Prognosis for restoration of function after rectal atresia. Is it possible to completely restore intestinal function with surgery? Diagnosis of anal atresia

Anal atresia is one of the rare diseases that is characterized by the absence of an opening in the right place.

According to statistics, approximately one child in five thousand is born with this pathology. After birth, a baby may have a completely or partially absent rectum or anus.

What are the reasons for the development this phenomenon and what to do if pathology occurs?

Causes

Atresia of the anus and rectum is considered a rare case. According to experts, the main cause of the disease is considered to be a violation of the development of the embryo.

Around the seventh week of pregnancy, the formation of the urogenital system and anal canal occurs. At this moment they begin to be separated by a partition. If this stage goes well, then the baby develops normally in the future.

If at this stage separation does not occur, then the organs begin to develop with various deformations. The pathological process can be primary or secondary.

The causes of the defect may be infectious diseases or disorders in the cardiovascular system.

This disease can develop with or without fistulas. It all depends on how the rectum is located relative to the muscle structures.

Types of pathology

Anal atresia is divided into several types, which include:

congenital narrowing of the anus. It is not detected immediately, since in the first months of life the baby passes liquefied stool. Problems begin when the child is introduced to the first complementary foods. Then there is a prolonged absence of bowel movement;
atypical location of the anus. The anus is located close to the genitals. In such cases, urgent surgery is required. The thing is that because of this situation, girls can suffer from vaginitis and diseases genitourinary tract chronic;
fistulas in the perineum. Often found in boys. Against this background, the baby develops partial intestinal obstruction;
fistulas that extend into the genitals. Often detected in girls. Anal passage located near the posterior vaginal commissure. Meconium and gas can come out through the fistula. This process can lead to chronic poisoning of the body;
anal atresia with a fistula that extends into the urinary organs. This type of illness is considered the most serious. It is detected in male children.

The disease can only be determined immediately after birth. A baby with pathology can be born healthy. But in some cases concomitant diseases are hydrocephalus and abnormalities of the skeletal system.

Symptoms

Anal atresia in newborns is detected immediately after birth by neonatologists.

Signs of an anomaly may be hidden in:

breast refusal, sleep disturbances, anxiety;
difficulty breathing, shortness of breath;
constant regurgitation. In the first weeks, the contents of the stomach;
absence of passage of meconium and excess gases. The child pushes regularly;
severe bloating. In some situations, distended intestinal loops are visible.

If the pathology is not detected in time, the child will vomit with bile or feces. This process leads to severe poisoning of the body, inflammation of the abdominal walls, and perforation of the intestines. Possible death.

If the baby has anal atresia with a fistula, then feces will come out through it.

In some situations, the rectum is formed as it should. But here anal hole covered by a septum of skin. Through it you can see the presence of meconium.

If there is an exit of the fistula into the genital organs, then intestinal obstruction does not develop. But this phenomenon leads to infection of the urinary organs. With this process, the baby develops cystitis or pyelonephritis.

Atresia of the anus with the release of the fistula into the genitourinary system is accompanied by the occurrence. This type of opening is very small for the passage of stool. This type The disease is diagnosed in boys.

The symptoms of fusion of the anus are expressed clearly, so it is difficult not to notice them. Often the pathology is diagnosed in the maternity hospital in the first days after labor.

Diagnosis

Rectal atresia is detected with great difficulty and not immediately. The thing is that the newborn has an anus in this situation, but the anomaly is located on the inside of the organ. This form The disease is detected only when the baby experiences severe symptoms in the form of vomiting bile and feces, and the absence of gases.

If there is a suspicion of the presence of pathology, then an examination should be carried out as soon as possible.

It includes:

examination by a surgeon;
performing ultrasound diagnostics in the perineal area;
performance x-ray examination Vangestin type. This method allows you to measure the height of atresia;
fine-needle puncture using the Sitkovsky method. In the place where the anus should be located, a small needle is inserted and the plunger is pulled until drops of meconium enter the syringe;
electromyography. Makes it possible to identify the location of the external sphincter.

If the anus is developed as it should, then probing of the rectum is performed using manual palpation. In the presence of fistulas, urethrocystography and fistulography are performed.

Magnetic tomography, ultrasound examination of the kidneys, heart and esophagus are used as additional diagnostic techniques.

Often, anal atresia develops with defects of other organs.

Therapeutic measures

It doesn’t matter what kind of anal atresia the baby has. In any case, surgical intervention is performed in the first days of life.

