congenital hypertrophic pyloric stenosis – description of the disease

This is an anatomical defect that is characterized by an overgrowth of the smooth muscles of the pylorus. The causes of this disease are still unclear. Some theories speak of abnormal development of ganglion cells in the pyloric wall; genetic factors and disorders of local nitric oxide synthesis. Pyloric stenosis is most often manifested by severe vomiting, which often leads to the dehydration of the child. Ultrasound is the basis for the diagnosis. The laparoscopic method is often used in the treatment.

What is the pylorus?

It is the olive-shaped end section of the stomach with a firm consistency. It is in the stomach that the initial digestion of the consumed food takes place, it is flexible, therefore its position and shape change depending on how full it is and the position of our body. The stomach is connected to the esophagus at the top. In turn, the pylorus connects with the duodenum – when we lie on our back, it is usually located under the right costal arch. It consists of smooth muscle fibers and plays an important role: it allows food to pass from the stomach to further parts of the gastrointestinal tract.

Frequency of appearance

Congenital hypertrophic pyloric stenosis occurs with a frequency of 2-5 cases per 1000 live births. Men are more often affected by this disease, especially African Americans and Asians (less often they are white people). In addition, studies show that this defect is more common in babies from the first pregnancy, especially when the pregnant woman had previously suffered from pyloric stenosis.

Causes of congenital hypertrophic pyloric stenosis

As mentioned before, the disease is associated with pyloric smooth muscle hypertrophy. Increasing contractility of the muscles and the formation of mucosa edema lead to impaired patency of the pylorus. Other possible causes of congenital hypertrophic pyloric stenosis are:

1. disturbances of the local synthesis of nitric oxide (reduced relaxation of the pyloric muscles),
2. abnormal development of ganglion cells in the pyloric wall,
3. increased local expression of growth factors,
4. genetic predisposition.

Congenital hypertrophic pyloric stenosis – symptoms

The first symptoms of this ailment usually appear in newborns between the third and eight weeks of life. In the initial phase, there is an inconspicuous downpour after a meal, which, as the narrowing of the pyloric lumen increases, becomes larger and larger. Over time, sloppy vomiting begins to join, without any admixture of bile. In 5 percent In cases of vomiting, blood is present. This situation leads to dehydration which, as it grows, causes constipation and defecation problems. The stools are small and appear as dense, dark brown lumps and then become thinner and thinner. In addition, there are problems with urination and weight loss of the child. This results in the development of water-electrolyte and acid-base disturbances in the form of hypokalemic and hypochloraemic metabolic alkalosis. The child is deficient in potassium and chlorine, and his blood pH is very elevated. It worsens the condition of the little patient: there are disturbances in consciousness, breathing and convulsions.

Curiosity: high appetite is observed in children with pyloric stenosis despite constant vomiting.

Congenital hypertrophic pyloric stenosis – diagnosis

Usually, the clinical picture of the disease is sufficient to make a proper diagnosis. However, in most patients, it must be confirmed for confirmation ultrasound examinationwhich is considered the basis of diagnostics. Usually, this examination confirms the presence of pylorostenosis. In children with pylorosthenosis, the pylorus is closed throughout the examination, and its morphological parameters remain unchanged. On the other hand, in the case of pyloric spasm, the wall thickness and length of the pyloric canal often exceed normal values, but they may change during the examination. In doubtful cases, an additional radiological examination is performed.

Diagnostics should also take into account other possible causes of persistent vomiting of the newborn. For this purpose, it is recommended to conduct a medical history and determine whether it is vomiting or downpour, which is not unusual in a newborn. You should look at how the baby is fed, what is the volume of food given and if there is too much of a hole in the nipple that causes the baby to swallow large amounts of air. In addition, the doctor should determine whether the child’s weight has decreased due to vomiting.

Treatment of congenital hypertrophic pyloric stenosis

The main treatment for this ailment is surgical procedure. It is highly effective and causes symptoms to be quickly eliminated. During the procedure, the doctor makes an incision in the circular and longitudinal layers of the pyloric smooth muscles (without disturbing the mucosa). As a result, the lumen of the pylorus regains its original size (most often an incision is made in the upper right quadrant of the abdominal wall).

A few hours after the operation, you can start giving your newborn a drink in the form of a glucose solution. After a few days of hospitalization, the toddler is discharged home.

Recently, it has become more and more popular pyloromiotomia laparoskopowawhich requires a much shorter hospital stay and a faster return to normal feeding. The number of postoperative complications is also much smaller.

Conservative treatment of this ailment is implemented only in light cases! Unfortunately, treatment is usually cumbersome and is associated with the risk of complications.