Heilmann G, Erckenbrecht J F
Klinik für Innere Medizin und Gastroenterologie, Florence-Nightingale-Krankenanstalten, Diakoniewerk Kaiserswerth.
Ther Umsch. 1994 Mar;51(3):208-15.
Intestinal pseudo-obstruction is a rare and heterogeneous syndrome caused by severe disorders of gastrointestinal motility. It affects the entire gastrointestinal tract or only segments, giving rise to symptoms and physical signs of a mechanical obstruction of the gut despite negative results of all imaging procedures. The disease may occur in an acute or chronic form. The acute and some of the chronic forms develop as complication of other gastrointestinal or extragastrointestinal diseases, e.g. pancreatitis or systemic sclerosis. The primary forms of chronic intestinal pseudo-obstruction are most often caused by genetic neuromuscular disorders of the gastrointestinal tract, e.g. familial visceral neuropathies. The diagnosis of intestinal pseudo-obstruction is based on the exclusion of a mechanical obstruction of the gut by fluoroscopy and endoscopy. Manometric studies may disclose the underlying disorder of gastrointestinal motility. In a few patients, results of all imaging procedures as well as motility studies are inconclusive, and laparatomy (with full thickness biopsy of the gut wall) has to be performed to exclude mechanical obstruction of the gut. Acute intestinal pseudo-obstruction is treated by elimination of the underlying intestinal or extraintestinal disease. In case of extensive colonic dilatation with imminent colonic perforation endoscopic decompression should be evaluated. Treatment of chronic pseudo-obstruction aims to correct the underlying motility disorder. Usually, restoration of normal gastrointestinal motility is attempted by prokinetic drugs, but often their effect is limited. Surgery may be helpful in the few patients in whom the disease is confined to small segments of the gut, leaving all other parts unaffected. Some patients with otherwise intractable disease may need long-term parenteral nutrition.
肠道假性梗阻是一种由胃肠动力严重紊乱引起的罕见且异质性综合征。它可影响整个胃肠道或仅累及部分节段，尽管所有影像学检查结果均为阴性，但仍会出现肠道机械性梗阻的症状和体征。该疾病可呈急性或慢性形式。急性和部分慢性形式是其他胃肠道或胃肠道外疾病（如胰腺炎或系统性硬化症）的并发症。慢性肠道假性梗阻的原发性形式最常由胃肠道的遗传性神经肌肉疾病（如家族性内脏神经病）引起。肠道假性梗阻的诊断基于通过荧光镜检查和内镜检查排除肠道机械性梗阻。测压研究可能会揭示潜在的胃肠动力紊乱。在少数患者中，所有影像学检查以及动力研究结果均无定论，必须进行剖腹手术（取肠壁全层活检）以排除肠道机械性梗阻。急性肠道假性梗阻通过消除潜在的肠道或肠道外疾病进行治疗。如果出现广泛的结肠扩张且即将发生结肠穿孔，应考虑内镜减压。慢性假性梗阻的治疗旨在纠正潜在的动力紊乱。通常，尝试使用促动力药物恢复正常的胃肠动力，但往往效果有限。对于疾病仅局限于肠道小部分节段而其他部分未受影响的少数患者，手术可能会有所帮助。一些患有其他难治性疾病的患者可能需要长期肠外营养。
