Congenital anorectal malformations

Introduction

Introduction to congenital anorectal malformation Congenital rectal anus deformity is the first common digestive tract malformation. It is the most common neonatal surgical disease with a high incidence rate of about 1:2500. The male is slightly higher than the female. The main form is anal atresia, with many forms of lesions and complicated classification. The operation method is complicated and the treatment results are different. In recent years, despite the uneven medical standards in various places, the overall level of treatment has made great progress, and the treatment methods are basically in line with the developed countries. Such operations are generally available in many large and medium-sized cities in China. basic knowledge The proportion of illness: 0.004% Susceptible population: newborn Mode of infection: non-infectious Complications: urethritis, urinary tract infection

Cause

Causes of congenital anorectal malformation

(1) Causes of the disease

The occurrence of anorectal malformation is the result of impaired embryonic development. The cause of anorectal developmental disorder is still unclear. In recent years, many scholars believe that it is related to genetic factors. According to the literature, anorectal malformation has a family history of less than 1%. Among the 350 cases of anorectal malformations reported by Saeki Shouyang, 3 family siblings suffer from the same disease. Van Gelder reports that in a family, 4 brothers and sisters suffer from the disease, the first child is an anal stenosis, and the second child is Rectal urethral fistula, both daughters are rectal vaginal fistula, Yano Bodo and other collections of 34 of the 38 cases of family disease, 19 groups with genetics, 16 groups of autosomal recessive or dominant inheritance, 3 groups of companion Sexual recessive inheritance, in which 13 pairs of twins and third-childs accounted for 1/3, and some people think that the possibility of this malformation in siblings of anorectal malformation is 25%.

Gu Chuanmins investigation of the cause of congenital anorectal malformation in pigs also proved that it is genetically related. In 122 cases of anorectal malformation of pigs, 28 cases (23%) were due to boars; 6 cases due to sows ( 4.9%); due to boars, the incidence of both sows was 28 (32%); the unexplained 60 (49.2%), so 62 cases of boars, sows and parents, accounted for 50.8 %, the scholar believes that there may be recessive genetic factors in pigs with anorectal malformation. In 1992, some people studied the genes of the family's disease, and found that the anorectal malformation is related to the HLA located on the short arm of chromosome 6. The malformed pathogenic gene is located near the HLA gene. Zhang Zhibo found that the anesthesia of the anorectal malformation was abnormal in the second exon conserved region, while the HoxD-13 second exon PCR-PstI restriction The endonuclease was not completely digested or incompletely digested, suggesting that HoxA-13, HoxD-13 gene abnormality may be one of the causative genes of anorectal malformation. Some people found an anorectal malformation in the SD rat gene mutation in normal house mice. Rat, said mouse is SD mouse, SD gene is semi-dominant Genetics, affect the development rectum, urinary and reproductive systems, axial skeleton system, and later, it was with heterozygous SD rats bred mice anorectal malformation, Aberdeen, SD explanation is closely related gene with anorectal malformation.

The occurrence of anorectal malformation is similar to the occurrence of other malformations. It may be related to the effects of viral infection, chemical substances, environment and nutrition during pregnancy, especially in early pregnancy (4-12 weeks). The earlier the location of the deformity is, the more complicated it is.

In 1974, Schwetz et al. used Spraque-Dawleg female rats to inhale different doses of chloroform during pregnancy (6-15 days gestation) to observe the teratogenic condition of the rats. The inhalation volume fraction was 100×10-6 chloroform for 7 hours. In addition to tail or short tail, subcutaneous edema, lack of ribs and delayed calcification of the sternum, some rats developed anorectal malformations.

In 1990, Hirai et al used ethylene thiourea to produce an anorectal malformed fetus in the 11-day-old Wistar rats, and the malformation type was very similar to that of humans; Feng Xiaolan and Liu Ying of China also used this method to make an animal model of anorectal malformation. Explain that teratogenic substances - ethylene thiourea is the direct cause of anorectal malformation in some embryonic animals.

Hashimoto's intraperitoneal injection of all-trans retinoic acid in pregnant female rats also caused anorectal malformations in the rats, with the highest malformation.

The above experimental results suggest that the application of teratogenic substances can not only produce anorectal malformation embryos, the incidence of malformation is as high as 30% to 90%, and also shows good prospects for the production of other various malformed animal models.

Prevention

Congenital anorectal malformation prevention

There is no effective preventive measure for this disease. Early detection and early diagnosis are the key to the prevention and treatment of this disease.

