Dore Mariela, Junco Paloma Triana, Andres Ane M, Sánchez-Galán Alba, Amesty Maria Virginia, Ramos Esther, Prieto Gerardo, Hernandez Francisco, Lopez Santamaria Manuel
Department of Pediatric Surgery, Hospital Universitario La Paz, Madrid, Madrid, Spain.
Deparment of Pediatric Gastroenterology, Hospital Universitario La Paz, Madrid, Spain.
Eur J Pediatr Surg. 2016 Feb;26(1):112-6. doi: 10.1055/s-0035-1567805. Epub 2015 Nov 4.
Intestinal failure (IF) requires a multidisciplinary management based on nutritional support, surgical and medical rehabilitation, and transplantation. The aim of this study is to review our experience with surgical rehabilitation techniques (SRTs: enteroplasty, Bianchi, Serial Transverse Enteroplasty Procedure [STEP]) in patients with short bowel syndrome (SBS) and poor prognosis due to complex abdominal pathology. We performed a single-center retrospective study of patients with IF evaluated for intestinal transplantation in the Intestinal Rehabilitation Unit who underwent an SRT. Nonparametric tests were used for statistical analysis.A total of 205 patients (107 males/98 females) with mean age of 25 ± 7 months were assessed for IF. A total of 433 laparotomies were performed on 130 patients including intestinal resection, enteroplasties, adhesiolysis, and transit reconstruction. SRT were performed in 22 patients: 12 enteroplasties, 8 STEPs, and 4 Bianchi procedures. All patients were parenteral nutrition (PN) dependent with different stages of liver disease: mild (13), moderate (5), and severe (4). The adaptation rate for patients who underwent enteroplasty, STEP, and Bianchi were 70, 63, and 25%, respectively, although the techniques are not comparable. Overall, intestinal adaptation was achieved in nine (41%) patients, and four (18%) patients showed significant reduction of PN needs. One child did not respond to SRT and did not meet transplantation criteria. The remaining eight (36%) patients were included on the waiting list for transplant: four were transplanted, two are still on the waiting list, and two died. Better outcomes were observed in milder cases of liver disease (mild 77%, moderate 40%, severe 25%) (p < 0.05). Conversely, a trend toward a poorer outcome was observed in cases with ultrashort bowel (p > 0.05). One patient required reoperation after a Bianchi procedure due to intestinal ischemia and six needed further re-STEP or adhesiolysis procedure several months later. The median follow-up was 62 (3-135) months. Overall mortality was 19%, and was due to end-stage liver disease and/or central venous catheter-related sepsis. SRT led to intestinal adaptation in a significant number of patients with poor prognosis SBS referred for intestinal transplantation. However, SRT requires a multidisciplinary evaluation and should be attempted only in suitable cases. Careful assessment and optimal surgical timing is crucial to obtain a favorable outcome.
肠衰竭（IF）需要基于营养支持、手术及药物康复治疗和移植的多学科管理。本研究的目的是回顾我们在患有短肠综合征（SBS）且因复杂腹部病变预后较差的患者中应用手术康复技术（SRTs：肠成形术、比安奇手术、系列横断肠成形术[STEP]）的经验。我们对在肠道康复科接受SRT评估以进行肠道移植的IF患者进行了单中心回顾性研究。采用非参数检验进行统计分析。
总共评估了205例平均年龄为25±7个月的IF患者（107例男性/98例女性）。对130例患者共进行了433次剖腹手术，包括肠切除术、肠成形术、粘连松解术和转运重建术。22例患者接受了SRT：12例肠成形术、8例STEP和4例比安奇手术。所有患者均依赖肠外营养（PN），且处于不同阶段的肝病：轻度（13例）、中度（5例）和重度（4例）。接受肠成形术、STEP和比安奇手术患者的适应率分别为70%、63%和25%，尽管这些技术无可比性。总体而言，9例（41%）患者实现了肠道适应，4例（18%）患者的PN需求显著减少。1名儿童对SRT无反应且不符合移植标准。其余8例（36%）患者被列入移植等待名单：4例接受了移植，2例仍在等待名单上，2例死亡。在肝病较轻的病例中观察到更好的结果（轻度77%，中度40%，重度25%）（p<0.05）。相反，在超短肠病例中观察到预后较差的趋势（p>0.05）。1例患者在比安奇手术后因肠缺血需要再次手术，6例患者在数月后需要进一步的STEP或粘连松解手术。中位随访时间为62（3 - 135）个月。总体死亡率为19%，死因是终末期肝病和/或中心静脉导管相关败血症。SRT使大量因预后差而转诊进行肠道移植的SBS患者实现了肠道适应。然而，SRT需要多学科评估，且仅应在合适的病例中尝试。仔细评估和最佳手术时机对于获得良好结果至关重要。