The only exception is those situations when the disease is accompanied by the formation of wide fistulas. This process allows the intestinal canal to empty normally. But they should have the operation before they reach one year of age.

In case of low atresia without the presence of concomitant defects of the heart muscle and kidneys, a one-stage operation called proctoplasty is performed.

Severe types of illness are treated with surgical procedure, which takes place in two stages. At the first stage, obstruction of the digestive canal is eliminated by applying a sigmostoma. This allows you to bring the end of the rectum to the anterior wall abdominal cavity.

The second stage is based on covering the sigmostoma. It is carried out six to twelve months after assessing the baby’s condition.

The postoperative stage is characterized by preventing narrowing of the anus. For this purpose, a device is used that artificially expands the anus and the end of the rectum.

After this, doctors prescribe gymnastics. It should be completed within three to four months. Drug therapy involves taking antibacterial agents.

If there is atresia with fistulas, then completely different treatment procedures are prescribed. Surgical manipulations are prohibited for children under two years of age if it is located in the bladder or vagina.

After this, children are prescribed a strict diet, taking large quantity liquids, constant enemas.

Preventive actions

The causes of this disease are not yet fully understood.

But, since the pathology develops precisely during pregnancy and is not hereditary in nature, doctors advise adhering to some preventive recommendations:

Refuse bad habits in the form and drinking.
Rest at least eight to nine hours a day.
Do not take medications without consulting a doctor.
Avoid colds and other infectious diseases in the first weeks of pregnancy.
Take care of proper nutrition. Refuse junk food, preservatives and fast foods.
Take regular walks.
Complete all tests and ultrasound diagnostics on time.
Avoid stressful situations, nervous experiences.
Register for pregnancy as early as possible.

If a woman has chronic diseases, then it is necessary to warn the doctor about this. It is better to plan pregnancy in advance. Before conception, you must undergo all tests for the presence of hidden infections and the amount of hormones.

Atresia of the anus and rectum is rare disease. But if a disease is detected, it requires an urgent examination by a doctor and surgical intervention.

Congenital anomalies occur in the form of complete atresia or atresia with fistulas.

Highlight:

anal atresia(in its place there is thin skin that can be easily pressed inside with a finger);

rectal atresia(in its place there is connective tissue, the anus may be absent, but it may be (it leads into a blind pocket 1-3 cm deep), sometimes combined with fistulas connecting the intestine with one of the pelvic organs: rectovaginal atresia, rectal-vaginal atresia, cystic atresia, rectal-urethral atresia);

atresia of the anus and rectum,

congenital narrowing of the rectum and anus,

ectopia of the anus,

congenital fistulas.

Clinical picture and diagnosis. Complete atresia of the anus or rectum is manifested by symptoms of intestinal obstruction from the first days after the birth of the child: bloating, failure to pass gases and feces, hiccups, belching, vomiting. Examination of the perineum allows you to make the correct diagnosis.

With atresia with fistulas, stool discharge occurs in an unusual place (through the vagina, urethra). With rectovesical atresia, the urine is cloudy and mixed with feces. The ectopic anal opening can be localized in the perineum or in the vestibule of the vagina (vestibular form).

Treatment. Complete atresia of the anus or rectum is an indication for urgent surgery. In case of anal atresia, the skin covering the exit from the intestine is cut, the mucous membrane of the rectum is reduced and it is sutured to the skin. Other types of complete rectal atresia require complex surgical interventions. In case of atresia with fistula, the operation is performed as planned; it is aimed at eliminating atresia and fistula.

Surgical treatment is indicated for the vestibular form of ectopia of the anus: a normally formed anus to the usual place (Stone's operation). Constrictions detected early are treated with bougienage. If there is no effect or stenosis is detected in adulthood, they resort to surgical treatment - dissection of the stenotic ring followed by intestinal plastic surgery or intestinal resection.

2. Diagnosis of acute pancreatitis, conservative therapy.

Classification

Hemorrhagic
Focal pancreatic necrosis
Pancreatic abscess
Total pancreatic necrosis

Clinical picture

constant intense pain of a girdling nature ( since the XI-XII intercostal nerves are involved in the process due to retroperitoneal edema, due to the entry of enzymes into the retroperitoneal space) with irradiation to the left costovertebral angle (Mayo-Robson symptom). Occurs after alcohol, fatty foods.

bloating in the epigastrium, due to the fact that the pancreas lies near the transverse colon(swelling of the pancreas leads to paresis of the colon). Palpation of muscle tension in the epigastrium.

vomiting that does not bring relief for pain in the epigastrium (since it is a reflex - swelling of the stellate ganglion. Undigested stool - “plasticine” stool.

