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 Table of Contents  
LETTER TO EDITOR
Year : 2014  |  Volume : 3  |  Issue : 1  |  Page : 70-72

Cronkhite-Canada syndrome: A rare form of gastrointestinal polyposis causing malabsorption


1 Department of Internal Medicine, Sher i Kashmir Institute of Medical Sciences, Soura, Srinagar, Jammu and Kashmir, India
2 Department of Gastroenterology, Sher i Kashmir Institute of Medical Sciences, Soura, Srinagar, Jammu and Kashmir, India

Date of Web Publication15-Apr-2014

Correspondence Address:
Faheem Arshad
133, Housing Colony Sanatnagar Rawalpora, Srinagar 190 005, Jammu and Kashmir
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2278-344X.130625

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How to cite this article:
Arshad F, Mir RM, Javaid G, Khan MA. Cronkhite-Canada syndrome: A rare form of gastrointestinal polyposis causing malabsorption. Int J Health Allied Sci 2014;3:70-2

How to cite this URL:
Arshad F, Mir RM, Javaid G, Khan MA. Cronkhite-Canada syndrome: A rare form of gastrointestinal polyposis causing malabsorption. Int J Health Allied Sci [serial online] 2014 [cited 2019 Sep 18];3:70-2. Available from: http://www.ijhas.in/text.asp?2014/3/1/70/130625

Sir,

Cronkhite-Canada syndrome (CCS) is rare non-familial disorder of unknown etiology reported for the first time in 1955 by Cronkhite and Canada [1] as a distinct clinical entity in two female patients with generalized gastrointestinal (GI) polyps and ectodermal manifestations. Since then only 467 cases have been reported in the world literature. This condition is characterized by diffuse GI polyposis, dystrophic changes in the fingernails, alopecia, cutaneous pigmentation, diarrhea, weight loss and abdominal pain. The purpose of reporting this rare case is to remind clinician that despite no definitive treatment, early recognition and initiation of appropriate symptomatic management can markedly improve quality of life in these patients.

A 55-year-old-male, chronic smoker was referred to our tertiary institute with 1 year history of loose stools and abdominal pain, alopecia for 3 months, lower limb swelling for 2 months, pigmentation over palms and soles for 1 month. There was no history of fever, drug abuse or similar complaints in family. On examination he was moderately built with height of 158 cm and weight of 47 kg, he had alopecia, hyperpigmentation on hands and feet, dystrophic fingernail changes [Figure 1] and lower limb edema. Oral cavity was normal. Systemic examination was unremarkable. On evaluation his hemogram and blood chemistry was normal except for hypoalbuminemia 2.3 g/dl (normal range 3.5-5.3) and low total protein level 3.7 g/dl (5.5-7.5). His stool examination was negative for fat and did not reveal any parasites, ova or cysts. His urinary D-xylose test was positive for malabsorption (0.7 g/5 h). Anti-nuclear antibody (ANA) was negative. Ultrasound scan abdomen revealed moderate fatty liver with two hepatic cysts in right lobe. Computed tomography enterography [Figure 2] showed multiple enhancing polypoidal lesions seen involving sigmoid, descending, transverse and ascending colon, few enhancing polyps were also seen in small bowel loops and liver had multiple hypodense lesions likely benign hepatic cysts. He underwent upper GI tract endoscopy, which revealed multiple scattered sessile polyps of varying sizes in the stomach involving proximal body and fundus, distal body and antrum show carpeting of polyps [Figure 3]. This was suspicious of polyposis syndrome in this patient and was subjected to colonoscopy which also revealed entire colon studded with polyps. Histopathological examination of biopsies taken from the polyps showed hyperplastic polyps [Figure 4]. Thus a detailed history, clinical features, endoscopic findings and histopathology was consistent with diagnosis of CCS. He was managed with a high protein diet, multivitamin supplements and H 2 receptor antagonists followed by proton pump inhibitors. After a month of his follow-up there was a marked improvement in his symptoms as well as hyperpigmentation, his laboratory parameters showed albumin levels of 3.2 g/dl and total protein 4.5 g/dl.
Figure 1: Hyperpigmentation and nail dystrophic changes

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Figure 2: Computed tomography enterography showing multiple enhancing polypoidal lesions seen involving sigmoid, descending, transverse and ascending colon

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Figure 3: Endoscopic view of carpet like polyposis of stomach

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Figure 4: Histopathological examination of biopsies taken from the polyps showed hyperplastic polyps