Complication

Congenital anorectal malformation complications Complications urethritis urinary tract infection

1. When the urinary tract infection is high or the levator ani muscle is deformed with urinary sputum, the urinary tract infection, penile inflammation and upper urinary tract infection may occur repeatedly.

2. Urinary incontinence high or levator ani muscle deformity combined with spinal deformity, if the sacral nerve development is involved, because its branch dominates the bladder and anal sphincter muscles, it may have urinary incontinence.

3. vaginitis rectal vestibule licking female sick child, because feces often flow out from the vestibular vestibule, can cause vaginitis or ascending infection in sick children.

Symptom

Congenital anorectal malformation symptoms Common symptoms Constipation does not have a fetus intestine atresia without anal anus rectal malformation intestinal stenosis connective tissue sputum loose stool rectal urethral fistula

There are many types of congenital anorectal malformations. The clinical symptoms are different, and the symptoms are different. Some children develop acute intestinal obstruction after birth. Some have difficulty in defecation for a long time after birth. Even a few children have not had a long time. Symptoms or symptoms are mild. Most children with anorectal malformation do not have an anus in the normal anus. If the baby does not have a fetus 24 hours after birth, an anorectal malformation should be considered. It should be checked in time, about 3/4 cases, including All innocent anorectal atresia and some of them have sputum, but the fistula is small and can not discharge meconium or only a small amount of meconium can be discharged, such as rectal bladder spasm, urethral fistula, etc., vomiting occurs after feeding, and spit is milk. It also contains bile, which can vomit fecal material in the future. The abdomen gradually expands and the condition becomes more and more serious. If it is not diagnosed and treated, it can die within 6 to 7 days. Another part of the case includes anorectal stenosis and vaginal fistula, vestibular fistula and perineum. If the sputum is thicker, there will be no symptoms of intestinal obstruction for a period of time after birth, but in a few weeks, months, or even years after the occurrence of difficulty in defecation, the note becomes , Abdominal swelling, and sometimes lower abdomen can be touched fecal great, this time to change the existing secondary megacolon.

1. High or levator ani muscle deformity

About 40% of anorectal malformations, boys are more common than girls, whether boys or girls often have fistulas, but because of the thin tube, almost all have intestinal obstruction symptoms, such children in the normal anal position, the skin is slightly depressed, The color is deeper, but there is no anus. When the child is crying or using hard, the depression does not bulge outward, and there is no impact when touched with the finger.

Girls often accompanied by vaginal fistula, more open in the posterior wall of the vagina, such children with genital dysplasia, is naive, due to no sphincter control, feces often flow out of the mouth, easy to cause genital tract infection, after constipation The heavier the gradual formation, the secondary megacolon is formed, which is characterized by abdominal bulging, which can often reach huge fecal masses. The child's general condition is not good and there are symptoms of chronic poisoning.

Urinary sputum is almost seen in boys, girls are rare, venting from the urethra and meconium is the main symptom of rectal urinary fistula. When the bladder is paralyzed, the meconium enters the bladder and mixes with urine. The urine in the whole process of urination Green, the last part of the urine is darker, and at the same time it can discharge the gas trapped in the bladder. For example, when the bladder is compressed, the meconium and gas are discharged more. When not urinating, it is controlled by the bladder sphincter, no gas is discharged, and the rectal urethra is paralyzed. Only a small amount of meconium is discharged at the beginning of urination, and is not mixed with urine, and the urine is transparent later, because there is no sphincter control, and the venting from the external urethra has nothing to do with urination.

The above symptoms are important for the diagnosis of urinary fistula, but because the thickness of the fistula is different, or is often blocked by sticky meconium, the degree of occurrence is not the same, or even does not appear at all, so the urine in the urine is routinely examined. Ingredients are necessary. A urine test is negative, and the presence of urinary fistula cannot be excluded. It must be checked several times.

Some cases can be diagnosed according to the gas or liquid level in the bladder of the X-ray film. It has been pointed out that calcification in the intestinal lumen is also the basis for the diagnosis of rectal urinary fistula. In urethral cystography, the contrast agent can only be filled with the mouth. There is a diverticulum-like shadow, and there is little contrast agent into the rectum. When the fistula of the urethral membrane is thick, the catheter is inserted along the posterior wall of the urethra and can enter the rectum through the fistula.

