Atresia of the anus and rectum - causes and signs of atresia of the anus and rectum

Depending on the length of the rectum, which ends blindly (with a gas bubble), atresias are divided into:

• low (sublevator, anal) - its closed end does not reach the skin by less than 2 cm;
• medium (intermediate);
• high (suplevator, anorectal) - the blind end is deeper than two centimeters from the surface.

A distinction is made between fistulous and non-fistulous (complete) forms of pathology. 90% of cases are related to the first of them.

Fistula deviations are characterized by the presence of passages that open into:

• inguinal region;
• reproductive system;
• urinary organs.

Variants of fistula-free atresia:

• anomaly of the anus and rectum;
• defectiveness of the anal canal, its absence or covering with a translucent layer of skin (meconium pigmentation);
• underdevelopment of the intestinal canal with the correct structure of the anus.

It is necessary to distinguish atresia from stenosis.

Causes

The disease is associated with a genetic predisposition, the presence of dysostosis of bone development, VATER syndrome, which includes a tendency to vertebral, anal, tracheoesophageal defects, dysplasia.

If there is also a susceptibility to the development of heart defects (hydrocephalus), the syndrome is called VACTER.

The association of defects, including incomplete formation of limbs, is designated VACTERL. Thus, when one anomaly is detected, others should be suspected.

Symptoms

The clinical picture depends on the pathology. In the case of a fistula-free nature, 10-12 hours after birth, the newborn begins to show anxiety, does not suckle, does not pass meconium, and shows symptoms of obstruction (ileus), namely, bloating and asymmetry of the abdomen, vomiting of gastric contents, then bile and feces, toxicosis and dehydration. The baby can strain, and with a low location of the gas bubble, the skin in this area protrudes.

Ileus also develops with a fistula form, since narrow fistula lumens have poor permeability for feces. In this case, it can be excreted through the urethra, genital slit or openings in the perineum. As a result, concomitant diseases occur - cystitis, pyelonephritis, vulvitis, urosepsis, which can provoke death. Therefore, when the first alarming signs appear, you should immediately make an appointment with a pediatrician.

Diagnostics

To make an accurate diagnosis, you may need to consult a pediatrician, gynecologist, proctologist, urologist and surgeon. The absence of an anus is detected during the initial examination by a neonatologist, other defects are recognized within the first day. In this case, the examination is carried out in a short time, including:

• invertography according to Kakovich-Vangensteen-Rice - when the anus is formed, a radiopaque marker is placed in it, the child is placed head down in front of the X-ray machine. The distance from the mark to the gas bubble determines the height of atresia;
• digital examination;
• rectoscopy;
• proctography;
• fine-needle puncture according to Sitkoevsky - the skin in the groin is pierced in the direction of the intestine, a needle is inserted with gradual pulling of the plunger. When meconium appears in the syringe, the analysis is considered complete;
• ultrasound, MRI, electromyography;
• diagnostic laparoscopy.

The location of the fistula canals is clarified by the method of urethrocystography, catheterization of the urethra, fistulography. To establish concomitant abnormalities, ultrasound of the heart, kidneys and other organs, esophagoscopy are prescribed.

Treatment

Correction is carried out exclusively by surgery, so the child will need a surgeon's consultation. The operation consists of artificial formation of the anus, its complexity depends on the severity of the defect.

Without surgical intervention, the child dies by the end of the first week of life. The prognosis of therapy is positive in 30-40% of patients, 11-60% of operations are accompanied by a fatal outcome.

With delayed passage of feces, complete atresia, surgical intervention is necessary in the first two days after birth. For other types, proctoplasty and sigmoid colostomy are performed. Fistula closure is performed at the age of six months to two years.

In the postoperative period, constant monitoring of the child's diet and bowel movements is indicated. There may be a need for enemas, antibiotics, and physiotherapy.

Prevention

It is almost impossible to prevent this developmental defect; there are no methods of prevention.