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Current understanding of CCS is based on anecdotal reports. The etiology remains unknown. Cronkhite and Canada described first 2 cases of this syndrome with generalized GI polyposis with alopecia, hyperpigmentation and nail changes, suggested ectodermal changes as a part of generalized deficiency state and their prominence may be related to extensive distribution of lesions in all segments of digestive tract in form of polyps. Associations with raised ANA and IgG4 levels, hypothyroidism and various autoimmune diseases such as systemic lupus erythematous, rheumatoid arthritis and scleroderma have been reported. [2] With an estimated incidence is 1/million, it usually manifests in the 5 th to 6 th decade and is characterized by presence of non-adenomatous juvenile type or hamartomatous polyps that occur throughout the GI excluding the esophagus. [3] The polyps are a part of a generalized GI mucosal disturbance that results in malabsorption and protein losing enteropathy as the disrupted mucosa cannot digest disaccharides or absorb carbohydrates and lipids. Blonski et al. [4] also attributed the ectodermal features and various clinical courses to malabsorption and profound malnutrition. Endoscopic findings of CCS vary from multiple sessile to semi pedunculated polyps ranging from 0.5 to 2 cm in diameter located principally in the colon but also in the stomach (carpet-like polyposis) and small intestine. Colonic polyps have been defined as sessile and "strawberry like" in one study. [5] Although rate of malignant transformation is low, Nagata et al. [6] suggest that CCS has a definite malignant potential observed in up to 15% of patients. Both gastric and colon adenocarcinomas have been reported. Thus highlighting the importance of regular follow-up with upper endoscopy and colonoscopy in these patients. Due to rarity of CCS no optimal treatment is recommended. Different treatment modalities have been used with varying degrees of success which include hyperalimentation, corticosteroids as anti-inflammatory, H 2 -receptor antagonists, proton pump inhibitors, antibiotics, surgery and combinations therapies. [7] The beneficial effects of antibiotics are attributed to small-bowel overgrowth. Supplementary nutrition can be life-saving in these patients as the first 2 reported cases of CCS died of starvation after onset of symptoms. Lipin et al. in their study have reported a case of CCS who showed complete recovery in symptoms after initiation of treatment with enteral supplements. [8] Our patient received multivitamin therapies and acid suppressants with marked improvement and is under endoscopic surveillance. Although a relentlessly progressive syndrome with a poor prognosis, but can be managed with nutritional support and reassurance that the syndrome is neither contagious nor familial.

 
  References Top

1.Cronkhite LW Jr, Canada WJ. Generalized gastrointestinal polyposis; an unusual syndrome of polyposis, pigmentation, alopecia and onychotrophia. N Engl J Med 1955;252:1011-5.  Back to cited text no. 1
[PUBMED]    
2.Sampson JE, Harmon ML, Cushman M, Krawitt EL. Corticosteroid-responsive Cronkhite-Canada syndrome complicated by thrombosis. Dig Dis Sci 2007;52:1137-40.  Back to cited text no. 2
    
3.Takeuchi Y, Yoshikawa M, Tsukamoto N, Shiroi A, Hoshida Y, Enomoto Y, et al. Cronkhite-Canada syndrome with colon cancer, portal thrombosis, high titer of antinuclear antibodies, and membranous glomerulonephritis. J Gastroenterol 2003;38:791-5.  Back to cited text no. 3
    
4.Blonski WC, Furth EE, Kinosian BP, Compher C, Metz DC. A case of Cronkhite-Canada syndrome with taste disturbance as a leading complaint. Digestion 2005;71:201-5.  Back to cited text no. 4
    
5.Mönkemüller K, Neumann H, Evert M. Cronkhite-Canada syndrome: Panendoscopic characterization with esophagogastroduodenoscopy, endoscopic ultrasound, colonoscopy, and double balloon enteroscopy. Clin Gastroenterol Hepatol 2008;6:A26.  Back to cited text no. 5
    
6.Nagata K, Sato Y, Endo S, Kudo SE, Kushihashi T, Umesato K. CT endoscopy for the follow-up of Cronkhite-Canada syndrome. Int J Colorectal Dis 2007;22:1131-2.  Back to cited text no. 6
[PUBMED]    
7.Ward EM, Wolfsen HC. Pharmacological management of Cronkhite-Canada syndrome. Expert Opin Pharmacother 2003;4:385-9.  Back to cited text no. 7
    
8.Lipin SP, Paul B, Nazimudeen E, Jacob BS. Case of Cronkhite Canada syndrome shows improvement with enteral supplements. J Assoc Physicians India 2012;60:61-4.  Back to cited text no. 8
    


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  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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