Center for Colorectal and Pelvic Reconstruction, Nationwide Children's Hospital, Columbus, Ohio, United States.
Department of Pediatric Surgery, University Hospital La Paz, Madrid, Spain.
Eur J Pediatr Surg. 2020 Aug;30(4):309-316. doi: 10.1055/s-0039-1694744. Epub 2019 Aug 20.
Total colonic Hirschsprung's disease (TCHD) presents a postoperative challenge due to multiple stools and perineal rash. We propose a protocol developed by pediatric surgeons and ostomy nurses to help prevent and treat hypermotility and severe perineal rash, especially in younger children who are not toilet trained.
We retrospectively reviewed our TCHD patients' charts from 2014 to 2017. All patients received a prescribed protocol for the treatment of hypermotility and perineal rash. We describe patients who underwent their pull through before and after the age of urine toilet training, and assessed the number of bowel movements, the perineal skin status, and growth.
We treated 25 patients. Out of 25, 9 patients received a straight ileoanal pull through before the age of 18 months. Nine of 25 patients presented for a second opinion and had redo pull through. The remaining seven presented for bowel management after having a pull through at another institution. All these were treated following the hypermotility protocol. In total, 19 of 25 patients were not toilet trained. The mean number of bowel movements in all groups was 4 (3-5). All had a resolution of perineal rash and liquid stools after 3 months. Eleven of the 25 patients presented with failure to thrive. Two older patients experienced severe proctalgia requiring replacement of the ileostomy.
TCHD patients who underwent definitive pull through had nine high incidence of multiple stool, perineal rash, and low growth. With the implementation of bowel management care to slow the stools and a perineal skin protocol to treat the skin, we believe that these symptoms can be minimized even in patients who are not toilet trained. Since the implementation of this protocol, we have changed our practice to perform the pull through in such patients between the age of 6 and 18 months.
全结肠型先天性巨结肠（TCHD）术后存在排便次数多和肛周皮疹等问题。我们提出了一套由小儿外科医生和造口护士制定的方案，旨在帮助预防和治疗过度活动和严重的肛周皮疹，特别是对于尚未接受如厕训练的年幼患儿。
我们回顾性分析了 2014 年至 2017 年期间 TCHD 患者的病历。所有患者均接受了治疗过度活动和肛周皮疹的既定方案。我们描述了在接受拖出术治疗前和接受治疗后仍未接受如厕训练的患儿，并评估了排便次数、肛周皮肤状况和生长情况。
我们共治疗了 25 例患者。其中 9 例患儿在 18 个月前接受了直接回肠肛管拖出术。25 例患者中有 9 例因第二次就诊和需要进行再次拖出术而接受治疗。其余 7 例患儿在另一家机构接受拖出术治疗后因肠道管理问题来我院就诊。所有这些患儿均根据过度活动方案进行治疗。25 例患儿中共有 19 例未接受如厕训练。所有患儿的平均排便次数均为 4（3-5）次。所有患儿在 3 个月后均出现肛周皮疹和稀便消退。25 例患儿中有 11 例存在生长不良。2 例年龄较大的患儿出现严重的直肠痛，需要更换肠造口。
接受确定性拖出术治疗的 TCHD 患儿排便次数多、肛周皮疹、生长不良的发生率高。通过实施减缓粪便排出速度的肠道管理护理方案和肛周皮肤护理方案，我们认为即使对于未接受如厕训练的患儿，这些症状也可以得到最小化。自实施该方案以来，我们已经改变了在该年龄段（6-18 个月）对这类患儿实施拖出术的做法。
