Cronkhite-Canada Syndrome: A case Series in Galway University Hospital
Rahim Khan, G Harkin, P Maheshwari, C Lane, S K Palaniappan, V Byrnes
Cronkhite-Canada syndrome (CCS) is a rare, sporadically occurring, non inherited disorder reported for the first time in 1955 by Leonard W. Cronkhite, Jr, and Wilma J. Canada with 2 female patients having gastrointestinal polyps, cutaneous pigmentation, alopecia, and onychodystrophy. Up to 2002 only 475 cases were reported worldwide and of that 75% are from Japan. Diagnosis is clinical and the predominate features are weight loss, malbasorption, edema, diarrhea, nail changes, hair changes and hyper pigmentation of the skin and cataract formation associated with characteristic non-adenomatous polyps. The management is usually supportive as replacement of the electrolytes orally or intravenously, food supplement for malnutrition, steroids for the inflammation of the gut, occasionally surgery for the intestinal obstruction.
Case Reports Case 1: A 48 year old man presented with symptoms of malabsorption, Carpo-pedal spasms, and diarrhoea and weight loss of 16 kg in 2 months. He developed nail changes 3 months ago. Also noticed was thinning of hairs and tendency to fall. Spasms mainly in the thumb and index fingers. No significant background history apart from 30 pack years smoking history. On examination she has bilateral pitting pedal oedema, positive trousseaus sign onychoschizia, hyper-pigmented spots on skin. Blood tests were normal apart from hypocalcaemia, Phosphate 1.0. PTH of 121. GI endoscopy showed diffuse polyposis throughout his GI tract confirmed as non-adenomatous polyposis on histology consistent with CCS. He was being followed up in the hospital regularly for electrolytes replacement and after two years he was admitted with overwhelming sepsis and significant electrolytes imbalance which he could not survive eventually. Case 2: A 45 year old Spanish lady who presented with intermittent loose stools 4-6 per day and anaemia. She had right hemicolectomy for bowel cancer at the age 29 years, carpal tunnel syndrome, cervical glandular intraepithelial neoplasia, and multiple polyps on previous colonoscopies. Multiple upper and lower GI endoscopies revealed hyperplastic and inflammatory polyps consistent with the diagnosis of CCS. She is being followed up regularly in GI outpatients.
CCS is very rare disorder but there are cases present, one need to be vigilant while investigating for weight loss, malabsorption and skin changes. In our cases, first case has classical features of CCS and the second case had bowel cancer at an early age but common feature in both the cases was malabsorption.