Congenital Esophageal Stenosis Research Articles

IMAGING DISEASES OF THE PEDIATRIC ESOPHAGUS

Numerous conditions can affect the pediatric esophagus and it is essential that physicians caring for children are familiar with the radiographic evaluation of esophageal disorders. Diseases include congenital conditions such as esophageal atresia, tracheoesophageal fistula, congenital esophageal stenosis, foregut duplication cyst, esophageal bronchus, vascular rings, and pulmonary sling, inflammatory conditions such as eosinophilic esophagitis and epidermolysis bullosa, traumatic conditions such as ingested foreign body and iatrogenic esophageal injury, neoplasms such as leiomyoma, carcinoma, and lymphoma, infectious conditions such as herpes simplex virus, cytomegalovirus and Candida esophagitis, gastroesophageal reflux and achalasia (1). The radiographic assessment of these conditions typically begins with an esophogram, which utilizes fluoroscopy and an ingested contrast agent. In the most recent issue of Peadiatrics Today, Kljucevsek (2) provide a review of the pediatric esophogram and offer a useful overCommentary

Fluoroscopically guided large balloon dilatation for treating congenital esophageal stenosis in children.

We aimed to evaluate the safety and clinical effectiveness of fluoroscopically guided large balloon dilatation for treating congenital esophageal stenosis in children. Our study included seven children (mean age 4.0 years) who underwent a total of ten balloon dilatation sessions. The initial balloon diameters were 10-15 mm. The technical success, clinical success (improved food intake and reduced dysphagia within 1 month following the first balloon dilatation), dysphagia recurrence, and complications were retrospectively evaluated. Technical and clinical success rates were 100 %. During the mean 38-month follow-up period after the first balloon dilatation, 3 (43 %) patients underwent only one additional balloon dilatation 4-5 months after the first balloon dilatation for dysphagia recurrence. Two of them showed improvement without further recurrence, while the remaining one underwent partial esophagectomy. Well-contained transmural esophageal rupture (type 2) occurred in two (29 %, 2/7) patients and during two (20 %, 2/10) balloon dilatation sessions. All ruptures were successfully treated conservatively. Our study showed that fluoroscopically guided large balloon dilatation seems to be a simple and effective primary treatment technique for congenital esophageal stenosis in children. Esophageal ruptures were not uncommon although they were not fatal.

Endoscopic management for congenital esophageal stenosis: A systematic review.

Congenital esophageal stenosis (CES) is an extremely rare malformation, and standard treatment have not been completely established. By years of clinical research, evidence has been accumulated. We conducted systematic review to assess outcomes of the treatment for CES, especially the role of endoscopic modalities. A total of 144 literatures were screened and reviewed. CES was categorized in fibromuscular thickening, tracheobronchial remnants (TBR) and membranous web, and the frequency was 54%, 30% and 16%, respectively. Therapeutic option includes surgery and dilatation, and surgery tends to be reserved for ineffective dilatation. An essential point is that dilatation for TBR type of CES has low success rate and high rate of perforation. TBR can be distinguished by using endoscopic ultrasonography (EUS). Overall success rate of dilatation for CES with or without case selection by using EUS was 90% and 29%, respectively. Overall rate of perforation with or without case selection was 7% and 24%, respectively. By case selection using EUS, high success rate with low rate of perforation could be achieved. In conclusion, endoscopic dilatation has been established as a primary therapy for CES except TBR type. Repetitive dilatation with gradual step-up might be one of safe ways to minimize the risk of perforation.

Balloon or Bougienage Dilation for Esophageal Stenosis in Children?

