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Total Colonic Aganglionosis: Reappraisal of Contrast Enema Study
全結腸型先天性巨結腸症：重新評估灌腸攝影之影像表現
摘要
全結腸型先天性巨結腸症是先天性巨結腸症中一種罕見的分類。此疾病於灌腸攝影的表現非常多樣化，造成診斷上的困難。本研究藉由回顧本院2001年至2009年的14位患有全結腸型先天性巨結腸症病人及53位患有非全結腸型先天性巨結腸症病人的素片及灌腸攝影影像，來分析比較其影像表現。在分析的影像表現中，結腸短小且僵直，小腸擴張，細小結腸，影像轉型區位於盲腸或是小腸是全結腸型先天性巨結腸症具統計意義的特徵。雖然全結腸型先天性巨結腸症是一種結腸的疾病，但不論在臨床上或是影像上大多都是以遠端小腸阻塞來表現。雖然其影像表現非常多樣化，但若於灌腸攝影發現有小腸擴張、結腸短小且僵直、位於小腸的影像轉型區、直腸擴張不良、結腸壁不規則、及延遲性排出顯影劑等表現，就應該懷疑有全結腸型先天性巨結腸症的可能性，並安排結腸切片來確定診斷。根據是否有細小結腸將病人分成兩組，再分析其他全結腸型先天性巨結腸症的影像表現，對於鑑別此疾病與其他小兒腸阻塞的疾病是有幫助的。
關鍵字
無資料並列摘要
Total colonic aganglionosis (TCA) is a rare form of Hirschsprung disease (HD). It is difficult to be diagnosed on contrast enema because the radiographic findings are variable. The study aims to re-evaluate the contrast enema findings of TCA. From 2001 to 2009, 14 patients (11 males, 3 females; ages from 1 day to 6 months) with pathologically proven TCA were reviewed for demographic features, clinical presentations, pathology reports, radiographic and contrast enema findings. In addition, the radiographic and contrast enema findings of 53 patients with non-TCA HD were reviewed and compared with those of TCA. Among the imaging findings, a short and rigid colon, small bowel dilatation, microcolon, and radiographic transition zone proximal to the cecum were statistically more significant in patients with TCA than in patients with non-TCA HD (P ＜ 0.001). In conclusion, TCA is a colon disease presenting as small bowel obstruction clinically and radiographically. Although the radiographic findings of TCA are variable, TCA could be suspected when the initial radiograph shows small bowel dilatation and contrast enema shows a short and rigid colon, radiographic transition zone proximal to the cecum, poor rectal distensibility, colonic wall irregularity and delayed contrast emptying. In addition, biopsy should be performed to make a definite diagnosis. According to the caliber of the colon on contrast enema, a subgrouping approach may be useful in the differential diagnosis and be helpful in early diagnosis and exclusion of TCA.