Cronkhite-Canada Syndrome (CCS) is a syndrome characterised by a constellation of signs including but not limited to onychodystrophy of the finger and toe nails, skin hyperpigmentation and alopecia. Endoscopic features showed hamartomatous polyps involving all segments of the gastrointestinal tract with the characteristic exception of being oesophageal sparring. These polyps show confirmation by the presence of eosinophils and mast cells at the lamina propria upon histological studies.
Intestinal Polyposis

No abstract available.
Intestinal Polyposis*

Humans ; Intestinal Polyps ; Colonic Polyps/surgery* ; Intestinal Polyposis ; Colorectal Neoplasms

The Cronkhite-Canada Syndrome (CCS) is a monfamilial disorder of adults characterized by diffuse gastro-intestinal polyposis, ectodermal changes consisting of alopecia, dystrophy of nails and cutaneous hyper-pigmentation. The pathogenesis and the causes of CCS remain unknwon but the symptoms such as diarrhea and malnutrition are generally progressive, and the prognosis is knwon to be poor. Though the definitive treatment is not well known, it has been reported that the conservative management is the most important treatment, and that the clinical sourse can be reversible. One case was experienced involving Cronkhite-Canada Syndromen in a patient who was managed by conservative treatment and an other who was treated by corticosteroid. These two cases are reported herein with a review of corresponding literature.
Adult ; Alopecia ; Canada* ; Diarrhea ; Ectoderm ; Humans ; Intestinal Polyposis ; Malnutrition ; Prognosis

The Cronkhite-Canada syndrome is a rare, non-neoplastic, non-hereditary condition which consists of gastrointestinal polyposis associated with alopecia, onychodystrophy and hyperpigmentation of the skin. It usually encountered with severe diarrhea, weight loss and other malabsorption symptoms. The etiology of the condition is unknown. Usually, this syndrome is associated with a poor prognosis due to the effect of malabsorption and generalized debility. We report a case of this syndrome showing unusually rapid improvement after 3-month following only conservative treatment. To our knowledge, this is the first case report of Cronkhite-Canada syndrome in the Karean dermatologic literature.
Alopecia ; Diarrhea ; Hyperpigmentation ; Intestinal Polyposis* ; Prognosis ; Skin ; Weight Loss

BACKGROUND/AIMS: A flexible spectral imaging color enhancement system was installed in new capsule software for video capsule endoscopy. Contrast image capsule endoscopy (CICE) is a novel technology using light-emitting diodes selected for the main absorption range of hemoglobin. We assessed the feasibility and diagnostic effi cacy for small bowel surveillance in patients with polyposis syndromes. METHODS: Six patients with polyposis syndromes, four with familial adenomatous polyposis and one each with Cowden syndrome (CS) and Cronkhite-Canada syndrome (CCS) were examined using CICE. We conducted three evaluations to assess the effect on the numbers of the detected polyps; compare polyp diagnostic rates between adenoma and hamartoma; and assess polyp visibility. RESULTS: The numbers of detected polyps and diagnostic accuracy did not differ signifi cantly between pre-contrast and contrast images. However, 50% of the adenomatous polyps displayed enhanced visibility on contrast images. CICE contrast images exhibited clearly demarcated lesions and improved the visibility of minute structures of adenomatous polyps. Hamartomatous polyp micro-structures in patients with CS and CCS were more clearly visualized on contrast than pre-contrast images. CONCLUSIONS: CICE is an effective tool for enhancing the visibility of polyps in patients with polyposis syndrome.
Absorption ; Adenoma ; Adenomatous Polyposis Coli ; Adenomatous Polyps ; Capsule Endoscopes ; Capsule Endoscopy ; Hamartoma Syndrome, Multiple ; Hemoglobins ; Humans ; Intestinal Polyposis ; Polyps

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.
Adenomatous Polyposis Coli ; Capsule Endoscopy ; Diagnosis ; Gastrointestinal Tract ; Intestinal Polyposis ; Intestine, Small ; Peutz-Jeghers Syndrome ; Prognosis ; Radiation Exposure

Laugier-Hunziker syndrone is a rare, pigmentary disorder of the lips, oral mucosa, and nails. The absence of intestinal polyposis differentiated it from Peutz-Jeghers syndrome. We describe a 16-year-old boy with multiple, brownish, lenticular macules on the lips, oral mucosa, perianal area and anal mucosa, and on the digits of his fingers and toes, in wham intestinal polyposis was not detectable.
Adolescent ; Fingers ; Humans ; Intestinal Polyposis ; Lip ; Male ; Mouth Mucosa ; Mucous Membrane ; Peutz-Jeghers Syndrome ; Toes

Familial juvenile polyposis is a rare intestinal polyposis characterized by the occurrence of multiple juvenile polyps in the gastrointestinal tract. We report a case of familial juvenile polyposis in a 17-year-old man with a review of the literature. This patient underwent total colectomy due to a 6 years history of rectal bleeding. Grossly, the colon showed 36 variable sized pedunculated polyps, measuring 2.5cm x 2cm from the largest size and 0.2cm x 0.2cm to the smallest size. Histologically, the polyps consisted of cystically dilated glands, lined by normal colonic epithelial cells, scattered in loose, edematous stroma containing inflammatory cell infiltration. There were no areas of tubular adenoma or malignancy in any of the polyp.
Adenoma ; Adolescent ; Colectomy ; Colon ; Epithelial Cells ; Gastrointestinal Tract ; Hemorrhage ; Humans ; Intestinal Polyposis ; Polyps

Desmoid tumors are defined as aggressive overgrowth of fibrous sheets and musculoaponeurotic structures. Although desmoid tumors are generally known as a benign neoplasm, it's aggresive local invasiveness and frequent recurrence indicate it's position lying between the benign and malignancy. The association of desmoid tumor and familial adenomtous polyposis(FAP) was first made in 1923 by Nichols. In 1951, Gardner reported the familial occurrence of intestinal polyposis, osteomas, fibromas, and epidermal or sebaceous cyst. Desmoid tumors are common in patients with FAP and Occur in 3.5~29% of patients with FAP whereas the incidence in the gerenal population is 2~5/1,000,000 person years. Surgical resection of desmoids in patients with FAP has been controversial because unresectability and recurrence are more common than cure. Palliative and curative resections have a high morbidity. Surgery should be reserved for those patients with symptomatic mesenteric desmoids. If a small mesenteric desmoid is encountered incidentally and is easily resectable, it should be resected. If surgery has been less than satisfactory in the treatment of these patients, several different medical approaches can be combined with or without surgical resection with mixed result. Authors report a case of unresectable mesenteric desmoid tumor, developing after surgery of FAP and literatures were reviewed
Deception ; Epidermal Cyst ; Fibroma ; Fibromatosis, Aggressive* ; Humans ; Incidence ; Intestinal Polyposis ; Osteoma ; Recurrence