Department of Surgery, Boston Children's Hospital, Boston, MA, USA.
Department of Surgery, Johns Hopkins All Children's Hospital, St. Petersburg, FL, USA.
J Pediatr Surg. 2024 Dec;59(12):161671. doi: 10.1016/j.jpedsurg.2024.08.011. Epub 2024 Aug 8.
The management of neonates with long-gap esophageal atresia (LGEA) combined with distal congenital esophageal strictures (CES) is challenging. We sought to review our approach for this rare set of anomalies.
We reviewed children with LGEA + CES surgically treated at two institutions (2018-2024). LGEA repair was performed using the Foker technique (traction-induced esophageal lengthening). A CES strategy was chosen based on preoperative evaluations and intraoperative findings. The configuration and length of the CES were assessed using retrograde flexible esophagoscopy via gastrostomy with contrast fluoroscopy.
Eight patients (75% male) with LGEA + CES were treated: Four had type A and four had type B EA. Median gap length was 3.5 cm. Three underwent thoracoscopic esophageal lengthening. After a median follow-up of 18 months (IQR: 9-25), all retained their native esophagus. However, those who had CES resection concurrent with the lengthening process or at the time of EA anastomosis had more challenging perioperative courses: one required additional time on traction and another required esophageal anastomotic stricture resection.
Our experience with LGEA and distal CES emphasizes tailoring surgical approaches to each patient's unique condition, avoiding a one-size-fits-all strategy. However, if the esophageal tissue above the distal CES is in good condition, our preference has shifted towards retaining the CES during traction, performing gentle dilation at anastomosis time, and conducting definitive endoscopic management subsequently. We would caution against making the assumption that salvage of the native esophagus is not possible or that resection of the CES is always needed.
Level III.
长间隙食管闭锁（LGEA）合并远端先天性食管狭窄（CES）的新生儿的处理具有挑战性。我们旨在回顾我们对此类罕见畸形的处理方法。
我们回顾了在两个机构（2018-2024 年）接受手术治疗的 LGEA+CES 患儿。采用 Foker 技术（牵引诱导食管延长术）进行 LGEA 修复。根据术前评估和术中发现选择 CES 策略。通过经胃造口逆行性软式食管镜检查并结合对比荧光透视术评估 CES 的形态和长度。
8 例 LGEA+CES 患儿（75%为男性）接受了治疗：4 例为 A 型，4 例为 B 型 EA。中位间隙长度为 3.5cm。3 例行胸腔镜下食管延长术。中位随访 18 个月（IQR：9-25）后，所有患儿均保留了自身食管。然而，那些在食管延长过程中或在 EA 吻合时同时切除 CES 或在吻合时切除 CES 的患儿围手术期过程更具挑战性：1 例需要额外的牵引时间，另 1 例需要切除食管吻合口狭窄。
我们在 LGEA 和远端 CES 的经验强调根据每个患者的独特情况定制手术方法，避免一刀切的策略。但是，如果远端 CES 上方的食管组织状况良好，我们倾向于在牵引时保留 CES，在吻合时进行温和的扩张，并随后进行明确的内镜管理。我们要提醒不要假设无法保留自身食管或总是需要切除 CES。
III 级。
