07:36,6,Aug,2010 | (1595/0/0) | Original

Congenital anorectal malformations


Congenital anorectal malformations performance and how to diagnose what?
Congenital anorectal malformations are many kinds of different clinical symptoms, time of onset of symptoms is different, some children with acute intestinal symptoms after students, some students appeared long after defecation difficulties, or even a small number of children with prolonged absence symptoms or mild symptoms, most children with anorectal malformation, the location is not in the normal anal anal, 24h after birth, meconium can not be excluded, it should be thought of anorectal malformations, should be checked in time, about 3 / 4 of the cases, including All anorectal atresia without fistula, and although part of the fistula, but a small fistula can not be discharged or can only discharge a small amount of meconium meconium persons, such as the rectum, bladder fistula, urinary fistula, vomiting after feeding, spit things as milk, and contains bile, fecal matter can later be spit, the gradual expansion of the abdomen, the condition becoming more serious, if not diagnosed and treated, 6 to 7 days to death, the other cases, including anorectal stenosis and fistula, vestibular fistula and perineal fistula fistula and coarser, in a period of time after birth, symptoms of intestinal obstruction does not appear, and in a few weeks, months, even years after defecation difficulties, note tapering, abdominal swelling, and sometimes the lower abdomen to reach a huge manure block, this time, changes in secondary megacolon.
1. High on the levator ani muscle abnormalities or anorectal malformations accounted for about 40% more common in boys than girls, both boys and girls tend to have the fistula exists, but smaller fistulas is almost always intestinal obstruction symptoms, such patients children in the normal position of the anus is slightly sunken skin color darker, but no anus, children crying or exert oneself, the depression is not bulging outward, with the touch of a finger where there is no impact on flu.
Girls are often associated with vaginal fistula, and more openings in the posterior vaginal fornix Department, these children with hypoplastic external genitalia, was naive, because there was no sphincter control, waste often flow from the fistula, can lead to reproductive tract infections, constipation and more later and more weight, and gradually form a secondary megacolon, with abdominal distension, fecal matter can often reach a huge, poor general condition in children, chronic symptoms of poisoning.
Found in almost all urinary fistula boys, girls rare, the exhaust from the urethra, and meconium in the rectum of the main symptoms of urinary fistula, bladder fistula due to mixture of meconium and urine into the bladder, in children with urinary voiding the entire process was green, the last part of the urine color depth, while retention in the bladder discharge gas, such as the oppression of meconium and gas from the bladder is more, without voiding
, due to the bladder sphincter control, non-gas emissions, when the rectal urethral fistula , only a small amount of urine excreted meconium beginning, not mixed with urine, and urine after it is transparent, because there is no sphincter control, the exhaust from outside the urethra and bladder movement has nothing to do.
The symptoms of the diagnosis of urinary fistula is important, but because of the different thickness of the fistula, or thick meconium are often blocked, there's not the same level, or is not there, so the urine routine examination of children without meconium component is necessary, a urine tests negative, except for urinary fistula can not exist, must be repeatedly checked.
Some cases, according to the X-ray gas or liquid within the bladder surface and confirmed, it was noted that the intestine with calcification of diagnosing urinary rectal fistula according to the urethra when the bladder imaging, contrast agents can only be filled with fistula are often the Ministry of diverticulum-like shadow appears, and the contrast agent into the rectum very few of the fistula in the membranous urethra thicker, the catheter can be inserted along the posterior wall of the urethra into the rectum through the fistula.
Associated with cases of urinary fistula in the neonatal period without received treatment, can be recurrent urethritis, penis head inflammation and upper urinary tract infection, or even outside the fistula, the other, the combined spinal deformity in children is more common, sacral nerve The development is also affected, and its branches to the urinary bladder and anal sphincter, the correction of deformity or the line after the surgery may also have the phenomenon of urinary incontinence.
2. The middle bit deformed about 15%, these abnormalities were classified as high in the past by some deformity, while others are classified as low this malformation, the blind end of the rectum without fistula were the ball in the urinary tract or vaginal sponge, the bottom edge near the muscle , surrounding the rectum puborectalis distal fistula with fistula are the Ministry of urethral opening in the vagina or vestibule lower segment, the anal area with high malformations similar to the appearance, but also from the urethra or vagina defecation, the probe through the fistula Road into the rectum, anus with your fingers touch the top of the probe can be touched.
Vestibular fistula rectal girls more common than vaginal fistula, fistula openings in the vestibule of the vagina scaphoid fossa, scaphoid fossa, also known as fistula, fistula hole big hole early infancy through the fistula to maintain normal bowel movements, or even older children can be normal defecation, loose stools when only incontinence phenomena, such as vestibular fistula rectal fistula is very narrow, its clinical manifestations and the various openings in the lower genital abnormalities similar to, but through the fistula into the probe, the probe to the head Traveling to the dorsal rather than the baby out of the feces due to frequent, such as nursing ill, often in the vaginal vestibule feces, can cause vaginitis, or ascending infection.
Stenosis is a rare anorectal malformation, stenosis involving the anus and lower rectum that may be confused with anal stenosis, fistula angiography can confirm the diagnosis.
3. Low or the levator ani muscle abnormalities account for about 40% of anorectal malformation, the lower rectum end position, end of line in shame, such a fistula associated with multiple malformations, but less complicated by other malformations.
Some clinical manifestations of depression in the normal locations of the anus, anal canal completely occluded by a layer of membrane, membrane sometimes thin, dark blue, children crying membrane was bulging outward, with your fingers clear when the impact of sense of touch, stimulate significant contraction, although some anal film break, but not exclusively, its diameter is only 2 ~ 3mm, defecation difficulties, some anal normal, but the location front, in the normal anus and the scrotum or the labia after the United root between anus called the perineum before, usually without any clinical symptoms, no treatment.
Some men, accompanied by low anal atresia anal skin fistula, which is full of meconium, the dark blue, fistula opening in the perineum to the scrotum or more before the number of stitches or any part of the ventral penis, concealed in the girl child manure is not easy to see, but if from the fistula into the probe, then go back directly to the line next to the skin.
