Megacysto-microcolon-intestinal hypomotility syndrome

　　The etiology of megacysto-microcolon-intestinal hypomotility syndrome is not yet clear, and it may be related to environmental factors, genetic factors, dietary factors, and emotional and nutritional factors during pregnancy. The pathogenesis of the disease is unknown, and some scholars believe it is due to the loss of innervation of intestinal cells, which belongs to a neurogenic hypomotility. Some scholars also believe it is related to habitual diet and chromosomal abnormalities, but this has not been confirmed.

　　Megacysto-microcolon-intestinal hypomotility syndrome often presents with acute onset. Megacysto-microcolon-intestinal hypomotility syndrome can be complicated by urinary tract infections, sepsis, and long-term malnutrition and other diseases.

　　Megacysto-microcolon-intestinal hypomotility syndrome often presents with acute onset, initially表现为sudden abdominal pain, followed by abdominal distension, nausea, vomiting, and no flatus or defecation from the anus. The twisted intestinal loops cause an asymmetric abdominal appearance, and sometimes intestinal shape or peristaltic waves, and abdominal tenderness can be observed. The degree of tenderness in different parts of the colon varies with the torsion of the colon segment.

　　The etiology of megacystis-microcolon-intestinal hypoperistalsis syndrome is unclear and is related to autosomal recessive inheritance. It is usually associated with consanguineous marriage. This disease cannot be prevented directly. For patients with a suspected family history of chromosomal abnormalities, genetic screening should be performed to avoid offspring being affected by chromosomal inheritance after marriage. At the same time, attention should be paid to strengthening prenatal nutrition, avoiding emotional excitement, and other adverse stimuli that affect embryonic development.

　　Ultrasound examination of megacystis-microcolon-intestinal hypoperistalsis syndrome shows that the bladder is significantly enlarged, but the contraction force of the bladder is reduced, which is prone to pressure urinary incontinence; colonoscopy shows that the colon is empty, meconium is less, or there is no meconium; abdominal X-ray examination shows that the colon is significantly distended, but no liquid level is seen.

　　Patients with megacystis-microcolon-intestinal hypoperistalsis syndrome should undergo要素diet through stoma infusion. 要素diet is a chemically refined food containing all the easily digestible and absorbable nutrients required by the human body, including free amino acids, monosaccharides, main fatty acids, vitamins, inorganic salts, and trace elements. When mixed with water, it can form a solution or a relatively stable suspension. Its main characteristics are: it can be directly absorbed and utilized by the intestines without going through the digestive process, providing the body with energy and nutrition. It is suitable for patients with severe burns and trauma, high metabolism, digestive tract fistula, nutritional support before and after surgery, non-infectious severe diarrhea, malabsorption, and other conditions.

　　The treatment of megacystis-microcolon-intestinal hypoperistalsis syndrome first adopts parenteral nutrition, followed by jejunostomy and gastrostomy, and then要素diet is administered through the stoma. Prevent and treat urinary tract and systemic infections.