• Imaging Science in Dentistry
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• 제46권4호
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• pp.267-272
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• 2016

Gardner syndrome is known as a variant of familial adenomatous polyposis. This syndrome is characterized by multiple intestinal polyposes, osteomas, and epidermoid cysts. In addition, dental abnormalities include an increased frequency of multiple odontomas, as well as supernumerary and impacted teeth. The authors report the case of a 7-year-old male patient with Gardner syndrome. Radiographic findings revealed multiple osteomas in both sides of the maxilla, multiple diffuse enostoses in both jaws, and a complex odontoma in the left mandibular body. Two years later, multiple epidermoid cysts on the scalp were found. Since this patient was suspected to have Gardner syndrome, the authors recommended gastrointestinal endoscopy to check for intestinal polyposis. Gastrointestinal endoscopic examination revealed multiple polyposes in the upper gastrointestinal tract and fundus of the stomach. As a result, the final diagnosis was Gardner syndrome.

• 대한소화기학회지
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• 제72권6호
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• pp.277-280
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• 2018

Although small bowel the mainly occupies the most part of the gastrointestinal tract, small intestine tumors are rare, insidious in clinical presentation, and frequently represent a diagnostic and management challenge. Small bowel tumors are generally classified as epithelial, mesenchymal, lymphoproliferative, or metastatic. Familial adenomatous polyposis and Peutz-Jeghers syndrome are the most common inherited intestinal polyposis syndromes. Until the advent of capsule endoscopy (CE) and device-assisted enteroscopy (DAE) coupled with the advances in radiology, physicians had limited diagnostic examination for small bowel examination. CE and new radiologic imaging techniques have made it easier to detect small bowel tumors. DAE allows more diagnosis and deeper reach in small intestine. CT enteroclysis/CT enterography (CTE) provides information about adjacent organs as well as pictures of the intestinal lumen side. Compared to CTE, Magnetic resonance enteroclysis/enterography provides the advantage of soft tissue contrast and multiplane imaging without radiation exposure. Treatment and prognosis are tailored to each histological subtype of tumors.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제6권2호
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• pp.208-214
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• 2003

저자들은 전신부종과 심한 저알부민혈증으로 내원한 환아에서 테그네슘표지 인혈청알부민 스캔을 이용하여 결장에서 단백이 소실되는 것을 확인하고, 바륨 관장 대장 조영술 검사, 상부 위장관 촬영, 소장 조영술, 대장과 십이지장 내시경 검사를 통해 단백 소실 장증으로 발현한 연소성 용종증을 진단하여, 전대장 절제술 후 단백 소실 장증이 호전된 증례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

• Asian Pacific Journal of Cancer Prevention
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• 제15권16호
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• pp.6945-6948
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• 2014

Background: Familial adenomatous polyposis (FAP) is a disease inherited in an autosomal dominant fashion. Most FAP patients develop upper gastrointestinal polyps; especially those in the antrum and duodenum are usually neoplastic. The aim of this study was to evaluate the prevalence of gastroduodenal polyps in Iranian FAP patients. Materials and Methods: 28 patients affected by FAP underwent front-view and side-view endoscopy. Papillary biopsies were performed in all patients. Location of polyps, their number and size, pathology study, patient general information (gender, age, family history of FAP or colorectal cancer and gastroduodenal polyps) were analyzed. Results: Gastric polyps were seen in 39.3 % of patients. Some 72.7% of the affected individuals had fundic gland polyps and 36.36% had hyperplastic polyps. Duodenal adenoma was observed in 25% of patients. While 57% of patients had tubular adenoma with low grade dysplasia, 42.8% showed tubulovillous adenoma with low grade dysplasia. Conclusions: Findings of this study indicated that the prevalence of gastroduodenal polyps in FAP patients is high and dysplasia may be evident in duodenal polyps. Therefore, it appears that routine gastroduodenal endoscopy in FAP patients is necessary.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제23권1호
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• pp.115-120
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• 2020

Klippel-Trenaunay syndrome (KTS) is a rare disorder characterized by a triad of abnormal bone and soft tissue growth, the presence of a port-wine stain, and venous malformations. Gastrointestinal (GI) manifestations of KTS are relatively common and generally do not cause significant problems. However, persistence can lead to chronic GI blood loss or even massive bleeding in rare cases. The majority of the severe GI manifestations associated with KTS present as vascular malformations around the GI tract and exposed vessels can lead to serious bleeding into the GI tract. Herein, we report a case of a 16-year-old boy with severe iron deficiency anemia who was previously misdiagnosed as hemorrhoid due to small amount of chronic bleeding. The actual cause of chronic GI bleeding was from an uncommon GI manifestation of KTS as rectal polyposis.

