Anuras S, Hade J E, Soffer E, Lu C C, Loening-Baucke V, Soper R T, Shirazi S S
J Clin Gastroenterol. 1984 Jun;6(3):205-10.
The natural history of adult Hirschsprung's disease was reviewed in 22 patients (four from our hospital and 18 from the literature). The diagnosis in nearly half the patients was made before age 30, and the oldest patients were 69 years of age. Male to female ratio was 4:1. Almost all patients had severe constipation since birth. Barium enema showed rectal narrowing in 77%, and colonic dilation in 100%. Anorectal manometry was performed in only four patients; three showed no relaxation with rectal distention. In all patients, rectal biopsy showed no ganglion cells. Appropriate operation for Hirschsprung's disease improved symptoms in over 80% of patients. Hirschsprung's disease should be considered in all adult patients who have had severe constipation since birth, especially if they are male.
对22例成人先天性巨结肠患者（4例来自我院，18例来自文献）的自然病史进行了回顾。近半数患者在30岁之前确诊，年龄最大的患者为69岁。男女比例为4:1。几乎所有患者自出生起就有严重便秘。钡灌肠检查显示，77%的患者直肠狭窄，100%的患者结肠扩张。仅对4例患者进行了肛门直肠测压；3例患者直肠扩张时无松弛反应。所有患者的直肠活检均显示无神经节细胞。针对先天性巨结肠进行的适当手术使超过80%的患者症状得到改善。所有自出生起就有严重便秘的成年患者，尤其是男性患者，均应考虑患有先天性巨结肠。
