Videofluoroscopy, CT angiography, and ultrasonography of congenital esophageal stenosis in a Labrador retriever.

A 7-week-old Labrador retriever presented for further investigation into acute onset regurgitation, following weaning from liquid to solid food. A videofluoroscopic swallow study demonstrated a severe, focal esophageal dilation in the mid-cervical region, with marked luminal narrowing distally. Computed tomography with angiography revealed esophageal stenosis, extending from C4-T2, secondary to circumferential esophageal wall thickening. With the concern for development of secondary aspiration pneumonia, the owners elected to euthanize the dog and consented to postmortem examination. A diagnosis of segmental, cervical esophageal muscular hypertrophy was made on necropsy, consistent with the fibromuscular thickening type of congenital esophageal stenosis reported in humans.

Complications After endoscopic balloon dilatation of esophageal strictures in children. Experience from a tertiary center in Shiraz - Iran (Nemazee Teaching Hospital).

BACKGROUND: Esophageal stricture is one of the most important complication of the caustic ingestion. OBJECTIVE: The aim of this study was to evaluate complications of balloon dilatation among children with esophageal stenosis. MATERIAL AND METHODS: In this retrospective study 82 children were included. Children who underwent balloon dilatation for esophageal stenosis were included in our study. Duration of study was 14 year starting from 2001. Mean age of the cases was 3.95±0.4 year (Min: 15 days, Max: 14 year). Chart review and telephone calling were the methods of data collection. Data was analyzed using SPSS. RESULTS: In this study, 47% of the patients were male and 53% of the cases were female. Caustic ingestion (33.7%) was the most common etiology for the esophageal stricture. Vomiting (87.8%) was the most common presenting symptom. Among our cases, 76.8% had no compliant after esophageal dilatation. Chest pain was the most common compliant after esophageal dilatation. Response rate was similar among boys and girls. Toddler age had the best treatment response after esophageal dilatation. CONCLUSION: Among our cases, 76.8% had no post procedural compliant after esophageal dilatation. Esophageal perforation was seen in 4.9% of the cases. Chest pain was the most common post dilatation complication.

Early and late outcomes after surgical management of congenital vascular rings.

Persistent respiratory or feeding problems in children may be associated with a congenital vascular ring. Surgical management is fairly standardized, but long-term outcomes are not well described. This study aims to investigate clinical presentation, surgical treatment, and risk factors for early mortality and late outcome. Our database revealed 62 surgically treated vascular ring patients between 1993 and 2014. Double aortic arch was the most common diagnosis (53%). Median age at operation was 1 year. Symptoms were mainly respiratory (89%) and feeding problems (32%). Median extubation time and hospital stay were 4 h and 5 days. Mean follow-up was 7.8 ± 5.8 years. Early mortality was 8% and was related to anatomical diagnosis, concomitant anomalies, and a need for preoperative intubation. Freedom from residual symptoms at 1 and 6 months was 63 and 82%, respectively. Freedom from inhalation therapy at the last follow-up was 82% and was influenced by a type of vascular ring and preoperative ventilation. Dysphagia symptoms always disappeared. CONCLUSION: Surgical relief of tracheoesophageal compression is commonly effective in vascular ring anomalies. Respiratory symptoms necessitating chronic inhalation therapy only persist in a minority of children. Patients with double aortic arch are at increased risk to remain symptomatic, particularly with infectious exacerbations.

Current characteristics and management of congenital esophageal stenosis: 40 consecutive cases from a multicenter study in the Kyushu area of Japan.