Cases with urinary fistula may not be treated in the neonatal period, recurrent urethritis, penile head inflammation and upper urinary tract infection, and even external hemorrhoids. In addition, these children are more common with spinal deformity, phrenic nerve The development is also affected, and its branches innervate the bladder and anal sphincters, that is, after the malformation surgery, there may be urinary incontinence.

2. Middle position malformation

About 15% of these malformations have been classified as high malformations by some people in the past, while others have classified this into low-level malformations. The blind end of the intestines is near the edge of the urethral sponge or the lower end of the vagina. Around the distal rectum, there is a fistula whose fistula is in the urethral bulb, the lower vagina or the vestibule. The appearance of the anus is similar to the high malformation. It can also be defecate from the urethra or vaginal. The probe can enter the rectum through the fistula. The tip of the probe can be touched by the finger touching the anus.

The rectal vestibule of the girl is more common than the vaginal vault. The pupil is open in the vestibular scapular fossa of the vagina. It is also called the scapular fossa. The pupil is large. The baby can maintain normal bowel movement through the pupil in the early stage. Even the older child can be normal. Defecation, incontinence only in the case of loose stools, such as the rectum of the rectal vestibule is very narrow, its clinical manifestations are similar to the various low-position malformations that open in the vulva, but the probe is inserted into the head, the probe is directed to the cephalic side Walking rather than to the dorsal side, due to frequent fecal outflows during infancy, such as poor care, there are often feces in the vestibule of the vagina, which can cause vaginitis or ascending infection.

Anorectal stenosis is a rare malformation. The stenosis involves the anus and the lower rectum. It may be confused with anal stenosis. The stenosis can confirm the diagnosis.

3. Low or levator ani muscle deformity

About 40% of anorectal malformations, the rectal end position is lower, below the shame line, this type of deformity combined with a fistula, but less complicated with other malformations.

Some clinical manifestations have depressions in the normal anal position, the anal canal is completely occluded by a layer of septum, and the diaphragm is sometimes thin and dark blue. When the child is crying, the diaphragm bulges outward obviously, and when touched with a finger, there is a significant impact. There is obvious contraction of the stimuli. Some of the anal membranes are broken, but not complete. The caliber is only 2~3mm, the bowel movement is difficult, and some anus is normal, but the position is forward, between the normal anus and the scrotum root or the posterior labia. , called the anus before the perineum, generally no clinical symptoms, no treatment.

Some male patients with low anal atresia are accompanied by anal skin fistula, which is filled with meconium, which is dark blue. The fistula is open to the perineum or more to any part of the scrotal suture or the ventral side of the penis. Feces are not easy to see, but if the probe is inserted from the mouth, it will go straight back under the skin.

In the girl, some low-position malformations have an opening near the genital area of the joint of the labia. The appearance is similar to that of the normal anus. It is called the vestibular anus or the genital anus. In the anal vestibule, the intestine has passed through the puborectal muscle, and the end of the anal canal passes. The small fistula is connected to the vestibule. In clinical practice, the fistula is different from the rectal vestibule. The probe inserted into the fistula is slightly moved to the dorsal side instead of the head side, and the probe is easily touched by the finger at the normal anus.

In addition, there are some rare deformities, such as the perineal fissure of the girl, there is a moist epithelial fissure between the anus and the vestibule of the vagina, and the baby has a rare cloaca deformity. The vulva develops in a naive form, and the labia majora is small and thin. An opening from which the urine is discharged.

Examine

Examination of congenital anorectal malformation

X-ray inspection

In 1930, Wangensteen and Rice designed inverted photogrammetry to diagnose anorectal malformation, which is still widely used today. The procedure is to place the sick child at the head low for 5-10 minutes after 12 hours of birth, and gently massage the abdomen by hand. The gas enters the rectum sufficiently, and a metal mark is fixed on the skin corresponding to the position of the normal anal point in the perineum, or a small amount of tincture is used as a mark, and the legs of the sick child are inverted for 1 to 2 minutes, and the X-ray center is perpendicular to the film, X-ray. The distance between the tube and the child is 2 meters, the hips are close to the flexion position (70°90°), the injection point is the pubic symphysis, the exposure is in the inhalation of the sick child, the lateral position and the anterior and posterior position are taken, the pelvic gas shadow is The distance between the metal marks represents the height of the distal end of the rectum. On the lateral slice, a line from the midpoint of the pubic bone to the junction of the appendix is a line of shame (PC line), and then the parallel line between the ischial and the sacral line is I line, the pelvic gas shadow is higher than the PC line for the high position deformity, just between the PC line and the I line is the middle position deformity, lower than the I line for the low position deformity, which is important for determining the treatment measures, the choice of surgery significance.

