Treatment
The treatment of polyps of the GI tract in children can vary from simple observation of benign lesions to wide excision and chemotherapy for malignant ones.
Once diagnosed, juvenile polyps should be removed endoscopically. This is done to stop the symptoms as well as establish the diagnosis. Most are within easy reach of a flexible sigmoidoscope and can be snared around the stalk. Cecal or ascending colonic polyps can be observed as long as the lesions do not grow and exceed 2 cm in diameter. However, with the widespread use of colonoscopy in pediatrics, endoscopic removal of these lesions is now the treatment of choice in this previously hard to reach area.
The overall guiding principle in the treatment of Peutz-Jeghers polyps should be one of bowel conservation. Since the entire gastrointestinal tract can be affected, excision of all affected areas could easily result in intestinal insufficiency. Regular surveillance through endoscopy or small bowel contrast studies should be done to detect polyps that are growing and could be malignant. Large polyps or those causing significant symptoms should be removed. Endoscopic removal should be attempted first. For lesions normally beyond the range of normal endoscopy, a combined endoscopic-surgical approach can be attempted where the surgeon at laparotomy guides the endoscopist through the small bowel for visualization and removal of large lesions. Resection of segments of bowel should be reserved only for cases where cancer has developed and invaded the submucosa, or in areas where polyps are particularly dense and causing severe symptoms. Duodenal polyposis may be a particularly difficult problem. Although most polyps can be excised endoscopically or surgically, diffuse duodenal involvement with bleeding has been treated with pancreaticoduodenectomy.
Lymphomatous lesions are surgically removed. Perforations or areas of intussusception are resected. Radical surgery with lymph node dissection is unnecessary as all these patients require systemic chemotherapy. Staging with computed tomography scanning and bone marrow sampling are necessary and most patients require insertion of subcutaneous reservoirs for long-term chemotherapy. The outlook for small bowel lymphoma depends on the staging and cellular subtype. Prognosis for this disease has been steadily improving with the advent of more effective chemotherapeutic regimens.
Treatment of children with FAP involves not only the individual involved but should also extend to others in the family. Family members should be screened and referred for genetic counseling and genetic analysis. Definite genetic markers have been identified in this family of disorders, which may include other syndromes such as Gardner's syndrome (multiple osteomas, fibromas, epidermoid cysts). The surgical treatment of FAP requires planning the process with the family and the patient. It is customary to do a complete colonic and rectal removal with a sphincter saving operation in early adolescence. This generally occurs at a time when the patient understands and can participate in treatment planning and execution.
A Soave type operation (endorectal pullthrough) is the one most commonly used. The mucosa of the distal rectum is stripped and the terminal ileum is pulled through. Both straight pullthroughs and reservoir operations in the form of a J or S pull through have been advocated. In the teenage group, patients have attained the size where a stapling device can be used to construct both the reservoir and perform the lower anastomosis. It is customary to protect the pouch and the anastomosis with a loop ileostomy which is then closed 4-6 weeks later after all the suture lines have healed and a contrast study of the pouch has demonstrated no leaks.
The prognosis when the disease is treated in a timely fashion is excellent. Periodic studies to inspect the remaining native anal mucosa are essential for the early detection of new lesions that can be easily ablated. Genetic counseling is essential so that all family members can be screened and so that all patients affected by the disease can consider the risks to their own children.
All other lesions are exceptionally rare in children including the adenomatous polyp or frank carcinoma. Treatment always includes endoscopic removal whenever possible, saving surgical resection for cases where this is impractical or contraindicated (i.e., invasive cancer).