Pediatric Gastroenterology, Hepatology & Nutrition

The Korean Society of Pediatric Gastroenterology, Hepatology and Nutrition (대한소아소화기영양학회)
권호 표지
DOI QR코드

DOI QR Code

Clinical, Endoscopic and Pathologic Findings of Colonic Polyposis in Korean Children

소아 대장용종증 환자의 임상양상 및 내시경적, 조직학적 소견

  • Lim, Mi-Sun (Department of Pediatrics, Seoul National University College of Medicine) ;
  • Seo, Jeong-Kee (Department of Pediatrics, Seoul National University College of Medicine) ;
  • Ko, Jae-Sung (Department of Pediatrics, Seoul National University College of Medicine) ;
  • Yang, Hye-Ran (Department of Pediatrics, Seoul National University College of Medicine) ;
  • Kang, Gyeong-Hoon (Department of Pathology, Seoul National University College of Medicine) ;
  • Kim, Woo-Sun (Department of Radiology, Seoul National University College of Medicine)
  • 임미선 (서울대학교 의과대학 소아과학교실) ;
  • 서정기 (서울대학교 의과대학 소아과학교실) ;
  • 고재성 (서울대학교 의과대학 소아과학교실) ;
  • 양혜란 (서울대학교 의과대학 소아과학교실) ;
  • 강경훈 (서울대학교 의과대학 병리학교실) ;
  • 김우선 (서울대학교 의과대학 영상의학교실)
  • Received : 2010.08.13
  • Accepted : 2010.08.28
  • Published : 2010.09.30
Download PDF
⟨ Previous Next ⟩

Abstract

Purpose: Colonic polyposis is less common in children than in adults. The clinical data pertaining to colonic polyposis in children are limited. Children with colonic polyposis have complications associated with numerous polyps, malignant transformation of the polyps, and extraintestinal neoplasms. We studied the clinical spectrum, endoscopic characteristics, and histologic findings of colonic polyposis in Korean children. Methods: We reviewed the clinical data of 37 children with multiple colonic polyps between 1987 and 2009. The mean age at the time of diagnosis of colonic polyposis was 8.0${\pm}$3.2 years. Results: Peutz-Jeghers syndrome, juvenile polyposis syndrome, familial adenomatous polyposis (FAP), and lymphoid polyposis was diagnosed in 22, 7, 6, and 2 children, respectively. The most common clinical presentation in children with colonic polyposis was hematochezia. A family history of colonic polyposis was noted in 7 children. The colonoscopic findings of colonic polyposis varied with the size and number of polyps. The majority of polyps were multi-lobulatd and pedunculated in children with Peutz-Jeghers syndrome. The polyps in children with juvenile polyposis syndrome were primarily round and pedunculated. For the children with FAP, the colon was carpeted with small, sessile polyps. There were multiple sessile polyps in the patients with lymphoid polyposis. Surgical polypectomy was performed in 14 children (38%). Intestinal segmental resection was performed in 13 children (35%). Four patients with FAP underwent total colectomy. Four children with Peutz-Jeghers syndrome had extraintestinal neoplasms. No malignant transformation of polyp was identified. Conclusion: Children with colonic polyposis should undergo a careful initial evaluation and require periodic re-evaluation.

목 적: 대장용종증은 소아에서는 드문 질환군으로 다수의 용종으로 인한 출혈, 복통, 장중첩증 등이 반복될 수 있고 용종의 악성화나 장외종양이 발생할 수 있으나 아직은 이에 관한 연구가 많지 않다. 본 연구에서는 소아 대장용종증의 임상 양상과 내시경적, 조직학적 특징을 살펴보고자 하였다. 방 법: 서울대병원 어린이병원에서 1987년부터 2009년까지 대장내시경을 시행 받은 2,956명의 소아 환자중에서 대장용종증으로 진단받은 37명의 환자를 대상으로 의무기록 분석을 시행하였다. 대장용종증 환자들의 진단 시 평균나이는 8세였다. 결 과: Peutz-Jeghers 증후군이 22예로 가장 많았으며 연소성 용종증 7예, 가족성 선종성 용종증 6예, 림프성용종증 2예이었다. 내원 시 가장 흔한 주소는 혈변이었다. 50% 이상의 환자에서 혈변과 복통이 동반되었고 일부에서 항문종괴, 설사, 변비가 동반되었다. 용종의 수와 크기는 다양하였고 위장과 소장에 용종이 동반된 환자는 각각 21명, 17명이었다. Peutz-Jeghers 증후군 환자에서는 주로 다엽성의 목이 있는 용종이 관찰되었다. 연소성 용종증 환자에서는 둥글고 목이 있는 용종이 대부분이었다. 가족성 선종성 용종증 환자에서는 작고 둥글며 목이 없는 용종이 관찰되었다. 림프성 용종증 환자에서는 목이 없는 용종이 관찰되었다. 모든 환자는 내시경적 용종절제술을 시행받았고 14명(38%)은 수술적 용종절제술을 시행받았다. 부분장절제술을 시행받은 환자는 13명(35%)이었고, 가족성 선종성 용종증 환자 4명은 전대장절제술을 시행받았다. Peutz-Jeghers 증후군 환자 중 일부에서 장외 종양이 발생하였으나 용종의 악성화는 없었다. 결 론: 소아의 대장용종증 환자는 출혈, 복통 등의 증상을 보이며 장중첩증 등의 합병증이 발생할 수 있어 조기 진단과 치료가 필요하며 정기적인 대장내시경 검사를 통해 합병증을 예방하고 용종의 악성화나 장외 종양 여부를 확인해야 한다.

