Hirschsprung's Disease-A Review of 17 Cases in 13 Years

HIRSCHSPRUNG氏病－13年17例之分析

張扶陽(Full-Young Chang) ； 程東照(Tung-Chao Chen) ； 方名熹(Ming-Shieh Fong)

《Journal of Medical Sciences》 5卷1期 (1983/05) Pp. 1577-1583

摘要

最近13年來，三軍總醫院共有17例經手術及病理檢查證實的Hirschsprung氏病。男女性別比率為3.2比1，平均年齡為41.0±76.2個月，計有：新生兒1例，嬰兒8例，幼童6例及10歲以上者2例。有1例曾有家族史，另1例出生時合併肛門閉鎖症及直腸陰道瘻管症。最常見症狀為便祕佔100%，腹脹94%及嘔吐41%。常見物理學檢查：腹部膨脹82%，空的直腸48%及腹部觸診有糞便硬塊者36%。大腸鋇劑對診斷極具效果，正確率82%。無神經節侵犯的部位比率分別是：直腸52%，直腸乙狀結腸交接區18%，乙狀結腸18%，降結腸6%及全部大腸6%。分別接受下列的手術：modified Duhamel氏手術13例，Swenson氏手術1例及直腸肌切除術3例。手術後並無死亡者或併發腸結腸炎。其中11例回覆問卷者顯示預後：良好者3例，其他則有輕度腹瀉或便祕但未妨礙日常生活。Modified Duhamel氏手術在本報告中是一理想的手術。

並列摘要

Seventeen patients with histologically confirmed Hirschsprung's disease were treated from 1969 to 1981. The male-female sex ratio was 3.2 to 1. there were 1 neonate, 8 infants, 8 children with 2 of them older than 10 years of age. The mean age was 41.0±76.2 months. Incidence of family history and associated abnormalities were 6% respectively. On admission, the most common symptoms were constipation (100%), distension (94%) and vomiting (41%). Six of 8 had a history of delayed passage of meconium. Initial physical examination showed abdominal distension (82%), empty rectum (48%) and palpable abdominal impaction (36%). Barium enema was helpful for the diagnosis with a positive rate of 82%. Two required the full-thickness rectal biopsy for diagnosis. The percentages of aganglionsis involving rectum, rectosigmoid junction, sigmoid colon, descending colon and total colon were 52%, 18%, 18%, 6% and 6% respectively. Major operations in this series included 13 modified Duhamel procedure, 1 Swenson procedure and 3 rectal myectomies. Enterocolitis and mortality never occurred after these procedures. Eleven respondents to our questionnaire showed a fair prognosis except some loose stools and tolerable constipations without influences to their daily life. It pointed out that modified Duhamel procedure are ideal to the treatment of Hirschsprung's disease.