Section of Pediatric Surgery, Children's Hospital, Helsinki University Central Hospital and University of Helsinki, Finland.
J Pediatr Surg. 2013 Feb;48(2):339-44. doi: 10.1016/j.jpedsurg.2012.11.014.
The aim of this study was to analyze outcomes of severe short bowel syndrome (SBS) treated with autologous intestinal reconstruction (AIR) surgery to facilitate independence of parenteral nutrition (PN).
PN dependence, growth, nutritional status, liver function, and survival were comparatively assessed in SBS children treated with (n=10) or without (n=18) AIR surgery.
Median follow-up was 9.2 (4.9-14) years. Residual small intestinal length was 28 cm at birth. A total of 13 AIR procedures were performed at median age of 16 (11-41) months: serial transverse enteroplasty (STEP; n=8), longitudinal lengthening and tailoring (LILT; n=2), and tapering enteroplasty (n=3). Following STEP and LILT, length of the remaining small intestine increased by 19 (15-38) cm or 43% (38%-64%). Two children required repeat STEP due to recurrence of symptoms and bowel re-dilatation. Median duration of PN was 12 (11-17) months before and 14 (0-19) months after AIR. Eight children remain off PN 6.9 (3.6-9.7) years after surgery, and one with ultra short bowel (12 cm) receives 12% of energy as PN - all with reassuring growth and nutritional status and normal liver function. Actuarial PN dependence, including those who died on PN, was similar (P=0.19) with or without AIR surgery being 30% and 20% at four years, respectively. Complications of AIR surgery (lymphocele, bowel obstruction, and staple line bleeding) resolved without operative interventions. Survival was 90% with and 83% without AIR procedures.
AIR surgery can provide long-term survival, independence of PN, and satisfactory general health in selected children with severe SBS.
本研究旨在分析自体肠重建(AIR)手术治疗严重短肠综合征(SBS)的结果,以促进患者脱离肠外营养(PN)。
对比分析接受(n=10)或未接受(n=18)AIR 手术治疗的 SBS 患儿的 PN 依赖、生长、营养状况、肝功能和存活率。
中位随访时间为 9.2(4.9-14)年。出生时残留小肠长度为 28cm。在中位年龄 16(11-41)个月时共进行了 13 次 AIR 手术:序贯横结肠成形术(STEP;n=8)、纵向延长和剪裁术(LILT;n=2)和缩窄肠成形术(n=3)。在进行 STEP 和 LILT 后,残留小肠长度增加了 19(15-38)cm,增加了 43%(38%-64%)。由于症状复发和肠再扩张,两名儿童需要再次进行 STEP。在进行 AIR 之前,PN 的中位持续时间为 12(11-17)个月,在进行 AIR 之后为 14(0-19)个月。8 名儿童在手术后 6.9(3.6-9.7)年无需 PN,1 名超短肠(12cm)儿童接受 12%的能量作为 PN-所有儿童的生长和营养状况及肝功能均正常。考虑到死亡在 PN 上的儿童,有无 AIR 手术的 PN 依赖性的累积发生率相似(P=0.19),分别为 4 年后的 30%和 20%。AIR 手术的并发症(淋巴囊肿、肠梗阻和吻合口出血)无需手术干预即可解决。有或无 AIR 手术的存活率分别为 90%和 83%。
在选择的严重 SBS 患儿中,AIR 手术可提供长期生存、PN 独立和良好的整体健康状况。