In the early stages of the onset of the disease, objective data are very scarce, especially in the edematous form: pale skin, slight yellowness of the sclera(for biliary pancreatitis), mild cyanosis. The pulse may be normal or accelerated, the body temperature is normal. After infection of foci of necrosis, it increases, as with any purulent process.

The abdomen is usually soft, all parts participate in the act of breathing, and sometimes some bloating is noted. Shchetkin-Blumberg's symptom is negative. In approximately 1-2% of seriously ill patients, bluish, sometimes with a yellowish tinge spots (Gray Turner's symptom) and traces of resorption of hemorrhages in the pancreas and retroperitoneal tissue appear on the left side wall of the abdomen, indicating hemorrhagic pancreatitis. The same spots may be observed in the navel area(Cullen's sign). Percussion high tympanitis is determined over the entire surface of the abdomen - intestinal paresis occurs due to irritation or phlegmon of the retroperitoneal tissue or concomitant peritonitis. When a significant amount of exudate accumulates in the abdominal cavity, there is a dullness of percussion sound in the sloping parts of the abdomen, which is more easily detected when the patient is positioned on his side.

On palpation abdomen, pain in the epigastric region is noted. Abdominal muscle tension in initial period There is no development of pancreatitis. Only sometimes resistance and pain are noted in the epigastrium in the area where the pancreas is located (Kerte's symptom). Palpation in the left costovertebral angle (projection of the tail of the pancreas) is often painful (Mayo-Robson sign). With fatty necrosis of the pancreas, an inflammatory infiltrate forms early. It can be determined by palpation of the epigastric region. Due to paresis and swelling of the transverse colon or the presence of infiltration, it is not possible to clearly determine the pulsation abdominal aorta(Voskresensky symptom). Peristaltic sounds are already weakened at the very beginning of the development of pancreatitis, disappearing as the pathological process progresses and peritonitis appears. Percussion and auscultation of the chest in a number of patients reveals a sympathetic effusion in the left pleural cavity.

With very severe pancreatitis, a systemic response syndrome to inflammation develops, the functions of vital organs are disrupted, respiratory failure occurs, manifested by an increase in respiratory rate, adult respiratory distress syndrome (interstitial pulmonary edema, accumulation of transudate in the pleural cavities), cardiovascular failure (hypotension , frequent thread-like pulse, cyanosis of the skin and mucous membranes, decrease in blood volume, central venous pressure, minute and stroke volume of the heart, signs of myocardial ischemia on the ECG), liver, kidney and gastrointestinal failure (dynamic intestinal obstruction, hemorrhagic gastritis). Most patients experience a mental disorder: agitation, confusion, the degree of disturbance of which should be determined using Glasgow scale scores.

Functional liver disorders are usually manifested by a icteric discoloration of the skin. With persistent obstruction of the common bile duct, obstructive jaundice occurs with increased levels of bilirubin, transaminases, and enlarged liver. For acute pancreatitis typical promotion amylase and lipase in blood serum. The concentration of amylase (diastase) in the urine and in the exudate of the abdominal and pleural cavities increases significantly. With total pancreatic necrosis, the amylase level decreases. A more specific test for the early diagnosis of pancreatitis is the determination of trypsin in the blood serum, α-chymotrypsin, elastase, carboxypeptidase and especially phospholipase A, which plays a key role in the development of pancreatic necrosis. However, the complexity of their definition hinders the widespread use of these methods.

The acid-base state undergoes a shift to acidosis, against the background of which the flow of intracellular potassium into the blood increases while its excretion by the kidneys decreases. Hyperkalemia, which is dangerous for the body, develops. A decrease in calcium content in the blood indicates the progression of fat necrosis, the binding of calcium by fatty acids released as a result of the action of lipase on fatty tissue in foci of necrosis. Small foci of steatonecrosis occur on the omentum, parietal and visceral peritoneum (“stearin spots”). Calcium levels below 2 mmol/l (normal 2.10-2.65 mmol/l, or 8.4-10.6 mg/dl) are an unfavorable prognostic indicator.

Diagnostics acute pancreatitis is based on medical history (appearance sharp pain in the abdomen after a heavy meal, drinking alcohol or exacerbation of chronic calculous cholecystitis), data from physical, instrumental and laboratory studies.

