Mann S D, Debinski H S, Kamm M A
St Mark's Hospital, Harrow, Middlesex, UK.
Gut. 1997 Nov;41(5):675-81. doi: 10.1136/gut.41.5.675.
Chronic idiopathic intestinal pseudo-obstruction, a syndrome of ineffectual motility due to a primary disorder of enteric nerve or muscle, is rare.
To determine the clinical spectrum, underlying pathologies, response to treatments, and prognosis in a consecutive unselected group of patients.
Cross sectional study of all patients with clinical and radiological features of intestinal obstruction in the absence of organic obstruction, associated with dilated small intestine (with or without dilated large intestine), being actively managed in one tertiary referral centre at one time.
Twenty patients (11 men and nine women, median age 43 years, range 22-67) fulfilled the diagnostic criteria. Median age at onset of symptoms was 17 years (range two weeks to 59 years). Two patients had an autosomally dominant inherited visceral myopathy. Major presenting symptoms were pain (80%), vomiting (75%), constipation (40%), and diarrhoea (20%). Eighteen patients required abdominal surgery, and a further patient had a full thickness rectal biopsy. The mean time interval from symptom onset to first operation was 5.8 years. Histology showed visceral myopathy in 13, visceral neuropathy in three, and was indeterminate in three. In the one other patient small bowel motility studies were suggestive of neuropathy. Two patients died within two years of symptom onset, one from generalised thrombosis and the other from an inflammatory myopathy. Of the remaining 18 patients, eight were nutritionally independent of supplements, two had gastrostomy or jejunostomy feeds, and eight were receiving home parenteral nutrition. Five patients were opiate dependent, only one patient had benefited from prokinetic drug therapy, and five patients required formal psychological intervention and support.
In a referral setting visceral myopathy is the most common diagnosis in this heterogeneous syndrome, the course of the illness is usually prolonged, and prokinetic drug therapies are not usually helpful. Ongoing management problems include pain relief and nutritional support.
慢性特发性肠道假性梗阻是一种由于肠神经或肌肉原发性疾病导致的无效蠕动综合征，较为罕见。
确定一组未经挑选的连续患者的临床谱、潜在病理、治疗反应及预后。
对某三级转诊中心同时积极治疗的所有具有肠梗阻临床和放射学特征但无器质性梗阻且伴有小肠扩张（伴或不伴有大肠扩张）的患者进行横断面研究。
20例患者（11例男性和9例女性，中位年龄43岁，范围22 - 67岁）符合诊断标准。症状出现的中位年龄为17岁（范围从两周至59岁）。2例患者患有常染色体显性遗传性内脏肌病。主要症状为疼痛（80%）、呕吐（75%）、便秘（40%）和腹泻（20%）。18例患者需要进行腹部手术，另有1例患者进行了全层直肠活检。从症状出现到首次手术的平均时间间隔为5.8年。组织学检查显示13例为内脏肌病，3例为内脏神经病变，3例不确定。另一例患者的小肠动力研究提示神经病变。2例患者在症状出现后两年内死亡，1例死于全身血栓形成，另1例死于炎性肌病。其余18例患者中，8例在营养上无需补充剂，2例通过胃造口术或空肠造口术喂养，8例接受家庭肠外营养。5例患者依赖阿片类药物，仅1例患者从促动力药物治疗中获益，5例患者需要正式的心理干预和支持。
在转诊环境中，内脏肌病是这种异质性综合征最常见的诊断，病程通常较长，促动力药物治疗通常无帮助。持续存在的管理问题包括疼痛缓解和营养支持。