After Wang Changlin fixed 50 pelvic corpses by 10% formalin solution, the anatomical view of the puborectalis muscle was observed. The lowest point of the lower edge was parallel to the PC line, which was SS' and LL', respectively. On the line photograph, the lowest point of the puborectal muscle and the lower edge of the lower edge are suitable for the boundary line of high, medium and low malformation. The observation results, the SS' line and the LL' line are respectively from the anus (1.75±0.3) cm and (1.04). ±0.27)cm, in order to facilitate clinical use, it is proposed that the rectal blind end gas shadow is 1.8cm and 1.1cm from the anal point, that is, the blind end is 1.8cm or more from the anal point, and the lower position is 1.1cm or lower. The position between 1.1 and 1.8 cm is a median malformation. The positional relationship between this standard and the PC line, M line and I line is basically the same. At the same time, 45 cases of anorectal malformation are determined by this method. 41 cases are correctly determined, and the rectum is blind. The position of the end and the anatomy of the puborectalis muscle were consistent with the preoperative judgment of the method; 4 cases were judged to be mistaken, mainly due to improper conditions of the projection, 1 case of premature infants with pneumonia, scleredema, 24h filming, There is very little colonic gas, which leads to misjudgment.

It is worth noting that the inverted side X-ray film sometimes encounters the following conditions can cause errors: 1 check too early (within 12 hours after birth), intestinal gas has not yet filled to reach the end of the rectum; 2 when the examination is less than the inverted time of the child 1 to 2 min; 3X line injection angle is not suitable and exposed when the sick child exhales.

When observing the X-ray inverted flat film, observe whether the appendix bone is deformed, recurved, fused, half-vertebral and absent, and observe the gas or liquid level in the bladder or calcification in the intestinal lumen. The fetal shadow, the blind end of the rectum is a reliable method for the diagnosis of urinary fistula, and it is found that this change is feasible retrograde urethral cystography. At this time, it can be seen that the contrast agent is filled with the fistula or enters the rectum, which is important for the diagnosis. Value, for patients with colostomy, intestine or sacral angiography, you can understand the length of the fistula, the direction of the sacral approach and the level of the end of the rectum.

2.B-ultrasound

Ultrasonic tomography scanner is used. The frequency of the mechanical sector scanning probe is 3~5MHz. The child does not need special preparation for the examination. The flat-cut stone position is taken. The probe contacts the perineal skin at the anal point of the sick child. The sagittal section scan can be used to obtain the anorectal rectum. In the area of the sonogram, the skin of the perineum is thin and strong echo; the sacral vertebral body often shows a beaded arrangement with a strong echo accompanied by a posterior sound shadow. The first atlas is wider and tilts forward and downward, forming the initial part of the distortion. It is easy to identify; the echo of the tubular structure with a diameter of about 1cm is generally seen in front of the atlas. The lumen is mostly an anechoic area, and there is a strong echo of the bubble in the middle; the bladder is filled in the upper part of the rectum, and the bladder wall is thin and strong. Echo, the interior is an echo-free zone, and the echo is enhanced behind the bladder.

If the echo of the perineal skin is unclear during the examination, a water sac can be added to the surface of the skin to increase the clarity of the interface between the water sac and the skin. If there is a sputum, the vestibular sputum can be inserted into the catheter through the external orifice of the fistula to inject saline 20~ 40ml, the best to take the head high and low 30 ° position, so that the rectal blind end is fully filled, to prevent water spillage, scan according to the above method, not only can show the distance between the rectal blind end and the anus skin, but also can observe the fistula direction and length, rectal bladder In the latter, the strong echo or strong echo spot of swimming is seen in the bladder, and the spot is obviously increased when the lower abdomen is pressed.