Ultrasonography . Ultrasound provides significant assistance in diagnosis, allowing one to establish etiological factors (cholecysto- and choledocholithiasis) and identify swelling and enlargement of the pancreas. CT scan is a more accurate method for diagnosing acute pancreatitis compared to ultrasound. There are no obstacles to carrying it out. The reliability of diagnosis increases with intravenous or oral enhancement with contrast material. Enhanced computed tomography can more clearly identify diffuse or local enlargement of the gland, edema, foci of necrosis, fluid accumulation, changes in peripancreatic tissue, “paths of necrosis” outside the pancreas, as well as complications in the form of abscesses and cysts. Magnetic resonance imaging is a more advanced diagnostic method. It provides information similar to that obtained from a computed tomography scan. Laparoscopy shown when unclear diagnosis, if laparoscopic installation of drains is necessary for the treatment of acute pancreatitis. Laparoscopy allows you to see foci of steatonecrosis (stearin spots), inflammatory changes in the peritoneum, gall bladder, penetrate into the cavity of the lesser omentum and examine the pancreas, install drainages for the outflow of exudate and rinsing the cavity of the lesser omentum. If it is impossible to use laparoscopy to take peritoneal exudate and perform diagnostic lavage, a so-called “groping” catheter can be inserted into the abdominal cavity through a puncture in the abdominal wall (laparocentesis). Electrocardiography is necessary in all cases for differential diagnosis with acute heart attack myocardium, and to assess the state of cardiac activity during the development of the disease.

Conservative treatment:

Adequate infusion therapy is the basis for the treatment of pancreatic necrosis. Treatment begins with transfusion of isotonic solutions and potassium chloride for hypokalemia. Since in acute pancreatitis hypovolemia occurs due to the loss of the plasma part of the blood, colloidal solutions (starch, proteins) are used. Improvement rheological properties blood is achieved by administering low molecular weight dextrans with pentoxifylline.

At the same time, measures are taken to suppress the functional activity of the pancreas, create “physiological rest,” and limit food intake for 5-7 days. An effective reduction in pancreatic secretion is achieved by aspiration of gastric contents through a nasogastric tube and gastric lavage with cold water. In order to reduce the acidity of gastric secretions, alkaline drinks and omeprazole are prescribed.

To suppress the secretory activity of the gastropancreatoduodenal zone in the first 3-5 days of active hyperfermentemia, somatostatin analogs (ocreotide acetate, stylamine) are used, and to eliminate enzyme toxinemia, protease inhibitors are used: contrical or gordox intravenously. For the purpose of systemic detoxification, extracorporeal methods are used: plasmaphoresis, blood ultrafiltration.

Antibiotic therapy: carbapenems + third generation cephalosporins + metronidazole + fluoroquinolones. The edematous form does not require antibiotics.

Parenteral nutrition is mandatory.

ATRESIA OF THE ANUS AND RECTUM honey.
Atresia of the anus and rectum - absence of the natural canal of the rectum and/or anus; developmental anomaly.

Frequency

1:500-1:5000 live births. The predominant gender is male (2:1).
Genetic aspects. Exist hereditary forms atre-zium
VATER and VACTERL syndromes. (192350). Clinical picture: atresia of the anus, esophagus, duodenum and rectum, esophageal-tracheal fistula, pulmonary hypoplasia, dysplasia radius, six-fingered, hypoplasia femur, abnormal location thumb hands, ventricular septal defect, renal agenesis, urethral atresia and vertebral defects (hemivertebrae)
VACTERL syndrome and hydrocephalus (314390, 8 or p). Clinical picture: combination of VACTERL syndrome and hydrocephalus
Vertebral-costal dysostosis with anal atresia and urogenital anomalies (271520, p): anal atresia, genitourinary anomalies, single umbilical artery, severe dysplasia of the ribs and vertebrae, unusual shape chest.

Classification

Atresia
Cloacal form*
cystic
vaginal
With fistulas
into the urinary system in boys (into the bladder and urethra)*
into the reproductive system in girls (uterus*, vagina*, vestibule of the vagina)
on the perineum in boys and girls (in boys also on the scrotum and penis)
No fistulas
atresia of the anus and rectum*
anal atresia
covered anus
atresia of the rectum with a normally developed anus
Note. An asterisk (*) indicates high forms of atresia (supralevator, the blind sac of the rectum is located above 2-2.5 cm from the skin). All others are classified as low (sublevator).