PURPOSE: Congenital esophageal stenosis (CES) is rare, and the available clinical data are limited. We explored the current diagnosis, treatment and outcomes of CES. METHODS: A questionnaire survey was performed using medical records at pediatric surgical centers in the Kyushu area. RESULTS: Over 10 years, 40 patients (24 males) had CES. The incidence of associated anomalies was 52.5% (21/40), and that of esophageal atresia was 20.0% (8/40). The mean age at the diagnosis was 12.0 months (range, 1 day-8.8 years). Seven (17.5%) patients were diagnosed in the neonatal period. Ten (25.0%) developed CES due to tracheobronchial remnants, 27 (67.5%) due to fibromuscular stenosis (FMS) and 1 (2.5%) due to membranous stenosis + FMS. Thirty-six (90.0%) were treated by balloon dilatation (mean, 3 times; range, 1-20). Perforation at dilatation occurred in 7 (17.5%) patients, and all were diagnosed with FMS. Eighteen (45.0%) patients underwent radical operation (3 primary, 15 secondary to dilatation). CONCLUSIONS: Our study clarified the characteristics and outcomes of CES, including neonatal diagnoses. CES occurred in 1 in every 33,000 births in the Kyushu area. Careful attention should be paid, even in cases of dilatation for FMS. CES requires long-term follow-up for symptom persistence after adequate and repeated treatment.

Steroid pulse therapy prevents restenosis following balloon dilatation for esophageal stricture.

PURPOSE: This study aimed to evaluate the effectiveness of intravenous steroid pulse therapy following balloon dilatation for esophageal stenosis and stricture in children. METHODS: The study enrolled six children, including three with congenital esophageal stenosis and three with anastomotic strictures after surgery for esophageal atresia, all of whom were treated by balloon dilatation combined with high-dose intravenous methylprednisolone pulse therapy. Methylprednisolone was injected intravenously at a dose of 20 mg/kg/day for 2 days, starting from the day of dilatation, followed by 10 mg/kg/day for 2 days, for a total of 4 days. RESULTS: Esophageal stricture recurred in all three patients with congenital esophageal stenosis despite repeated balloon dilatation without methylprednisolone. However, the symptoms of dysphagia improved and did not recur after systemic steroid pulse therapy following balloon dilatation. Symptoms also resolved in all three patients with anastomotic strictures following balloon dilatation with systemic steroid pulse therapy. All six patients remained asymptomatic after 6-21 months follow-up, with no complications. CONCLUSION: Intravenous methylprednisolone pulse therapy following balloon dilatation is safe and effective for the treatment of esophageal stenosis and strictures in children.

Congenital esophageal stenosis associated with esophageal atresia.

Congenital esophageal stenosis (CES) is a rare clinical condition but is frequently associated with esophageal atresia (EA). The aim of this study is to report the diagnosis, management, and outcome of CES associated with EA. Medical charts of CES-EA patients from Lille University Hospital, Sainte-Justine Hospital, and Montreal Children's Hospital were retrospectively reviewed. Seventeen patients (13 boys) were included. The incidence of CES in patients with EA was 3.6%. Fifteen patients had a type C EA, one had a type A EA, and one had an isolated tracheoesophageal fistula. Seven patients had associated additional malformations. The mean age at diagnosis was 11.6 months. All but two patients had non-specific symptoms such as regurgitations or dysphagia. One CES was diagnosed at the time of surgical repair of EA. In 12 patients, CES was suspected based on abnormal barium swallow. In the remaining four, the diagnostic was confirmed by esophagoscopy. Eleven patients were treated by dilation only (1-3 dilations/patient). Six patients underwent surgery (resection and anastomosis) because of failure of attempted dilations (1-7 dilations/patient). Esophageal perforation was encountered in three patients (18%). Three patients had histologically proven tracheobronchial remnants. CES associated with EA is frequent. A high index of suspicion for CES must remain in the presence of EA. Dilatation may be effective to treat some of them, but perforation is frequent. Surgery may be required, especially in CES secondary to ectopic tracheobronchial remnants.

Eosinophilic esophagitis: a paradigm shift for pathology.