Purpose: Esophageal stenosis (ES) in children is defined as a fixed intrinsic narrowing of the esophagus caused by numerous etiologies. Treatment mainly consists of intraluminal dilation using balloon or Savary-Gilliard bougies. Methods: We retrospectively reviewed all medical records of children with ES treated by balloon or bougies. Our aim of this study was to compare both method of esophageal dilation in terms of safety, short- and long-term outcome. Results: Forty-two children were included in this study, 22 males (52%), mean age at diagnosis was 3.9 ± 5.7 years. This group underwent 190 dilation procedures in our institution between 1994-2013. The median treatment period was 5.5 months and the median follow-up after the last dilation was 2.25 years. The average number of dilations was 4.5 (range 1-22). Twenty-four patients had anastomotic stricture after surgical treatment of esophageal atresia (57.1%), 8 had stenosis following caustic ingestion (19%), 3 had functional stenosis due to esophageal motility disorders (achalasia) (7.1%) and the others (16.7%) had congenital ES, eosinophilic esophagitis, foreign body ingestion, or were post-fundoplication. Dilations were defined as failures in 11 children (26.2%). The success rate was 87% for the bougienage group (13 children) and 67% for the balloon group (18 children) (p=NS). The success rate was 75% (24 children) after caustic ingestion and surgical correction of esophageal atresia. There were 6 (3.1%) procedure-related complications that included 2 cases of aspiration pneumonia and 4 esophageal perforations. Conclusions: Esophageal dilation in children is a safe procedure with a high rate of long-term success. Longterm success of dilation among children with ES depends primarily on the etiology of stenosis and less on the method of dilation.

English

Congenital esophageal stenosis is a rare entity with a frequently delayed diagnosis. Patients are often treated according to diagnosis of GERD until intolerance to semisolid diet appears and CES is suspected. It usually presents in infancy and childhood. We are reporting a case of congenital esophageal stenosis in twenty-one days old male child. It is very rare to diagnose

Congenital esophageal stenosis: report of a case

Congenital esophageal stenosis is a rare condition, the most common cause is the persistency of embryonic tracheobronchial remnants. A tentative diagnosis is based on a physical examination and imaging studies. Dysphagia and vomiting are the most common complaints. A definitive diagnosis is based on a histopathologic study. The treatment of choice is surgical excision. We report a case of a four–year old girl with congenital esophageal stenosis succesfully treated with surgical excision.

Congenital triple oesophageal stricture and megaoesophagus in a neonatal colt

SummaryCongenital oesophageal stenosis is rarely described in horses. This case report describes a neonatal colt with signs of a milky nasal discharge and cervical swelling present from birth. The diagnostic evaluation revealed a triple oesophageal stricture, identified as congenital fibromuscular oesophageal stenosis. A megaoesophagus was seen cranial to the strictures, but its aetiology remains unexplained. To the authors’ knowledge, multiple oesophageal stenosis together with megaoesophagus has not previously been described in the horse.

The natural history of steroid-naïve eosinophilic esophagitis in adults treated with endoscopic dilation and proton pump inhibitor therapy over a mean duration of nearly 14 years

Despite the vast focus of research in eosinophilic esophagitis (EoE), the natural history of untreated EoE remains undefined. Current expert consensus panels are calling for natural history studies to define long-term risks, adverse events, and progression of the disease. To address the natural course and long-term adverse events of EoE. Retrospective, single-center study. Tertiary-care center. A cohort of patients from the year 1988 initially diagnosed as having congenital esophageal stenosis who were later reclassified as having EoE. Ninety-five patients, with 13 meeting entrance criteria for idiopathic EoE with follow-up >5 years. Anti-acids and esophageal dilation. Clinical response, adverse events, long-term clinical outcomes, and progression of disease. Thirteen patients (mean age at diagnosis 30.3 years, 10 male) were evaluated over a 13.6-year mean follow-up (range 5-24 years). All patients experienced daily dysphagia, with 12 presenting with food impactions. Patients were treated with esophageal dilation (64% Maloney, 34% Savary, 2.5% through-the-scope balloon) and daily anti-acids. Patients were initially treated with an average of 3.2 dilations over the first year (range 1-6) to achieve a luminal size of 15.8mm (range 14-18mm). They were maintained successfully with dilations every 2 years, on average, based on symptoms. Two patients not adhering to recommended dilation schedules experienced repeat impactions. One adverse event from a mucosal tear required hospitalization (1 of 157, 0.6%). Seven of 13 had Barrett's esophagus, average length 2.4 cm (range 1-4 cm), 3 on initial EGD and 4 identified over a mean duration of 9.4 years. No patient developed dysplasia or malignancy. Retrospective, small sample. The course of EoE over a 13.6-year mean duration, although persistent, appears benign and not associated with cancer risk. A program of regular esophageal dilations based on symptom recurrence appears to be a safe, long-term treatment.

