2008 年 07 月 13 日

2008-07-13 10:13 Hirschsprung Hirschsprung

Hirschsprung’s intestine without ganglion cells, also known as disease. As hirschsprung be described in detail, it is usually called Hull Shipu Long disease (hirschsprung’s-Disease), is due to the distal rectum or colon bowel continuous convulsions, fecal stasis in the proximal colon, which make the intestinal hypertrophy, expansion in children common congenital intestinal malformations.

Hirschsprung disease is a partial or complete colonic obstruction, combined intramural ganglion cells, characterized by absence of a baby common gastrointestinal abnormalities. Understanding and Development of the disease has more than 200 years of history. 1691 Ruysch first report of this disease until later in Berlin until 1886 Hirschspmng pediatric Hirschsprung the General Assembly to be on a detailed description of the system, so the disease was named after him Hirschspmng disease. Megacolon is Mya (1894) advocated use. First proposed in 1901, Tittle and Hirschsprung’s lack of ganglion cells. Tiffin in 1940 so that the ganglion megacolon is a lack of early occurrence of intestinal motility disorder of the results. Discussed in detail in 1964 Ehrenpries Hirschs-prong disease etiology and pathogenesis. 1950, Swenson was the ganglion from the pathological lack of Hirschsprung disease and other types of megacolon distinguished. The disease is characterized by involvement of distal bowel absence of myenteric nerve cells, so that contraction of intestinal spasm produced, narrow, loss of capacity creep. Proximal intestines expansion, the expansion of secondary compensatory hypertrophy. Incidence rate from 2000 to 5000 births in 1 case, after anorectal malformation, malformation of the gastrointestinal tract in neonatal ranks No. 2. According to the Shanghai area (1966 ~ 1975) data, the incidence of gastrointestinal malformations accounted for 24.67% of congenital malformations. The malformation of the gastrointestinal disease accounted for the 4th. Passarge reported 414 cases so far, male to female ratio of 5 to 10:1. And a clear tendency of familial disease, the data indicate that the disease may be polygenic. The past 20 years due to histology, histochemistry, electron microscopy, immunohistochemistry means of research progress. People Hirschsprung pathology, physiology histology, embryonic development, genetic diagnosis and treatment have a lot of progress.

pathology: Hirschsprung basic pathological changes of the distal bowel wall involvement myenteric plexus and submucosal ganglion cells in congenital absence of the nerve plexus, parasympathetic nerve fibers were significantly higher than normal hyperplasia. The pathological changes of tissue anatomy, resulting in occurrence of the physiology involved bowel dysfunction – normal peristalsis disappeared, replaced by spasmodic contraction. For this dysfunction, there are four kinds of explanation:

1. Absence of nerve cells: narrow bowel myenteric plexus (Auerbach plexus) and the submucosal plexus (Meissner plexus) within the ganglion cells absent, the distal nerve plexus is difficult to find. Thickening of nerve fibers, the number increased, neat wavy type. Although sometimes found in individual ganglion cells, form is not normal. Narrow bowel wall proximal colon gradually found in normal nerve plexus, ganglion cells also gradually increased. Lanes are different degrees of mucosal lesions, colonic lamina propria widened and accompanied by lymphocytes, eosinophils, plasma cells and macrophage infiltration, sometimes seen with superficial ulcers.

2. Cholinergic nervous system abnormalities: international study found that lesions of large bowel parasympathetic preganglionic fibers in the intestinal wall thickening growth of abnormal elevation of acetylcholine, which is about more than twice normal, acetylcholine esterase activity was also correspondingly increased, resulting in a large number of cholinergic neurotransmitter in the intestinal smooth muscle cholinergic receptors, intestinal disease caused by a strong sustained contraction convulsion, which no ganglion cells is caused by spastic contraction of the main reasons for intestinal disease .

3. Adrenergic (sympathetic) Exception: Garrett’s study found that submucosal and myenteric fluorescence intensity and distribution of the sympathetic nervous manner, bowel disease and . Sympathetic nerve fibers (postganglionic fibers) decreased thickening, wavy buckling winds, loss of the original network structure.

