Two Cases Of Cronkhite-canada Syndrome Reports With Literature Review

Background Cronkhite-Canada syndrome is a rare clinical syndrome which has beenreported by Cronkhite and Canada firstly in1955.It has been called gastrointestinalpolyposis-cutaneous hyperpigmentation-alopecia-dystrophic changes in the fngernailssyndrome,a noninherited condition characterized by gastrointestinal hamartomatouspolyposis and ectodermal dysplasia.An autoimmune mechanism may be involved inCCS,but the pathogenesis is unclear.Objective To summary the manifestation,Endoscopic characteristics,pathologicalcharacteristics of the polyps,treatment and prognosis about Cronkhite-Canadasyndrome.Method Two cases of Cronkhite-Canada syndrome in General Hospital of BeijingMilitary Region were collected,and46cases from1990till now were retrieved aboutthe clinical manifestations,endoscopic and pathological characteristics,treatment andprognosis.Results31cases of men,17cases of women were included in domestic48cases.48cases were characterized by the presence of diffuse gastrointestinal polyposis andectodermal dysplasia. Chronic diarrhea, abdominal pain,loss of appetite and weight losswere the most common clinical symptoms.Polyps were mainly distributed in thestomach and colon,which was histologically abnormal, revealing edema, congestion,and inflammation of the lamina propria.Hyperplastic polyps,adenomatous polyps andhamartoma polyps were among them and2patients with polypcanceration.Corticosteroids plus nutrition support therapy can improvesymptoms,Surgical treatment was suitable for the polyps canceration,digestive tractobstruction and protein loss of bowel disease.11cases were only treated withsymptomatic support, among them,8cases ameliorated,2cases died,1case of illnessaggravated, after1years follow-up, of8cases2cases were dead,6cases improved,1case of aggravation was dead; after1years follow-up2patients treated with endoscopic polyps resection and symptomatic support improved;2cases with colonic polypcanceration,2cases by protein loss of bowel disease,1case with intussusception weretaken colon resection,2cases of glucocorticoid therapy at the same time, postoperativefollow-up of1year,1case was dead by malnutrition, systemic failure,3cases improved,1case was lost to follow-up;1case were accepted treatment of traditional Chinesemedicine, after1years follow-up improved;29of glucocorticoid treatment, after1years follow-up of18cases improved symptoms,2cases of the illness aggravated,4cases of secondary infection,malnutrition and systemic failure were dead,5cases werelost to follow-up.Conclusions Cronkhite-Canada syndrome is a rare disease characterized by thepresence of diffuse gastrointestinal polyposis and ectodermal dysplasia.Etiologypathogenesis is unknown, the lack of growth factors,infection, arsenic poisoning andimmune system disorders may be related to the disease.Mental stimulation,fatiguelong-term medication are risk factors of CCS.Given the rarity of CCS,diagnosis isdifficult based on the clinical characteristics, laboratory performance, endoscopic andhistologic findings and there are no systematic investigations of medical or surgicalinterventions have been conducted to guide management.The treatments includehyperalimentation, corticosteroids, H2-receptor antagonists, antibiotics, acidsuppression, cromolyn sodium, anabolic steroids, surgery etc.Surgical treatment wassuitable for the polyps canceration,digestive tract obstruction and protein loss of boweldisease.Surgery cannot be used to simply solve the symptoms, especially for those withpoor conditions.At present surgical treatment is mainly suitable for the treatment ofvarious complications.Domestic cases through active treatments, some patients withCCS available relieved, but long-term prognosis was poor, often died of malnutrition,anemia, systemic failure, secondary infection.The disease of CCS prognosis is notoptimistic.