A Survey of Pulmonary Function Abnormalities Following Thoracotomy

To study the incidence and type of pulmonary function abnormalities after thoracotomy in children. Children below 12y of age who had undergone thoracotomy for any condition and have at least 2y follow up were included in the study. Detailed assessment of the patients included history and general examination, clinical assessment of pulmonary function, bedside tests to assess pulmonary function and laboratory pulmonary function test using portable spirometer. Fifty two patients were included in the study. Twenty-seven were cases of esophageal atresia with trachea-esophageal fistula (EATEF), nine pulmonary metastasis from abdominal solid tumors, six mediastinal masses, three hydatid cyst, three eventration of diaphragm, two bronchiectasis, and one each of H-type TEF and congenital esophageal stenosis. The mean age at the time of evaluation was 6.3y (range 2-18y). While all the patients were clinically assessed, only 25 (48%) were eligible for bedside tests and 23 (44%) for spirometery. The incidences of abnormalities picked were: dyspnea during exercise 8/52 (15.4%), dyspnea on exercise and on climbing stairs 1/52 (2%), decreased breath holding time 2/25 (8%), abnormal incentive spirometry 1/25 (4%), mild restrictive pattern on pulmonary function test (PFT) 11/23 (47.8%), moderate restrictive pattern on PFT 2/23 (8.7%). None had an obstructive pattern on PFT. Though the incidences of pulmonary function abnormalities were high, these were of mild grade. Close follow up of patients after thoracotomy would be needed for early pick up and appropriate management of these abnormalities to prevent long-term consequences.

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.

Histopathologic profile of esophageal atresia and tracheoesophageal fistula

Purpose Few reports are available in the literature on the histology of the congenital atretic esophagus in humans. Histologic abnormalities including congenital esophageal stenosis (CES) may contribute toward the abnormal esophageal motility after successful repair of esophageal atresia (EA) and tracheoesophageal fistula (TEF). The main aim of this study is to document the histopathologic profile in cases of EA. Methods One hundred and nineteen surgical specimens were collected from 69 consecutive EA patients who underwent surgical repair at the Aseer Central Hospital, Abha, and Armed Forces Hospital Southern Region, Saudi Arabia, from May 1999 through May 2009. This included 62 cases with EA and distal TEF, five cases of pure EA, and two cases of N-type TEF. Samples from tips of the upper pouch (UP), lower pouch (LP), and mid portion of the TEF were preserved in 10% formalin, sectioned, and stained with hematoxylin and eosin. Results The combined three elements of tracheobronchial tissue were observed in only three LP specimens. Gastrictype mucosa was seen in one UP and one LP specimen. Except for one N-type fistula, all sections showed fullthickness muscle coats. Distortion of muscles by fibrosis was most commonly seen in the UP. The muscle layer in the LP was more commonly distorted by glands with or without cartilage. Fourteen samples (10.8%) showed a histological picture consistent with CES. Conclusion Glands in the submucosa may be abnormal innumber and type, and may extend to different esophageal coats. Muscle distortion by fibrosis, glands, or cartilage and associated CES may contribute toward esophageal dysmotility and stricture after surgery. Cutting the TEF B3–5mm distal to its origin from the trachea is adequate histologically for primary anastomosis of the atretic esophagus. The histological changes associated with the TEF need to be revised. Keywords : congenital esophageal stenosis, esophageal atresia, esophageal dysmotility, histopathology

