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

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+/-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.

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

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+/-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.

Non-familial paediatric colorectal cancers - an 18 years’ experience.

Background: Primary gastrointestinal malignancies constitute only 1% of all paediatric neoplasms. Aim: The aim of this study was to describe our 18 years’ experience with non-familial paediatric colorectal malignancies, outlining pertinent features of diagnosis, treatment and outcome. Methods: 9 patients of non-familial paediatric colorectal malignancies were admitted in PGIMS, Rohtak, Haryana between 1990 and 2008. After the initial surgical management, the advanced cases underwent chemotherapy and radiation therapy where required and were followed up. Results: There were six male and three female patients (age range: 7 to 16 years). Three tumours arose in the rectum, three in the sigmoid colon, one each in the splenic flexure and appendix, and there was one case of diffuse colonic polyposis. All cases presented with obstruction and rectal bleeding. Two cases of sigmoid carcinoma were unresectable and expired 4 months post-surgery. The rest responded to radical resection. Three patients required palliative radiation therapy. Due to the advanced stage, chemotherapy was given to all the carcinoma patients. One patient had local recurrence after 5 months and another developed distant metastasis. The rest are on follow-up and clinically and radiologically disease free. Conclusion: Paediatric colorectal malignancy is a rare entity, usually diagnosed in the later stages, culminating in advanced disease. A majority of cases undergo radical resection due to the advanced stage of presentation. Advanced stages may also require chemotherapy and radiation therapy.

Diagnosis of Cowden's Disease Based on Gastrointestinal Manifestations / 대한소화기내시경학회지

BACKGROUND/AIMS: Cowden's disease is an autosomal dominant hereditary disease characterized by the various hamartomatous and neoplastic lesions of multiple organs. We analyzed gastrointestinal manifestations of 5 cases of Cowden's disease and suggest several findings which are helpful to gastroenterologists for the early diagnosis. METHODS: The clinical characteristics of 5 unrelated patients with Cowden's disease were evaluated. Four patients were male, one patient was female, and their ages at the time diagnosis ranged from 17 to 49 years. All patients had the pathognomonic mucocutaneous lesions and thyroid nodules. RESULTS: In all patients, the esophagus was affected by acanthosis. In 4 patients, the stomach was affected by numerous variable sized polyps. In 4 patients, the duodenum was involved by several polyps. In 4 patients, the entire small bowel and in one patients, only the terminal ileum was affected by numerous polyps. In all patients, the colon, especially the sigmoid colon and rectum, showed numerous variable sized polyps. Family history was positive for stomach cancer in two patients. CONCLUSIONS: Cowden's disease should be considered in patients with esophageal acanthosis among patients with colonic polyposis, although the mucocutaneous lesions, unfamiliar to gastroenterolgists, are pathognomonic criteria for the diagnosis.

A Case Report of Segmental Non-Familial Colonic Polyposis / 대한소화기내시경학회지

Segmental non-familial colonic polyposis was first reported by Chiang et al. in 1992. It is characterized by segmental distributlion of colonic polyposis usually confined to the descending colon, absence of family history of polyposis, large bowel malignancy, inflammatory bowel disease, or other pre-malignant colonic conditions. We experienced a nineteen-year-old male, who suffered from 2 years watery diarrhea about five to ten times a day, intermittent hematochezia, and weight loss of 12 kg in a year. He had no family history of colonic polyps, colon cancer, or inflammatory bowel disease. Colon study showed variable sized multiple colonic polyps on the rectum and sigmoid colon. Colonoscopy showed that 0.5 to 1.5 cm sized multiple polyps were scattered from the 6cm site to the 30cm site above the anal verge and the intervening mucosa between polyps was edematous. Colonoscopic biopsy revealed hyperplastic, adenomatous, and mixed hyperplastic and adenomatous polyps, After partial colectomy, we discovered 169 polyps from the resected specimen. After operation, diarrhea and abdominal pain had disappeared. We experienced a case of segmental non-familial polyposis and report it with review of the literatures related to it.