Congenital short colon

Introduction to congenital short colon Congenital short colon (congenitalshortcolon), also known as congenital pouchcolon or pouchcolonsyndrome, is a rare intestinal malformation. Simple short colon is rare, and more combined with high anorectal malformation. basic knowledge The proportion of illness: 0.003% Susceptible people: seen in young children Mode of infection: non-infectious complication:

Congenital short colon cause

(1) Causes of the disease

Chadha et al believe that the regional incidence of congenital short colon is different and may be related to race, diet or environmental factors. Because these cases are sporadic, there is no evidence of family inheritance.

Stephens (1983) believes that when the human embryo is 4 to 6 weeks (length 4 to 16 mm), the cloaca is separated by a downwardly growing urinary rectal septum or cloaca. The cloaca is divided into two parts, the anterior part is the urogenital sinus, and the The part is rectum. At the same time, the distal cloaca membrane is also divided into two parts: the anterior part is the urinary genital membrane, the posterior part is the anal membrane, and the human embryo begins to enlarge the anal membrane at the 5th week and gradually deepens. Membrane rupture The rectum communicates with the outside of the body. If the cloaca is not fully grown, it leads to high anorectal malformation: the boy is a rectal urethral fistula, the girl is a rectal cloaca, and the anal membrane does not rupture, an anal atresia occurs, Dickinson (1967) proposed, intrauterine In early development, vascular damage in the posterior intestine is the cause of sacral colon malformation. Bourdelat et al. (1988) studied the supply of arteries in the human rectum and the anorectal region of the fetus. It is suggested that vascular ischemia is the basis of anorectal malformation. They found that the anus The rectal blood vessels are originally derived from the superior rectal artery of the inferior mesenteric artery, which is a terminal artery without an anastomosis, but can fully ensure the development of the hindgut. Chadha et al believe that the vascular damage hypothesis is fully capable. The etiology of a short colonic malformation and its series of anatomical features: vascular damage in the vicinity of the urethral septum that grows downward in the cloaca before and after the fourth week of pregnancy can affect the differentiation of the cloaca; blind endarterial lesions near the hindgut in differentiation Will affect the development of the cloaca; different ranges of mesenteric vascular damage determine the pathological and clinical classification of the congenital short colon.

(two) pathogenesis

Chadha et al. believe that "congenital saccular colon and anorectal hypoplasia" can accurately summarize the pathological features and changes of congenital short colon. Narasimharao et al. will congenital short colon type 4: type I: no normal colon, short colon In the form of a bag, the ileum is directly connected with it; type II: a short cecum between the ileum and the saclike colon; type III: a section between the ileum and the saclike colon (the newborn is 7-8 cm long) with a normal shape; Type IV: The colon is close to normal, but the distal end (rectal and sigmoid colon) is in the shape of a bag, type IV is only the distal vascular damage of the inferior mesenteric artery; type III is the proximal vascular lesion of the inferior mesenteric artery; type II is the submucosal The main artery of the artery is damaged. The blood vessels of the short colon are derived from the superior mesenteric artery. In addition to the main injury of the inferior mesenteric artery, the type I has the most distal branch of the superior mesenteric artery. The type I is the most, and the type IV is rare.

Most short colon expansion, hypertrophy in the form of a bag or sac, type I, very like the inverted stomach, occupying most of the abdominal cavity; II, III type, because there is a cecum and ascending colon, so the short colon is mainly located in the left abdominal cavity; Type IV, the short colon is located in the lower abdomen, the ileum, the cecum or a colon is connected to the right upper part of the short colon; the distal end of the short colon is short and thin, which is a hypoplasia rectum, often connected to the bladder, urethra or vagina with a wide fistula. The short colon has no colonic band and colonic pocket; the omentum is also stunted, which is connected with the short colon in the cord; the gastric colon ligament is short and thin, and some short colons have poor peristaltic function, and no peristalsis is seen after decompression. Some short colons have peristaltic function, so defecation can be maintained through the fistula, histological examination, in addition to full-thickness hypertrophy, edema, mucosal inflammation can be changed, ganglion cells in the short colon can be normal, reduced or absent.

Congenital short colon prevention

Pay attention to your diet and pay attention to your usual diet.

Congenital short colon complications Complication

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Congenital short colon symptoms Common symptoms Bloating constipation Colonic vascular dysplasia

Most of the disease occurs after birth, the clinical manifestations are similar to the high anorectal malformation: the anal atresia is trace-like, not full and no impact, the anus development of some sick children is normal, only rectal atresia, no fistula or fistula is smaller, born After the occurrence of low intestinal obstruction symptoms: obvious abdominal distension, abdominal wall tension, shiny, superficial vein dilatation; larger fistula, transurethral or vaginal discharge of feces and gas, although the position of defecation is abnormal, due to smooth bowel movements without abdominal distension, so visit or The onset time is later. If the ganglion cells in the short colon are reduced or absent, there are symptoms of chronic constipation.

