Malone Frances R, Horslen Simon P
Frances R. Malone, ARNP, PhD Children’s Hospital Regional Medical Center, Seattle, WA 98105, USA.
Curr Treat Options Gastroenterol. 2007 Oct;10(5):379-90. doi: 10.1007/s11938-007-0038-7.
Until recently, extreme short bowel due primarily to massive resection in the neonatal period had been considered incompatible with long-term survival. Indeed, parents of infants with midgut volvulus or other causes of very extensive intestinal necrosis still may be informed that resection is futile. The advent of intestinal transplantation as a potential therapy and its evolution into a standard therapy for irreversible intestinal failure have led to changing attitudes regarding these catastrophic gastrointestinal events. The experience gained from aggressively maintaining infants with little if any functional small bowel while awaiting transplantation has led to the increasing recognition that long-term survival is possible in many of these children with and often without intestinal transplantation. Even children with very small lengths of residual intestine ultimately may adapt and grow sufficiently to allow enteral autonomy. Achievement of these outcomes requires early referral to a dedicated multidisciplinary intestinal care team well versed in the management options for such children. Initial assessment often involves an inpatient evaluation followed by very close outpatient follow-up. Aggressive management is imperative for all patients with intestinal failure, allowing time for full enteral adaptation before complications become life-threatening; those with no possibility of significant adaptation can achieve optimal growth while awaiting transplantation. Along with medical and nutritional therapy and nontransplant surgery, intestinal transplantation should be seen as one of many modalities available for the optimal management of this population of patients. Thus, patients with irreversible intestinal failure and those with indications for transplantation (even those for whom hope remains that sufficient enteral adaptation still may occur) should be evaluated by the transplant team. If there is no intestinal transplant program at the center undertaking the intestinal failure management, strong links and regular communication with an intestinal transplant program that can partner in the care of these patients should be established. Multicenter collaborative and interventional studies are necessary to clearly demonstrate outcomes and to move the field forward.
直到最近，主要由于新生儿期大量肠切除导致的极短肠仍被认为无法长期存活。事实上，患有中肠扭转或其他导致广泛肠坏死原因的婴儿的父母，可能仍然被告知切除手术是徒劳的。肠道移植作为一种潜在治疗方法的出现，以及它发展成为不可逆肠衰竭的标准治疗方法，已经改变了人们对这些灾难性胃肠道事件的态度。在等待移植期间积极维持几乎没有功能性小肠的婴儿所获得的经验，使人们越来越认识到，许多这类儿童无论是否接受肠道移植都有可能长期存活。即使是残留小肠长度非常短的儿童最终也可能充分适应并成长，实现肠内营养自主。要实现这些结果，需要尽早转诊至一个专门的多学科肠道护理团队，该团队要精通此类儿童的管理方案。初始评估通常包括住院评估，随后进行非常密切的门诊随访。对于所有肠衰竭患者，积极的管理至关重要，以便在并发症危及生命之前有时间实现完全的肠内适应；那些没有显著适应可能性的患者在等待移植期间可以实现最佳生长。除了医学和营养治疗以及非移植手术外，肠道移植应被视为对这类患者进行最佳管理的多种方式之一。因此，患有不可逆肠衰竭的患者以及有移植指征的患者（即使是那些仍寄希望于可能会发生充分肠内适应的患者）都应由移植团队进行评估。如果负责管理肠衰竭的中心没有肠道移植项目，应与一个能够参与这些患者护理的肠道移植项目建立紧密联系并定期沟通。多中心协作和干预性研究对于明确展示治疗结果并推动该领域向前发展是必要的。
