Tambucci Roberto, Wautelet Océane, Haenecour Astrid, François Geneviève, Goubau Christophe, Scheers Isabelle, Halut Marin, Menten Renaud, Schmitz Sandra, de Toeuf Caroline, Pirotte Thierry, D'hondt Beelke, Reding Raymond, Poncelet Alain
Pediatric Surgery and Transplantation Unit, Department of Surgery, Saint-Luc University Clinics, Brussels, Belgium.
Pediatric Intensive Care Unit, Emergency Department, Saint-Luc University Clinics, Brussels, Belgium.
Front Pediatr. 2020 Nov 20;8:605143. doi: 10.3389/fped.2020.605143. eCollection 2020.
Abnormal connections between the esophagus and low respiratory tract can result from embryological defects in foregut development. Beyond well-known malformations, including tracheo-esophageal fistula and laryngo-tracheo-esophageal cleft, rarer anomalies have also been reported, including communicating bronchopulmonary foregut malformations and tracheal atresia. Herein, we describe a case of what we have called "esophageal trachea," which, to our knowledge, has yet to be reported. A full-term neonate was born in our institution presenting with a foregut malformation involving both the middle esophagus and the distal trachea, which were found to be longitudinally merged into a common segment, 3 cm in length, located just above the carina and consisted of esophageal tissue without cartilaginous rings. At birth, the esophagus and trachea were surgically separated via right thoracotomy, the common segment kept on the tracheal side only, creating a residual long-gap esophageal atresia. The resulting severe tracheomalacia was treated via simultaneous posterior splinting of such diseased segment using an autologous pericardium patch, as well as by anterior aortopexy. Terminal esophagostomy and gastrostomy were created at that stage due to the long distance between esophageal segments. Between ages 18 and 24 months, the patient underwent native esophageal reconstruction using a multistage traction-and-growth surgical strategy that combined Kimura extra-thoracic esophageal elongations at the upper esophagus and Foker external traction at the distal esophagus. Ten months after esophageal reconstruction, prolonged, refractory, and severe tracheomalacia was further treated via anterior external stenting using a semitubular ringed Gore-Tex® prosthesis, through simultaneous median sternotomy and tracheoscopy. Currently, 2 years after the last surgery, respiratory stabilization, and full oral feeding were stably achieved. Multidisciplinary management was crucial for assuring lifesaving procedures, correctly assessing anatomy, and planning for multiple sequential surgical approaches that aimed to restore long-term respiratory and digestive functions.
食管与下呼吸道之间的异常连接可由前肠发育的胚胎学缺陷引起。除了众所周知的畸形，包括气管食管瘘和喉气管食管裂，还报道了一些罕见的异常情况，包括连通性支气管肺前肠畸形和气管闭锁。在此，我们描述了一例我们称之为“食管气管”的病例，据我们所知，该病例尚未见报道。一名足月儿在我们机构出生，患有涉及食管中段和气管远端的前肠畸形，发现它们纵向合并成一个长3厘米的共同节段，位于隆突上方，由无软骨环的食管组织组成。出生时，通过右胸切开术将食管和气管手术分离，共同节段仅保留在气管侧，导致残留长间隙食管闭锁。由此产生的严重气管软化通过使用自体心包补片对病变节段进行同步后路夹板固定以及前路主动脉固定术进行治疗。由于食管节段之间距离较长，在该阶段进行了终末食管造口术和胃造口术。在18至24个月龄之间，患者采用多阶段牵引和生长手术策略进行了原位食管重建，该策略结合了上段食管的木村胸外食管延长术和下段食管的福克外牵引术。食管重建术后10个月，通过使用半管状带环的戈尔泰克斯®假体进行前路外部支架置入，同时进行正中胸骨切开术和气管镜检查，对持续存在的、难治性的严重气管软化进行了进一步治疗。目前，在最后一次手术后2年，呼吸稳定，完全经口喂养得以稳定实现。多学科管理对于确保挽救生命的手术、正确评估解剖结构以及规划旨在恢复长期呼吸和消化功能的多个连续手术方法至关重要。
