Cronkhite Canada syndrome complicated by pulmonary embolism-A case report

Abstract

Introduction: Cronkhite Canada Syndrome (CCS) is a rare syndrome, described in 1955 by Americans, Leonard Wolsey Cronkhite and Wilma Jeanne Canada in the New England Journal of Medicine [1]. About 450 cases have been reported. Complications, like malignant transformation, unprovoked thromboembolism is known. Since there is wide variability in medical presentation, no definitive diagnostic and treatment protocol s have been set. The mortality remains at 55%.

Case presentation: We report a case of a 50 year old male patient presenting with diarrhea, weight loss, abdominal pain, ectodermal features. His upper (UGI) and lower Gastrointestinal (LGI) endoscopy showed multiple polypoidal and carpet like lesions in fundus, body and antrum of stomach. Videocolonoscopy showed multiple sessile and pedunculated polyps. Multiple biopsies were taken, proving malignancy. Because of poor nutrition, total parenteral nutrition was given for four weeks. After nutritional optimization, he underwent laparoscopic assisted subtotal colectomy. His post-operative course was complicated by the occurrence of pulmonary embolism and anastomotic leak.

Discussion: CCS is an ailment of unknown pathophysiology. Considering what is known so far, patients suffering from CCS are at highest risk of thromboembolic episodes. This seems to be irrespective of surgical intervention. Patients of CCS should have thromboembolic prophylaxis started as soon as a diagnosis is made. They should have thrombophilia profile, fibrinogen level and Factor 8 tested before any intervention is planned.

Conclusion: If CCS presents with a surgical indication, namely malignancy, the patient should be categorized as highest risk for thromboembolic complications and both mechanical and pharmacological prophylaxis be instituted.

Keywords: Cronkhite Canada syndrome; Gastrointestinal polyps; Post-operative; Pulmonary embolism.

Fig. 1

Dysnychia of hand nails.

Fig. 2

Duodenal bulb showing large sessile and pedunculated polypoidal lesions involving ampulla.

Fig. 3

Videocolonoscopy showing multiple small sessile and large pedunculated polyps.

Fig. 4

CTPA showing thrombi in both right and left main pulmonary arteries.

Fig. 5

Multiple hamartomatous polyps and tubular adenomas.

Fig. 6

Few tubular adenomas show moderate to high grade dysplasia.