OVERVIEW
Overview
Melanoma-gastrointestinal polyposis syndrome is a hereditary disorder characterized by melanoma visible on and around the lips and mouth, accompanied by polyposis of the gastrointestinal tract.
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Department of Medicine
Gastroenterology
Clinical symptoms
Visible melanin spots on the oral mucosa and lips.
Hazards
This disease can lead to intussusception, intestinal bleeding, gastrointestinal bleeding, and intestinal obstruction.
Complications
Intussusception, intestinal bleeding, gastrointestinal bleeding, intestinal obstruction, etc.
Examination
Barium meal X-ray examination of the whole digestive tract, X-ray double contrast of the colon, electronic fiber endoscopy, histopathological examination, etc.
Diagnosis
Diagnosis is based on the manifestation of melanin spots on the oral mucosa and lips, combined with X-ray film, electronic fiber endoscopy, histopathology and other examinations.
Treatment principle
Mild intestinal bleeding can be treated conservatively with internal medicine. Severe bleeding, internal medicine treatment is ineffective, feasible caesarean section. For intestinal obstruction, large number of polyps or large polyps, surgery or endoscopic polypectomy is feasible.
Curability
There is no cure for this disease, but active treatment can improve the symptoms, and rarely occur malignant changes.
Dietary advice
Eat a light and nutritious diet, avoid spicy and irritating foods.
Causes
Causes
This disease is related to heredity.
Symptoms and Diagnosis
Typical Symptoms
Oral mucosa and lips have obvious melanin spots, which can be single or multiple, brown or black, ranging from 1 to 5mm in diameter, irregular round or oval in shape, darkest before and after puberty, lighter in early childhood and old age, and individual patients also have varying degrees of pigmented spots on the face and hands. Polyps mostly occur in the small intestine, generally no conscious symptoms, there are occasional intussusception and intestinal bleeding, a large amount of gastrointestinal bleeding, more suggestive of gastric, duodenal polyps. Polyps can directly or indirectly induce intussusception, when abdominal cramps and a series of intestinal obstruction symptoms. Compared with pediatric intussusception or intussusception caused by tumors in the elderly, the abdominal pain caused by this disease is relatively mild, and the abdominal pain can be relieved in 10 to 15 minutes.
Diagnostic basis
1. Melanin spots can be seen around the lips and mouth, accompanied by multiple polyposis in the digestive tract. 2. Barium meal X-ray of the whole digestive tract: polyps of different sizes, most of them within 2 cm, most of them are with tips, densely and widely distributed, and those with obvious lesions can involve the whole colon, even the stomach and the small intestine, the edges of which appear lace-like when the intestinal tube is filled, and countless small, dense filling defects can be seen when pressure is applied. 3. X-ray of the colon Duplex contrast: honeycomb patterned shadow can be seen.4. Electronic fiberoptic endoscopy: polyps of stomach, duodenum, rectum and colon can be detected.5. Histopathological examination: glandular hyperplasia and dendritic hyperplasia of mucosal muscularis layer can be seen as the main ones.
Treatment
Treatment guideline
Mild intestinal bleeding should be treated conservatively with internal medicine. Severe bleeding, internal medicine treatment is ineffective, feasible caesarean section. For intestinal obstruction, large number of polyps or large polyps, surgery or endoscopic polypectomy is feasible.
Surgery
If there is intestinal obstruction, a large number of polyps or large polyps, surgery or endoscopic polypectomy is feasible.
Other treatments
This disease is a congenital hereditary disease, so there is no special radical treatment. Mild intestinal bleeding should be treated conservatively by internal medicine. If the internal medicine treatment is ineffective in severe bleeding, caesarean section is feasible to find the bleeding site and do appropriate surgical treatment, generally not suitable for polypectomy.
Prognosis
Prognosis can be improved by active treatment.
Nursing care
Daily care
1. Keep the environment quiet and comfortable, reduce the adverse stimulation and psychological pressure on the patient. 2. Strengthen dietary hygiene and dietary nutrition, and develop regular eating habits. 3. Exercise appropriately, avoid excessive exertion, and enhance the resistance of the organism. 4. Maintain a good state of mind, and be strong in overcoming the disease.
Diet regulation
Eat a light and nutritious diet, avoid spicy and stimulating food.