Department of Pediatric Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430022, China.
Pediatr Surg Int. 2021 Jun;37(6):799-805. doi: 10.1007/s00383-021-04902-5. Epub 2021 Apr 21.
It is challenging to establish a definitive diagnosis and initiate timely management for skip segment Hirschsprung's disease (SSHD). Herein, we report three cases of SSHD at our institution between December 2008 and March 2018. Patient #1 was misdiagnosed and underwent three successive operations within 2 years. Patient #2 and #3 were diagnosed timely based on previous experience. A segmental narrowing detected by barium enema may raise the possibility of SSHD. Laparoscopic-guided multipoint biopsy is regarded as a mini-invasive and purposeful way to take representative samples after locating the segmental narrowing intraoperatively and accurate the final diagnosis. The laparoscopic-assisted pull-through procedure with radical resection from the distal rectum to the most proximal margin of the diseased skip segment is safe and effective in treating patients with SSHD.Level of evidence: Level III.
对于跳跃节段型先天性巨结肠(skip segment Hirschsprung's disease,SSHD),明确诊断和及时进行治疗具有一定挑战性。本文报告了我院 2008 年 12 月至 2018 年 3 月期间诊治的 3 例 SSHD 患儿的临床资料。病例 1 患儿误诊,2 年内经历了 3 次手术。病例 2 和病例 3 根据既往经验及时诊断。钡剂灌肠显示节段性狭窄可能提示 SSHD。腹腔镜引导下多点活检术可在术中定位狭窄段后获取有代表性的标本,从而明确诊断,是一种微创、有针对性的方法。对于 SSHD 患儿,从远端直肠至病变跳跃段最近端行腹腔镜辅助经肛门拖出术联合根治性切除术是安全有效的。循证医学等级:III 级。