In girls, some low deformity near a joint at the labia vulva with an open, similar in appearance to normal anus, anus or genitals, said vestibular anus, vestibular fistula in anus, bowel has passed puborectalis, anal canal by the end of interlinked with the vestibule of a small fistula, such clinical vestibular fistula and rectal fistula The difference, as the mouth of the probe into the fistula rather than go slightly dorsal cephalic, with a normal anus and easy finger touch probe head.
Also, there are rare deformities, such as the girl's perineum fissures, anal and vaginal vestibule in between the epithelium with a moist fissures, baby still rare cloacal malformation, its external Yinfa Yu was naive, skinny labia majora, only see an opening, urine will since discharge port.
The diagnosis of congenital anorectal malformations in clinical general is not difficult, but more important is accurate determination of the height of rectal atresia, rectal end of the relationship with the puborectalis and the availability of urinary fistula and the presence of spinal deformity, in order to more reasonable treatment measures, this should be based on clinical symptoms and signs the necessary examinations.
Congenital anorectal malformations

The names of diseases (English) congenital anus-rectum malformation Pinyin XIANTIANXINGGANGMENZHICHANGJIXING Classification of Diseases code alias Western congenital disease name Western medicine defines disease classification codes congenital anorectal malformation is a normal embryonic development of the disorder in the results. Is the most common digestive tract abnormalities, and often with multiple deformities. Traditional Chinese Medicine Explained Western cause cause seasonal population with men slightly more than women. High malformations in the male 50% female 19%. The incidence of a variety of fistula was 90% in women, men 72%. Intensity and spread of the disease incidence rate of 1:50 O-5000, associated with other congenital malformations of the occurrence rate of about 30% -50%. Most associated with urinary tract malformations (25%), followed by spinal deformity (15%), cardiovascular malformations (10%), esophageal atresia (8%). Then malrotation, omphalocele, Down's syndrome, neural tube defects and so on. When embryonic pathogenesis 4mm (4 weeks), after intestinal phase expansion and the formation of allantoic cloaca, the cloacal membrane separated coverage and in vitro embryo 16mm (6 weeks), the cloaca separated ventral urogenital sinus as, the formation of dorsal rectum. The separation process of the Chinese and mesoderm tissue to the caudal fold and the direction of growth Tourneux interstitial direction from the wall to the inside of the proliferation of Rothke fold, blending synthetic urogenital septum. If separation occurs in problems of rectal urethral fistula (male) and rectovaginal fistula or cloaca malformation (female). Cloacal membrane was also divided into anterior and posterior urogenital sinus membrane rectal membranes. Constitute the original perineum. Embryonic 19-30mm (7-8 weeks), the anal membrane rupture, the original communication between the anal canal and rectum. The formation of reproductive organs and perineum simultaneously with the above process. After the transfer process if the barriers and anal dysplasia occurred perineal bulbar urethral fistula in the rectum, anus skin fistula (male) and the vestibular fistula or anal skin, anal fistula (women) and other deformities. TCM pathological classification of anorectal malformations range in 1970 to develop an international classification method to the terminal rectum and levator ani muscles, especially with the lowest level of the puborectalis relationship as the standard. Rectum ends above the levator ani muscle in the levator ani muscle is known on the type or high type; terminal rectum at the level of the levator ani, puborectalis muscle was surrounded by an intermediate position type; terminal rectum through the puborectalis muscle has been located in the pelvic floor muscle by the levator ani muscles below the lower type or low type. This method is more comprehensive way to accurately guide the choice of surgery and prognosis, but in clinical practice, are classified generally too complex and difficult to categorize.
In view of this, revisit the 1984 International Conference, is still the basis of international classification, for a more streamlined program. First, by gender into male and female groups, the end of each line is still shame "PC" demarcation, divided into high, medium and low three types, according to the type of anatomical abnormalities in a number of different subtypes of another column. Female cloacal malformation (previously known as cloacal fistula rectal) as anatomic variations and more complex malformations, it is to the independent group, according to the cloaca, "length" is divided into high, medium and low.
Male:
1. High (1) anorectal agenesis: rectal prostatic urethral fistula: fistula opening in the urethra. No anal sphincter, external sphincter is not obvious, urine or meconium gas, blind-side in the PC line (end line of Shame: the lateral pelvic X-ray films, from the pubis to the sacrococcygeal bone body drawn between the midpoint of a line) on; no fistula: between the blind-side and the urethra can be a fiber cable with connectors. No anal sphincter, only traces of the external sphincter. Dead-end PC line level or above. (2) Rectal atresia: blind-side stop at different heights rectum. Anus and anal canal normal, anus, external sphincter and levator ani muscles, and to maintain normal relations with the anal canal.
2. The middle bit (1) Department of rectal urethral fistula: rectal blind side bulb in the corpus cavernosum urethra muscle above the puborectalis fistula surrounding the rectum, the blind-side, absence of anal sphincter. Gas and meconium in the urine. Dead-end PC line in the rectum and â…  line (consistent with shame by the end of ischial ridge line parallel to the line) between. (2) anal dysplasia, no fistula: rectal urethral bulb finally blind-side above the cavernous muscle, puborectalis muscle around the rectum blind side. Absence of anal sphincter. Only see traces of the external sphincter. Blind-side in the rectum â…  PC line and between the lines.
3. Low (1) anal skin fistula: Fistula in the anus to the urethral opening line of any part of the middle back to the Ministry of the scrotum up, anal flap was like, slit the skin fistula was meager cover. Puborectalis normal. (2) anal stenosis: the anus and the internal and external sphincter is normal.
Women:
1. High (1) anorectal agenesis: rectovaginal repeated, rectal vaginal wall blind-side openings in the middle of meconium discharge from the vagina; no fistula. (2), rectal atresia.
2. The middle bit (1) vestibular fistula rectal: rectal blind-side or slightly in the PC line, the fistula long 1 ~ 2cm, through the puborectalis muscle, the posterior wall of the vaginal opening along the nest in the vestibule of the vagina. (2), rectovaginal fistula: fistula opening in the hymen at the top, around the rectum, puborectalis blind side and fistula. (3) anal dysplasia, no fistula: vaginal finally dead end of the lower rectum plane. Normal urethra and vagina. Blind-side line in the rectum or under â… .
