Stafford-Brady F J, Kahn H J, Ross T M, Russell M L
Department of Medicine, Women's College Hospital, University of Toronto, Canada.
J Rheumatol. 1988;15(5):869-74.
Severe intestinal hypomotility in scleroderma is an uncommon but difficult management problem. We present a case of advanced scleroderma bowel complicated by colonic pseudo-obstruction, which was dramatically improved by subtotal colectomy. Benign pneumoperitoneum due to pneumatosis cystoides intestinales later developed. Symptoms and the pneumoperitoneum resolved with oxygen therapy and total parenteral nutrition, followed by an elemental diet. Our case illustrates how a judicious combination of medical and surgical therapy can be successful in advanced scleroderma bowel. The pathology and pathogenesis of intestinal scleroderma are reviewed to provide an understanding of these complications and a rationale for management.
硬皮病患者出现严重肠道动力不足是一个不常见但处理棘手的问题。我们报告一例晚期硬皮病肠道病变合并结肠假性梗阻的病例，该病例经结肠次全切除术后病情显著改善。随后发生了因肠壁囊样积气症导致的良性气腹。通过氧疗和全胃肠外营养，随后采用要素饮食，症状和气腹均得以缓解。我们的病例表明，在晚期硬皮病肠道病变中，合理联合药物和手术治疗可能取得成功。本文对肠道硬皮病的病理和发病机制进行了综述，以增进对这些并发症的理解并为治疗提供理论依据。
