Congenital esophageal stenosis caused by tracheobronchial remnants: A case report.

Congenital esophageal stenosis caused by tracheobronchial remnants is a rare anomaly in infants and children that results in dysphagia and recurrent pneumonia. An accurate diagnosis is needed because this anomaly does not respond well to instrumental dilatation, such as bougienage and balloon dilatation, and requires surgical resection. Herein, I report a case of congenital esophageal stenosis caused by tracheobronchial remnants with characteristic imaging findings.

Congenital esophageal stenosis caused by tracheobronchial remnants: a case report.

We describe a case of congenital esophageal stenosis in which the patient underwent ineffective balloon dilatation twice and eventually required surgery. The case was initially misdiagnosed as achalasia. Pathological findings revealed tracheobronchial remnants (TBRs) in the muscular layer of strictured esophageal tissue. Most TBR strictures are located in the middle and lower thirds of the esophagus. Esophagography is the main examination method for esophageal stricture, and the appearance of the "rat tail sign" is a key diagnostic indicator. Endoscopic ultrasonography can reveal hypoechoic cartilaginous structures. The gold standards for TBR treatment include esophageal stricture resection, end-to-end esophageal anastomosis, and the construction of structures to prevent reflux. At present, endoscopic longitudinal resection and transverse anastomosis of the anterior esophageal wall with partial cartilage resection without pyloroplasty are novel and practical TBR procedures. To avoid further complications, patients with congenital esophageal stenosis should be promptly treated surgically if balloon dilatation is ineffective.

Laparoscopic-Assisted Longitudinal Incision and Transverse Anastomosis: A Novel Surgical Approach for Treatment of Esophageal Stenosis Caused by Tracheobronchial Remnants.

Purpose: To review the treatment of lower congenital esophageal stenosis caused by tracheobronchial remnants (TBR) and to introduce a novel technical approach through laparoscopic surgery. Methods: Patients with TBR who underwent surgery in our single-center from January 2016 to December 2019 were enrolled. Resection of cartilage with stenotic esophageal segment and end-to-end anastomosis was the traditional surgery. Since 2018, longitudinal incision with partial resection of cartilage loop in the anterior esophageal wall and the transverse suture was conducted endoscopically. We reviewed the treatment, followed-up with these patients, and discussed the new procedure's preponderance. Main Results: Thirteen patients underwent surgery and were followed-up for 0.5-45 months (M = 13) after surgery. Twelve patients showed good physical development with a regular diet. One patient, who was 2 weeks after the surgery, was fed by a soft diet and regularly followed-up at our clinic. In 13 cases, five patients underwent traditional laparotomy with pyloroplasty. Two patients who went through anastomotic leakage were cured by drainage and conservative treatments. Anastomotic stricture that occurred in two cases was improved by one-time of dilation. The administration time of parenteral nutrition (PN) was 9.0 ± 1.4 days. The length of hospitalization was 36.6 ± 5.2 days. Eight cases underwent the new surgical approach through laparoscopy or thoracoscopy. Pyloroplasty was avoided since the vagal close to the posterior wall of the esophagus was protected. Gastric motility disorder did not occur as expected. No leakage occurred postoperatively. The anastomotic stricture was found in six cases and improved after one to five times of dilations. The length of hospitalization dropped to 18.6 ± 6.9 days significantly (P < .001). Conclusions: Longitudinal incision and transverse anastomosis of the anterior wall of the esophagus with partial resection of cartilage without pyloroplasty through endoscopy is a novel practical surgical approach to treat patients with TBR.

Characteristics and treatment of congenital esophageal stenosis: A retrospective collaborative study from three Japanese children's hospitals.

