Pediatric gastrointestinal polyposis includes familial polyposis of the colon, pigmented-polyposis of the gastrointestinal tract (Peutz-Jeghers syndrome), Gardner syndrome, juvenile polyposis, and polypoid protein-losing enteropathy, with the first three being the most common.
I. Familial polyposis of the colon
The disease is an autosomal dominant disease, the tendency of malignant change up to 80% or more, the polyp distribution is extremely wide, the colon is the preferred site, the heavy rectum and cecum mucosa is completely covered with polyps, can not be counted.
Diagnosis
1.Symptoms appear slowly, early with increased number of bowel movements, with mucus, or mixed with fresh or old blood, and then the symptoms gradually worsen.
2.The polyps in the rectum can prolapse out of the anus after increasing in size, forming rectal prolapse, and a large number of polyps of different sizes can be seen on the mucosa.
3.After a long period of disease, there are often intermittent abdominal discomfort, abdominal pain, weakness, emaciation, anemia, loss of appetite, etc.
4, rectal finger diagnosis and colonoscopy can be diagnosed.
5.Barium enema or gas-barium double contrast radiography can clearly show the polyps with filling defects or ring shadows.
Treatment
1, Leucommune infusion retained enema can be used for long-tipped adenomatous polyps, the cure rate of 23%.
2.Surgical treatment, it is recognized that early resection of the diseased colon can prevent malignant transformation. According to the extent of the lesion, partial colectomy, hemicolectomy or subtotal colectomy can be chosen. If the whole colon to cecum is involved, the whole colon and rectum can be removed and a permanent ileostomy can be performed, but it is generally not easily accepted by parents. Soave surgery is also feasible to preserve the function of the sphincter muscle, and the postoperative control of defecation is satisfactory.
Peutz-Jeghers syndrome
Half of them have family history of this disease, which is also dominantly inherited. Polyps can occur in any part of the gastrointestinal tract, with the highest incidence in the jejunum and ileum, accounting for more than 90%. Polyps can be malignant, but are much less common than familial polyposis.
Diagnosis
1, clinical features are skin and mucous membrane pigmentation. In the mouth and lips, cheek mucosa, as well as around the mouth and eyes, the palm surface of the hands and feet, the perianal area, etc., we see small light brown to black spots, linear, oval or irregular, not fused with each other.
2. Some cases have early abdominal pain, mucous bloody diarrhea, combined with intestinal entrapment and intestinal obstruction. Long-term blood in stool may cause anemia and weakness.
3, barium meal and intestinal examination can be seen in the gastrointestinal polyp shadow, barium enema, fiber colonoscopy can help the detection of polyps in the colon.
Treatment
1.Segmental intestinal resection anastomosis can be performed for dense polyps in the small intestine with limitations.
2, scattered polyps can be removed simply or together with part of the mucosa.
Gardner syndrome
Gardner syndrome, also known as hereditary intestinal polyp syndrome, is characterized by colonic polyposis combined with multiple osteomas and soft tissue tumors. It is autosomal dominant, and the malignant rate of colon polyps is very high, and the incidence is similar in men and women.
Diagnosis
1. The clinical symptoms are basically the same as those of familial polyposis of the colon.
2, often combined with bone tumors, mostly benign, can occur in any bone, to award, jaw, frontal, occipital and other common, such as in the tubular bone is often symmetrical.
3.More often combined with soft tissue tumors, such as epidermoid cysts, lipomas, smooth muscle tumors, etc.
4.Barium meal, barium enema and fiber colonoscopy, cranial and long bone X-ray are helpful for the diagnosis of this disease.
Treatment principle
Once diagnosed, resection of the diseased colon should be performed to prevent cancer. Soft tissue tumors are prone to recurrence after resection.