Hort Amy, Hameed Ahmer, Middleton Peter G, Pleass Henry C
Department of Surgery, Westmead Hospital, Sydney, New South Wales, Australia.
Centre for Transplant and Renal Research, The Westmead Institute for Medical Research, Sydney, New South Wales, Australia.
ANZ J Surg. 2020 May;90(5):681-686. doi: 10.1111/ans.15357. Epub 2019 Jul 30.
As life expectancy for those with cystic fibrosis (CF) now exceeds 40 years of age, adult hospitals away from specialized CF services are being exposed more frequently to people with acute complications of CF. Well-known manifestations of CF include pulmonary disease and pancreatic insufficiency with malabsorption. However, a less well-known entity is distal intestinal obstruction syndrome (DIOS), which is an important cause of obstructive symptoms in people with CF that must be differentiated from other causes of bowel obstruction. However, one confounding factor is that adults with CF may have elements of both DIOS and mechanical bowel obstruction due to adhesions from previous operations. A recent tragic outcome in a young adult with CF highlights the need for all doctors, both junior and senior, especially those who are not directly involved in day-to-day CF care, to understand the features of DIOS and the appropriate management, which differs from that of a mechanical bowel obstruction. This review aims to highlight the clinical and pathophysiological features of DIOS, differentiate it from other causes of bowel obstruction and contrast management strategies. Improved knowledge of DIOS will help to facilitate appropriate recognition and permit optimal, multidisciplinary management of this CF complication.
由于囊性纤维化（CF）患者的预期寿命现已超过40岁，远离专业CF服务机构的成人医院越来越频繁地接触到患有CF急性并发症的患者。CF的常见表现包括肺部疾病和伴有吸收不良的胰腺功能不全。然而，一种不太为人所知的情况是远端肠梗阻综合征（DIOS），它是CF患者出现梗阻症状的一个重要原因，必须与其他肠梗阻原因相鉴别。然而，一个混杂因素是，成年CF患者可能同时存在DIOS和因既往手术粘连导致的机械性肠梗阻。一名年轻成年CF患者近期的悲惨结局凸显了所有医生，无论资历深浅，尤其是那些未直接参与CF日常护理的医生，都需要了解DIOS的特征及恰当的处理方法，因为其处理方法与机械性肠梗阻不同。本综述旨在强调DIOS的临床和病理生理特征，将其与其他肠梗阻原因相鉴别，并对比管理策略。对DIOS有更深入的了解将有助于促进正确识别，并实现对这种CF并发症的最佳多学科管理。
