Etskovitz Haley, Kim Rosa S, Wang Sarah Ziqi, Nandivada Prathima
Department of General Surgery, Boston Children's Hospital, Boston, Massachusetts, USA.
World J Pediatr Surg. 2024 Dec 31;7(4):e000916. doi: 10.1136/wjps-2024-000916. eCollection 2024.
Hirschsprung disease (HSCR) is the most common congenital motility disorder of the intestine, characterized by the absence of ganglion cells in the myenteric and submucosal plexuses, leading to functional bowel obstruction. Short-segment Hirschsprung disease (SS-HSCR) accounts for the majority of cases, with surgical resection being the cornerstone of treatment. Despite advances in surgical techniques, considerable variability exists in practice regarding the timing of surgery, the choice of technique, and the length of aganglionic rectal cuff to resect. This review synthesizes the current evidence surrounding surgical management of SS-HSCR, discussing technique-specific outcomes and areas for future research, with a focus on optimizing patient care and functional outcomes.
先天性巨结肠症(HSCR)是最常见的先天性肠道动力障碍性疾病,其特征是肌间神经丛和黏膜下神经丛中缺乏神经节细胞,导致功能性肠梗阻。短段先天性巨结肠症(SS-HSCR)占大多数病例,手术切除是治疗的基石。尽管手术技术有所进步,但在手术时机、技术选择以及切除无神经节直肠袖带的长度等实际操作方面仍存在很大差异。本综述综合了目前关于SS-HSCR手术治疗的证据,讨论了特定技术的结果和未来研究领域,重点是优化患者护理和功能结局。