4. Non-adrenergic non-cholinergic (NANC) exception: recent findings (1987) Gastrointestinal responses of each segment were inhibited by the NO (CO)-mediated, such as 1990, Butt provided of intestinal non-adrenergic non-cholinergic (NANC) nerve excited the evidence after the release of NO, it is still called a peptide), SP (P material), ENK (enkephalin), SOM (somatostatin), GRP (gastrin-releasing peptide), CGRP (calcitonin gene-related peptide) have all disorder, has a different degree of lack of or even disappear. megacolon of NO research in foreign countries has just begun. is to confirm that the main NANC neurotransmitters, relaxation of gastrointestinal reactions mediated by NO. The role of carbon monoxide caused a great concern. It is inherent in the intestinal wall evoked by the produce. as neurotransmitters play an inhibitory effect. has been demonstrated in the absence of ganglion cell paragraphs of carbon monoxide because of lack of muscular plexus synthase (enzyme required to produce carbon monoxide.) scattered within the muscle nerve fibers may afferent nerve endings for the alien. so you can narrow section of bowel that spasms and bowel without ganglion cells produce NO lack of nerve on.

Hirschsprung’s disease can be seen from the gross specimen, distal bowel – generally remote from the anus to the sigmoid colon, small bowel slightly higher than normal, easy to stiffness, lack of peristalsis, the appearance of normal color; proximal colon – including the sigmoid colon and the upper part of the descending colon, transverse colon or even a small number of cases of cecum and ascending colon, abnormal expansion of intestinal can be rough than normal 1 to 3 times the fraudulent claims of up to 10cm or more, slightly pale appearance and color, mucosal edema, or a small ulcer. luminal plot has hard as stone debris. narrow and expansion between, there is a Mobile District, funnel-shaped, ranging from 4.0 ~ 8.0cm long.

Some physicians will expand the proximal bowel segment called secondary expansion. This section is only intestinal expansion, but not the intestinal wall hypertrophy, normal intestinal tissue. ganglion cells appear gradually from the distal to proximal.

the scope of the above pathological intestines can have great variation. ganglion cells in the narrow area without 75% starting from the anus to the distal sigmoid colon. About 80% of cases without ganglion cells is limited to the rectum remote area, known as a short segment area. which such individual cases of pathological intestinal segments shorter than that, only the terminal rectum The 3.0 ~ 4.Ocm, that is part of the internal sphincter, that the short segment area. have the opposite situation, about 20% of cases, ganglion cells in areas without pathology intestines, can be extended to the descending colon, splenic flexure, and even Most of the transverse colon, it is also known as the long segment type Hirschsprung. there are 20% of cases are the complete absence of ganglion cells, called ganglion cells in the colon without disease, very few cases of bowel or even pathological jejunum, ileum and even up to, called the entire colon – ileum (or jejunum) without ganglion cell disease. the absence of ganglion cells, only a portion of the colon from the beginning, continue until the anus to the remote.

neonatal period colon wall is thin, distal bowel obstruction spasm, leading to expansion of the entire colon. At the same time as the ileocecal valve dysfunction, resulting in expansion of the small intestine are involved, clinical presentation whole abdominal swelling, severe vomiting, not into the milk. 2 -3 months later, improving function of the ileocecal valve, expansion of the intestines is limited to the colon, small bowel function returned to normal, does not affect the eating and stop vomiting. later with age, the muscles will gradually strengthen the intestinal wall, near the spasm segment intestine due to the resistance maximum, gradually expanding and a high degree of bowel wall thickening. Thus, on the one hand the driving force to strengthen its local; other hand, the expansion increases the capacity of the intestinal wall, thereby reducing the resistance of proximal intestine, so normal proximal colon, and finally the formation of the limitations of megacolon. These changes produce symptoms, spasms and convulsions of the intensity and the length of the intestine, strong and the elderly are a serious obstruction, the neonatal period is a serious and even life-threatening symptoms; On the contrary, the neonatal period may be obvious symptoms, older before symptoms appear megacolon.