BACKGROUND: Eosinophilic esophagitis (EoE) is an increasingly recognized disease only reported in the adult literature since 1993. Our facility has the opportunity to evaluate steroid-naive EoE patients since 1988, allowing us to describe the evolution of the histologic diagnosis of EoE. METHODS: This retrospective study analyzed 95 patient charts with initial diagnosis of congenital esophageal stenosis/EoE from 1988 to 2012. Patients had dysphagia and met endoscopic criteria consistent with current EoE histologic criteria while on chronic proton-pump inhibitor therapy. Histologic slides were obtained for only 10 patients because of limited slide availability; and the slides were reviewed by a single pathologist. EoE is defined as ≥1 biopsy specimens demonstrating >15 eosinophils/HPF. RESULTS: Following review of histologic analysis reports, pathologists in our 2 academic hospitals began recognizing EoE as separate disease entity starting in 2007, coincidental with first EoE guidelines. After 2007, there was a clear surge in histologic diagnosis of EoE. Slides from 10 patients from 1988 to 2012 were reviewed. Of 35 biopsy sets, 19 were previously interpreted as reflux esophagitis (RE) or acute/chronic inflammation, 3 as RE with eosinophils, 2 as normal, and 11 as EoE. Reevaluation revealed EoE in 79% specimens with RE/inflammation and 100% with RE and eosinophils; remaining specimens had confirmed original diagnosis. All 10 patients had at least one set of slides meeting current EoE histologic criteria. CONCLUSIONS: EoE as a disease has been present for at least 2 to 3 decades. This is the first report of a clearly demarcated time point reflecting a paradigm shift in the histologic diagnosis of EoE as a distinct entity resulting from a seminal consensus report.

[Congenital esophageal stenosis: a case report]. / Estenosis esofágica congénita: A propósito de un caso.

Congenital esophageal stenosis, a rare disease of unknown cause which reports have increased in the last few years, requires a high index of suspicion for its diagnosis and treatment. It can be classified in three types based on the etiology of the stenosis: tracheobronchial rest, fibromuscular hypertrophy and membranous diaphragm. Symptoms may vary depending on location and severity of the stenosis. Treatment options are based on clinical suspicion of the histologic type and they can be balloon dilation or surgical resection of the stenotic segment. The definitive diagnosis is the histological study.

Esophagitis with eosinophil infiltration associated with congenital esophageal atresia and stenosis.

BACKGROUND: The esophagus is physiologically devoid of eosinophils, so their presence would suggest some underlying pathology. The prevalence of eosinophilic esophagitis (EoE) has steadily increased in Western countries. Previous studies have described EoE in association with congenital esophageal atresia (CEA), which is the most common congenital anomaly of the esophagus. However, the association remains unclear. METHODS: We performed a retrospective histological analysis examining for eosinophil infiltration in the esophagus of patients with CEA following surgical repair or congenital esophageal stenosis (CES) who underwent esophageal biopsy or surgical resection in our hospital between 2005 and 2012. RESULTS: There were 6 patients with CEA following surgical repair or CES who had eosinophil-dominant infiltration in the esophagus. All had associated allergic disorders, including food allergies in 4. Moreover, all except for one fulfilled the histological criteria of EoE. Impairment of eosinophil infiltration and symptomatic improvement were observed in those treated with a proton pump inhibitor (PPI), either alone or in combination with steroids after esophageal dilatation. CONCLUSIONS: These findings suggest that CEA repair or CES in conjunction with allergic conditions and coexisting gastroesophageal reflux disease (GERD) may induce greater esophageal eosinophilic inflammation. In addition, esophageal dilatation followed by PPI treatment, alone or with steroids, may be a therapeutic strategy that can provide symptomatic relief by reducing eosinophilic inflammation in esophageal strictures or GERD associated with CEA or CES.

[Congenital esophageal duplication cysts: progressive dysphagia in adulthood]. / Kongenitale zystische Duplikatur des Ösophagus: Progrediente Dysphagie im Erwachsenenalter.

Esophageal duplications are congenital abnormalities of the foregut. We present the case of a 33-year-old woman suffering from progressive dysphagia who had surgery for esophageal duplication. The following three criteria define the cystic lesion: an intimate attachment to the esophageal wall, the presence of a smooth muscle coat and a mucosal lining consisting of squamous and/or ciliated respiratory epithelium. Diverticula, bronchogenic cysts and cystic neoplasms have to be considered in the differential diagnosis. Congenital cystic esophageal duplication is a rare cause of dysphagia in adulthood.

Congenital distal esophageal obstruction caused by intraluminal mucosal web.