3. Low (1) Anal vestibular fistula: fistula is very short, closely adjacent to the rectum and vagina. Puborectalis normal, signs of anal sphincter, external anal sphincter is sometimes exist, the Ministry of fistula in the vestibule of the vagina, the mucous membrane around the fistula. (2) anal skin rash. (3) anal stenosis.
4. Cloacal malformation: classification by Raffenspergers law. (1) "long" cloaca with the urethra, vagina and rectum at the top of the joint: a. Absence of the vagina with water. b. Vaginal water. c. Diaphragm. d. Separated with rectal bladder vaginal fistula. (2) cloaca with the anus, rectum and urogenital sinus hypoplasia. (3) The cloaca and rectum with vaginal atresia urethral fistula. (4), rectovaginal connection (common control), the vagina and urethra form the cloaca. (5) "short" cloaca. (6) cloaca with penile urethra. (7) water cloaca with double vagina, rectum and vagina of a connection. (8) cloaca with double vagina, a vaginal obstruction, another free vaginal obstruction. (9) short cloacal segment. Traditional Chinese Medicine diagnostic criteria for pathological diagnosis of physiological and diagnostic criteria for Western Western diagnostic checks based on the perineum can be made after birth found deformities. Occasionally, not for inspection, then the gradual emergence of symptoms of low intestinal obstruction, vomiting and severe abdominal distension. In the absence of fistula, and associated with small fistula cases, often in the early stage after birth, low acute complete obstruction symptoms. If there is a big anorectal stenosis and cases of fistula, stenosis and fistula according to their thickness, can be weeks, months or even years after defecation difficulties, more constipation, fecal stone formation, such as chronic megacolon secondary obstructive symptoms.
Inverted lateral X-ray measurement of the gas in the rectum and the perineal skin (coated barium marker) the distance between the blind side to draw the height of the rectum. According to "Shame tails (PC)" and "sciatic point (â… )" is divided into high, intermediate and low-bit type. Shame the end line (PC) is the center of the greater trochanter to the lateral pelvic line, the midpoint of the body from the pubis to the lower edge of the body 5 sacrum (the lowest of the ossification points) a dotted line, this line and the puborectalis The lateral part of the corresponding joint; sciatic point (â… ) is seen on X-ray film ischial the lowest point of this point and puborectalis fistula surrounding the rectum or the corresponding central portion of the muscle fibers together. Such as blind-side gas in the rectum "PC" line are for the high type, in the "PC" and "â… " intermediate position between point type, in the "â… " below the low point type. In order to clarify the location of fistula, size and direction of the applied probe or fine catheterization, if necessary, can be used for fistula imaging, urethrography, vaginal contrast and so on.
Men, such as the perineum are open, according to its location, size, can be diagnosed as a low type and fistula. If no open or intermediate position, compared with high-type type, microscopic examination of urine or meconium see the mixed keratinocytes were combined that high type fistula rectal prostatic urethra or in the middle bit-type combined rectal fistula urethral bulb. Malformations and fistulas of the common male type of skin and rectal urethral fistula and anal fistula.
Women, the perineum is only one opening, is the cloacal malformation; has two openings (urethra and vagina), can be diagnosed as high-or intermediate-bit-type rectovaginal fistula; there are three openings in the anal fistula before or multi-colored, and may for the middle or low rectum anus vestibular vestibular fistula, anal skin fistula. History of symptoms and signs of disease diagnostic imaging medical diagnostic laboratory diagnosis of power in urine feces blood cerebrospinal fluid immunological histology other diagnostic test certificate in Traditional Chinese Medicine Western differential diagnosis evaluation standard assessment of curative effect, the past record of different standards, making it difficult to make uniform evaluation, are advised to clean according to the degree perineal surgery were divided into clean, fecal contamination, incomplete and complete incontinence incontinence four score, while each of the emptying of the rectum and then record whether the feces were saved, and the need to be made for treatment. During surgery, deformity or other complications to be found in separate records, which may be the main factors affect the score. Complications of the treatment outcome of anorectal malformations Western treatment methods and timing should be based on the type and combination of different circumstances in which the fistula, high and intermediate position for colostomy first, wait until 6-10 months further the purposes of Anoplasty its way to the perineum via the sacral by abdominal sacral perineal, or abdominal perineal and so on, out of the rectum must puborectalis ring. Vestibular fistula such as rectal fistula hole larger, can first dilation effect 6 months after angioplasty. For low-type anal fistula after resection of the skin, anus and anal cut diaphragm for angioplasty, anal stricture dilation and so on.
Chinese Acupuncture and Chinese Medicine Integrative Medicine Massage preventive care and rehabilitation of historical research
Congenital anorectal malformations (anal atresia) New Progress in diagnosis and treatment
Congenital anorectal malformations in children accounted for first in the digestive tract abnormalities, in children with high imperforate anus after defecation most have varying degrees of dysfunction. In addition to children with their muscles, nerves on the development of good and bad, but mainly related to surgical methods and surgical techniques.
In order to get the best free anal bowel function in children, to avoid the occurrence of anal incontinence, Union Hospital, Huazhong University of Science and Technology Pediatric Surgery doctors staff the pathogenesis of the disease, clinical classification, the choice of surgical approach and treatment of in-depth research, active use of the world's most advanced scientific research, so that the effect of the treatment of the disease in the domestic leading level. In the diagnosis, we took the lead in the domestic part of the normal routine application of MRI for children and every case, children with high anorectal comprehensive assessment, the establishment of a system of normal children of normal rectal MRI anal sphincter morphology and objective criteria; use of MRI accurately display the anus level and possible closure of the fistula, the correct diagnosis rates were 100% and 97%, avoiding the traditional inspection methods rely only on X-ray and inverted bit error caused by B-ultrasound; and from different angles, visual display of quantitative anal sphincter development indicators and the development of the sacral circumstances reasonable for doctors to design and create the correct surgical procedure program provides reliable protection from: Medical Education Network www.med66.com. In terms of treatment, according to the clinical classification of MRI for different surgical options. The median of Pena anal surgery used routinely (after sagittal approach), on the high side from the anal free anal blind nest is less than 3.5-4.0cm (without fistula 3.5 cm, a fistula 4.0cm) by Pena surgery, more than Laparoscopic surgery uses this distance ,95-98% of the patients were using an operation, make full use of electrical stimulation and fiber surgical technology, not only to avoid the huge abdominal trauma, but also ensures accurate through the rectal sphincter new center to achieve the best bowel function. Source: Medical Education Network
Laparoscopic treatment of high anal atresia advantages:
(1) of the children with little damage and quick recovery.
