Muff Julian L, Sokolovski Filipp, Walsh-Korb Zarah, Choudhury Rashikh A, Dunn James C Y, Holland-Cunz Stefan G, Vuille-Dit-Bille Raphael N
Department of Pediatric Surgery, Children's University Hospital, 4056 Basel, Switzerland.
Department of General and Visceral Surgery, Kepler University Hospital, 4020 Linz, Austria.
Children (Basel). 2022 Jul 10;9(7):1024. doi: 10.3390/children9071024.
Short bowel syndrome (SBS) is a devastating disorder with both short- and long-term implications for patients. Unfortunately, the prevalence of SBS has doubled over the past 40 years. Broadly speaking, the etiology of SBS can be categorized as congenital or secondary, the latter typically due to extensive small bowel resection following diseases of the small intestine, e.g., necrotizing enterocolitis, Hirschsprung's disease or intestinal atresia. As of yet, no cure exists, thus, conservative treatment, primarily parenteral nutrition (PN), is the first-line therapy. In some cases, weaning from PN is not possible and operative therapy is required. The invention of the longitudinal intestinal lengthening and tailoring (LILT or Bianchi) procedure in 1980 was a major step forward in patient care and spawned further techniques that continue to improve lives for patients with severe SBS (e.g., double barrel enteroplasty, serial transverse enteroplasty, etc.). With this review, we aim to provide an overview of the clinical implications of SBS, common conservative therapies and the development of operative techniques over the past six decades. We also provide a short outlook on the future of operative techniques, specifically with respect to regenerative medicine.
短肠综合征(SBS)是一种严重的疾病,对患者有短期和长期的影响。不幸的是,在过去40年里,SBS的患病率翻了一番。一般来说,SBS的病因可分为先天性或继发性,后者通常是由于小肠疾病(如坏死性小肠结肠炎、先天性巨结肠或肠闭锁)后进行广泛的小肠切除所致。目前尚无治愈方法,因此,保守治疗,主要是肠外营养(PN),是一线治疗方法。在某些情况下,无法停用PN,需要进行手术治疗。1980年纵向肠道延长和裁剪(LILT或比安奇)手术的发明是患者护理方面的一大进步,并催生了进一步的技术,这些技术继续改善重症SBS患者的生活(如双腔肠成形术、连续横向肠成形术等)。通过本综述,我们旨在概述SBS的临床影响、常见的保守治疗方法以及过去六十年来手术技术的发展。我们还简要展望了手术技术的未来,特别是在再生医学方面。