• Maxillofacial Plastic and Reconstructive Surgery
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• 제35권6호
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• pp.421-426
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• 2013

Gardner syndrome, an autosomal dominant inherited condition, is a subtype of familial adenomatous polyposis. It causes lesions in bones, skin, and teeth, as well as multiple gastrointestinal polyps, which, if left untreated, become malignant. Because patients with colorectal cancer have a low survival rate, early diagnosis and treatment of Gardner syndrome is critical. Therefore, the characteristic lesions of Gardner disease that appear on the face, jaws, and oral cavity must be understood; these can be evaluated by oral and maxillofacial clinicians. This report describes a case that was diagnosed and treated earlier with the help of a routine oral and maxillofacial examination and has had a seemingly good prognosis so far.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제7권1호
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• pp.112-118
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• 2004

위장관에서 용종성 병변이 있을 때 Cowden 증후군의 감별이 필요하며 소아 연령에서는 모든 증상이 발현되지 않으므로 추적 관찰이 필요하리라 판단된다. 특히, 식도의 극세포증은 모습이 독특하고 거의 대부분의 증례에서 보고되고 있어 최근 Cowden 증후군의 진단 시 질병특유의 기준으로 활용되기도 하므로 특히 염두에 둘 필요가 있으며, 그러나 소아 연령에서는 적절한 나이가 될 때까지는 극세포증의 발현이 지연될 수 있음도 반드시 고려하여야 할 것으로 판단된다.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제13권1호
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• pp.1-6
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• 2010

The small bowel is the most difficult intestinal segment to examine. Radiologic tests are mostly insensitive and double-balloon enteroscopy is unsuitable for the younger child. Capsule endoscopy is a novel wireless method of investigation of the small bowel. The primary indications for capsule endoscopy include evaluation of obscure gastrointestinal bleeding, small bowel Crohn's disease, and polyposis syndromes. Capsule endoscopy offers an accurate and effective means of investigating the small bowel in children. Capsule retention is a potential complication of capsule endoscopy. This review provides the indications, safety, and limitations of wireless capsule endoscopy in children.

• Journal of Digestive Cancer Reports
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• 제9권2호
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• pp.60-67
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• 2021

Tumors of the small intestine are rare and generally asymptomatic or with nonspecific symptoms. The small intestine is difficult to approach using conventional endoscopy, and early diagnosis of the small intestinal tumors is difficult. Therefore, many of the small intestinal tumors are diagnosed at an advanced stage, which makes the prognosis poor. Premalignant lesions of the small intestine or known risk factors of small bowel cancer are sporadic adenoma, adenoma associated with familial adenomatous polyposis, hamartomatous polyp associated with Peutz-Jeghers syndrome, Crohn's disease, and celiac disease. Therefore, it is necessary to recognize that the small bowel cancer can occur in these patients with premalignant lesions or risk factors of small bowel cancer. To reduce the possibility of small bowel cancer or to detect at an earlier stage, attention should be paid to screening and surveillance of these patients with premalignant lesions or risk factors of the small bowel cancer.

• Advances in pediatric surgery
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• 제3권1호
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• pp.54-60
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• 1997

Eight pediatric patients with Peutz-Jeghers syndrome were treated from 1984 to 1994 at the Department of Surgery, Seoul National University Children's Hospital. We reviewed the clinical features of our own 8 cases as well as 56 cases reported in Korean literatures. The results were compared to those of western reports. Gastrointestinal complications in our series were more frequent than in the Korean series, but the distribution of polyps was similar. The Korean series showed the following characteristics compared to the western reports; demographic backgrounds were similar; gastrointestinal symptoms were more common; location of the polyp was more frequent in colon (2 times); and the most prevalent site of malignant change was the colon in Korean cases.