Argov Samuel, Levandovsky Olga, Kerner Hedviga, Ben-Yizhak Ofer, Klinehouse Uriel, Reissman Ptahia
Department of Surgery, Elisha Hospital, Haifa.
Harefuah. 2012 Jan;151(1):18-9, 63.
Hirschprung disease is well known. The basic pathogenesis is lack of parasympathetic ganglia in the large bowel wall, part of it, or all of it. The absence of these ganglia paralyzes the involved segment leading to obstruction. Almost all babies are diagnosed and operated upon in their first year. Few rare cases manage to grow and reach maturity without diagnosis. These patients are characterized by severe constipation and grade III-IV hemorrhoids. This is adult type Hirschprung's disease. Two first cases in Israel were diagnosed, patients were operated upon, and recovered. Adult type Hirschprung's disease is reviewed and various surgical options are discussed.
先天性巨结肠病广为人知。其基本发病机制是大肠壁部分或全部缺乏副交感神经节。这些神经节的缺失使受累节段麻痹，导致梗阻。几乎所有患儿在一岁内就会被诊断并接受手术。极少数罕见病例在未被诊断的情况下成长至成年。这些患者的特征是严重便秘和III - IV级痔疮。这就是成人型先天性巨结肠病。以色列诊断出首例两例患者，对其进行了手术，患者康复。本文对成人型先天性巨结肠病进行了综述，并讨论了各种手术选择。