(2) can handle the urethra or rectum, rectovaginal fistula.
(3) laparoscopic pelvic muscle tissue for the observation of the state provides a new way, as a normal rectum clearly visible through the path, reducing the tissue damage of the pelvic floor.
(4) postoperative sphincter around the anus in a new powerful, symmetrical contraction in order to prevent fecal incontinence has provided a guarantee.
Figure 15-year-old normal girls cross-sectional structure of anorectal and perineal body phased-array MRI T1WI STIR images (PC plane). Puborectalis muscle development, PRWR = 1.28/2.8 = 0.46. [Medical Education Network finishing pm]
Figure 25 month high anorectal boy, axial T1WI STIR images, PC plane no rectal cavity structure, puborectalis dysplasia, PRWR = 0.3/2.1 = 0.14.
Figure 39-month boy, high imperforate anus (4.8cm) associated with urethral fistula rectal fistula transverse, sagittal FSE T2WI, Landrace arrow to the urethra, the short white arrow at the fistula into the urethra, the black arrows for the puborectalis , BL for the bladder. Closed position can accurately display the level, there is the development of fistula and the sphincter situation.
Figure 41-year-old girl, Currarino syndrome, sagittal FSE T2WI images, low imperforate anus with rectovaginal fistula (white arrow), presacral cystic mass (black arrow), absence of sacral vertebrae below 5. Can display the associated with anal atresia malformations.
The treatment of congenital anorectal malformations [Medical Education Network finishing pm]
A, Pena surgery (in children with high imperforate anus)
Second, laparoscopic-assisted surgery (children with high imperforate anus)
Section surgical indications and contraindications
[Indications]
1, high anorectal malformation, the blind-side distance from the anal fossa is less than 3.5 (no fistula) or 4.0cm (a fistula 4).
2, a complex cloacal malformation
[Contraindications] low or median anorectal malformations
[Prep]
1. Fasting, gastrointestinal decompression line set nasogastric tube to prevent vomiting and aspiration.
2. Preoperative rehydration to correct dehydration and electrolyte imbalance, antibiotics.
3. Who have fistula, fistula can be used for preoperative imaging and cleaning enema.
4. Line MRI examination, to understand the situation and the development of fistula and the sphincter relative to neighboring organs.
5. A high degree of abdominal distention in children, first transverse colon or sigmoid colostomy, in March after the surgery.
6. Colostomy patients have been OK to line both sides of the intestine through the stoma imaging.
Surgical methods and procedures II
1, take the first low-lying supine, place the catheter, disinfect the skin covering the abdomen, perineum and lower limbs.
2, the puncture hole location choice: the center line between the navel and the xiphoid, umbilical 1-2cm away from the place the first 5mmTrocar, and then set the two sides of abdominal 5mmTrocar, sometimes need to be placed in the left lower abdomen, the fourth Trocar easy to operate.
3, no line of neonatal colostomy, rectal significant expansion of the blind-side, you can puncture the intestine through the abdominal wall decompression, or urethra, vagina through the fistula to the rectum and colon hydrotherapy tube decompression.
4, rectum and sigmoid colon laparoscopic incision line break at the peritoneal membrane, ultrasonic knife or electric knife cautery heartbroken piping from the film, close to the mesangial ligation, on the breaking of the rectum and sigmoid colon arteriovenous arteriovenous; bring the rectum, close to the separation of the distal rectal wall, when the rectal tapering into a cone, you can show to the urethra or vaginal fistula.
5, close to the urethra or vaginal wall, cut off the fistula (suture ligation, cut sutures, or titanium clip)
6, the lens from the center of Trocar guide people, look pelvic floor, separating the fatty tissue of the pelvic floor to expose the pubic bone on both sides of the coccyx pelvic floor muscle belly.
7, electrical stimulation to find the center of the external sphincter, the anal sphincter central longitudinal skin incision 1.5cm. Stimulate the muscle at the same time, laparoscopy can clearly see the pelvic floor muscle contraction, identify the center of muscle contraction.
8, from the center of the perineal muscle contraction to the pelvic floor with Trocar puncture, under the surveillance of the laparoscopic pelvic pubococcygeus from the muscle belly between the two center shell. Withdraw the needle core, using 10 mm Trocar introduction, the formation of pelvic floor tunnel
9, the leaders grasping forceps through the tunnel, the rectum (colon) pulled out from the tunnel. Sigmoid colostomy were on the line, distal and proximal fistula bowel motility dysfunction expansion needs to be cut or distal rectal fistula is too short, can be free in the laparoscopic proximal intestine, removal of the stoma, the proximal normal colon pulled out from the center of the pelvic floor muscles. 6-0 absorbable suture to suture the rectum and perineum, the skin phase.
Complications and Prevention Section
1, the laparoscopic pneumoperitoneum caused by high CO2 acidosis, respiratory and circulatory function of potentially dangerous, surgery can be avoided by means of intermittent.
2, alert pelvic infection, urinary tract in dealing with the rectum or vaginal fistula, should be double ligation of the middle cut, fistula thick, use a cutting stapler. Meconium contamination pelvic surgery should be washed promptly.
3, with a comeback due to pelvic separate knife or cautery, often with different degrees of thermal damage to nerve, postoperative urinary retention are often required to place the catheter around 1 week after surgery when free as possible from the bowel and bladder fistula.
4, the perineum is circular surgical anastomosis, often narrow, extended in time after 2 weeks anus.
