890 COLON

Table 8-3. SUMMARY OF FEATURES CHARACTERIZING GASTROINTESTINAL POLYPOSIS SYNDROMES*

Symptom Onset Usual Age (yrs.) 15 to 30 15 to 30 10 to 30 Teens 40 to 70 Distribution Hereditary Transmission Dominant Dominant Dominant Recessive None 100% Stomach Less than 5% -5% 25% Less than 50% Small Bowel Less than 5% -5% 95% Colon 100% 100% 30% 100% 100% Histology Adenomas Adenomas Cellular hamartomas Adenomas Inflammatory glandular dilatation Additional Features Prognosis

Syndrome Multiple polyposis Gardner's Peutz-Jeghers Turcot CronkhiteCanada

Coton catcjnomcT Coton carcinoma Soft tissue tumors, osteomatosis Pigmented skin lesions With or without Gl tract carcinoma CNS tumors CNS tumors Alopecia, onychia. hyperpigmentation Diarrhea with protein and electrolyte losses Death often of cachexia

Juvenile polyps (common type)

Less than 10

Usually none

100%

Inflammatory

Diarrhea with protein Regression, loss possible autoamputation

From Youker JE, Dodds WJ, Welin S: Colonic polyps. In Margulls AR, Burhenne HJ (eds): Alimentary Tract Roentgenology 2nd ed. SI Lous, C. V. Mosby Co., 1972.

been demonstrated. Imaging by double-contrast barium enema is the method of choice, since the polypi may be extremely small and exhibit minimal serration of the border of the colon, merely resembling a thickening of the mucosal folds and a velvety appearance. The average age at diagnosis of patients with familial polyposis is 9 years. The total incidence of colon carcinoma in children with familial polyposis of the colon is 6.6 per cent (Franken). Peutz-Jeghers syndrome has already been characterized elsewhere (Chapter 7) as a disorder of mucocutaneous pigmentation and gastrointestinal polyposis. The melanotic macules on the lips or buccal mucosa are noteworthy. These hamartomata occur more frequently in the small intestine, but they may be demonstrated in the colon. There is an increased incidence of bowel cancer in patients with Peutz-Jeghers syndrome, but the frequency of malignancy is low. With Gardners syndrome, there are not only multiple adenomatous polyps of the colon but osteomas of the skeleton and multiple soft tissue tumors as well; and the adenomatous polyps of this syndrome are premalignant and colectomy is advised. With juvenile polyposis of the colon there may be many polyps throughout the entire colon, and involvement of the small bowel is unusual. However, there is no predisposition to malignancy. There may be a few to thousands of polyps, both sessile and pedunculated, throughout the colon. Usually, a partial colon resection for chronic blood loss or proteinlosing enteropathy is the only treatment carried out. Miscellaneous polypoid diseases of the bowel in children include the Cronkhite-Canada syndrome (alopecia, nail changes, skin pigmentation, and polyposis), Turcot syndrome (polyposis and central nervous system tumors), intestinal ganglioneuro- matosis (polypoid colon with thyroid carcinoma and pheochromocytoma), trichuriasis, and polyposis with Hirschsprungs disease.

Lymphosarcoma of the Colon in Children

In children this lesion is usually located in the ileocecal area, mostly in males under 13 years of age. Intussusception may occur in about one-fourth of the cases. Radiologically, a mass lesion is demonstrated or there may be an intussusception. The prognosis, when proven, is poor.

Sigmoid Volvulus
This is very rare in children (1 per 5000 hospital admissions) (Hunter and Keats). Usually, in children the small intestine is the organ that undergoes volvulus and sigmoid volvulus is rare. When it does occur, however, the features are very similar in both children and adults, except that bowel necrosis has a much more rapid course in children. Moreover, the large, air-filled sigmoid colon, readily diagnosed on plain films of the adult, is usually not present. For the most part, diagnosis must be made by a barium enema.

Eraserophagia
This refers to ingestion of abrasive ink or pencil eraser fragments, which produce a characteristically dense, opaque particulate appearance in the colon, closely resembling the appearance of dried inspissated particles of barium. Abrasive erasers ordinarily contain approximately 70 per cent barium sulfate

Carcinoma of the Colon

This is very rare in children, with the most frequent locations being the rectum and sigmoid. These, for the most part, occur in the second decade of life, after chronic recurrent abdominal pain, recta', bleeding, and constipation. The radiographic appearance is typical, and the prognosis is poor.