Clinical picture

Usually detected when initial examination newborn in the maternity hospital.
If the child is not examined after birth for some reason, then by the end of the day the newborn begins to worry, profuse regurgitation appears, vomiting of stomach contents, then bile, and then late dates- mekonyom. The abdomen gradually becomes bloated. Meconium and gases do not pass. A picture of low intestinal obstruction develops.
Physical examination in the first hours after birth: establish the type of atresia and its height.
A covered anal hole is the most light form low atresia. A translucent membrane is identified at the site of the anus.
Anal atresia: also classified as low. At the site of the anus, a slight depression of a pigmented area of skin is detected. When pressing on it, they feel a ballot in view low position rectum.
Atresia of the anal canal and rectum: classified as high forms of atresia. The perineum is usually reduced in size and underdeveloped. Often the coccyx is underdeveloped or absent, sometimes the sacrum. At the site of the anus, the skin is most often smooth.
Rectal atresia (isolated) can be high or low. The anus with a well-formed external sphincter is located on usual place. To establish a diagnosis, it is enough to insert a catheter through the anus or conduct a digital examination.
Fistula into the reproductive system occurs exclusively in girls. The main symptom is the release of meconium, and then feces and gases through the genital opening.
A fistula in the urinary system (bladder, urethra) occurs almost exclusively in boys. The main symptom is the passage of meconium and gases through the external opening of the urethra.
The cloacal form of atresia is the most complex of all anorectal malformations. During the examination, the absence of the anus is discovered. In the place where the external openings of the urethra or vagina should be, there is one thing - the exit of the cloaca, into which the urethra, vagina and rectum open. Anatomical forms Cloaca are diverse, so a detailed examination is necessary.
Perineal fistula occurs in boys and girls. Diagnosis is based on external examination.
Combined malformations in various combinations often occur with atresia of the anus and rectum (renal aplasia, hypospadias, megaureter, hypospadias, duplication of the kidney and ureter, etc.).

Research methods

X-ray according to Vangestein. The natural location of the anus is marked with a radiopaque object (coin, pellet, etc.). The baby is wrapped in diapers, leaving his legs open, placed under an X-ray screen and lowered upside down. The height of the atresia is judged by the distance between the blind end of the intestine (gas bubble) and the mark.
Hole method according to Sitkoesky. Using a thin needle with a syringe, pierce the skin of the perineum at the natural location of the anus and, pulling the piston, insert the needle towards the intestine until a drop of meconium appears in the syringe. The height of the atresia is judged by distance
Ultrasound
Catheterization of the urethra - diagnosis of a fistula in its membranous part
Urethrocystography facilitates the diagnosis of fistula in the urinary system
Laparoscopy is indicated to clarify the condition of the internal genital organs.

Treatment:

Methods

surgical interventions
Simultaneous radical operations - perineal and abdominoperineal proctoplasty
An unnatural anus is applied only if there are contraindications to a one-stage radical operation.
Contraindications to one-stage radical surgery
Severe combined malformations
Accompanying illnesses
High rectal atresia in a child with prematurity II-IV degrees
Presence of fistulas in the urinary system
The surgeon lacks experience in abdominal-perineal proctoplasty in a newborn. Postoperative management
The child is placed in a heated incubator in the supine position. Legs spread, bent in knee joints and pull it to the stomach, fixing it with a bandage in the spreading position. This position is maintained for 5-10 days.
Perineal toilet is carried out several times a day, after each physiological departure
Catheter from Bladder removed on day 2, and if the child has a urethral fistula, the catheter is left for 3-5 days
Sutures in the area of the created anus are removed for 10-12 days
Feeding a child after perineal proctoplasty surgery is prescribed from the first day according to the usual age scheme; after abdominoperineal proctoplasty, it is first prescribed parenteral nutrition, and by 8 days the patient begins to receive normal nutrition for his age
Prescribing antibiotics and physiotherapy
To prevent narrowing of the anus or eliminate it, bougienage is carried out (start from 15-20 days, continue for
3-4 months, and longer if necessary).

Forecast

Without surgical intervention, the patient dies later
4-6 days after birth. Mortality during surgical treatment
- 11-60%. Good results Treatment is achieved in no more than 30-40% of patients.
see also

ICD

Q42 Congenital absence, atresia and stenosis of the large intestine

MSh

192350 VATER and VACTERL syndromes
271520 Vertebral-costal dysostosis with anal atresia and urogenital anomalies
314390 VACTERL syndrome and hydrocephalus Notes: VATER - Vertebral defects, Anal atresia, Iracheoesophageal fistula, Јsophageal atresia, Radial dysplasia; VACTERL
- Y_ertebral anomalies, Dpal atresia, Cardiac malformations,
Iracheoesophageal fistula, Esophageal atresia, Renal anomalies, Limb
anomalies

Literature

Jarcho S, Levin PM: Hereditary malformation of the
vertebral bodies. Bull. Johns Hopkins Hasp. 62:216-226, 1938

Directory of diseases. 2012 .