Li Zhendong et al reported 10 cases of sick children, 9 cases of short colon with fistula, 4 cases of colonic bladder fistula, 3 cases of colonic vaginal fistula, 2 cases of colonic urethral fistula; Chadha et al reported 32 cases of male sick children, 29 cases of colonic bladder, the other 3 cases of innocent, respectively, II, III, IV type of short colon disease, type II, III, sputum was replaced by short fiber strands attached to the posterior wall of the bladder, type IV was distal to the short colon The blind end is located in the pelvic cavity; of the 9 female patients, 6 have high colonic vaginal fistula, and the other 3 have an anal deformity.

Concurrent malformations: 10 cases of sick children reported by Li Zhendong, 8 cases of Meckel's diverticulum, lack of appendix, short intestine, abdominal wall hernia and congenital heart disease; 41 cases of children reported by Chadha et al., in genitourinary Digestive, skeletal, cardiovascular and other systems have 53 malformations: 14 cases of vesicoureteral reflux, 5 cases of double appendix, unilateral kidney deficiency, hydronephrosis, Meckel's diverticulum and some tibial dysplasia in 4 cases, 3 For example, a point of anal, trace-shaped appendix, intestinal insufficiency, unilateral testicular insufficiency, separation of the vagina, multiple spine vertebral bodies in 2 cases, and other rare deformities.

The incidence of high anorectal malformation with congenital short colon is high. Therefore, after clinical diagnosis of high anorectal malformation, X-ray examination should be performed to determine whether there is a congenital short colon. The characteristic change of X-ray is to diagnose congenital The main basis of the short colon is characterized by a large liquid plane on the flat plain of the erect position, and the width exceeds 50% of the maximum transverse diameter of the abdominal cavity. The pupil of the urethra or vagina has a huge capsular change. Also, do a full inspection of the sick child as much as possible in order to discover other fatal deformities.

Congenital short colon examination

1. Abdominal palpation: It can touch the colonic shape of the colon that expands in a square shape.

2. Digital rectal examination: helpful for diagnosis. In addition to excluding the rectum, anus without congenital atresia and stenosis and other organic lesions. First of all, it means that there is a sense of emptiness in the rectum of the rectum, no large amount of meconium stagnation, and after the fingers are pulled out, there will be a large amount of meconium and a lot of odors. After this explosive excretion, the abdominal distension will improve. .

3. X-ray examination: X-ray is one of the important means to diagnose this disease. The abdomen plain film can be seen that the colon is inflated, and there is a continuous empty columnar translucent area in the periphery of the abdomen. The small intestine also has flatulence, but there is no large liquid level and small intestinal obstruction. Identification. No gas in the rectum ampulla is also an important difference. Some people suggest that colon allergy should be inverted and the lateral side of the abdomen, pelvic film, such as gas can not rise to the rectum, the diagnosis is more reliable.

4. The sputum enema X-ray film for the diagnosis of lesions in the rectum, sigmoid case, the accuracy rate of more than 90%. The lesion showed continuous stenosis of the rectum, which was funnel-shaped and connected with the dilated bowel segment. The dynamic image showed strong and regular colonic peristalsis. After the sputum was thickened due to thickening of the intestinal wall and mucous membrane, there were obvious wrinkles in the intestinal lumen, similar to normal. The jejunal folds are known as the so-called "colon jejunalization" changes. Most children can not be drained in time, and observing sputum function is an important diagnosis of neonatal megacolon. In the case of colitis, the outline of the proximal dilated colon is blurred on the X-ray, the shape is stiff, and most irregular burrs protrude.

5. Rectal biopsy: In theory, rectal biopsy is the most reliable diagnosis of this disease. However, due to the narrow anus of the newborn, the tissue should be cut more than 4.Ocm from the anal margin, and the depth should reach the full thickness of the rectum, so the operation is difficult. In addition, the rectal ganglion cells of the anal canal are sparse, and the ganglion cells in the internal sphincter are absent, and the location of the cut tissue is low, which is easily misdiagnosed. In addition, neonates, especially premature babies, have particularly small ganglion cells and poor nuclear display, which must be diagnosed by pathologists with extensive experience. Therefore, it is not necessary to do this check except for a few cases.

6. Colonic allergy internal pressure measurement: due to lack of rectal stenosis caused by rectal dilatation caused by anal sphincter relaxation, but also lack of anorectal reflex, so when the balloon is inflated, stimulate the rectal wall after the anal canal pressure does not drop , can be suspected of congenital megacolon. Therefore, in recent years, it has been advocated to use the method of rectal internal pressure measurement as a method for diagnosing congenital megacolon.

Diagnosis and diagnosis of congenital short colon

X-ray examination is the main basis for the diagnosis of this disease. The inferior lateral X-ray film of the children with congenital short colon disease is a change of high anorectal malformation, abdominal X-ray film, except for intestinal dilatation, gas accumulation and liquid level. In addition, the characteristic change is a significantly expanded capsular colon image. Singh et al. (1972) concluded that the large liquid plane width of the erect abdominal plain film is more diagnostic than the 50% of the maximum transverse diameter of the abdominal cavity, Chadha et al. Of the 41 cases, 27 (66%) had this characteristic change, of which 24 were type I and type II, 3 were type III; 10 (24%) had gas shadow in the bladder, and 6 cases were I Type II female sick children, because of the large genitourinary tract, without the above-mentioned typical X-ray changes, there are still 2 cases of type I disease, due to short colon perforation and pneumoperitoneum.

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