BACKGROUND/PURPOSE: There is no consensus on treatment strategy of congenital esophageal stenosis (CES). This study aimed to assess appropriateness of the treatment we have provided to patients with CES over the past four decades. METHODS: We carried out a retrospective chart review of 83 CES patients treated at three children's hospitals between 1973 and 2015. Each patient underwent an initial treatment with either surgery or a series of dilation that was followed by surgery if dilation failed to improve esophageal transit. Demographic data, course of treatment, outcomes, and complications were analyzed. RESULTS: During this initial treatment, 19 and 64 patients underwent surgery and dilation, respectively. Out of the 64 patients who underwent dilations as an initial treatment, 26 patients eventually required surgery. Out of all patients who required surgery (19 initial treatments + 26 failed dilations), 29 had tracheobronchial remnants and 16 had fibromuscular hypertrophy. Six patients experienced esophageal perforation during dilation and ten experienced anastomotic leakage after surgery. No patients had swallowing difficulties at the latest follow up, 141(9-324) months. CONCLUSIONS: Dilation is recommended as an initial therapy, especially if histological diagnosis of CES is uncertain. Persistent swallowing difficulties after 2 series of dilation may be an indication for surgery. LEVELS OF EVIDENCE: level IV.

Congenital Esophageal Stenosis in Children: From Etiology to Prognosis

Congenital esophageal stenosis (CES) is a rare disease that has been reported to occur once in every 25,000 to 50,000 births. According to its etiology, CES is divided into 3 subtypes, tracheobronchial remnants (TBR), fibromuscular hypertrophy (FMH) and membranous diaphragm (MD). Symptoms begin at the weaning period and the introduction of solid food around 6 months with dysphagia and vomiting. Esophagography is first screening test and endoscopic ultrasonography plays important roles to diagnose subtypes deciding therapeutic plan. TBRs were generally treated with surgical resection and end-to-end anasotomosis, whereas FMH and MD had good response rate to endoscopic or radiologic guided dilatation. This article reviews the literature on the etiology, clinical course, diagnosis and management of CES including recent opinion.

Esophageal duplication and congenital esophageal stenosis.

Esophageal duplication and congenital esophageal stenosis (CES) may represent diseases with common embryologic etiologies, namely, faulty tracheoesophageal separation and differentiation. Here, we will re-enforce definitions for these diseases as well as review their embryology, diagnosis, and treatment.

Management of long segment congenital esophageal stenosis: A novel technique.

Congenital esophageal stenosis (CES) is a rare cause of dysphagia in children. Diagnosis is often apparent on esophagogram. Surgical treatment for the subtype with tracheobronchial remnants (TBR) includes resection and anastomosis of the stenosed segment, myectomy, enucleation of cartilage, etc., These procedures are not suitable if the stenosed segment is long. We present a case of a 5-year-old boy who was diagnosed as CES and was successfully managed with stricturoplasty with some innovative modifications.

The Diagnosis and Treatment of Congenital Esophageal Stenosis

PURPOSE: Congenital esophageal stenosis (CES) is a rare cause of esophageal narrowing due to intrinsic esophageal wall abnormalities such as tracheobronchial remnants (TBR), web, and fibromuscular dysplasia (FMD). It is sometimes associated with esophageal artesia (EA). METHODS: The medical records of children, who underwent operation due to CES at the Asan Medical Center from Jan 1990 to Dec 2007, were retrospectively reviewed. RESULTS: Among 12 patients (M : F=6 : 6), 9 patients had TBR and 3 had FMD. The median age of operation was 25 months (7 mo~6 years). Four patients underwent operation for esophageal atresia at neonate, one underwent operation for duodenal atresia and one for ventricular septal defect. Recurrent vomiting or dysphagia was developed at weaning period or introduction of solid foods. The lesions of stenosis were low-esophagus in 11 and mid-esophagus in 1. Segmental resection of lesion and end-to-end anastomosis of esophagus were completed successfully by means of laparotomy in 9 and thoracotomy in 3 (left in 1, right in 2). One patient treated with ballooning at first, suffered from mediastinitis due to esophageal perforation. Postoperative complications were anastomosis leakage in 2 and lung abscess in 1. Esophageal ballooning for anastomosis stricture was performed in 2 patients and an esophageal stent was needed in 1 patient. All patients are relieved from dysphagia and able to eat solid food. CONCLUSION: CES is rare but should be considered for recurrent vomiting and dysphagia, especially for those who underwent operation for EA. Differential diagnosis from other causes such as gastro-esophageal reflux is essential and surgical treatment is treatment of choice.

