Umberto Bosio Center for Digestive Diseases, AO SS Antonio e Biagio e Cesare Arrigo, Alessandria, EU, Italy.
Unit of Minimally Invasive and Robotic Surgery, Department of Surgery, Ospedale Pediatrico Bambino Gesù, IRCCS, Piazza di Sant'Onofrio, 4, 00165, Rome, Italy.
Pediatr Surg Int. 2020 Dec;36(12):1507-1510. doi: 10.1007/s00383-020-04763-4. Epub 2020 Oct 23.
Extended aganglionosis (TIA) is the presence of some viable aganglionic gut distal to the levelling jejunostomy. Different surgical procedures (including transplantation) have been proposed with inconclusive results. We conceived a new procedure named skipped aganglionic lengthening transposition (SALT) consisting of multiple pedicled isoperistaltic transpositions of aganglionic ileal loops interposed to normoganglionic jejunum. The innovative aspect consists of taking advantage of the propulsive effect of normoganglionated bowel to progress enteric content throughout interposed aganglionic loops down to the stoma. The procedure was adopted in a male patient who was born with 30 cm of normoganglionated jejunum. SALT was performed when the baby was 18 months. Three 5-cm pedicled isoperistaltic aganglionic loops of small bowel were interposed each 10 cm of normoganglionic jejunum with an overall 36% length gain (from 42 to 57 cm). Postoperative course was uneventful. 6 months postoperatively, an upper gastrointestinal series showed normal progression without dilatations. A laparoscopic gastrostomy was performed due to food aversion 6 months postoperatively, demonstrating impressive anatomic and functional postoperative results. The procedure provides promising and unique opportunity for patients with TIA with encouraging outlook for the near future.
延长性无神经节细胞症（TIA）是指在水平空肠造口术以下存在一些有活力的无神经节细胞肠道。已经提出了不同的手术程序（包括移植），但结果不一致。我们设计了一种新的手术程序，称为跳过无神经节细胞延长转位（SALT），包括多个有蒂等蠕动转位，将无神经节细胞回肠环插入到正常神经节细胞空肠中。创新之处在于利用正常神经节细胞肠的推进作用，使肠内容物通过插入的无神经节细胞环向下到达造口。该手术程序应用于一名出生时具有 30 厘米正常神经节细胞空肠的男性患者。当婴儿 18 个月大时进行了 SALT 手术。三个 5 厘米有蒂等蠕动无神经节细胞小肠环每隔 10 厘米正常神经节细胞空肠插入一次，总长度增加了 36%（从 42 厘米增加到 57 厘米）。术后过程顺利。术后 6 个月，上消化道系列检查显示正常推进，无扩张。由于术后 6 个月出现食物厌恶，进行了腹腔镜胃造口术，显示出令人印象深刻的解剖和功能术后结果。该手术程序为 TIA 患者提供了有希望的独特机会，对近期前景充满期待。
