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Generalized Juvenile Polyposis Coli: Clinical Management Based on Long-term Observations

Generalized Juvenile Polyposis Coli: Clinical Management Based on Long-term Observations Abstract • Generalized juvenile polyposis occurred in five patients (age range, 18 months to 16 years). Clinical findings included abdominal pain, weakness, rectal bleeding, diarrhea, rectal prolapse, intussusception, clubbing, and failure to thrive. Laboratory findings included anemia, hypoalbuminemia, hypokalemia, and skin test anergy. Diagnosis is achieved by double contrast enema, endoscopy, and biopsy. Unlike patients with solitary juvenile polyps, patients with generalized involvement require surgical intervention. Subtotal colectomy and ileoproctostomy are the procedures of choice, and we performed them in four cases. An ileoanal-endorectal pull-through procedure was required in one patient with continued rectal disease. All five patients are currently alive and well. Long-term follow-up is important as polyps may persist into adult life. Family members are at risk for developing gastrointestinal tract tumors and should be screened. (Arch Surg 1986;121:530-534) References 1. Holgerson LO, Miller RE, Zintel HA: Juvenile polyps of the colon . Surgery 1971;69:288-293. 2. Shermeta DW, Morgan WW, Eggleston J, et al: Juvenile retention polyps . J Pediatr Surg 1969;4:211-215.Crossref 3. Alexander RH, Beckwith JB, Morgan A, et al: Juvenile polyps of the colon and their relationship to allergy . Am J Surg 1970;120:222-225.Crossref 4. Soper RT: Intestinal polyps , in Holder T, Ashcraft K (eds): Pediatric Surgery . Philadelphia, WB Saunders Co, 1980, pp 481-494. 5. Haggitt RC, Pitcock JP: Familial juvenile polyposis of the colon . Cancer 1970;26:1232-1238.Crossref 6. Franken EA Jr, Gamet DJ, Fitzgerald JF, et al: Juvenile polyposis of the colon . Ann Radiol 1975;18:499-504. 7. Sachatello CR, Pickren JW, Grace JT: Generalized juvenile gastrointestinal polyposis: A hereditary disorder . Gastroenterology 1970;58: 699-708. 8. Peck DA, Watanabe JS, Trueblood HW: Familiar polyposis in children . Dis Colon Rectum 1972;15:23-29.Crossref 9. Watne AL, Lai H, Carrier J, et al: The diagnosis and surgical treatment of patients with Gardner's syndrome . Surgery 1977;82:327-333. 10. Turcot J, Despres MP, St Pierre F: Malignant tumor of the central neurosystem associated with familial polyposis of the colon . Dis Colon Rectum 1959;2:465-468.Crossref 11. Duzois R, Judd ES, Dahlin DC, et al: The Peutz-Jeghers syndrome: Is there a predisposition to the development of intestinal malignancy? Arch Surg 1969;98:509-517.Crossref 12. Cronkhite LW Jr, Canada WJ: Generalized gastrointestinal polyposis: An unusual syndrome of polyposis, pigmentation, alopecia and onychotrophia . N Engl J Med 1955;252:1011-1015.Crossref 13. Diner WC: The Cronkhite-Canada syndrome . Radiology 1972;105: 715-716.Crossref 14. Franken EA Jr: Lymphoid hyperplasia of the colon . Radiology 1970;94:329-334.Crossref 15. Gathright JB Jr, Cofer TW Jr: Familial incidence of juvenile polyposis coli . Surg Gynecol Obstet 1974;138:185-188. 16. Veale AM, McColl I, Bussey HJR et al: Juvenile polyposis coli . J Med Genet 1966;3:5-16.Crossref 17. Smilow PC, Pryor CA, Swinton NW: Juvenile polyposis coli: A report of three patients in three generations of one family . Dis Colon Rectum 1966;9:248-254.Crossref 18. McColl I, Bussey HJR, Veale AM, et al: Juvenile polyposis coli . J R Soc Med 1964;57:896-897. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Surgery American Medical Association

Generalized Juvenile Polyposis Coli: Clinical Management Based on Long-term Observations

Grosfeld, Jay L.; West, Karen W.
Archives of Surgery , Volume 121 (5) – May 1, 1986
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References (21)