5, urethral stricture, urethral diverticulum, urethral fistula associated with children of preoperative fistula angiography should be performed to understand the trend of the fistula, the site of ligation of the fistula in the rectum into the top of the vertebral shape 0.5cm. Children with congenital anorectal malformation MRI diagnosis, treatment status and progress of Pediatric Surgery, Union Hospital, Tang Shaotao
Advances in MRI diagnosis
Recent studies indicate that congenital anorectal malformations (ARM Anorectal Malformations) is often accompanied by pelvic floor muscles, sacrococcygeal bone and sacral nerve, the development of the genitourinary system malformations, early embryonic tail dysplasia syndrome. Press Wingspread classification can be divided into high, medium and low malformations, most of the fistula, and urinary tract abnormalities or reproductive tract connected. Children with different types of surgery is not the same effects are different. Simple low anorectal malformation surgery with excellent results, and abnormal high-exist with malformations, such as kidney, heart, spine and spinal cord deformities often affect the fetus or newborn death and the main reason for postoperative effects. Now it has come to realize the pelvic floor striated muscle complex (Striated Muscle Complex, SMC) in the postoperative prognosis is good or bad play a decisive role [1,2,3]. Consequently, the comprehensive and accurate grasp of children with anorectal malformation pathology is the choice of treatment methods to determine the prognosis of the key. Now is the pre-operative imaging assessment of ARM only means of these pathological changes.
1930 Wangensteen and Rice designed the film to diagnose the inverted bit ARM, when urinary leakage occurs when the diagnosis of retrograde contrast has important value. Under both methods can determine the height and rectal fistula blind-side form, but can not observe the state of perianal muscle development. B-type ultrasonic around the anal sphincter can be assessed, the main drawback is that ultrasound of soft tissue resolution is poor, experience by the observer impact, difficult to define the muscle border. CT scan can clearly show the puborectalis and external sphincter anatomical structure, but is limited to a cross section [5,7]. Multiplanar MRI can accurately show the level of ARM atresia, with or without fistula, clearly shows the development of pelvic floor SMC and distal rectal conditions and the relationship between the sacrococcygeal vertebrae, the sacral and other abnormalities of the genitourinary system, clearly the new rectal surgery relationship with the SMC and whether the abnormal tissue between the two, are also found these abnormalities to date can only imaging method. Meanwhile, a clear MRI scan and pathogenesis of ARM during the development of great help, this article on MRI in these areas in recent years, new progress in the research are reviewed.
A, MRI technology
1, MRI systems: Recent literature shows that magnetic resonance imaging examination on the ARM using a different field strength MRI system, a 0.5T, 0.6T, 1.0T, 1.5T of magnetic resonance (MR). General low-field MR cheap, low operating costs, but for a long time, signal to noise ratio (SNR) is low, mostly in the early in the literature. Reported in the literature the recent high-field MR images can provide a better signal to noise ratio, is easy to get thin, high spatial resolution and image quality. So, You Tiaojian institutions should be adopted in high-field MR examination of children with ARM [4,6,7], low field MR of adjustment pixels, matrix and other parameters to be awarded Kekao image [8]. 2, the scan sequence and parameters: According to recent reports and our experience shows that all the pathological changes of ARM need spin-echo (Spin echo, SE) T1 weighted sequence (T1WI), fast spin-echo (Fast spin echo, FSE ) of the weighted image (T2WI) and short time inversion recovery pulse (Short TI inversion recovery, STIR) sequences [9,10]. Traditionally, the main display anatomical structures T1WI, T2WI reflects the pathological information. Nievelstein applications such as FSE T2WI sequences can be particularly improved the detection rate of fistula, showing more abundant level of anal canal anatomy, STIR sequence can seriously inhibit fat signal to display more prominent muscle morphology, muscle hypoplasia especially useful in children [11 , 12].
3, scanning techniques and procedures: no enema before the test, infants and young children within the water supply of oral or rectal chloral hydrate anesthesia (90 ~ 100mg/kg), older children can be given by rectal thiopental (30mg/kg) [13] . Cod liver oil pills marked with anal nest, insert the catheter to drain the bladder, a fistula fistula were from 2-3mm into the plastic tube into the rectum to facilitate imaging, all patients check with the supine position, straighten the legs and buttocks. Slice thickness 3 ~ 5mm, spacing 0 ~ 1.25mm, sweep time is 20 to 30 minutes. Body phased-array coil MRI (newborns, infants with a head coil) can clearly show various aspects of anorectal malformation anomalies, phased array coil magnetic resonance images acquired with high signal to noise ratio, wide field of vision, the image quality [14]. Built-coil MRI is also used for anal sphincter system, but outside the scope of 3cm from the coil will be the rapid decay of signal strength, so that anorectal malformation and other diseases can not be displayed. And will cause varying degrees of discomfort of patients, coupled with a larger diameter coil is not suitable for children and children with congenital anorectal malformations inspection [1,10]. First, the upper edge of the pubic symphysis TIWI down for axial scanning (parallel to the pelvic floor) and then for TIWI and T2WI sagittal scanning requirements can be observed through the catheter and the catheter length of the anal canal to determine the anal canal central to The center for the coronal scan, the last under the sagittal and coronal images (perpendicular to the pelvic floor), from the upper edge of the anal canal and anal canal to the lower edge of the vertical axis for axial T2WI scan. Sagittal scans should also include lumbosacral vertebrae and spinal cord, kidneys and, if anomalies should be coronal and axial scans, which can observe and analyze three-dimensional form of the anal muscles, blocking levels and possible fistula and associated malformations [7].