食道閉鎖症・先天性食道狭窄症術後の下部食道の通過障害に対し逆流防止術が有効であった1 例

食道閉鎖症・先天性食道狭窄症術後の下部食道の通過障害に対し逆流防止術が有効であった1 例

Characteristics and management of congenital esophageal stenosis: findings from a multicenter study

BackgroundCongenital esophageal stenosis (CES) is a rare condition frequently associated with esophageal atresia (EA). There are limited data from small series about the presentation, treatment, and outcomes of CES.MethodsMedical records of all patients with CES included in the French Network on Esophageal Malformations and Congenital Diseases were reviewed retrospectively with regard to diagnosis, treatment, and outcome.ResultsOver 18 years, 61 patients (30 boys) had CES, and 29 (47%) of these patients also had EA. The mean age at diagnosis was 24 months (1 day to 14 years) and was younger in patients with CES and EA than in those with isolated CES (7 vs. 126 months, p < 0.05). Twenty-one of the 61 patients with CES had no clinical symptoms: in three patients, the findings were incidental, and in 18 of the 29 patients with associated EA, CES was diagnosed at the time of surgical repair of EA or during a postoperative systematic esophageal barium study. In the 40 other patients, at diagnosis, 50% presented with dysphasia, 40% with vomiting, 50% with food impaction, and 42% with respiratory symptoms. Diagnosis of CES was confirmed by esophageal barium study (56/61) and/or esophageal endoscopy (50/61). Sixteen patients had tracheobronchial remnants (TBR), 40 had fibromuscular stenosis (FMS), and five had membrane stenosis (MS). Thirty-four patients (56%) were treated by dilation only (13/34 remained asymptomatic at follow-up); 15 patients were treated by dilation but required later surgery because of failure (4/15 remained asymptomatic at follow-up); and nine patients had a primary surgical intervention (4/9 were asymptomatic at follow-up). Dilation was complicated by esophageal perforation in two patients (3.4%). At follow-up, dysphagia remained in 36% (21/58) of patients, but the incidence did not differ between the EA and the isolated CS groups (10/29 vs. 7/32, p = 0.27).ConclusionsCS diagnosis can be delayed when associated with EA. Dilation may be effective for treating patients with FMS and MS, but surgical repair is often required for those with TBR. Our results show clearly that, regardless of the therapeutic option, dysphagia occurs frequently, and patients with CES should be followed over the long term.

Congenital esophageal stenosis owing to tracheobronchial remnants

OBJECTIVE To emphasize the need of an accurate diagnosis of congenital esophageal stenosis due to tracheobronchial remnants, since its treatment differs from other types of congenital narrowing. CASE DESCRIPTION Four cases of lower congenital esophageal stenosis due to tracheobronchial remnants, whose definitive diagnosis was made by histopathology. Except for the last case, in which a concomitant anti-reflux surgery was not performed, all had a favorable outcome after resection and anastomosis of the esophagus. COMMENTS The congenital esophageal stenosis is an intrinsic narrowing of the organâ€(tm)s wall associated with its structural malformation. The condition can be caused by tracheobronchial remnants, fibromuscular stenosis or membranous diaphragm and the first symptom is dysphagia after the introduction of solid food in the diet. The first-choice treatment to tracheobronchial remnants cases is the surgical resection and end-to-end anastomosis of the esophagus.

Successful Left Thoracoscopic Esophagectomy for Congenital Esophageal Stenosis with a Useful Adjunct of Preoperative Endoscopic Marking

Abstract Introduction: Minimally invasive surgery is an alternative to open surgery in esophageal resection. Several studies have described thoracoscopic esophagectomy for esophageal stenosis in children.1–3 It is standard to conduct esophagectomy from the right thoracic cavity, so the aorta does not obstruct the procedure. So far, there have been no reports on left thoracoscopic esophagectomy in children. In a case of congenital esophageal stenosis, where the lesioned part was close to the esophagogastric junction, we show a successful left thoracoscopic esophagectomy with a useful adjunct of preoperative endoscopic marking. Materials and Methods: A 3-year-old female with Down's syndrome presented with the chief complaint of vomiting after eating solid food. The esophagogram and the esophageal endoscope revealed a pinhole stenosis in the lower esophagus, diagnosed as congenital esophageal stenosis. Balloon dilation was performed twice; however, esophageal perforation occurred on the second dilation. The ...