The normal rectum passes through the pelvic levator ani muscle and the external sphincter to the anus before the iliac crest. The rectal blind end formed by the rectal atresia is often separated from the perineal skin by soft tissue. The soft tissue at the pelvic floor often shows heterogeneity in the ultrasound examination. Strong echo, the rectal blind end is filled with meconium, and the blind tube-shaped hypoechoic is displayed in the ultrasound examination, but it should be noted that the thick meconium in the blind cavity may cause differences in echo, and the thin meconium is hypoechoic or approximately echoless, such as When there is gas in the blind cavity, there is a strong echo zone. When the body position changes, the strong echo position often moves. When the child's crying and intra-abdominal pressure changes, the blind end of the lumen swings up and down with the breath. At this time, the rectal blind should be treated. Stop the frame when the end image is moved to the closest position to the skin, or change the direction of the probe to scan the coronal section and stop the frame to measure the shortest distance from the skin of the anus.

The whole school model applied B-ultrasound examination of 15 cases of anorectal malformation. Except for 1 case, the difference between the operation and the B-ultrasound was 1.0cm, the other 14 cases were within 0.3cm. In addition, Wang Chuwen also reported the use of B-ultrasound to detect anal atresia combined with rectal fistula. 16 cases, including 8 cases of vestibular fistula, 5 cases of perineal fistula, 3 cases of bladder spasm and urethra fistula, 15 cases of rectal blind end and perianal skin distance measurement and surgical test results, only 1 case error > 5mm.

3. CT examination

The anal sphincter muscles include the internal and external sphincters and the puborectalis muscle. The degree of formation and development is the most important factor in determining the prognosis of children with anorectal malformation. The relationship between the rectal blind end and the puborectal muscle ring is directly understood by CT. The therapeutic effect of anorectal malformation is extremely important.

The pelvic cavity scan was performed with CT instrument. The pre-existing children were fasted for 4-6 hours, intramuscular injection with thiopental 10-15 mg/kg, or ketamine 3-6 mg/kg, supine position, and both lower extremities were fixed at the examination table. On the upper side, the lower edge of the pubic symphysis is zero, every 5mm is a plane, the scanning is started from the -5mm position, and the tomographic examination is performed sequentially to the head end. A total of 8 cross-sectional images are obtained, and the surface of the perineal skin is head-to-head. At right angles to the body axis, scan every 5mm. The center of the CT horizontal window is positive 30, width 250, and the lower edge of the pubic symphysis is used as the standard scanning plane. In the scanning limit, the pelvic outlet muscle distribution area is calculated by the computer. The exit triangle is based on the lower edge of the two ischial tuberosity, and the pubic symphysis is the apex. The triangle area is calculated by computer. The ratio of the area of the pelvic outlet muscle distribution to the pelvic outlet triangle area is multiplied by 100%. Compared with normal age, the ratio of normal children to age is basically the same, which is 37.2%±5.7%. There is a significant difference in the ratio of children with different types of anorectal malformations. The distribution of the perianal muscles and the area ratio of children with low and intermediate anal malformations are basically the same. Near normal, and high malformation muscles have a small distribution range and low ratio. Therefore, the application of CT provides reliable morphological data, which is helpful for the design of surgical plans. Lang Shimin et al reported 2 cases without anal CT: 1 case of 2 days baby boy High anorectal atresia, CT examination: in the obturator muscle plane, clearly show that the intestine passes through the muscle ring; in the -5mm plane, the sphincter soft tissue mass is visible in the midline anus, the other is also male, 6 months due to high anorectal atresia CT examination after colostomy: no intestinal lumen in the muscle plane of the obturator, only a soft tissue mass including the posterior urethra and muscle ring behind the pubis; only a linear shape in the plane of the anal area, no sphincter Block shadow, 10 days after surgery, inserting the No. 14 catheter in the intestine, showing a thicker muscle layer around the intestine; after 3 months of re-examination, the child has a good ability to control bowel movements.

4. MRI examination

0.5h/kg 5% chloral hydrate solution 0.5h/kg before the examination, the supine position of the child, in the normal anal point position and the pupil with the cod liver oil pill as a marker, can be sagittal, coronal and cross-sectional scan of the pelvic cavity, each A 5 mm section, sagittal, coronal section was scanned outward and backward from the center of the rectum, and the cross section was scanned upward from the anal marker.

The normal neonatal perianal muscles appear on all MRI sections: the puborectalis muscle is located in front of the appendix in the sagittal plane at the PC line; the coronal plane is located on the distal side of the rectum; the cross section is located in the anterior and posterior posterior rectum, anus The external sphincter is located at the distal end of the rectum in a cross section, and is surrounded by a circular muscle bundle around the anus; in the sagittal, coronal plane is located before or after the anal canal or left and right.

