Clinical Analysis Of Total Colonic Aganglionosis

BackgroundHirschsprung disease is developmental abnormalities of the digestive tract characterized by the lack of intestinal intestines ganglion cells. Children’s incidence of hirschsprung disease is about 1/2000 ~ 5000. There have been reports that this disease was influenced by more than 10 kinds of genetic mutations mainly RET gene. Fetal intestinal nerve migration dysplasia lead to intestinal wall mucosa and submucosa of the absence of ganglion cells. Transitional process take place in embryonic 6 ~ 12 weeks, total time is about 7 weeks. The enteric nervous system is neural network structure composed of gastrointestinal tract neurons and glial cells. Most ganglion cells is located in between the intestinal wall ring muscle and longitudinal muscle muscle bundle and the submucosa, mucosa has a small amount of ganglion cells. Muscle ganglion cells and mucous membrane ganglion cells dominate gastric bowel movement. Many functions of bowel, including movement、 secretion、 blood vessels、 tension, etc., were mainly controlled by the enteric autonomic nervous system. The lack of the mucosa and submucosa of ganglion cells lead to bowel lack of corresponding normal peristalsis. Major clinical manifestations including meconium discharge delay, abdominal distension and constipation, vomiting, intestinal obstruction, severe cases with digestive tract perforation. According to involvement in the work of clinical pathological changes the length of the colon, hirschsprung disease mainly divides into the ultra short, short period(S-HD), and long(L- HD) type, long type is mainly divided into colon, the total colonic aganglionosis(TCA), the whole colon and small intestine. Hirschsprung disease conventional auxiliary examination including a rectal suction biopsy( RSB), contrast enema(CE), rectal pressure(anorectal manometry, ARM), etc. Influenced by age and pathological changes of the intestine, the positive rate of auxiliary examinations above is low.The diagnosis of disease eventually based on the pathological examination intraoperative bowel wall tissue. The treatment of surgery of congenital megacolon is best.In recent years, Surgical treatment has made great progress, classic surgical procedures were Rehbein、 Swenson 、Duhamel、Soave surgery, etc. With the development of the medical and health undertakings, the surgery way gradually improve. improved Duhamel surgery, improved Soave surgery, Martin are widely applied in clinical Improving Soave surgery has a advantages of slight trauma, widely used in domestic and abroad. In recent years, with the clinical application of minimally invasive technology, laparoscopic assisted megacolon radical prostatectomy is widely used in clinic, reducing the incidence of the complications of open operation. The involvement of total colonic aganglionosis is from the anus to back to the blind department level, no more than blind proximal 50 cm of small intestine. The incidence of TCA in hirschsprung disease accounts for about 2% to 13%. The total colonic aganglionosis is an heavy illness, clinical manifestations lack of specificity,the positive rate of auxiliary examination is low, so early clinical diagnosis and correct treatment are more difficult. ObjectiveAt home and abroad, hirschsprung literature reports of total colonic aganglionosis is less. Long-term follow-up data is lacking. In this paper, from January 2008 to January 2016, clinical data of 10 cases of the total colonic aganglionosis of the first affiliated hospital of zhengzhou university were retrospectively analyzed. Combined with related literature at home and abroad, the analysis and summary of clinical commonness and characteristics give help for the clinical treatment of total colon hirschsprung. Methods:In this paper, from January 2008 to January 2016, clinical data of 10 cases of the total colonic aganglionosis of the first affiliated hospital of zhengzhou university were retrospectively analyzed. The preoperative examination and clinical performance characteristics of total colon hirschsprung was analyzed. According to the operation way, this group of children can be divided into two categories: one-stage improved Soave megacolon radical operation; one-stage intestinal colostomy of ileum and two-stage megacolon radical operation then. The postoperative recovery of children were follow-up by outpatient service and telephone for a long time. Combined with related literature at home and abroad, Sorting and comparing complications of various operation was analyzed by statistical analysis. ResultsThree months later,compared with radical operation, postoperative bowel movement situation of stage surgery was obviously improved. According to the questionnaire of follow-up project, one year after megacolon radical surgery, assessment of improved Soave megacolon radical was taken place. the scores of this group are compared with scores of modified Duhamel surgery cases, According to the data, postoperative recovery of improved Soave is better than the improved Duhamel megacolon radical operation for recurrent abdominal distension. Compare the Short-term postoperative patients recovery of improved Soave surgery and Martin surgery. About short-term postoperative recovery, Statistical data show improved Soave surgery better than Martin surgery. Conclusion1. About TCA radical recovery, staging is better than the first phase of the radical surgery,but postoperative complications of stage surgery are more than radical surgery.2.About TCA treatment of short-term postoperative complications, improved Soave megacolon radical better than Martin megacolon radical; about long-term enterocolitis of postoperative complications, Improved Soave megacolon radical is better than the improved Duhamel megacolon radical prostatectomy.