Publisher
American Medical Association
Copyright
Copyright © 1986 American Medical Association. All Rights Reserved.
ISSN
0004-0010
eISSN
1538-3644
DOI
10.1001/archsurg.1986.01400050040005
Publisher site
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Abstract

Abstract • Generalized juvenile polyposis occurred in five patients (age range, 18 months to 16 years). Clinical findings included abdominal pain, weakness, rectal bleeding, diarrhea, rectal prolapse, intussusception, clubbing, and failure to thrive. Laboratory findings included anemia, hypoalbuminemia, hypokalemia, and skin test anergy. Diagnosis is achieved by double contrast enema, endoscopy, and biopsy. Unlike patients with solitary juvenile polyps, patients with generalized involvement require surgical intervention. Subtotal colectomy and ileoproctostomy are the procedures of choice, and we performed them in four cases. An ileoanal-endorectal pull-through procedure was required in one patient with continued rectal disease. All five patients are currently alive and well. Long-term follow-up is important as polyps may persist into adult life. Family members are at risk for developing gastrointestinal tract tumors and should be screened. (Arch Surg 1986;121:530-534) References 1. Holgerson LO, Miller RE, Zintel HA: Juvenile polyps of the colon . Surgery 1971;69:288-293. 2. Shermeta DW, Morgan WW, Eggleston J, et al: Juvenile retention polyps . J Pediatr Surg 1969;4:211-215.Crossref 3. Alexander RH, Beckwith JB, Morgan A, et al: Juvenile polyps of the colon and their relationship to allergy . Am J Surg 1970;120:222-225.Crossref 4. Soper RT: Intestinal polyps , in Holder T, Ashcraft K (eds): Pediatric Surgery . Philadelphia, WB Saunders Co, 1980, pp 481-494. 5. Haggitt RC, Pitcock JP: Familial juvenile polyposis of the colon . Cancer 1970;26:1232-1238.Crossref 6. Franken EA Jr, Gamet DJ, Fitzgerald JF, et al: Juvenile polyposis of the colon . Ann Radiol 1975;18:499-504. 7. Sachatello CR, Pickren JW, Grace JT: Generalized juvenile gastrointestinal polyposis: A hereditary disorder . Gastroenterology 1970;58: 699-708. 8. Peck DA, Watanabe JS, Trueblood HW: Familiar polyposis in children . Dis Colon Rectum 1972;15:23-29.Crossref 9. Watne AL, Lai H, Carrier J, et al: The diagnosis and surgical treatment of patients with Gardner's syndrome . Surgery 1977;82:327-333. 10. Turcot J, Despres MP, St Pierre F: Malignant tumor of the central neurosystem associated with familial polyposis of the colon . Dis Colon Rectum 1959;2:465-468.Crossref 11. Duzois R, Judd ES, Dahlin DC, et al: The Peutz-Jeghers syndrome: Is there a predisposition to the development of intestinal malignancy? Arch Surg 1969;98:509-517.Crossref 12. Cronkhite LW Jr, Canada WJ: Generalized gastrointestinal polyposis: An unusual syndrome of polyposis, pigmentation, alopecia and onychotrophia . N Engl J Med 1955;252:1011-1015.Crossref 13. Diner WC: The Cronkhite-Canada syndrome . Radiology 1972;105: 715-716.Crossref 14. Franken EA Jr: Lymphoid hyperplasia of the colon . Radiology 1970;94:329-334.Crossref 15. Gathright JB Jr, Cofer TW Jr: Familial incidence of juvenile polyposis coli . Surg Gynecol Obstet 1974;138:185-188. 16. Veale AM, McColl I, Bussey HJR et al: Juvenile polyposis coli . J Med Genet 1966;3:5-16.Crossref 17. Smilow PC, Pryor CA, Swinton NW: Juvenile polyposis coli: A report of three patients in three generations of one family . Dis Colon Rectum 1966;9:248-254.Crossref 18. McColl I, Bussey HJR, Veale AM, et al: Juvenile polyposis coli . J R Soc Med 1964;57:896-897.

Journal

Archives of Surgery – American Medical Association

Published: May 1, 1986

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