Shivanand Desai Center for Digestive Disorders, Deenanath Mangeshkar Hospital and Research Center, Maharashtra, India.
Department of Internal Medicine, Rochester General Hospital, Rochester, NY, USA.
Minerva Gastroenterol (Torino). 2023 Jun;69(2):175-183. doi: 10.23736/S2724-5985.22.03166-7. Epub 2022 Mar 28.
Hirschsprung's disease (HD) is a congenital disorder characterized by absence of intrinsic ganglion cells of the hindgut. It commonly presents in infancy with refractory constipation and failure to thrive. Short segment HD affecting the rectosigmoid region is the commonest variant. Although surgical or laparoscopic single or multi-stage pull-through procedures have been the gold standard for more than six decades, these procedures are associated with significant morbidity, recurrence, and often multi-stage procedures. Per-rectal endoscopic myotomy (PREM) is a recently described novel minimally invasive procedure based on the principles of third space endoscopy. It is based on the principle to open spastic aganglionic bowel segments by performing a myotomy through a submucosal tunnel. This review describes the patient selection and preparation and technique of PREM and discusses the status of PREM for treatment of HD.
先天性巨结肠（HD）是一种以先天性缺乏内胚层神经节细胞为特征的疾病。它通常在婴儿期表现为难治性便秘和生长发育不良。累及直肠乙状结肠的短段型 HD 是最常见的类型。尽管六十多年来，外科或腹腔镜的单阶段或多阶段拖出术一直是金标准，但这些手术与较高的发病率、复发率有关，而且通常需要多阶段手术。经直肠内镜肌切开术（PREM）是一种最近描述的基于第三空间内镜原理的新型微创方法。它基于通过黏膜下隧道进行肌切开术来打开痉挛性无神经节细胞肠段的原理。本文综述了 PREM 的患者选择、准备和技术，并讨论了 PREM 在治疗 HD 中的地位。