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During normal embryonic development The rectum descends into the perineum within a funnel-shaped muscle complex. Top part complex is formed by a structure known as the levator muscle, Bottom part — external sphincter. These striated muscles are controlled voluntarily. When the muscles contract, the rectum rises and is pulled forward, squeezing and closing, holding feces. Internal sphincter in lower section the rectum consists of circular involuntary smooth muscles; the internal sphincter is in a closed state, closing the anal canal, with the exception of the act of defecation.

Anal atresia is the absence of a normal open anus. Anal atresia is associated with incomplete prolapse of the rectum, hypoplasia of the infundibulum muscle complex described above, and the internal anal sphincter. Anal atresia in children occurs with a wide range of malformations of the anorectal region, which are classified according to gender and the need for colostomy, which is shown in early period treatment. About 10% of defects in boys are defects above the levator ani muscle. With such high defects there is serious violation muscle development, abnormal development of the sacrum, impaired sensitivity and neuromuscular innervation, as well as poor control of defecation after surgical correction. In the remaining 90% of cases in boys, the lesion is limited to the area between the levator ani muscle and the perineum, and there is often a rectourethral fistula. Fistula communication between the rectum and the genitourinary tract is also observed in girls. Almost a third of girls with anal atresia have a fistula between the rectum and the vestibule of the vagina. 40% of girls are diagnosed with a developmental defect in the form of a cloaca. Cloaca is a malformation of the anorectal region in which the rectum is connected to the vagina and ureters to form a common canal, which can be short (3 cm). Boys and girls can have anal atresia without fistula.

According to the literature, the prevalence of anorectal malformations is 1 in 5000 live newborns; in general, boys are more often affected and they tend to have higher defects. Anal atresia in newborns is associated with various birth defects development and some syndromes such as Down syndrome and association with VACTERL. Anomalies genitourinary system observed in approximately 50% of patients; the higher the defect, the more often it is combined with severe urological disorders. Cloaca, with a prevalence of 1 in 50,000 births, is an example of high anorectal malformation and severe genitourinary anomaly. Cloacal exstrophy is a complex malformation associated with anal atresia; in such children, omphalocele and exstrophy of the bladder are detected, into which the cecum and blindly ending colon open.

Symptoms of anal atresia and diagnosis

The first step is to determine the level of anorectal defect and associated malformations. To exclude esophageal atresia and gastric decompression, a nasogastric tube is inserted. Carefully examine the genitals and perineum to detect a fistula, which may appear as a whitish, barely noticeable subcutaneous tract along the perineal suture or the lower suture of the scrotum. A high or medium fistula may be suspected in boys with a split scrotum or atypical hypospadias. If there is no evidence of a fistula and the perineum and genitals appear normal, a piece of gauze is placed on the top of the penis to catch any meconium that may be excreted in urine. Such a finding is evidence of the presence of a rectourethral or rectouteral fistula and is an indication for colostomy. Similarly, in girls with anal atresia and a normal perineum, it is necessary to trace the passage of meconium through the fistula of the vaginal vestibule or high vaginal fistula. If the vulva is small and there is no hymen, there is likely a cloaca-type malformation. In this case, the urethra is not identified; urine and meconium are simultaneously released through a single opening.

In most cases, the decision to perform a colonostomy should be delayed until the child is 18 to 24 hours old. During this time, the distal rectum is distended, which helps identify a perineal fistula. If a fistula is not found and the child has not passed meconium through the penis or vagina, a lateral X-ray should be taken in the prone position with a marker on the perineum. If the x-ray shows a gas-filled rectum located at a distance of 1 cm from the perineum, a low defect is determined, which is treated during the neonatal period using anoplasty. If the defect is located above 1 cm from the perineum, a colostomy is indicated. Until this point, a chest x-ray is necessary to identify abnormalities of the upper vertebrae and ribs. X-ray examination of the abdominal cavity and pelvic organs in the supine position helps to identify abnormalities of the lumbar sacral region spine and, depending on the degree of agenesis of the sacral region, determine the initial prognosis. Echocardiography allows you to diagnose concomitant anomalies of the heart, abdominal organs - anomalies of the kidneys and ureters. Limb anomalies, especially radial forearm anomalies, can be evaluated clinically and radiographically to provide evidence in favor of VACTERL. Before the child is discharged or during early follow-up, excretory cystoureterography is indicated, which helps to identify possible vesicoureteral reflux. In addition, because spinal cord pathology is common, which can be detected even in patients with normal AP fluoroscopy and low defects, spinal cord ultrasound or MRI is indicated in all neonates with anal atresia to rule out occult spinal pathology, such as a tethered spinal cord. or spinal cord.

