Intestinal Rehabilitation and Transplant Center, University of Pittsburgh School of Medicine, PA, USA; Thomas E. Starzl Transplantation Institute, University of Pittsburgh School of Medicine, PA, USA; Department of Surgery, University of Pittsburgh School of Medicine, PA, USA.
Intestinal Rehabilitation and Transplant Center, University of Pittsburgh School of Medicine, PA, USA; Thomas E. Starzl Transplantation Institute, University of Pittsburgh School of Medicine, PA, USA.
Surgery. 2020 Aug;168(2):297-304. doi: 10.1016/j.surg.2019.12.001. Epub 2020 Mar 3.
Historically, adults with ultra short bowel syndrome (USBS) have been considered candidates for lifetime parenteral nutrition (PN) or are referred for visceral transplantation. We examined the surgical and nutritional outcomes of adult patients with USBS managed at a single intestinal rehabilitation center.
We retrospectively reviewed data on 588 adult patients referred to our center between January 2013 and December 2018. USBS was defined as residual small bowel (SB) length ≤ 50 cm.
Forty-five patients (7.6%) with a mean age of 46.7 years (range 17-78) were identified. Indications for enterectomy included mesenteric ischemia (n=17) and internal hernias (n=6), followed by large intraabdominal fibroids, trauma, and allograft enterectomies, with five cases each. Median SB length was 18.0 cm; 20 patients (44.4%) had their entire SB resected. Thirteen patients had an intact colon, of which nine had preservation of the ileocecal valve. Patients who underwent autologous reconstruction of their gastrointestinal (GI) tract required a lower total PN volume (29.0 ± 7.6 vs. 40.8 ± 13.2 ml/Kg/day, p=0.002) and presented better short- and long- term survival (p=0.005). Patients with no gut had higher mortality (p=0.036). Hormonal therapy with the glucagon-like peptide-2 analog teduglutide was used in nine patients (20%) five of whom were weaned off TPN. Excluding patients with no gut (n=20), discontinuation of total PN rate for patients with an end ostomy or tube decompression (n= 6), jejunocolostomy (n= 10), and jejunoileostomy (n=9) were 0%, 40%, and 77.7%, respectively. Eleven patients (44%) with some residual small intestine achieved nutritional autonomy in an average of 20 months after GI reconstruction. Fifteen patients were listed for transplantation (33.3%). Seven patients underwent isolated SB transplantation and achieved nutritional autonomy in an average of three months after transplantation. One-year patient and graft survival were 100%. After a 37-month median follow-up period, 36 of 42 patients followed by our center were still alive (85.7%).
Nutritional autonomy can be achieved in a significant number of patients with USBS in specialized centers with surgical and/or hormonal therapy. The presence of an intact colon and ileocecal valve can significantly increase the adaptation rate. Moreover, restoration of GI tract continuity has a positive impact on medical management and survival.
历史上，患有超短肠综合征（USBS）的成年人被认为是终身肠外营养（PN）的候选者，或被转介进行内脏移植。我们研究了在一个单一的肠道康复中心接受治疗的成年 USBS 患者的手术和营养结局。
我们回顾性地分析了 2013 年 1 月至 2018 年 12 月期间在我们中心就诊的 588 例成年患者的数据。USBS 定义为剩余小肠（SB）长度≤50cm。
确定了 45 名（7.6%）平均年龄为 46.7 岁（范围 17-78 岁）的患者。肠切除术的指征包括肠系膜缺血（n=17）和内疝（n=6），其次是大型腹腔内纤维瘤、创伤、同种异体肠切除术，各有 5 例。SB 长度中位数为 18.0cm；20 名患者（44.4%）接受了整个 SB 切除术。13 名患者保留了完整的结肠，其中 9 名保留了回盲瓣。接受胃肠道（GI）自体重建的患者需要更少的总 PN 量（29.0±7.6 vs. 40.8±13.2ml/Kg/天，p=0.002），且具有更好的短期和长期生存率（p=0.005）。没有肠道的患者死亡率更高（p=0.036）。九名患者（20%）使用了胰高血糖素样肽-2 类似物 teduglutide 进行激素治疗，其中五名患者成功停用 TPN。排除无肠道患者（n=20），接受肠造口术或管减压（n=6）、空肠结肠吻合术（n=10）和空肠回肠吻合术（n=9）的患者完全停止 PN 的比例分别为 0%、40%和 77.7%。11 名（44%）有部分残留小肠的患者在 GI 重建后平均 20 个月达到营养自主。15 名患者被列入移植名单（33.3%）。7 名患者接受了单纯 SB 移植，平均在移植后 3 个月实现营养自主。1 年患者和移植物存活率为 100%。在中位随访 37 个月后，我们中心随访的 42 名患者中有 36 名（85.7%）仍存活。
在具有手术和/或激素治疗的专业中心，大量 USBS 患者可实现营养自主。完整结肠和回盲瓣的存在可显著提高适应率。此外，恢复 GI 道连续性对医疗管理和生存率有积极影响。