Second, ARM MRI assessment of embryonic development
The current process of embryonic development of the ARM study is surgery and autopsy, and MRI can provide a good living ARM pathological studies [11]. According to MRI observations, ARM can be divided into the normal location of ectopic abnormal anus and the anus. Formerly known as ectopic anal fistula may be opening in the urethra, bladder or reproductive tract, is due to early embryonic dorsal part of the cloaca and cloacal membrane caused by developmental disorders. Dorsal cloacal membrane or absence of dysplasia, the dorsal part of the cloaca does not exist, resulting in closer to the top after the intestinal opening. Dorsal cloacal opening position depends on the degree of development, severe dysplasia, the hindgut into the urogenital sinus at a high level, mild dysplasia, after opening in the lower intestine or the perineum, therefore, according to the views of embryonic development, ARM fistula combined the most better described as ectopic anus. Do not merge for high anorectal malformation fistula, surgery is often found in the rectum and the urogenital system, blind-side fiber cable between the tape, the fiber cable is with traces of ectopic occlusion. Cloacal malformation is due to the rear and the front side of the cloacal membrane dysplasia, a direct result closer to the top of intestinal opening, and urogenital openings further back, just the middle of the cloaca and cloacal membrane exists, the formation of intestinal, urinary, reproductive Road joint openings. The abnormal normal position, including anal membrane anal atresia and anal stenosis, and now view that the formation of early embryos anorectal 7 weeks after fertilization in the anus anal wall adhesions, epithelial cells form a "peg", causing a temporary rectum and anal canal occlusion, and then cell apoptosis pathway through the anus and then pass the block to form the anus [15]. Therefore, the location is not the normal formation of abnormal urinary rectum, anus and cloacal membrane fusion across barriers, but because of the late embryonic stage occlusion of the anus there is recanalization defects. Based on these findings, according to a new MRI imaging AMR classified as: early and late embryonic developmental defects of embryonic development defects, including defects in early embryonic development abnormal fistula or anal openings patients without fistula (high deformity), late embryonic developmental defects, including anal stenosis, deformities such as anal membrane is not broken (low abnormality) [11].
ARM associated with the assessment of the spine and spinal cord abnormalities, Nievelstein that the spine and spinal cord abnormalities in early embryonic neural tube formation primary interference, and tethered spinal cord of the embryo and differentiation of late degradation caused by interference. Therefore it is not difficult to understand is often clinically abnormal anal opening the spine and spinal cord malformation associated with abnormal, and abnormal position of the normal anal malformation associated tethered cord. The anal opening was rarely seen clinically tethered cord malformation associated anomalies may be specific to the basic form of disorder caused by the activation process occurs [11,16].
Third, ARM Evaluation of preoperative MRI
As MRI technology continues to improve the application of the coil and a high signal to noise ratio, MRI can clearly show visually blind-side and related rectal muscle system, which can accurately determine the extent and type of deformity, making MRI a diagnosis of anorectal malformation can not be replaced tools [13,17], the majority of cases through the T1-weighted image can show abnormalities of the closed level, while T2-weighted images to assess the low deformity more helpful, because the mucous membrane of rectum and anal canal high-strength signal to the sphincter complex and and perineum tissue contrast and better imaging. Blind-side rectal atresia Neonatal meconium is an excellent MRI contrast agent, in the high signal on T1WI (due to the high mucus or lipids), can clearly show the level of ARM's closed. Reported in the literature [7,17], through the cross-sectional MR imaging in the PC plane and I flat panel display latch rectum can determine the level of the blind side. In fact sagittal and coronal imaging can more accurately show the level of locking position, so lock in the position to determine axial image is not so important, and only in the low deformity, it helps to determine the exact location of the anal canal and with sphincter complex relationship. For reference, we recommend using the perineal superficial transverse muscle of the urogenital diaphragm muscle near the dorsal, anal normal muscle in the dorsal, anal and the muscle while at the same level or in the ventral, the existence of the anal canal ectopic before and outside of the sphincter complex.
Formerly known as ectopic anal fistula ARM merge its different forms. But so far, MRI showed the fistula is still not reliable [7,13,17], while the fistula to develop the full display of the surgical procedure and surgical operation is important [18]. Nievelstein application FSE T2-weighted sagittal and transverse imaging, has greatly increased the detection rate of fistula, 25 patients the fistula was 100% [11], but still difficult to accurately show the fistula into the urinary and reproductive system of the entrance, To solve this problem, Taccone and other proposed Colostomy the distal colon into the Vaseline oil (composition and meconium similar) underwent MRI examination before surgery in the reconstruction is expected to identify the existence of the fistula [19].
Studies suggest that in most of the high ARM perianal muscles varying degrees of developmental abnormalities, and puborectalis and external sphincter direct impact on the development status of anal function postoperatively, the preoperative state of muscle development in judging the prognosis is important. Multi-dimensional imaging by MRI can clearly show the anal sphincter [7,13,19], coronal T1-weighted image can be a good indication of the levator ani muscle (puborectalis) and its relationship with the anal sphincter, and transection surface can fully reveal the puborectalis muscle, the development of anal sphincter status. Although the general development of the sphincter complex situation easy to judge, but so far, the normal thickness of these muscles is still a lack of objective criteria. Subjective visual assessment by some scholars [17], and some scholars, the computer will check the combination of vector manometry and MRI study [20]. Vade, etc. by CT examination of the puborectalis normal thickness varies with age, and found puborectalis gradually thickened with age, and to determine the normal infants less than three months, the muscle thickness of not less than 2.5mm [21] . Recently Wuhan Union Hospital Division of Pediatric Surgery and application of high signal to noise ratio magnetic resonance coils, 1.5T MRI in transverse and coronal measurements of two muscles on the relative thickness and width as the muscle development indicators show normal anatomical level T2WI rich, STIR sequence of the levels of muscle morphology becomes more prominent, even in infants and young children limit the muscle can also be legible, when the relative thickness of the puborectalis (PRTR) <0.18, relative thickness of external sphincter (EASTR) <0.15 when the difference as the development of the diagnostic criteria of sphincter .
Fourth, ARM postoperative MRI evaluation
MRI for assessment of persistent postoperative fecal incontinence is important [13,20,21],
First, MRI axial T1WI can clearly show the new down puborectalis muscle and the rectum and anal sphincter position relationship.
Second, when the puborectalis and external anal sphincter dysplasia, even if the new center is located in the rectum, fecal incontinence can also occur. Arnbjornsson other study found that the degree of bowel control sphincter thickness (CT scan) was no statistical difference between the significant correlation [22,23], Da-yong and other measuring the width of the sphincter and the two ischial support for the evaluation of the ratio of distance between the midpoint of the sphincter development index, as ratio of <0.13, 60% had fecal incontinence; and when the index> 0.13, 100% good bowel control, showing sphincter sphincter development index can be used as development of quantitative indicators to determine, according to the thickness of the muscle is the absolute difficult to determine bowel function [24]. In addition, Fukuyoi MRI measurement applications such as the postoperative evaluation of anorectal angle and found good and bad clinical results after reconstruction in patients with anorectal angle between the statistically significant difference [20].
