Department of Pediatric Surgery, Kuopio University Hospital, Finland and University of Helsinki, Finland.
Department of Pediatric Surgery, Tampere University Hospital, Tampere, Finland.
J Pediatr Surg. 2024 Nov;59(11):161622. doi: 10.1016/j.jpedsurg.2024.07.007. Epub 2024 Jul 14.
We aimed to evaluate disease characteristics, associated malformations and surgical outcomes of congenital intestinal atresia.
We identified all patients with jejunoileal (JIA) or colonic atresia (CA) treated at the Helsinki University Children's hospital during 1947-2019 and collected clinical data retrospectively from archived and electronic medical records.
Of the 180 included patients, 156 had JIA and 24 CA. Overall survival improved markedly from 34% during 1947-1977 to 93% during 1978-2019. Rate of primary anastomosis (81% in JIA, 21% in CA) remained unchanged while early surgical complications decreased (32% vs 18%, P = 0.04) and prematurity rate increased (21% vs 45%, P = 0.002) among JIA patients over time. Around half of patients had associated, mostly gastrointestinal malformations which occurred comparably in JIA and CA. During 1978-2019, 20 (21%) JIA patients, but none of CA patients, developed short bowel syndrome. Presence of type 3b atresia (n = 16) or gastroschisis (n = 14), but not the extent of intestinal resection or surgical complications, was associated with development of short bowel syndrome without decreasing survival. Shorter primary bowel resection without tapering surgery in JIA predisposed to repeated resections due to obstructive symptoms, and prolonged duration of parenteral nutrition.
Survival of patients with JIA and CA has improved remarkably over time and is currently high despite frequent prematurity and associated malformations. In patients with JIA, apple peel atresia and gastroschisis predisposed to short bowel syndrome without jeopardizing high survival rates.
IV.
本研究旨在评估先天性肠闭锁的疾病特征、相关畸形和手术结果。
我们确定了在 1947 年至 2019 年间在赫尔辛基大学儿童医院接受治疗的所有空肠和回肠(JIA)或结肠闭锁(CA)患者,并从存档和电子病历中回顾性地收集临床数据。
在 180 例纳入的患者中,156 例为 JIA,24 例为 CA。总体存活率明显提高,从 1947-1977 年的 34%提高到 1978-2019 年的 93%。JIA 患者的初次吻合率(81%)保持不变,而早期手术并发症减少(32%比 18%,P=0.04),早产儿率增加(21%比 45%,P=0.002)。大约一半的患者存在相关的胃肠道畸形,在 JIA 和 CA 中发生率相似。在 1978-2019 年期间,20 例(21%)JIA 患者发生短肠综合征,但无 CA 患者发生。存在 3b 型闭锁(n=16)或腹裂(n=14),但不限于肠切除范围或手术并发症,与短肠综合征的发生有关,但不会降低存活率。JIA 患者中较短的初次肠切除而没有逐渐缩小手术,容易因梗阻症状而导致再次切除,并延长肠外营养的持续时间。
JIA 和 CA 患者的生存率随着时间的推移显著提高,目前即使早产和相关畸形频繁发生,生存率仍然很高。在 JIA 患者中,苹果皮样闭锁和腹裂容易导致短肠综合征,但不会危及高生存率。
IV。