Congenital esophageal stenosis associated with esophageal atresia: new concepts

Abstract

Congenital esophageal stenosis (CES) is suspected by a fixed intrinsic narrowing of the esophagus that affects the normal swallowing mechanism. The diagnosis is only confirmed by histopathologic picture, which may show fibromuscular disease (FMD) or tracheobronchial remnants (TBR). The latter involves ciliated pseudo stratified columnar epithelium, seromucous glands or cartilage each alone or in combination. The aim of this study is to document the usefulness of histologic picture of surgical specimens obtained from the lower esophageal pouch (LEP) during primary repair in detecting cases of CES associated with esophageal atresia (EA) with or without tracheoesophageal fistula (TEF). Over an 8-year period, 57 consecutive cases operated upon for EA with or without TEF were subjected for histologic examination of surgical specimens obtained from the tip of the LEP. Cases that histologically showed FMD or TBR were included. The usefulness of this histologic picture as a diagnostic and therapeutic aid is assessed. Methods of treatment and outcome were also reviewed. Eight patients out of 57 (14%) had a histologic picture suggestive of CES, two with FMD, four with TBR without cartilage and two with cartilage. Out of 57 patients, 23 developed strictures, six of them had positive biopsies suggestive of CES. One patient with TBR without cartilage did not have stricture. Another case of pure atresia had LEP resection and gastric pull up showed cartilage involving the whole lower esophagus. Excluding the case of pure EA with gastric pull up, all patients suffered from feeding problems and recurrent aspiration. Fluoroscopic barium studies showed late onset minor dysmotility in five patients and late onset major dysmotility in two. All cases studied showed significant gastro-esophageal reflux (GER). Stricture was seen at the anastomotic site extending distally in the two fibromuscular cases and one case with cartilage, at the anastomotic site in three cases with TBR without cartilage. Anti reflux surgical procedures were performed in four patients without benefit in two patients with major dysmotility. Dilatation was successful in the three patients with TBR without cartilage. One patient with cartilage had resection of the anastomotic site and required frequent dilatations and is now doing well. A case of FMD did not improve after frequent dilatations and myotomy together with Nissen's fundoplication and required resection while the other case of FMD responded partially to dilatations. Cartilage in cases of CES requires surgical resection. Those with TBR without cartilage may not develop stricture. If stricture develops, it responds well to dilatation and patients have good clinical outcomes. Unlike isolated CES, GER is a significant feature in CES with EA. Anti reflux procedures should be avoided before definitive surgery for the stricture and if necessary a partial wrap with gastrostomy is recommended. CES should be considered in the etiology of anastomotic stricture. Taking a surgical specimen routinely from the tip of th...

Management of congenital esophageal stenosis associated with esophageal atresia and its impact on postoperative esophageal stricture

“…CES at the anastomotic site was defined as histology showing tracheobronchial remnants (TBR) or fibromuscular abnormality consistent with fibromuscular stenosis (FMS). From our previous and present experience [3], a histology similar to this will present sooner or later with stenosis and/or esophageal dysmotility. CES distal to the anastomotic site was diagnosed at primary repair either by failure to pass a size 6 nasogastric tube distally or by having a high index of suspicion during initial esophagogram.…”

“…The authors of the present study believe that the middle third of the esophagus corresponds to the anastomotic site in EA. It has been reported earlier that CES can affect the anastomotic site [3,6] or distal to it [2,7,8]. The diagnosis of CES at the anastomotic site can be confirmed by means of histological examination of specimens obtained from the tips of the esophageal pouches during primary repair of EA [3,6].…”

“…In a study on 61 patients with CES, 29 had associated EA [5]. CES can affect the anastomotic site [3,6] or distal to it [2,7,8]. The incidence of anastomotic esophageal stricture (ES) after repair of EA ranges from 18 to 50% [9,10].…”

“…The incidence of congenital esophageal stenosis (CES) associated with esophageal atresia (EA) ranges from 0.4 to 14% [1][2][3]. According to four observational studies, the overall incidence of CES among patients with EA and/or tracheoesophageal fistula (TEF) was 9.6% [4].…”

Quality of reporting of the literature on gastrointestinal reflux after repair of esophageal atresia–tracheoesophageal fistula

“…Managing GER medically in EA-TEF patients is important not only for symptom relief, but also to reduce stricture formation, the potential for malignancy and the need for surgical intervention [21,34,40,44,57]. However, there exists wide variation in the medical management of postoperative GER in EA-TEF; pediatric surgeons must base their clinical decision-making on the available, heterogeneous literature.…”