We underwent MRI in 9 children with anorectal malformation, 6 patients aged 1 to 3 days, 3 patients 5 to 6 months, 4 patients with high malformation, 2 patients with median malformation, and 3 patients with low malformation. The results showed: median 1 case of malformation, 3 cases of high malformation with patella straight and/or recurve, 3rd, 4th, 5th vertebral fusion and 5th sacral sinus, 1 case, 5 cases of low, medial malformation puborectalis Well developed, and of the 4 cases of high malformation, only 1 case of this muscle development is better, 2 cases are weakly developed (all confirmed by surgery), the other 1 case is unclear, 5 cases of normal anal external sphincter development, 1 case of high position malformation There is abundant muscle tissue image behind the urethra below the blind end of the rectum, indicating that the external sphincter is well developed. One middle and three high deformities have poor development of the external and external sphincters, and a large amount of adipose tissue is filled between the blind end of the rectum and the anal point. Three patients who had undergone surgery were confirmed.

Innocent anorectal malformation in children with rectal blind end dilatation, filled with meconium, can clearly see its relationship with the PC line and the distance from the anal point, children with sputum or colostomy after the rectum emptiness, in On the MRI, the development of the appendix can be seen on the MRI. The internal sphincter changes can not be seen on the MRI. Because the rectal end of the rectum is filled with meconium, the intestinal wall is thin, and the blind end of the rectum is empty, and the image of the intestinal wall is unclear.

MRI examination of anorectal malformation can observe the changes of muscles around the anus, and can determine the type of deformity and the presence or absence of deformity of the appendix. MRI has no damage to the child. The changes of the perianal muscle group can be observed from three aspects. Compared with CT, it only has a more comprehensive cross section. In order to obtain a clear image, a certain amount of sedative is given before the examination, and the anal point is well marked. If necessary, it is necessary to inject the gas into the rectal blind end through the mouth to make the image clearer.

5. Other inspections

Some people use the method of puncture to determine the height of the rectal end, that is, use a sterile syringe to connect the thick needle, puncture from the center corresponding to the position of the normal anus, and push up while pushing upwards. When the needle tip enters the end of the rectum, there is a fetus. Or gas discharge, the length of the needle penetration represents the distance between the end of the rectum and the skin. After the fetus is sucked out, the contrast agent can also be injected to take a picture to determine the type of deformity and the position of the intestine. This method may be very high for rectal atresia. Invalid and dangerous, so be careful.

Checking the fistula with a probe is also a convenient way to determine the length, width and width of the sinus. For example, by simultaneously touching the tip of the probe with the fingertip at the normal anus position, the distance between the blind end and the skin can be roughly estimated.

For girls with rectal vaginal fistula, the position of the fistula can be directly observed with a nose.

Diagnosis

Diagnosis and diagnosis of congenital anorectal malformation

diagnosis

Congenital rectal anus deformity is the first common digestive tract malformation. It is the most common neonatal surgical disease with a high incidence rate of about 1:2500. The male is slightly higher than the female. The main form is anal atresia, with many forms of lesions and complicated classification. The operation method is complicated and the treatment results are different. In recent years, despite the uneven medical standards in various places, the overall level of treatment has made great progress, and the treatment methods are basically in line with the developed countries. Such operations are generally available in many large and medium-sized cities in China.

Differential diagnosis

Pregnancy mothers with too much amniotic fluid and abnormal fetal movement should be given enough attention. Prenatal ultrasound examination usually can indicate suspicious lesions. Refer to the results of prenatal ultrasound examination and obvious surgical signs after birth, combined with typical symptoms such as no abnormal anus opening, ectopic fetus, vomiting, abdominal distension, abdominal flat, abdominal ultrasound and gastrointestinal angiography Most of the necessary auxiliary inspection methods can be clearly diagnosed.

The manifestations of congenital anorectal malformations vary, mainly in the abnormal position of the fistula and the length and direction of the fistula. The performance of both mouth and fistula is different for men and women. Men are relatively simple, mainly manifested as perineal skin and urethral fistula, while women have perineum, vestibular spasm, scapular fossa and rare vaginal fistula, and even "acupoint anal "A variety of expressions." Usually the diagnosis is easy, no need to carefully identify, but special emphasis on the diagnosis of "one-acupoint anus", the vulva development should be carefully observed before surgery, the external urethra and vaginal opening should be carefully searched, and if necessary, the drug should be calmed and then further examined. Strive to make a clear diagnosis before surgery.

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