Treatment of anal atresia and prognosis

Newborns with low-grade anorectal anomalies, such as an anterior anus or a posterior perineal fistula, usually undergo perineal anoplasty in the first few days of life. Newborns with average or high level lesions, an end colostomy should be formed in the lower left quadrant of the abdomen. Colostomy allows for subsequent posterior sagittal plasty. Before performing restorative treatment for anal atresia, it is highly advisable to perform fistulography (irrigoscopy through the foot). This test involves injecting a water-soluble contrast agent into the stoma under fluoroscopic X-ray television (X-ray) guidance to determine the length of the distal lesion and where it enters the bladder or urethra. Sufficient pressure is applied to fill the bladder. Then, if the child is passing urine, a cystourethrogram is performed. If a cloaca is detected, it is necessary to additionally conduct a contrast study of a single opening. This study will determine the anatomical relationships of all three structures within the cloaca.

Posterior sagittal anorectoplasty is the gold standard for reconstructive intervention for anal atresia and associated anorectal and genitourinary defects development. The operation is performed with the child lying on his stomach. A median incision is made from the coccyx along the perineum, the tissue between the sphincter muscles and the posterior wall of the rectum is deeply dissected. Rear wall the anterior urinary fistula (if present) is isolated and crossed. The urinary fistula is sutured with absorbable suture. The rectum is carefully separated from the genitourinary tract and brought down to the perineum. Reconstruction of the perineum is performed, the rectum is located inside the muscular complex and parasagittal fibers of the external sphincter.

Laparoscopic anorectal resection is performed for high anal atresia. This minimally traumatic technique allows for precise positioning of the rectum within the levator ani muscles and the external anal sphincter muscle complex. The advantages of this method are excellent exposure rectal fistula and surrounding structures, precise location of the intestine along the anatomical midline and levator ring, minimal trauma abdominal wall and perineum. Optimal method reconstruction is total urogenital mobilization in combination with posterior sagittal anorectoplasty.

After reconstruction of mid and high anorectal anomalies, the most common problem is. Such patients are divided into two groups. Treatment for children with incontinence and constipation usually involves a large-volume enema through the anus or a Mallon appendicectomy. Patients with incontinence and a tendency to diarrhea are usually taught according to a specific program.

The long-term prognosis for incontinence is different and depends on the type and level of the defect, concomitant spinal defects, and the motivation of the patient and parents.

The article was prepared and edited by: surgeon

The frequency of rectal pathologies among newborns is 0.02%. Exact reasons their occurrence is unknown, but scientists as one of key factors consider heredity. Rectal atresia is a disease of newborns, which is characterized by underdevelopment of the anus. A fused rectum is more common in boys.

Atresia of the anus and rectum is congenital pathology digestive system, as well as atresia of the esophagus, bile ducts, etc. The disease occurs in one newborn out of 5 thousand and is diagnosed twice as often in boys. In a third of cases, the anomaly is combined with congenital diseases cardiovascular and urinary systems, kidneys, genitals.

The pathology requires emergency surgical assistance in the first few days after birth. The development of new methods for diagnosing and treating defects is an acute problem for neonatologists, pediatric surgeons, proctologists, urologists and other specialists.

The clinical manifestation of complete atresia occurs in the first 24 hours of life. There is no meconium, the baby is restless, and there are symptoms of poor intestinal obstruction. In the fistulous form, feces exit through the canal in the perineum, the external opening of the urethra, and the genital slit. Diagnostics consists of the results ultrasound examination, visual examination, cystourethrogram, fistulography.

Causes

Partial or complete intestinal obstruction develops during the antenatal period and is associated with fetal developmental disorders. The anomalies are the absence of an opening on the proctodeum and the non-division of the cloaca, which should be separated into the anorectal and genitourinary parts by the eighth week of the embryo's life. During the same period, two openings are formed in the cloaca, which are further transformed into the anus and urethra.

