Boston Children's Hospital, Boston, MA, USA.
Kapiolani Medical Center for Women and Children, Honolulu, HI, USA.
J Pediatr Surg. 2014 Jan;49(1):104-7; discussion 108. doi: 10.1016/j.jpedsurg.2013.09.037. Epub 2013 Oct 5.
Since its introduction as an alternative intestinal lengthening technique, serial transverse enteroplasty (STEP) has been increasingly used as the surgical treatment of choice for patients with refractory short bowel syndrome (SBS). While primary STEP for the treatment of congenital conditions was proposed in the original description of the procedure, emphasis was placed on a delayed or staged approach to these patients. To date, a comprehensive review of the outcomes from this sub-population has not been reported by the International STEP Data Registry.
A retrospective review of the International STEP Data Registry was performed to identify all patients who underwent STEP as a primary operative procedure for the treatment of congenital SBS. Changes in pre- and post-STEP values were assessed using paired t-tests with significance set at p<0.05. Data are presented as mean ± standard deviation.
Fifteen patients underwent primary STEP for congenital SBS between September 1, 2004, and April 10, 2012. Thirteen patients had follow-up information available. Causes of congenital SBS included closing gastroschisis, small bowel atresia, and midgut volvulus. Twelve patients had pre- and post-STEP bowel measurements taken. Average pre- and post-STEP bowel lengths were 32 ± 16 cm and 47 ± 22 cm, respectively. Intestinal length was increased by a mean of 15 ± 12 cm for a relative small bowel length increase of 50.4 ± 27.3% (p<0.001). Only one patient required an ostomy at the time of primary STEP. A second patient required a temporary ostomy at 3months of age that was later closed. There was one death from intestinal failure associated liver disease (IFALD). Another patient experienced IFALD progression and required liver and intestinal transplantation. The most commonly reported complication following primary STEP was obstruction or bowel re-dilatation requiring additional operative interventions. Nine patients underwent second STEP procedures under these circumstances. Eight patients remain dependent on parenteral nutrition, while three patients achieved enteral autonomy.
Primary STEP is a feasible and safe surgical option for the treatment of congenital conditions resulting in SBS. Primary STEP establishes early bowel continuity, creates intestinal length from congenitally dilated bowel, and appears to obviate the need for interval stomas and their associated loss of bowel length in neonates with congenital SBS. However, with recent changes in SBS management emphasizing intestinal rehabilitation, additional studies are needed to assess the long-term impact on intestinal adaptation of STEP performed in the neonatal period prior to adoption of this technique.
自作为替代肠延长技术引入以来，连续横向肠成形术（STEP）已越来越多地被用作治疗难治性短肠综合征（SBS）患者的首选手术治疗方法。虽然最初描述该手术时提出了用于治疗先天性疾病的原发性 STEP，但重点是对这些患者采用延迟或分期方法。迄今为止，国际 STEP 数据登记处尚未对此亚人群的结果进行全面审查。
对国际 STEP 数据登记处进行回顾性审查，以确定所有接受 STEP 作为治疗先天性 SBS 的主要手术治疗的患者。使用配对 t 检验评估 STEP 前后的值变化，并将显著性设置为 p<0.05。数据表示为平均值±标准偏差。
2004 年 9 月 1 日至 2012 年 4 月 10 日期间，15 名患者因先天性 SBS 接受了原发性 STEP。13 名患者有随访信息。先天性 SBS 的病因包括闭合性腹裂、小肠闭锁和中肠旋转不良。12 名患者进行了 STEP 前后的肠道测量。平均 STEP 前后肠长度分别为 32±16cm 和 47±22cm。肠长度平均增加 15±12cm，相对小肠长度增加 50.4±27.3%（p<0.001）。只有 1 名患者在原发性 STEP 时需要造口术。另一名患者在 3 个月大时需要临时造口术，后来关闭。有 1 例患者死于与肠衰竭相关的肝病（IFALD）。另一名患者 IFALD 进展，需要进行肝肠移植。原发性 STEP 后最常见的并发症是梗阻或肠道再扩张，需要进一步手术干预。9 名患者在这种情况下进行了第二次 STEP 手术。8 名患者仍依赖于肠外营养，3 名患者实现了肠内自主。
原发性 STEP 是治疗导致 SBS 的先天性疾病的可行且安全的手术选择。原发性 STEP 建立了早期肠道连续性，从先天性扩张的肠道中创造了肠道长度，并似乎避免了先天性 SBS 新生儿中间隔造口术及其相关的肠道长度损失的需要。然而，随着 SBS 管理的最新变化强调肠道康复，需要进一步的研究来评估在采用该技术之前在新生儿期进行的 STEP 对肠道适应的长期影响。