Here, we report a case with intraluminal membrane (web) located in the lower esophagus causing complete obstruction. Esophagogram revealed complete obstruction near the esophagogastric junction. Surgical excision of the esophageal membrane was performed. To our knowledge, only a few cases with membranous esophageal atresia have been reported. It must be remembered in neonates who cannot tolerate feeding.

Surgical correction of an aberrant right subclavian artery in a dog.

A diagnosis of an aberrant right subclavian artery was made in a 3-month-old Boston terrier. Surgical correction was performed after confirming adequate collateral circulation. Reports of surgical correction and evaluation of the perioperative thoracic limb blood pressure are rare in dogs.

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.

Congenital esophageal stenosis: the differential diagnosis and management.

Congenital esophageal stenosis (CES) is a rare congenital abnormality that is difficult to diagnose and often masquerades as other types of structural esophageal disease. We report three cases of CES with different presenting symptoms. We advocate for balloon dilation as the preferred first approach to therapeutic intervention. CES is an important clinical entity in the evaluation of pediatric esophageal disorders and should be suspected in young infants with dysphagia.

[A case of congenital esophageal stenosis that remained untreated until adulthood and was improved by endoscopic balloon dilatation].

A 20-year-old man had suffered from dysphagia since primary school. Upper gastrointestinal and endoscopy examinations revealed severe circumferential stenosis of the upper intra-thoracic esophagus. Secondary stenosis due to factors such as inflammation did not appear present, so congenital esophageal stenosis (CES) was diagnosed. Dysphagia improved after two endoscopic balloon dilatations. Almost all cases of CES are treated in baby-hood, and individuals who remain untreated until adulthood are rare. Check ups and diagnoses should be made taking CES into consideration, even in adults.

Endoscopic incisional therapy and other novel strategies for effective treatment of congenital esophageal stenosis.

BACKGROUND & AIMS: Congenital esophageal stenosis (CES) is an inborn condition of the esophagus that can be refractory to endoscopic dilation. Surgical intervention is not curative, with patients experiencing frequent ongoing need for therapy for anastomotic stricture postoperatively. We hypothesized that novel methods of endoscopic CES management including endoscopic incisional therapy (EIT) would lead to less surgical intervention. METHODS: We retrospectively reviewed the medical records of all patients with CES treated by our tertiary care center who had at least one endoscopy between July 2007 and July 2019. Statistical comparison of cohorts who underwent advanced endoscopic therapy involving EIT versus traditional endoscopic therapy with balloon dilation was performed. Primary outcome measure was need for surgical intervention. RESULTS: Thirty-six patients with CES met inclusion criteria. Thirty-four ever had at least one endoscopic intervention such as balloon dilation, steroid injection, stenting, and/or endoscopic incisional therapy (EIT) at their CES. Esophageal vacuum assisted closure (EVAC) was used for treatment or prevention of esophageal leak. Odds of surgical intervention were significantly lower in the group who received therapeutic endoscopy with EIT (odds ratio (OR) 0.1; p = 0.007). Clinical feeding outcomes were similar in the endoscopic and surgical management groups. Odds of complications after therapeutic endoscopies involving EIT were significantly greater than those without EIT (odds ratio 6.39; 95% confidence interval (2.34, 17.44); p < 0.001), though our rates of esophageal leak significantly decreased over time as our use of EVAC increased (Spearman's ρ = -0.884; p = 0.004). CONCLUSION: Complementary endoscopic techniques such as EIT broaden the toolbox of the treating physician and may allow for avoidance of surgery in CES. LEVEL OF EVIDENCE: Level III.

Laparoscopic treatment of esophageal stenosis due to tracheobronchial remnant in a child.

Esophageal stenosis due to the presence of abnormal tracheal cartilage in the wall of the esophagus is an uncommon disease, which presents with vomiting or a failure to thrive. Surgical excision of the cartilaginous segment is the preferred treatment. This is a report of a new technique for the treatment of esophageal stenosis in children. We performed a laparoscopic lower esophageal stricturoplasty with anterior fundoplication for esophageal stenosis caused by a tracheobronchial remnant in the wall of the lower esophagus.