diagnosis: 1, X-ray examination is an indispensable diagnostic tool in this disease, abdominal plain film can only understand the proximal intestine cavity expansion and inflation, barium enema for diagnosis of a relatively large value. X-ray findings of characteristics: the rectum was spasticity, no expansion; all normal or less than the diameter of the normal colon, but little different from the fetal colon; colon bags shape disappeared, the intestinal wall become smooth. enema pressure increased, the barium can be counter-flow to the ileum, but there is no significant expansion of the colon; colon length shorter than the normal children, especially the left colon short colon splenic flexure was seen obtuse angle, and shift inwards, when the visible colonic enema abnormal motility; lateral spine films before, no general expansion of Hirschsprung’s disease seen in the transverse colon inflated. X ray findings of abdominal plain film and more upright display low colonic obstruction. barium enema lateral and anteroposterior enema photos can be seen in the typical spastic bowel and expansion of intestines, exclusive of barium function is poor, there are still 24 hours after the barium retention, if not promptly washed out barium enema, barium can form stones, combined enteritis bowel wall when the expansion of serrated performance, expansion of the neonatal period after birth, more than half of bowel can only see the comparison. 2, medical history and signs more than 90% of children 36 to 48 hours after birth, no meconium in the future that is intractable constipation and bloating, must be enema, laxatives or anus suppository service to a history of bowel movements. often malnutrition, anemia and loss of appetite. abdomen shows a high degree of expansion and wide in the intestinal, rectal digital rectal examination was pot empty stomach can not be touched feces, more than cramps side section to the expansion hit stool in paragraph 3. biopsies taken more than 4cm from the anal submucosa and muscular rectal wall of a small piece of tissue to check the number of ganglion cells, giant colon in children the lack of ganglion cells. 4, Determination of anorectal manometry and rectal reflex anal sphincter pressure changes, can be diagnosed Hirschsprung’s disease and identify other causes of constipation. in the normal and functional constipation in children, when the rectal stimulation by the expansion after internal sphincter relaxation reflex occurs immediately, the pressure drop, internal sphincter in children with Hirschsprung’s disease not only not relaxed, and the apparent contraction, the pressure increased. This method of newborns within 10 days can sometimes cause false positive results 5. mucosa which was calculated based histochemical test for mucous membrane and muscle spasm segment of ganglion cells at the absence of proliferation, hypertrophy of the parasympathetic preganglionic fibers continue to release large amounts of acetylcholine and choline enzyme, the chemical method Determination of the number and activity of both normal children than the 5 to 6 times, contribute to the diagnosis of Hirschsprung’s disease, can be used for newborns.

identification: the neonatal Hirschsprung’s disease to and other causes of low intestinal obstruction such as atresia, colonic atresia, meconium constipation, such as identification of neonatal peritonitis. the larger infants, children should be with anorectal stenosis, the lumen caused by tumor compression inside and outside the secondary megacolon , colon weakness (such as hypothyroidism caused constipation in children), habitual constipation and idiopathic megacolon in children (more than 2 years after the sudden onset, as the internal sphincter dysfunction, pregnant with conservative therapy integrated), etc. It identification. concurrent with the viral enterocolitis, bacterial enteritis or septicemia enteroparalysis identification.

treatment: 1. newborns, infants generally poor, symptoms are severe, or severe combined congenital malformations enterocolitis , should be temporary enterostomy. to be generally improved, about 6 to 12 months after radical surgery. (where the length of bowel cramps, severe constipation, the need for radical surgery, is currently the most used type of surgery is out of the rectum ① sigmoid resection (Swenson’s operation); ② after colon resection colon rectal surgery pull (Duhamel’s operation); ③ stripped colonic mucosa cents out of the sheath of the rectum muscle resection (Soave’s surgery). If children acute enterocolitis, or nutritional risk as developmental disorders, can not tolerate a radical surgery, intravenous fluid transfusion should improve the general situation after the radical surgery, can not control, such as enteritis, abdominal distension and vomiting, it is timely for the intestinal fistula, after radical surgery again. six months old are generally more satisfied with the surgical results after 1 year postoperatively usually can be fully restored defecation function.)

2 children generally were good, conservative treatment can be used . non-surgical therapy: for newborns, children, and the limitations of short-segment Hirschsprung.

A therapy enema to assist defecation and exhaust, to reduce bloating and vomiting in children, in order to ensure normal feeding, maintenance of children growing up, according to the severity of symptoms to consider surgery. enema is inserted through the anal canal with a 24-26, bowel spasm, and then injected into the exhaust 50ml saline, keep the anal canal; massage the abdomen, so that gas and feces as emptying through the anal canal.

B expansion of the rectum and anal canal: once a day, every 30 minutes. extended to the large number of anal small devices.

C. ear and Point closure therapy: acupuncture ear kidney, sympathetic, subcortical, and other points lower rectum once a day, every 30 minutes; points closed: Shenshu injection of ginseng injection, colon shu neostigmine injection; or two alternately, once a day.

D internal medicine: qi yang commonly used drugs, such as Codonopsis, raw Astragalus, Morinda officinalis, Citrus aurantium, Magnolia; line gas capture drugs, such as Yuli Ren, cows son, Magnolia officinalis, Citrus aurantium; Qi and blood, moistening of qi circulation drugs, such as Codonopsis, angelica, raw land, cultivated land, Cistanche, Magnolia, citrus aurantium, peach kernel, safflower. transfected Abstract

Congenital long colon! Hirschsprung

Tags: Health Medical Pharma, bowel myenteric plexus, gastrointestinal abnormalities, septicemia enteroparalysis identification.treatment

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