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.

Thoracoscopic treatment of congenital esophageal stenosis in combination with H-type tracheoesophageal fistula

Congenital esophageal stenosis combined with H-type tracheoesophageal fistula is a rare clinical entity that is often not diagnosed until months or sometimes many years after birth. We present a case of a boy who presented both these entities and was treated by thoracoscopy.

Preoperative Diagnosis of Congenital Esophageal Stenosis Caused by Tracheobronchial Remnants Using Miniprobe Endoscopic Ultrasonography in a Child

Congenital esophageal stenosis (CES) can be classified into three types based on the etiology of stenosis: tracheobronchial remnants (TBRs), fibromuscular hypertrophy (FMH), and membranous diaphragm (MD). It is important to make a differential diagnosis because the therapeutic plan for CES is determined by its etiology. Most cases of FMH and MD can be managed with balloon dilatation, whereas cases of TBRs require resection and anastomosis. Thus, the preoperative distinction of TBRs is critical. Recently miniprobe endoscopic ultrasonography (EUS) with a maximum diameter of 2.5 mm has been useful for distinguishing TBRs from FMH in pediatric patients with CES. EUS shows hyperechoic lesions indicating TBR cartilage. Miniprobe EUS is recommended for choosing the correct therapeutic method for CES. We report a case of CES due to TBRs in which a preoperative diagnosis was made in a child using miniprobe EUS without any difficulties. (Pediatr Gastroenterol Hepatol Nutr 2012; 15: 52∼56)

Preoperative Diagnosis of Congenital Esophageal Stenosis Caused by Tracheobronchial Remnants Using Miniprobe Endoscopic Ultrasonography in a Child

Preoperative Diagnosis of Congenital Esophageal Stenosis Caused by Tracheobronchial Remnants Using Miniprobe Endoscopic Ultrasonography in a Child

Endoscopic management of congenital esophageal stenosis

Background/PurposeCongenital esophageal stenosis (CES) is a rare malformation. Endoscopic dilations represent a therapeutic option. This study retrospectively evaluated the efficacy and safety of a conservative treatment of CES. Patients and MethodsPatients diagnosed with CES since 1980 by a barium study or endoscopy were reviewed. Endoscopic ultrasonography (Olympus UM-3R-20-MHz radial miniprobe, Olympus Corporation, Tokyo, Japan), available from 2001, allowed for the differential diagnosis of tracheobronchial remnants (TBR) and fibromuscular hypertrophy (FMH) CES. All children underwent conservative treatment by endoscopic dilations (hydrostatic and Savary). ResultsForty-seven patients (20 men) had CES. Fifteen were associated with esophageal atresia; and 8, with Down syndrome. Mean age at the diagnosis was 28.3 months (range, 1 day to 146 months). Symptoms were solid food refusal, regurgitation, vomiting, and dysphagia. Congenital esophageal stenosis was located in the distal esophagus. Endoscopic ultrasonography demonstrated TBR and FMH in 6 patients. One hundred forty-eight dilations in 47 patients were performed. The stenosis healed in 45 (95.7%). Complications were 5 (10.6%) esophageal perforations, hydrostatic (3/32, or 9.3%), and Savary (2/116, or 1.7%). At follow-up, 1 patient with FMH CES and 1 patient with TBR CES required operation for persistent dysphagia. ConclusionsThe conservative treatment yielded positive outcomes in CES. Endoscopic ultrasonography allows for a correct diagnosis of TBR/FMH CES. A surgical approach should be reserved for CES not responsive to dilations.