Sigmoid redundancy Basic overview Congenital long colon is also called sigmoid redundancy. The colon consists of six parts: cecum, ascending colon, transverse colon, descending colon, sigmoid colon, and rectum. The ascending colon, descending colon, and lower part of rectum are inter- or extrinsic organs of the peritoneum, which are relatively fixed and cannot be redundant. The cecum and transverse colon are redundant and can cause abdominal pain, bloating and other digestive symptoms due to excessive mobility. The sigmoid colon is a fecal storage organ, and a redundant sigmoid colon can cause chronic constipation. Some authors examined the ganglion cells in the intestinal wall of three children with sigmoid redundancy, of which two cases were normal and one case was reduced. There are two possible causes of chronic constipation due to sigmoid redundancy: one is primary (HDAD); the other is secondary (simple redundancy). The etiology of sigmoid redundancy needs further study. (ii) Pathogenesis Kusi (1967) dynamically observed more than a thousand children with chronic constipation and frequent abdominal pain and found that 25% had sigmoid redundancy; Trofimova (1968) examined more than a thousand children with chronic constipation and 30% to 40% had sigmoid redundancy. Lyonyushkin suggested that sigmoid redundancy is not only a pathological basis for clinical symptoms, but also a normal variant, i.e., no clinical symptoms. Klimanov (1973) studied the histology of the redundant segment of the sigmoid colon and found degeneration of the muscle fibers of the intestinal wall with connective tissue edema, lymphocytic infiltration on the basis of hypertrophy of the muscle fibers, atrophy of the intestinal mucosa, and widening of the lymphatic gap. The nerve plexus was unchanged, but the number of ganglion cells was reduced, and the nuclei were wrinkled and vacuolated. Electron microscopic changes in the synapses of the distal sigmoid colon were seen to be primary lesions. The above changes are well consistent with the pathological changes of congenital megacolon homozygous disease (HAD). Symptoms and signs are similar to congenital megacolon in some cases and chronic constipation in others. In Lyonyushkin’s data, in 60% of children with sigmoid redundancy, constipation occurs when breastfeeding changes to artificial feeding or the addition of complementary foods within 1 year of age; in 40% of children, constipation occurs between 3 and 6 years of age, and abdominal pain results from the accumulation of intestinal contents, distention, and bending or partial torsion of intestinal collaterals, adhesions or scarring of the mesentery. Sometimes the abdominal pain is accompanied by vomiting. This author dynamically observed children aged 3 to 14 years and divided the clinical manifestations of sigmoid redundancy into 3 stages: compensated, subcompensated and decompensated. The compensated stage: characterized by occasional intestinal dysfunction and sigmoid redundancy on X-ray, which is actually found in healthy children. Some of them complain of occasional lower abdominal pain. Some abdominal pain is accompanied by vomiting and abdominal distention. Some were operated for suspected appendicitis and the abdominal pain was not relieved after surgery. The development of the child at that stage was consistent with that of a child of the same age. There are no physical signs. Subcompensatory phase: The main symptom is intermittent constipation, i.e., occasional 1 bowel movement in 2 to 3 days. Many parents confirm that the symptoms appear after 2 years of age. It may be related to the amount of fruits and vegetables in the diet, with frequent constipation in winter and the beginning of spring, and relief of constipation in summer and autumn. This stage of the disease is characterized by frequent abdominal pain and bloating. There are often signs of fecal accumulation along the colon, so parents often use laxatives. Decompensated phase: This phase is characterized by more pronounced intestinal dysfunction. Constipation can last for more than 5 days, and some children are unable to have spontaneous bowel movements and can only have bowel movements after an enema. Sometimes the lower abdomen is enlarged (flatulence). Some of these symptoms are similar to those of congenital megacolon, but their degree is completely different: sigmoid redundancy has a more moderate clinical presentation, with a later onset and often alternating constipation with spontaneous defecation. The diagnosis of sigmoid redundancy is based on a careful study of the clinical manifestations and barium enema x-ray data. According to Lyonyushkin, children with sigmoid redundancy do not have sigmoid dilatation in the early stages of the disease, so giant long sigmoid and giant sigmoid are just further dysfunctional and morphologic changes of sigmoid redundancy. Children diagnosed with sigmoid redundancy should be monitored dynamically and reviewed annually: once in the compensated phase, twice in the subcompensated phase, and three times in the decompensated phase. The child should be monitored dynamically. Treatment (a) Treatment There are 2 kinds of treatment methods: non-surgical and surgical. Non-surgical treatment is the main treatment for sigmoid redundancy and is also suitable for all children with sigmoid redundancy. Non-surgical treatment is a combination of defecation training, diet and medication, and requires repeated treatment. Defecation training and diet are of great importance in the prevention of defecation dysfunction and are also suitable for children with existing defecation dysfunction. The oral administration of liquid paraffin is beneficial for removing the accumulated stool in the sigmoid colon and promoting the recovery of sigmoid colon function. However, it takes at least 1 to 2 months to restore the function of sigmoid colon, so other methods can be used to assist in the treatment: oral or injectable neostigmine (under inpatient conditions); B vitamins; electrical stimulation of the lower abdomen (sigmoid colon), 1 time/d for 10-15 min each time. The earlier and more persistent the non-surgical treatment, the better the outcome. Cure standard: clinical symptoms disappear within 2~4 years after treatment. The indications for surgery of sigmoid redundancy are very strict, and the power function of the sigmoid colon should be judged by comprehensive clinical and X-ray data. (1) Indications for surgery: persistent constipation that has not been treated by non-surgical treatment; progressive dilatation of the sigmoid colon during non-surgical treatment or decreased power on electromyography; abdominal pain that is not relieved during non-surgical treatment, except for other causes. (2) The surgical method is usually Rehbein or Soave method.