If the development of the embryo is disrupted at this time, anorectal defects appear. The pathology is often included in a complex of combined anomalies (VATER/VACTERL), which cover multiple malformations spinal column, heart (IVS defect), gastrointestinal tract, lungs, kidneys, limbs (polydactyly), etc.

Classification

Rectal atresia is divided into:

to high (supralevator);
average;
low (sublevator).

There are also total atresia (fistula-free), which accounts for up to 10% of cases, and the fistula form (90%).

Types of non-fistula atresia:

atresia of the rectum and anus;
rectal atresia;
anal atresia;
covering the anus.

The fistula form is divided into the following types:

fistulas extend into the organs of the urinary system (urethra, bladder);
exit into the organs of the reproductive system (entrance to the vagina, vagina, uterus);
extend into the perineum.

In addition to fusion, congenital stenosis of the rectum and anus also occurs, with a normal anus, and ectopia of the anus.

Symptoms

If health workers were unable to immediately recognize the pathology, Clinical signs manifest themselves after 11–12 hours. The baby sleeps poorly, is capricious, refuses to feed, and strains. By the end of the first 24 hours of life, signs of intestinal obstruction appear:

non-excretion of original feces;
heaving;
vomiting of stomach contents, later - bile and feces;
intoxication;
dehydration.

Lack of help leads to the death of the child from gastrointestinal perforation and peritonitis.

With low localization in the anal area, a small depression in the form of a funnel or a skin elevation with folds along the radius is observed. The anus may be covered with a film through which meconium can be seen. The overgrown intestine, as a rule, is localized 1 cm from the exit to the perineum, therefore, when tense, a “push” symptom occurs (bulging into the anus).

With a high position, the “push” symptom is absent. Atresia with a fistula in the urinary organs is also accompanied by intestinal obstruction, since the opening of the fistula is small and does not allow the passage of feces.

Fistulas in the urinary system mainly occur in boys; in girls, this type is observed very rarely. With rectourethral and rectovesical fistula, meconium is visualized in the urine, and gas bubbles emerge from the urinary canal when straining.

As a result of intestinal contents entering urinary tract complications develop:

cystitis;
pyelonephritis;
urosepsis.

A sign of esophageal atresia with a fistula into the vagina is the release of original feces through the genital opening. Acute intestinal obstruction is atypical for this form, however, excretion of intestinal contents through the genitals contributes to the development of urinary tract infection, vulvitis.

Perineal fistulas open near the anus, scrotum, or base of the penis. On skin Weeping is noted at the fistula opening. Normal bowel movements are impossible, which explains fast development intestinal obstruction.

Diagnostics

All newborns are checked for congenital anomalies. The area of the anus must be examined. With pathology, instead of the anus there is a slight depression, sometimes there is none.

It is important for doctors to determine the form of the anomaly. If only the anus is missing, and the rectum is developed normally, when the child cries, a protrusion in the area of the anus is clearly visualized.

The child is x-rayed using a special technique: the newborn is turned upside down, and an iron mark is placed on the anus area. This allows you to determine the severity of the pathology and the location of the atresia.

The greatest difficulty is presented by cases in which the anus is present, but the rectum is fused in some segment. Primary visual inspection does not allow you to determine the defect. Atresia is suspected only when vomiting occurs and there is no bowel movement.

For staging accurate diagnosis and exclusion of other diseases is carried out additional research. It is possible to palpate the rectum, during which the doctor will feel an obstacle.

Treatment

Infants with low anorectal abnormalities undergo perineoplasty in the first few days. In children with average and high atresia An end colostomy is formed in the lower outer quadrant of the abdominal wall. Then a final correction can be made.

Before performing an intervention to restore the anus, it is advisable to do a fistulogram. The manipulation consists of introducing contrast into the stoma in order to determine the length of the defective segment and localize the entry into the urinary canal or bladder.

Considerable pressure is applied to fill the bladder. Next, when the child produces urine, cystourethrography is done.

For combined anorectal and urogenital anomalies, sagittal anorectoplasty is performed. During the operation, the child is positioned on his stomach. The surgeon makes a median incision from the coccyx to the perineum, cutting the tissue between the sphincter muscles and rear arch rectum. Urinary fistulas are divided and sutured with self-absorbing thread. The rectum is separated from the urogenital tract and brought down to the perineum. Next, reconstruction of the perineum is performed.

In case of high localization, anorectal resection is performed laparoscopically. This technique allows you to position the intestine strictly along anatomical path. The advantage of the technique is minimal trauma to the abdominal wall.