Dagorno Claire, Montalva Louise, Capito Carmen, Lavrand Frederic, Guinot Audrey, De Napoli Cocci Stéphan, Gelas Thomas, Dubois Rémi, Dariel Anne, Dugelay Emmanuelle, Chardot Christophe, Bonnard Arnaud
Department of Pediatric Surgery, Robert-Debré Children's University Hospital, APHP, Paris, France; Department of General Pediatric Surgery, Necker-Enfants Malades Hospital, APHP, Paris, France.
Department of Pediatric Surgery, Robert-Debré Children's University Hospital, APHP, Paris, France; Paris-Cité University, Paris, France; NeuroDiderot, INSERM UMR1141, Paris, France.
J Pediatr Surg. 2025 Jan;60(1):161909. doi: 10.1016/j.jpedsurg.2024.161909. Epub 2024 Sep 26.
Short Bowel Syndrome (SBS), secondary to various underlying diseases, is one of the main causes of intestinal failure in children. Surgical management by serial transverse enteroplasty (STEP), is feasible in selected cases, but results on long-term follow-up are scarce. The aim of this study was to report long-term outcomes of the STEP procedure in children with SBS.
We performed a multicenter national retrospective study reviewing medical charts of children who underwent a STEP technique between 2000 and 2022 in 6 university hospitals. Collected data included demographics, SBS history, surgical procedures, nutritional support (enteral or parenteral), STEP management, complications, and outcomes (nutritional support, digestive symptoms, growth).
STEP was performed in 36 SBS, resulting from 14 gastroschisis, 10 intestinal atresia, 8 necrotizing enterocolitis (NEC), 2 midgut volvulus, and 2 intestinal Hirschsprung disease. Median age at first STEP was 10.8 months [4.5; 63.8]. Bowel length significantly increased (47 vs 70 cm, p < 0.01), with a median gain of 16 cm [11; 25] or 34%. At surgery, 33 children had parenteral nutrition (PN). A second STEP was performed in 11 patients, at a median age of 4.2 years [2.8; 6.8]. One patient underwent a third STEP. Two children required intestinal transplantation. Median follow-up was 7 years [4.4;11.4]. Out of 33 children requiring PN before surgery, 14 children were weaned off PN. The remaining 19 children still required PN, but their dependency decreased by 19%. Out of 17 children presenting severe intestinal dilations and bacterial overgrowth, 12 became asymptomatic. Out of 20 with chronic obstructive symptoms, 8 patients still required supplementary PN whereas 12 achieved complete enteral autonomy, including 3 requiring supplementary enteral support.
STEP technique remains a surgical option in the management of these children, enabling a decrease in PN dependency, resulting in weaning off PN in some cases, as well as an improvement of clinical symptoms.
Retrospective multicenter observational study.
Level 3.
短肠综合征（SBS）继发于多种基础疾病，是儿童肠衰竭的主要原因之一。对于部分病例，采用系列横断肠成形术（STEP）进行手术治疗是可行的，但长期随访结果较少。本研究旨在报告STEP手术治疗儿童SBS的长期疗效。
我们进行了一项全国多中心回顾性研究，回顾了2000年至2022年期间在6家大学医院接受STEP手术的儿童的病历。收集的数据包括人口统计学资料、SBS病史、手术操作、营养支持（肠内或肠外）、STEP治疗情况、并发症及预后（营养支持、消化症状、生长发育）。
36例SBS患儿接受了STEP手术，病因包括14例腹裂、10例肠闭锁、8例坏死性小肠结肠炎（NEC）、2例中肠扭转和2例先天性巨结肠。首次接受STEP手术的中位年龄为10.8个月[4.5；63.8]。肠管长度显著增加（47 vs 70 cm，p < 0.01），中位增加16 cm[11；25]，即增加34%。手术时，33例患儿接受肠外营养（PN）。11例患儿接受了第二次STEP手术，中位年龄为4.2岁[2.8；6.8]。1例患儿接受了第三次STEP手术。2例患儿需要进行肠移植。中位随访时间为7年[4.4；11.4]。术前需要PN的33例患儿中，14例患儿停用了PN。其余19例患儿仍需要PN，但对PN的依赖程度降低了19%。17例出现严重肠扩张和细菌过度生长的患儿中，12例症状消失。20例有慢性梗阻症状的患儿中，8例仍需要补充PN，12例实现了完全肠内营养自主，其中3例需要补充肠内营养支持。
STEP技术仍是治疗这些患儿的一种手术选择，可降低对PN的依赖程度，在某些情况下可停用PN，并改善临床症状。
回顾性多中心观察性研究。
3级。
