Toyosaka A, Okamoto E, Okasora T, Nose K, Tomimoto Y
First Department of Surgery, Hyogo College of Medicine, Nishinomiya, Japan.
Clin Auton Res. 1993 Aug;3(4):243-8. doi: 10.1007/BF01829013.
One hundred and forty-eight cases of congenital large intestinal motor dysfunction (pseudo-Hirschsprung's disease) were reported by members of the Japanese Society of Pediatric Surgeons during the past 20 years. The disorder was defined as a congenital, non-mechanical obstruction of the intestine with the presence of intramural ganglia in the terminal rectum. Intramural ganglia were abnormal in 77 cases, normal in 42, and could not be determined in 29. Of those with abnormal intramural ganglia, 54 had immature ganglia or hypoganglionosis (oligoganglionosis), 15 had neuronal intestinal dysplasia, and eight had a segmental anomaly. Of those with a normal myenteric plexus, 22 had chronic and twelve had suspected idiopathic intestinal pseudo-obstruction syndrome; eight had megacystis-microcolon-intestinal hypoperistalsis syndrome. While cases with both hypoganglionosis and normal intramural ganglia had normal acetylcholine esterase activity, a significantly greater number of patients with hypoganglionosis lacked normal rectoanal reflexes. Patients with hypoganglionosis, chronic idiopathic intestinal pseudo-obstruction syndrome, and megalocystis-microcolon-intestinal hypoperistalsis syndrome had poor prognoses with an overall mortality of 36.9%. These findings indicate that congenital large intestinal motor dysfunction remains a serious disease of childhood.
日本小儿外科学会的成员在过去20年里报告了148例先天性大肠运动功能障碍（假性先天性巨结肠症）。该疾病被定义为一种先天性、非机械性肠梗阻，终末直肠存在壁内神经节。壁内神经节异常的有77例，正常的有42例，无法确定的有29例。在壁内神经节异常的患者中，54例有神经节发育不成熟或神经节减少症（神经节过少症），15例有神经元性肠发育异常，8例有节段性异常。在肌间神经丛正常的患者中，22例有慢性疾病，12例疑似特发性肠假性梗阻综合征；8例有巨膀胱-微结肠-肠蠕动减弱综合征。虽然神经节减少症且壁内神经节正常的患者乙酰胆碱酯酶活性正常，但神经节减少症患者中缺乏正常直肠肛门反射的人数明显更多。神经节减少症、慢性特发性肠假性梗阻综合征和巨膀胱-微结肠-肠蠕动减弱综合征患者的预后较差，总死亡率为36.9%。这些发现表明，先天性大肠运动功能障碍仍然是一种严重的儿童疾病。
