Mario Trompetto M.D., Giuseppe Clerico M.D., Ezio Ganio M.D., Alberto Realis Luc M.D.
Surgical approach to juvenile gastrointestinal polyposis
Colorectal Eporediensis Centre – Unit of Coloproctology – Ivrea – Italy
Familial adenomatous polyposis and Gardner syndrome are rare dominantly inherited syndromes characterized by hundreds to thousands of colonic adenomatous polyps. Colonic cancer occurs at a young age in both of them unless the colon is removed. Peutz-Jeghers syndrome and familial juvenile polyposis are inherited hamartomatous polyposis conditions with a low but well defined risk of colonic cancer. These four polyposis syndromes together account less than 1% of cases of colonic malignancies.
Familial juvenile polyposis is an autosomal dominant condition in which affected patients develop upper or lower gastrointestinal juvenile polyps, or both, and have a high possibility to develop a cancer of the gastrointestinal tract. Up to date the risk of G1 cancer in these patients has not been well defined because of the small number of families and lack of follow-up.
Single or few polyps can be frequently found in patients of any age and can occur also in infancy.
Polyps in children are classified as malignant or benign (tab. 1).
Table. 1. Pediatric tumors of the GI tract.
| Benign | Malignant |
| Polyps | |
| Non neoplastic | - |
| mnJuvenile | - |
| mnHamartomatous | - |
| mnInflammatory fibroid | - |
| mnLymphoid | Lymphomas |
| Neoplastic |
-
|
| mnAdenomatous | Carcinomas |
| | mnNon familial |
| | mnFamilial |
| | mnmnPolyposis |
| | mnmnNon polyposis |
| Non polypoid | |
| mnMesenchymatous | - |
| mnmnLipomas | - |
| mnmnAngiomas | - |
| mnmnLeiomyomas | Leiomyosarcomas |
| mnNeurogenic |
-
|
| mnmnNeurofibromas | Neurosarcomas |
| mnmnGanglioneuromas | - |
Isolated inflammatory polyps (juvenile or retention polyps) are the most frequent and occur often in the first decade of life, particularly between 3 and 4 years of age. Some studies confirm their presence in 1% to 2% of children of this age. More than 60% of them are localized in the distal part of the colon or in the rectum.
Juvenile polyposis coli is characterized by multiple inflammatory polyps in the colon in contrast with generalized juvenile polyposis in which polyps can be found anywhere in the gastrointestinal tract.
Histologically juvenile polyps are hamartomas with distended mucus filled glands, oedema, cystic dilation and multiple blood vessels. The mucus retention and criptic dilation give them also the term of retention polyps.
Endoscopically they appear as pedunculate red polyps with a frable, ulcerated surface and often bleeding. The presence of a peduncle is the only reliable distinction between a juvenile and a Cronkhite-Canada polyp.
Painless rectal bleeding and rectal prolapse in the first decade of life are the most common clinical features of juvenile polyposis coli while in the generalized juvenile polyposis the symptoms are more severe including not only haemorrhage and possible intussusception but also diarrhoea, disturbances of the electrolytic balance, anaemia, hypoalbuminemia, delay in growth.
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Piśmiennictwo
1. Reed K., Vose P.C.: Diffuse juvenile polyposis of the colon: a premalignant condition? Dis. Colon Rectum 1981, Apr. 24(3):205-10. 2. Grosfeld J.L., West K.W.: Generalized juvenile polyposis coli. Clinical management based on long-term observation. Arch Surg. 1986, May, 121(5):530-4. 3. Jarvine H., Franssila K.O.: Familial juvenile polyposis coli: increased risk of colorectal cancer. Gut 1984, Jul, 25(7):792-800. 4. Longo W.E. et al.: Malignant potential of juvenile polyposis coli. Report of a case and review of the literature. Dis. Colon Rectum 1990, Nov, 33(11):980-4. 5. Mazier W.P. et al.: Juvenile polyps of the colon and rectum. Surg. Gynecol. Obstet 1982, Jun, 154(6):829-32. 6. Rozen P., Baratz M.: Familial juvenile colonic polyposis with associated colon cancer. Cancer 1982, Apr, 1 49(7):1500-3. 7. Howe J.R. et al.: The risk of gastrointestinal carcinoma in familial juvenile polyposis. Ann Surg. Oncol. 1998 Dec. 5(8):751-6. 8. Agnifili A. et al.: Juvenile polyposis: case report and assessment of the neoplastic risk in 271 patients reported in the literature. Dig. Surg. 1999, 16(2):161-6. 9. Hoffenberg E.J. et al.: Symptomatic colonic polyps in childhood: not so benign. J. Ped. Gastroenterol. Nutr., 1999, Feb. 28(2):175-81. 10. Pashankar D. et al.: Life-threatening gastrointestinal haemorrhage due to juvenile polyposis. Am. J. Gastroenterol 2000, Feb. 95(2):543-5.
