Congenital megacolon

  Congenital megacolon (no ganglion cell syndrome) is one of the most common gastrointestinal malformations in pediatric surgery, characterized by constipation and the absence of ganglion cells in the diseased intestinal segment, with a high incidence of about 1:5000, slightly higher in males than females, and a familial tendency to develop. In the last decade, with the improvement of surgical techniques and the rapid improvement of perioperative monitoring and treatment, the mortality rate has decreased significantly.  The typical clinical manifestations are intermittent or progressive abdominal distension, difficulty in defecation, incomplete intestinal obstruction in severe cases, prolonged inability to eat normally and resulting in water-electrolyte imbalance, combined with enteritis, local and systemic toxic symptoms of infection will occur, and even giant colon crisis, delayed treatment can cause intestinal perforation, peritonitis, sepsis due to severe abdominal distension, the condition will rapidly deteriorate and eventually death.  Auxiliary examination △ abdominal x-ray standing plain film: routine examination, can initially determine the lesion site, the severity of the lesion and the possible prognosis.  △ lower gastrointestinal tract barium contrast examination: the examination means that may cause radiation injury, still widely used in clinical practice, and is currently one of the most important and accurate examination means. It can clearly diagnose the location and scope of the lesion, the dilatation of the intestinal canal and the barium discharge, and also help in the differential diagnosis.  Ultrasound examination: Ultrasound examination has developed rapidly in recent years and has partially or even completely replaced the diagnostic functions of traditional imaging, tomography and other large auxiliary instruments, which are simple, low equipment requirements, objective and harmless to the body, but requires ultrasonographers to have rich clinical experience and certain specialized skills in surgical anatomy, which can be done by non-specialized hospitals and ultrasonographers with extremely specialized training. The ultrasonographer should be able to do it. This year, our hospital has made a large number of accurate clinical diagnoses through simple outpatient ultrasound examinations.  Rectal manometry: It is more commonly used in foreign countries and Hong Kong and Taiwan, and the contents of manometry are internal sphincter relaxation reflex and pressure changes in various parts of the anal canal, which is safe and simple, but requires large and expensive medical equipment.  △ Rectal mucosal biopsy and enzymatic examination: the physical examination and radiological examination methods commonly used in clinical practice still cannot accurately determine the cause of the disease, rectal mucosal biopsy and enzymatic examination can be considered, which is accurate, objective and reliable in diagnosis, but it is an invasive examination method, so it is often forced to be used in clinical work.  Once the diagnosis of megacolon is established, surgery should be performed at a later date to ensure safety, otherwise compensatory pathological changes may occur in the normal intestinal tube involved in the diseased intestinal segment, which may be forced to be removed during surgery and cannot be regenerated, in addition, delayed surgery is bound to cause significant or even serious delay in the growth and development of the affected children compared to normal children of the same age. Some patients with short and ultra-short segments can be treated conservatively.  There are many surgical methods and approaches commonly used at home and abroad, but the ultimate goal is to remove the diseased spastic intestinal segment, restore local normal intestinal peristalsis, eliminate abdominal distension, and be able to defecate on their own. Combined with the specific situation in China (such as affordability, traditional thinking, etc.), the principle of surgery is to perform a one-time surgical cure as far as possible.  △ Modified soave surgery (transanal megacolon radical surgery): In the past decade, this surgical method has been popularized all over the world, and because the surgical design is more in line with pathophysiological anatomy, the surgical method has become simpler and safer, and the postoperative complications and sequelae have been significantly reduced, making the soave surgery widely accepted. During the operation, the diseased intestine is dragged out and removed through the anus, and the proximal “normal” colon, which is severely dilated and hypertrophied, is appropriately resected, and the severed end of the colon is sutured to the incised edge of the anal canal on the dentate line to restore the patency of the intestine.  △ First-stage enterostomy: Patients with macrocolon crisis, partial long-segment macrocolon, or other reasons that cannot tolerate one-time surgery should be operated in stages, and enterostomy should be performed first, and the location of the fistula should be chosen at the dilated intestinal segment, and then surgery is usually performed 2 months after the fistula for radical treatment.  △ Transabdominal or laparoscopic minimally invasive methods to assist in the radical treatment of giant colon: in case of long spastic intestine or serious local adhesions of intestine, short colonic mesentery and high tension, it is difficult to drag out the intestine during surgery, so transabdominal or laparoscopic assisted ligation can be done to cut off the secondary vessels of colonic mesentery to facilitate the drag out of the intestine for radical treatment.  △ Duhamal giant colon radical surgery: traditional surgical method, the surgical method is relatively complex, need special metal instruments to assist, because of the surgical design and part of the diseased intestinal wall can not be completely removed, more postoperative complications and sequelae, often have the performance of fecal filth, slightly worse independent defecation, has been abandoned, but some backward cities and regions in China are still delayed.  △ Thomas operation Rehbein operation Swenson operation, etc.: Nowadays, it is rarely used.