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Proctocolectomy for persistent haematochezia in a patient with Cronkhite-Canada Syndrome
N Wijekoon1, M Samarasinghe1, U Dalpatadu1
1University of Colombo, Sri Lanka.
Insights
Cronkhite-Canada syndrome, a rare gastrointestinal polyposis, presents with diarrhea and taste changes. This case highlights surgical management for severe bleeding, leading to a full recovery.
Area of Science:
- Gastroenterology
- Gastrointestinal Pathology
- Medical Case Reports
Background:
- Cronkhite-Canada syndrome (CCS) is an exceptionally rare disorder characterized by diffuse gastrointestinal polyposis.
- Key features include diarrhea, dysgeusia (altered taste), and ectodermal abnormalities.
- Polyps typically affect the entire GI tract, excluding the esophagus.
Purpose of the Study:
- To report a unique case of Cronkhite-Canada syndrome presenting with severe, recurrent hematochezia.
- To illustrate the diagnostic process and management of medically refractory bleeding in CCS.
- To highlight the successful surgical outcome in a patient with severe gastrointestinal manifestations of CCS.
Main Methods:
- A comprehensive literature review was performed.
- Clinical presentation, diagnostic investigations, and treatment of a 50-year-old male patient with CCS were detailed.
- The patient underwent proctocolectomy for intractable hematochezia.
Main Results:
- The patient presented with recurrent severe hematochezia due to colonic polyps.
- Diagnosis of Cronkhite-Canada syndrome was confirmed.
- Proctocolectomy resulted in complete resolution of symptoms, with the patient remaining asymptomatic.
Conclusions:
- Cronkhite-Canada syndrome requires careful diagnosis and management, especially when presenting with severe gastrointestinal bleeding.
- Surgical intervention, such as proctocolectomy, can be an effective option for refractory cases.
- This case underscores the importance of considering rare conditions in patients with complex gastrointestinal symptoms.
Abstract:
Cronkhite-Canada syndrome is an extremely rare condition of gastrointestinal polyposis in which the main presenting features are diarrhoea and dysgeusia. The polyps in this condition are characteristically distributed throughout the entire gastrointestinal tract except the oesophagus, and these patients exhibit unique ectodermal abnormalities. Herein, we report a 50-year-old male who had recurrent episodes of severe haematochezia from the polyps in the colon. Further examination and investigations revealed a diagnosis of Cronkhite-Canada syndrome. Proctocolectomy was carried out for medically refractory haematochezia and the patient is asymptomatic at present.
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