Third, MRI can clearly show the new rectum and puborectalis or anal sphincter associated with the mesenteric fat tissue between, showing the region of focal high signal in T1WI and low signal the existence of the bowel wall and muscle significant signal contrast. In recent years, the INITIATIVE PenÇŽ sagittal Road after angioplasty rectum and anal canal (posterior sagittal anorectoplasty, PSARP) is the middle bit and the high standard of surgical deformities, MRI can PSARP after the new relationship between the rectum and the muscles, the sphincter development was so an accurate assessment of [27]. Tsuji et al [25] 23 patients with MRI examination in children after surgery, I plane is equivalent to the lower edge of striated muscle complex, M plane is equivalent to the upper edge of the complex. All the children in the plane I did not deviate from the striated muscle complex rectal center, and 21% in the M plane is not in children with rectal muscle center. In the I plane for PSARP can be placed under direct vision into the rectum to the new central muscle, and striated muscle complex from the rear and the lower edge of the perineum can stimulate the search for power, but only from the upper edge of the rear look, this could explain why some patients In the M plane deviate from the rectum, so stressed PSARP sure exactly when the incision line to keep in the middle, which is where the main points of the surgery. deSouza application testing PSARP anal and conventional MRI after surgery anal sphincter and internal sphincter development and found that regardless of the lack of surgical patients are part of the two muscles, but after the PSARP can see below the midline muscle scar, visible rectal After the MRI, on the characteristics of children with perianal muscles provide valuable information on the composition [26]. 1987 Lambrecht in animal models found that all ARM rectal fistula at blind-side and were seen there in the proximal sphincter around the fistula, but very different forms, some of the same as a normal sphincter funnel-shaped, most of the internal sphincter distribution of a wide, round or flat dish discoid, clinical blind-side and to keep rectal fistula can get a good bowel function. MRI of fistula use PSARP and retention (fistula-preserving technique, FPT) anal sphincter (internal anal sphincter, IAS) morphology is found, MRI can show the new IAS anal tissue forming the anatomical features, but the muscle forms a very irregular in shape, the direction of thickness was measured 4 asymmetric manometry had a normal resting pressure, most of RAIR was positive. Accept fistula resection, rectum out of the surgical procedures in children with thin and can not be measured smooth muscle, and have lower resting pressure, without RAIR. But the authors also pointed out, IAS-like structure of the MRI whether the shape and size of constipation, fecal incontinence or fecal contamination related needs further study [8]. The latest research suggests that PSARP pathological postoperative constipation is due to blind-side and the rectal wall fistula caused by abnormal endogenous neurons.
V., ARM ARM associated with abnormal MRI images are often associated with other malformations, the rate was 25 ~ 80%, and some very serious defects, a direct impact on the prognosis of children with ARM. Another unique advantage is that MRI can accurately detect these associated malformations, especially in the spinal cord, spinal and genitourinary abnormalities [30]. Currently more reported a spinal dysraphism is associated with a high rate of abnormal [7,26-29], because these malformations and progressive neurodegenerative often related to whether the screening of anorectal malformation with spinal cord, spinal deformity appeared particularly important. 44% of children with anorectal malformation associated with spinal dysraphism, and often associated with spinal deformities. However, such Mosiello found that children with anorectal malformation, even if the normal spine, there are still 21% of children with varying degrees of spinal dysraphism, therefore, stressed on the normal spinal deformity in children should rule out the possibility [29 ]. 30 ~ 40% of children with anorectal malformation combined urinary and reproductive system abnormalities, MRI can accurately show these congenital malformations such as renal agenesis, renal dysplasia, ectopic kidney horseshoe kidney, kidney and megaureter multi-table psychosis. Tethered cord is often because there is no sacral anomaly exists in the low ARM, conventional MRI examination of such conduct can be found tethered spinal cord, combined with dynamic checks can be found in the urethra neurogenic bladder dysfunction [29]. In addition MRI can clearly show the various cloacal malformations such as double vagina, double uterus, MRI can show a more comprehensive rare triad of malformations Currarin all aspects, including the rectum, anal stenosis, sacrococcygeal bone hypoplasia, presacral mass [19 , 28].
VI summarizes the domestic application of MRI examination of children with ARM and 10 years time, but check the slow technological development, only 1 to 2 general hospitals to carry out, involving part of the preoperative diagnosis and associated with the detection of abnormalities [30]. In this application abroad for 15 years of history, inspection equipment and techniques are maturing in all aspects related to ARM, its significance becoming more apparent [21]. Although the MRI scan time longer, children need a sedative, but it was clear the embryonic development and pathogenesis of ARM, preoperative diagnosis, classification and assessment of muscle development, assessment and after surgery assessment of ARM associated with malformations of other imaging can not be replaced. Therefore we suggest that children with both high on the ARM or low deformity should be used as a routine MRI examination assessment. Status and progress of treatment
First, the treatment of stage
The purpose of treatment by surgery, anorectal malformation development of orthopedic surgery can probably be divided into three periods:
One. In the past to 1948 to Rhoads    successful use of abdominoperineal anorectal pull operation as a symbol of this period is the pursuit of surgery intended solely intestinal continuity, create a new bowel opening, it is relatively simple surgical procedure , including the initial task of colostomy and two ways by Stephens and De Vries pulled abdominal perineal anorectal surgery, this time the main problem after surgery is anal incontinence and some mortality;
Second .1953-1982 years, this period began in Stephens published a new surgical procedure, surgical focus in the protection of anorectal sphincter, pelvic floor and sacral nerve plexus, the period of surgery for the abdominal sacral perineal pull anorectal surgery , the main problems after sphincter dysfunction, fecal incontinence and mucosal prolapse;
III. 1982 to date, this period of loss of form for the post-surgical approach Anoplasty (PSARP), pioneered by the Pena and De Vries, the main problem is that after intestinal disorders, chronic constipation and overflow incontinence.
Second, the treatment of choice for surgical treatment of anorectal malformation is generally believed that:
1) high deformity, should be done in the neonatal period transverse colostomy, to be about 6 months abdominal sacral perineal, abdominal perineal sagittal approach rear out of the anus rectum angioplasty.