Keywords

References

  1. Kim SJ, Kim SM, Kim YJ, Jeong DC, Lee WB, Chung SY, et al. Colonic polyps; experience of 34 cases in two hospitals. Korean J Pediatr 2004;47:756-61.
  2. Gelb AM, Minkowitz S, Tresser M. Rectal and colonic polyps occurring in young people. N Y State J Med 1962; 62:513-8.
  3. Mestre JR. The changing pattern of juvenile polyps. Am J Gastroenterol 1986;81:312-4.
  4. Poddar U, Thapa BR, Vaiphei K, Singh K. Colonic polyps: experience of 236 Indian children. Am J Gastroenterol 1998;93:619-22. https://doi.org/10.1111/j.1572-0241.1998.176_b.x
  5. Durno CA. Colonic polyps in children and adolescents. Can J Gastroenterol 2007;21:233-9. https://doi.org/10.1155/2007/401674
  6. Latt TT, Nicholl R, Domizio P, Walker-Smith JA, Williams CB. Rectal bleeding and polyps. Arch Dis Child 1993;69:144-7. https://doi.org/10.1136/adc.69.1.144
  7. Longo WE, Touloukian RJ, West AB, Ballantyne GH. Malignant potential of juvenile polyposis coli. Report of a case and review of the literature. Dis Colon Rectum 1990;33:980-4. https://doi.org/10.1007/BF02139111
  8. Goh PG, Moon HS, Sung JK, Jeong HY, Song KS. A case of Peutz-Jeghers syndrome with intraductal papillary mucinous carcinoma of pancreas. Korean J Gastroenterol 2010;55:73-7. https://doi.org/10.4166/kjg.2010.55.1.73
  9. Kim JO, Kong HP, Park KS, Kim WS, Lee CH. A case of nonfamilial generalized gastrointestinal juvenile polyposis with pancytopenia. Korean J Pediatr 1996;39: 423-9.
  10. Kwak JW, Kim HY, Park JH. Polypectomy by intraoperative total gut endoscopy in a child with Peutz- Jeghers syndrome. Korean J Pediatr Gastroenterol Nutr 2005;8:76-80.
  11. Jean-Francois M, Sylviane O, Michel P, Intestinal polyps and polyposis. In: Walker WA, Walker SJ, editors. Pediatric gastrointestinal disease. 5th ed. Hamilton: BC Decker Inc, 2008:637-52.
  12. Elitsur Y, Teitelbaum JE, Rewalt M, Nowicki M. Clinical and endoscopic data in juvenile polyposis syndrome in preadolescent children: a multicenter experience from the United States. J Clin Gastroenterol 2009;43:734-6. https://doi.org/10.1097/MCG.0b013e3181956e0c
  13. Kang BY, Han SJ, Lee JE, Choi SK, Kim JM, Hong YJ, et al. A case of juvenile polyposis presented with protein losing enteropathy. Korean J Pediatr Gastroenterol Nutr 2003;6:208-14.
  14. Chang MS, Kim YI. Intestinal polyposis: a clinicopathologic analysis of 28 resected cases with special reference to surgical intervention. Korean J Gastroenterol 1990;23:62-74.
  15. Farmer RG, Hawk WA, Turnbull RB, Jr. The spectrum of the Peutz-Jeghers syndrome. Report of 3 cases. Am J Dig Dis 1963;8:953-61. https://doi.org/10.1007/BF02232093
  16. Calva D, Howe JR. Hamartomatous polyposis syndromes. Surg Clin North Am 2008;88:779-817. https://doi.org/10.1016/j.suc.2008.05.002
  17. Jass JR. Colorectal polyposes: from phenotype to diagnosis. Pathol Res Pract 2008;204:431-47. https://doi.org/10.1016/j.prp.2008.03.008
