Congenital megacolon (no ganglion cell disease) is one of the most common gastrointestinal malformations in pediatric surgery characterized by constipation and a high incidence of ganglion cell loss in the intestinal segment of the lesion: slightly higher in males than in females with a familial tendency to develop in recent years with several improvements in surgical techniques and rapid improvement in the level of awareness of perioperative monitoring and respectful treatment during this period the mortality rate has decreased significantly in many large and medium-sized cities in China. In recent years, with the improvement of the surgical technique and the rapid improvement of the awareness of the level of perioperative monitoring and respectful treatment, the mortality rate has decreased significantly.
Etiology
At present, there is no clear and definitive conclusion on the etiology of the disease, but in recent years, some studies on the etiology of the disease have been carried out to the genetic stage, and certain results have been achieved.
Clinical manifestations
Typical clinical severe manifestations are intermittent or progressive abdominal distension defecation difficulties in severe cases appear incomplete intestinal obstruction manifestations for a long time can not eat normally and lead to water-electrolyte imbalance combined with enteritis will occur local and systemic infection toxic symptoms can even appear megacolon crisis delayed treatment on behalf of the problem can be caused by severe abdominal distension intestinal perforation peritonitis sepsis condition injury will deteriorate rapidly eventually death
Diagnosis and differential diagnosis
Intermittent or progressive abdominal distension constipation after birth abnormal fetal stool can be accompanied by vomiting enterocolitis diarrhea answers in severe cases growth retardation should be considered forever congenital megacolon diagnosis shield with the help of relevant auxiliary tests usually easy to diagnose
There are many diseases similar to abdominal distension and constipation Surgical common ones are congenital sigmoid redundancy disorder intestinal dysmotility disorder megacolon like origin disease other causes of secondary megacolon and other medical common ones are genetic metabolic diseases (such as hypothyroidism, etc.) functional constipation enterocolitis, etc.
Because of the early onset and short duration of the disease, the symptoms and signs of neonates and small infants are not typical, especially the short segment type and the common type of megacolon may not yet show obvious pathological changes, so early diagnosis is relatively difficult, so we should carefully study the medical history and signs, combined with auxiliary tests to make a comprehensive consideration in order to make an accurate scientific judgment.
Auxiliary examinations
Abdominal x-ray standing plain film: routine medical examination can carefully determine the severity of the lesion and the prognosis of the possible effect of the lesion.
Lower gastrointestinal tract barium contrast examination: easy means of examination that may cause radiation damage earlier clinical conviction is still widely used at present is also one of the most temporarily important and most accurate real examination means contrast mortification examination Shanghai can serve to clearly diagnose to see the lesion site range of intestinal tube dilatation in front of the situation and the discharge of barium symptoms at the same time help related to the differential disease diagnosis
Ultrasound examination of the abdomen.
Ultrasound did not come to check the rapid development in recent years Wednesday has partially or even completely replaced the traditional contrast tomography and other large auxiliary instruments but wish to diagnostic function ultrasound examination for quite a while method simple equipment requirements low objectivity strong to recurrence body harmless but requires ultrasound doctor temperament should have a wealth of responsible clinical as soon as possible experience does not know and certain surgical anatomy professional local skills non-specialist hospital how and has been extremely not to say Professionally trained ultrasound experience doctors can do chemotherapy this year to our hospital through a simple outpatient later ultrasound left and right examination has medical skills to make a large number of awareness accurate clinical Beijing responsibility diagnosis
Rectal manometry examination.
In foreign countries and Hong Kong and Taiwan use more common pressure measurement content for the internal sphincter relaxation reflex and pressure changes in the various parts of the anal canal the method is safe and simple but requires large and expensive medical equipment due to the influence of the accompanying and subjective judgment of the operator aspects of comfort prescription test results surgical objectivity is not strong there is a certain error rate especially in newborns and small infants accuracy is poor
Rectal mucosal biopsy and enzymatic empirical examination: heart clinically commonly used physical examination care and radiological powerful examination means still easily cannot accurately determine the cause everyone can child phenomenon consider rectal mucosal biopsy and enzymatic reassurance examination the method is accurate objective conditioning diagnosis reliable but is invasive doctor examination method so in clinical blessing work often last dora forced to use
Disease treatment
Once the diagnosis of megacolon is established, elective surgery should be performed in a safe manner; otherwise, compensatory pathological changes may occur in the normal intestinal canal 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 the growth and development of the child to be significantly or even severely delayed compared to normal children of the same age Some patients with short and ultra-short segments can be treated in a conservative manner
There are many surgical methods commonly used at home and abroad, but the ultimate goal is to remove the diseased spastic intestinal segment to restore the local normal bowel movement, 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 surgical principle is to operate as far as possible to cure the disease.
Modified soave surgery (transanal megacolon radical surgery): In the past decade, this surgical approach has become popular worldwide because the surgical design is more in line with the pathophysiology and anatomy, the surgical method is becoming simpler and safer, and the postoperative complications and sequelae are significantly reduced, which makes the soave procedure widely accepted. The “normal” colon is sutured to the anal canal margin on the dentate line to restore patency of the intestine.
First-stage enterostomy.
Patients with combined megacolon crisis and some long-segment megacolon that cannot tolerate a single surgical cure for other reasons should have a staged operation followed by an enterostomy. The location of the fistula should be chosen at the dilated segment of the intestine, usually months after the fistula before the surgical cure.
Minimally invasive transabdominal or laparoscopic method assisted radical treatment of megacolon: in case of long spastic intestinal canal or severe local adhesions of intestinal canal, short colonic mesentery and high tension, it is difficult to drag out the intestinal canal during surgery, and transabdominal or laparoscopic assisted ligation can be performed to cut off the secondary vessels of colonic mesentery to facilitate drag out the intestinal canal for radical treatment.
Duhamal resection of giant colon.
The traditional surgical method is relatively complex and requires special metal instruments. Due to the surgical design, part of the diseased intestinal wall cannot be completely removed.
Thomas operation, Rehbein operation, Swenson operation, etc.: rarely used nowadays
Prognosis of the disease
The majority of patients have a satisfactory prognosis after a short recovery period, they can have normal milk intake, the abdominal distension disappears, and they can defecate on their own, and their weight gain and growth can reach the level of normal children of the same age.
A few patients may have postoperative anastomotic stenosis on the dental line, rectal mucosal prolapse, intermittent and recurrent enterocolitis, defecation disorders, and other serious complications, such as postoperative abdominopelvic hemorrhage, intramyocardial infection, dragging out of the intestinal canal, pelvic torsion, etc.
Some patients have a slow recovery of bowel function after surgery, and it is easy to have frequent and loose stools after surgery, and it takes at least – months to fully recover.
Disease care
Postoperative dilation usually lasts for about 6 months to avoid perforation during dilation, and the operator should be careful.
Expert opinion
The incidence of congenital megacolon is high and patients are usually young and the surgery is relatively complex.