Benign Neoplasms of the Colon and Rectum
Philip H. Gordon, Santhat Nivatvongs, Lee E. Smith, Scott Thorn Barrows, Carla Gunn, Gregory Blew, David Ehlert, Craig Kiefer, Kim Martens in Neoplasms of the Colon, Rectum, and Anus, 2007
Bowel resection is reserved for cases in which complications such as carcinoma, bleeding, intussusception, and rectal prolapse develop (115). Surgery is not usually performed for improvement of protein-losing gastroenteropathy because the protein losing is usually not localized (120). Hanzawa et al. (120) reported a patient with Cronkhite-Canada syndrome with numerous polyps in the stomach, duodenum, and from cecum to transverse colon. The patient had severe hypoproteinemia and peripheral edema, unresponsive to conservative treatment including elemental diet and hyperalimentation. Scintigraphy with technetium TC99m-labeled human albumin (121,122) demonstrated a protein-losing region in the ascending colon. An ileo-right colectomy was performed. After the operation, the protein-losing enteropathy stopped; the ectodermal changes improved, and other polyps that was a secondary cause to malnutrition regressed.
Nail changes in systemic diseases and drug reactions
Eckart Haneke in Histopathology of the NailOnychopathology, 2017
Cronkhite–Canada syndrome is a very rare condition characterized by nonhereditary gastrointestinal polyposis with diarrhea, dysgeusia, fatigue, weight loss, skin hyperpigmentation, alopecia, and nail dystrophy. Fecal occult blood is often positive. Hemoglobin, serum potassium, calcium, and protein may be below the normal range. Colonoscopy reveals multiple polyps of different types.79 Nail alterations are almost always present with a peculiar soft spongy area of the proximal nail and thickened irregular hard distal nail.80 Nail shedding often occurs.81 The disease is usually progressive and may eventually be fatal. Recently, an autoimmune pathogenesis was assumed as there is a strong IgG4 staining in the polyps.82 The risk of colorectal cancer is estimated to be 25%. Nutritional support and systemic corticosteroids are the treatment of choice.83
Cronkhite−Canada Syndrome
Dongyou Liu in Handbook of Tumor Syndromes, 2020
Cronkhite−Canada syndrome (CCS) is a rare sporadic/non-inherited disorder with an adult-age onset. Clinically, CCS is characterized by epithelial disturbances in both the gastrointestinal tract and epidermis, ranging from gastrointestinal hamartomas, juvenile type gastrointestinal polyps (often associated with diarrhea), colorectal serrated adenomas, ectodermal dysplasia, skin hyperpigmentation (darkening skin on the hands, arms, palms, soles, neck, and face), alopecia (hair loss), onychodystrophy (nail atrophy), peripheral edema (excess fluid accumulation in arms and legs), to malabsorption. This is accompanied by increased malignant transformation (with gastrointestinal cancer and multiple myeloma or Kahler's disease occurring in up to 15% of cases) and unprovoked thromboembolism, leading to a high mortality of 55% [1,2].
Cronkhite-Canada syndrome: a retrospective analysis of four cases at a single medical center
Published in Scandinavian Journal of Gastroenterology, 2022
Xing Yu, Chengdang Wang, Mi Wang, Yinchen Wu, Linlin Zhang, Qinyu Yang, Long Chen
Cronkhite-Canada syndrome (CCS) is an extremely rare disease with a prevalence rate of 3.7 (male: 4.0; female: 3.5) per 1,000,000 population [1] and is characterized by multiple gastrointestinal polyps and ectodermal abnormalities [2]. Since the disease was first reported by Cronkhite and Canada in 1955 [3], more than 500 cases have been documented worldwide, of which 75% were from Japan [4]. Analysis of CCS data from Japan and China showed that the average age of onset was 63.5 and 57.39 years, respectively, and the condition tended to be more common in males than females [5,6]. At present, the diagnosis of CCS mainly depends on typical symptoms, including gastrointestinal disorders, ectodermal dysplasia, and endoscopic observations [7]. Usually, patients have at least one manifestation of alopecia, nail dystrophy, and skin pigmentation. Gastrointestinal symptoms, such as diarrhea, abdominal pain, and hypogeusia have also been observed [4]. Diffuse multiple polyps of various shapes can be distributed throughout the digestive tract, most commonly in the stomach and colon [5].
Cronkhite–Canada syndrome causing pouch outlet obstruction 5 years after roux-en-y gastric bypass
Published in Acta Chirurgica Belgica, 2019
Ben Gys, Joren Mertens, Martin Ruppert, Guy Hubens
Cronkhite–Canada syndrome is a rare noncongenital gastrointestinal polyposis syndrome characterized by epithelial disturbances in the gastrointestinal tract and epidermis. Symptoms consist of diarrhea, weight loss, abdominal pain and dermatological problems like cutaneous hyperpigmentation, dystrophic changes of finger- and toenails and alopecia. The syndrome typically presents itself in the 5th decade and has a slight male predominance (3:2 ratio) [3].
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