2) the median rectal urethral fistula or malformation with vaginal fistula in the neonatal period should be transverse colostomy 6 months later Anoplasty sacral perineal, or posterior sagittal anorectal plasty approach.
3) The median malformations without fistula in the neonatal period may Anoplasty perineal sacral line, the surgical injury to the children small, the effect is good.
4) the median rectal malformation malformations and low vestibular fistula and anal stenosis who may be expanded or extended anal fistula to maintain bowel, six underwent perineal type Anoplasty.
5) low malformation without fistula or fistula may have skin that line the perineum during the neonatal period Anoplasty style, function recovered well after surgery, because this type of nerve and the anal sphincter in children with normal development, operation against are very small, better after surgery. With the improved surgical methods and the use of new technologies, and now advocates a multi-anus forming, which can mitigate against children and reduce operation costs, while results and staging after surgery there was no significant difference. For the medium and high malformations and anal atresia complicated fistula, previously advocated colostomy and two anal first forming, there are many reports of such a shape. Adeniran2002 published in the literature, anal atresia with vestibular fistula rectum anus forming a successful one, to avoid the pain of three operations. A high point for the treatment of anal children is still a difficulty, to reduce children with mental and physical suffering. One-time advocate of early completion of the current urethra, vagina and anus angioplasty; for combined vaginal fistula bladder incontinence in children, intraoperative bladder neck and urethra by rebuilding after the children can still get a good urinary control. Children's Hospital, Zhejiang University has congenital anal atresia with rectal navicular fossa improved to an anal fistula angioplasty. With the continuous promotion of laparoscopic techniques, the domestic Wuhan Union Hospital and Beijing Children's Hospital has assisted laparoscopic high anal atresia a Anoplasty reported. Also, because of laparoscopic surgery in the anus forming a wide range of applications, but also improved the quality of Anoplasty. Yamataka professors reported in 2001, laparoscopic surgery during laparoscopic levator ani muscle stimulation can be directly observed under laparoscopic vision to the contraction of the levator ani muscle and to evaluate the function of the levator ani muscle, help to improve bowel out of the precise positioning of the time increase the satisfaction of bowel control after surgery. At the same time he published in 2002 reported in the literature, through clinical validation, laparoscopic ultrasound during the operation out of the intestine to ensure the symmetry of the pelvic floor muscles were wrapped, greatly improving the accuracy of positioning out of the intestine. Lin reported that Hong Kong, China, plastic surgery laparoscopic anorectal anorectal appears more relaxed earlier reflection, the prognosis after the loss of form than the traditional approach to better anorectal plasty. In addition, after Shizhuang approach, there are reports of other surgical approaches, such as high, with a median anorectal malformations in children via the tail Heng Anoplasty incision, using the former technique modified Mollard approach Shi-shaped rectum colon actually blind Bag muscle of intrathecal treatment of high imperforate anus prolapse. In addition to surgical treatment, as well as automatic intermittent treatment of electromagnetic attraction, CO2 laser treatment of anal incompetence, etc., but did not find more support in the literature.
Congenital anal atresia surgical treatment of many. Difficulty is how to improve, the median abnormal return to normal physiological function after surgery. In recent years, it was found malformations of the rectum and anal canal distal circular muscle at the limitations of the intestinal wall thickening, a prototype of the internal sphincter, it is proposed to retain the rectal blind side. For the understanding of the importance of the perianal muscles, the current high, the median abnormal operation principle is the use of electrical stimulation and fiber surgery significantly improved postoperative quality of life.
Third, although the treatment of postoperative complications Anoplasty been greatly improved, as well as laparoscopic assisted, but the problem still exists after surgery, such as intestinal disorders, chronic constipation, such as overflow incontinence, the most most of the quality of life to the number of incontinence. Silva2004 proposed in the literature dealing with fecal incontinence after anal atresia surgical point of view of the new, artificial bowel sphincter and gracilis muscle on behalf of the anal sphincter, and verified by the two methods of clinical effectiveness. Postoperative constipation, Pe.na surgical applications, in recent years without anal incontinence after a significant reduction in the incidence, the second the following sacrum sacrum were rarely well-developed fecal incontinence. Combined phenomenon of constipation without rectal surgery is increasing people's attention, the trend is the lower rectum, the higher the combined incidence of constipation, for intractable constipation with giant rectum and sigmoid colon among those advocating surgery. For the prevention and treatment of postoperative bowel dysfunction, with information presented through the intestinal tract management, adjusting the diet, bowel training, drug therapy, enema, psychological behavioral therapy, biofeedback therapy, surgical treatment to adjust and treatment. Foreign scholars have also emphasized both anorectal malformations in children after surgery for bowel dysfunction psychological and behavioral therapy, and received good results, they believe that psychological and behavioral treatment of anorectal malformations after treatment of bowel dysfunction, an important component Part one.
Incontinence after surgery to assess progress
Traditional assessment of anorectal function of bowel control is by clinical score, divided into excellent, good, bad a few other. But with such a scoring system to study the efficacy of surgery for obvious defects, and even made after different surgical methods there is no comparable surgical treatment, and the traditional scoring system does not take into account the chronic constipation. Because, different scholars may choose a different surgical approach, a different scoring system, the study was mixed, the selected object anal atresia type of surgery is inconsistent, so under these conditions is to compare different surgical unscientific . Holschneider literature published in 2002 proposed scoring system altogether, according to the treatment to compare the efficacy of
surgery. He believed that a patient needs treatment after surgery to divide the control of postoperative anorectal function. Anorectal control functions will be divided into I, II, III three levels, I completely normal level, including indicators of whether fecal pollution, no constipation, spontaneous activity of intestinal motility; II part of the normal level, divided into three types, -A need to control through diet and / or slow release agents to maintain normal bowel function, B-severe constipation, fecal overflow of sewage, need to ease the enema, C-type part of the sphincter dysfunction, fecal contamination, and no chance of constipation; type III incontinence, sphincter function caused complete loss of fecal incontinence, bowel intractable constipation due to insufficient power, often accompanied by urinary incontinence. Through the study found that classification of anorectal function using this method can be more effective more accurate comparison of treatment effects.
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