A Case of Congenital Esophageal Stenosis Due to Tracheobronchial Remnants in Adult / 대한소화기내시경학회지

Congenital stenosis of the esophagus is a rare form of esophageal stenosis in adults. The main causes of congenital esophageal stenosis are the esophageal web, esophageal stricture due to tracheobronchial remnants, and idiopathic esophageal muscular hypertrophy. Recently we have experienced a 32-year-old male with dysphagia, indigestion, postprandial chest discomfort who was diagnosed as congenital esophageal stenosis due to tracheobronchial remnant. The esophagogram showed stricture of the distal esophagus with secondary proximal dilatation and endoscopic finding revealed marked stenosis on the distal esophagus with normal surrounding mucosa. The esophageal manometric finding showed decreased body peristalsis and incomplete relaxation of the lower esophageal sphincter. The patient was treated by surgical resection of the stenotic segment with end to end anastomosis. We report this rare case of adult type tracheobronchial remnant with analysis of various worldwide report and with brief review of literature.

Congenital Esophageal Stenosis: with Special Reference to Diagnosis and Postoperative Complications

PURPOSE: Congenital esophageal stenosis(CES) is one of the rare causes of recurrent vomiting during infancy and childhood. We studied the diagnostic and therapeutic tools and postoperative complications for early diagnosis and adequate management of CES. METHODS: Fourteen cases of CES were evaluated for clinical manifestations, findings of esophagogram and esophagoscopy, classification of pathologic findings and postoperative complications. RESULTS: Most common clinical manifestations at onset were non-projectile vomiting(14), dysphagia to solids(13). Age at onset of symptoms corresponded with the introduction of solids in 11 cases. Esophagogram showed segmental stenosis of variable length in the lower portion of the esophagus in all cases with marked proximal dilatation in 11 cases. Esophagoscopy revealed no signs of esophagitis or ulcer at the area of stenosis. Segmental resection and primary anastomosis were performed as a definitive treatment modality in all cases except one with fibromuscular stenosis. Bronchial cartilage were present in all cases of tracheobronchial remnants(10). Abnormal arrangement and thickening of muscularis mucosae and inner circular muscle were found in all cases of fibromuscular stenosis(4). Postoperative complications were gastroesophageal reflux(5), stricture of anastomotic sites, reflux esophagitis, and so on. CONCLUSION: CES is rare but should be considered as a cause of recurrent vomiting and dysphagia to solid food beginning in infancy and childhood especially in the weaning period. Esophagogram and esophagoscopy are useful tools for diagnosis and differential diagnosis. The stricture of anastomosis site, gastroesophageal reflux and esophagitis need to be evaluated in the follow-up postoperative periods.

Four Cases of Congenital Esophageal Stenosis Due to Tracheobronchial Remnants

Esophageal stenosis due to tracheobronchial remnants is a rare cause of congenital esophageal stenosis. The cause is thought to be esophageal sequestration of tracheobronchial remnants during embryonic separation. Errors in diagnosis are common and high index of suspicion is required for accurate diagnosis, and resection of primary site with anastomosis is recommanded. Although a rare entity, esophageal stenosis due to tracheobronchial remnants should be considered in patients with esophageal stenosis, when esophageal stenosis fails to respond to standard therapy including bougienage and balloon dilatation, and esophagoscopy shows normal mucosa on the stenotic segment. Four cases of congenital esophageal stenosis due to tracheobronchial remnants were reported, the first case without accompanied anomaly, the second case accompanied by tracheoesophageal fistula, the third case accompanied by Down syndrome, and the last case accompanied by tracheoesophageal fistula and congenital heart disease.