Kosloske A M, Goldthorn J F
Surg Gynecol Obstet. 1984 Mar;158(3):233-7.
Four decades ago, the mortality of congenital aganglionosis of the distal part of the colon (Hirschsprung's disease) approached 100 per cent. The development of diagnostic methods and curative operations lowered the mortality to 5 to 10 per cent. Enterocolitis remained the major cause of death. In this series of 18 children with Hirschsprung's disease, the majority were diagnosed as neonates. Although enterocolitis was present at diagnosis in four (22 per cent), all children survived after prompt surgical therapy. Neonatal diagnosis and treatment may prevent the development of fatal enterocolitis, virtually eliminating the mortality of Hirschsprung's disease.
四十年前,结肠远端先天性无神经节症(赫希施普龙病)的死亡率接近100%。诊断方法和治疗手术的发展将死亡率降低到了5%至10%。小肠结肠炎仍然是主要死因。在这组18例赫希施普龙病患儿中,大多数在新生儿期被诊断出来。虽然有4例(22%)在诊断时就存在小肠结肠炎,但所有患儿在及时接受手术治疗后均存活。新生儿期诊断和治疗可预防致命性小肠结肠炎的发展,几乎消除赫希施普龙病的死亡率。