  18. Kojima M, Itoh H, Motegi A, Sakata N, Masawa N. Localized lymphoid hyperplasia of the rectum resembling polypoid mucosa-associated lymphoid tissue lymphoma: a report of three cases. Pathol Res Pract 2005;201:757-61. https://doi.org/10.1016/j.prp.2005.07.007
  19. Seo JK. Therapeutic colonoscopy in children: endoscopic snare polypectomy and juvenile polyps. Seoul J Med 1993;34:285-94.
  20. Howe JR, Ringold JC, Hughes JH, Summers RW. Direct genetic testing for SMAD4 mutations in patients at risk for juvenile polyposis. Surgery 1999;126:162-70. https://doi.org/10.1016/S0039-6060(99)70150-9
  21. Schmid C, Vazquez JJ, Diss TC, Isaacson PG. Primary B-cell mucosa-associated lymphoid tissue lymphoma presenting as a solitary colorectal polyp. Histopathology 1994;24:357-62. https://doi.org/10.1111/j.1365-2559.1994.tb00537.x
  22. Byrne WJ, Jimenez JF, Euler AR, Golladay ES. Lymphoid polyps (focal lymphoid hyperplasia) of the colon in children. Pediatrics 1982;69:598-600.
  23. Scully RE. Sex cord tumor with annular tubules a distinctive ovarian tumor of the Peutz-Jeghers syndrome. Cancer 1970;25:1107-21. https://doi.org/10.1002/1097-0142(197005)25:5<1107::AID-CNCR2820250516>3.0.CO;2-7
  24. Van Lier MG, Wagner A, Mathus-Vliegen EM, Kuipers EJ, Steyerberg EW, Van Leerdam ME. High cancer risk in Peutz-Jeghers syndrome: a systematic review and surveillance recommendations. Am J Gastroenterol 2010; 105:1258-64. https://doi.org/10.1038/ajg.2009.725
  25. Barnard J. Screening and surveillance recommendations for pediatric gastrointestinal polyposis syndromes. J Pediatr Gastroenterol Nutr 2009;48(Suppl 2):S75-8. https://doi.org/10.1097/MPG.0b013e3181a15ae8
  26. Attard TM, Young RJ. Diagnosis and management of gastrointestinal polyps: pediatric considerations. Gastroenterol Nurs 2006;29:16-22; quiz 3-4. https://doi.org/10.1097/00001610-200601000-00003
  27. Park JG, Kim IJ. Hereditary colorectal cancer. Korean J Gastroenterol 2005;45:78-87.
  28. Vasen HF, Moslein G, Alonso A, Aretz S, Bernstein I, Bertario L, et al. Guidelines for the clinical management of familial adenomatous polyposis (FAP). Gut 2008;57: 704-13. https://doi.org/10.1136/gut.2007.136127
  29. Merg A, Howe JR. Genetic conditions associated with intestinal juvenile polyps. Am J Med Genet C Semin Med Genet 2004;129C:44-55. https://doi.org/10.1002/ajmg.c.30020
  30. Volikos E, Robinson J, Aittomaki K, Mecklin JP, Jarvinen H, Westerman AM, et al. LKB1 exonic and whole gene deletions are a common cause of Peutz- Jeghers syndrome. J Med Genet 2006;43:e18.
  31. Yang HR, Ko JS, Seo JK. Germline mutation analysis of STK11 gene using direct sequencing and multiplex ligation-dependent probe amplification assay in Korean children with Peutz-Jeghers syndrome. Dig Dis Sci 2010

Cited by

  1. Peutz-Jeghers Syndrome with Adenomatous Change in a Fifteen-month-old Boy vol.66, pp.2, 2010, https://doi.org/10.4166/kjg.2015.66.2.106
  2. Juvenile Polyp associated with Hypovolemic Shock Due to Massive Lower Gastrointestinal Bleeding vol.22, pp.6, 2010, https://doi.org/10.5223/pghn.2019.22.6.613