bluuebunny/crossref_metadata_2025_split huggingface dataset:
DOZ047.47: Flourish device for treatment of esophageal atresia
Authors: Slater Bethany J , Zaritzky Mario 
Published: June 2019

Abstract
Esophageal atresia (EA) is usually repaired soon after birth. However, there are conditions in which early repair is not possible including prematurity, a large distance between the ends, failed attempt at primary anastomosis, and postoperative complications. Cases in which the ends of the esophagus are not able to be brought together without significant tension, or long-gap esophageal atresia (LGEA), remain a challenge to manage. A variety of techniques have been used to establish esophageal continuity consisting of extensive mobilization, myotomies, esophageal flaps, and traction of the segments.
The use of magnets is a nonsurgical alternative for esophageal anastomosis. There have been a few series demonstrating initial success and accomplishment of magnamosis in EA patients. A prospective, single-arm, observational study is currently enrolling patients to evaluate the safety and benefit of the Flourish Device (Cook Medical), a catheter-based, magnetic device used to lengthen the atretic esophageal ends and create an anastomosis. The primary outcomes include stricture requiring dilation or surgery, anastomotic leaks or other adverse events with follow-up for two years.
The distance of the esophageal gap is measured with metal probes. All patients require a mature gastrostomy and atretic segments < 4 cm apart. The proximal catheter has a suction port and the distal portion has a channel for feeds. Placement is done under fluoroscopy with anesthesia or sedation. Daily chest radiographs are obtained until union of the magnets occur. Successful anastomosis is identified by saliva in the gastrostomy catheter, feeds in the esophageal catheter, or with a contrast study. One day after confirmation, the magnets may be removed.
When the catheters are aligned, the magnets attract. Once the magnets have connected, the tissue between them sloughs off while the outer rim heals establishing the anastomosis. The Flourish device may be particularly useful in patients in whom additional operations are not ideal such as those with congenital anomalies or who have undergone previous operations or complications.
DOZ047.57: Eosinophilic esophagitis in an infant with repaired esophageal atresia and tracheoesophageal fistula
Authors: Mustafa A , AlKhabaz A , Mallik A A , Kumar S 
Published: June 2019

Abstract
Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) requires early surgical intervention in life, following which symptoms related to esophageal dysmotility, gastroesophageal reflux, and strictures may develop. Eosinophilic esophagitis (EoE) is an immune-mediated disorder presents with vomiting and dysphagia. The appearance of exudates, rings, and furrows during endoscopy and the presence of >15 eosinophils/HPF in biopsy confirm the diagnosis. Treatment options include elimination diet, proton pump inhibitors (PPI), and topical steroids.
We report a full-term male who had EA and TEF repaired in the second day of life. At 11 months of age, he developed progressive vomiting, contrast study showed esophageal dysmotility with slow emptying and no stricture. The initial endoscopy showed a mild nonobstructive narrowing 5 cm above the gastroesophageal junction, below which the mucosa was erythematous, biopsies showed >25 eosinophils/HPF. Started treatment with PPI and endoscopy repeated three months later showed same endoscopic features and biopsies showed >45 eosinophils/HPF. Allergy testing was positive for milk, wheat, oat, and rice. Topical steroids and elimination diet were added to PPI. At 32 months of age, the child was asymptomatic, not on elimination diet and treatment was stopped. Repeat endoscopy showed longitudinal furrows and erosions of the lower esophagus, no stricture, and biopsies showed >15 eosinophils/HPF. Steroids, PPI, and elimination diet were all resumed.
Despite moderate esophagitis due to EoE and poor compliance to treatment, our patient shows normal growth, development, and symptoms resolution. Asymptomatic children with repaired EA and treated EoE may require close follow-up and repeat endoscopy.
DOZ047.58: The familial occurrence of congenital esophageal atresia with or without a fistula is an extremely rare pathology
Authors: Smigiel R , Dzielendziak A , Toczewski K , Rozensztrauch A , Bloch M , Patkowski D 
Published: June 2019

Abstract
We report on two siblings (boy and girl), which were treated for congenital esophageal atresia with the fistula, at the Department of Pediatric Surgery and Urology in Wroclaw, at an interval of two years. These children have two healthy siblings. Both newborns were born on time (38 and 42 week of pregnancy). Prenatally hydramnios was observed in younger child.
They were postnatally diagnosed with congenital esophageal atresia with a distal fistula. Newborns were operated on the second day of life. Bronchoscopy and right-sided thoracoscopy were performed. Both patients had a short-gap esophageal atresia with a distal fistula. The fistula was proximally closed with sutures and divided distally. Subsequently, the one-layered esophageal anastomosis was performed. There were no early postoperative complications. Both patients underwent two esophageal dilatations. Siblings are tolerating oral feeding. Occasionally, parents observe choking in the older child. Patients remain under the constant care of a pediatric surgery clinic.
Moreover, children were evaluated by clinical geneticist and exome sequencing (WES) study on the base of DNA isolated from lymphocytes and esophageal tissues were performed in both siblings.
DOZ047.67: Comparison of mesenchymal stem cells from pediatric normal and esophageal atresia patients for use in regenerative medicine
Authors: Jensen T , Wanczyk H , Thaker S , Mitchell A , Sharma I , Finck C 
Published: June 2019

Abstract
Esophageal atresia (EA) is a congenital defect in which the esophagus is not fully connected. Current treatments to bring together long gaps between the esophagus have very high complication rates and the replacement tissue does not possess the correct structure or physiologic properties. Tissue engineering and regenerative medicine have been suggested as a new patient-specific treatment for EA utilizing the patient's own cells and a tubular scaffold. Our group recently published on the successful regeneration of an esophagus using a retrievable scaffold seeded with adipose derived mesenchymal stem cells (AD-MSC) in a porcine model of esophageal loss. The goal of this study is to evaluate phenotypic differences between AD-MSCs from normal (N-AD-MSC) and EA patients (EA-AD-MSC) to determine if EA-AD-MSC could be used for autologous tissue engineering approaches. Subcutaneous fat was obtained from pediatric patients undergoing surgery (IRB#). The cells from all patients expressed mesenchymal stem cell markers CD90, CD105, CD73, CD44 and did not express hematopoietic markers CD45, CD34 and HLA-DR by flow cytometry. Growth kinetics revealed similar population doubling times and morphology was similar between the two groups. Some of the EA-AD-MSC demonstrated upregulated expression of ATNX1, BMI1, FOXF1, GLI2, GLI3, MYC, HOXC4 and NOG, known genotypic abnormalities in EA patients. None of the N-AD-MSCs had upregulated expression of these genes. Furthermore N-AD-MSCs and EA-AD-MSCs underwent osteogenic and adipogenic differentiation with positive staining for Oil Red O and Alizarian Red after 2 weeks in culture. The integrin profiles of these cells were also similar across all patients as demonstrated by flow cytometry. In conclusion, in vitro, the EA-AD-MSCs behaved similarly to N-AD-MSCs in growth kinetics, mesenchymal phenotype and differentiation ability. Genotypically there were some differences and how this will impact the growth of these cells on scaffolds is currently being investigated.
DOZ047.69: Tracking outcomes of tracheoesophageal fistula and esophageal atresia in pediatric population
Authors: Sharma I , Thaker S , Jensen T , Finck C 
Published: June 2019

Abstract

Purpose
The purpose of this study is to conduct a retrospective chart review of patients with a rare congenital deformity, tracheoesophageal fistula, and esophageal atresia treated at a small, freestanding Children's Hospital. The goal is to identify important variables to inform clinical pathways and prospective database creation. Current national surgical quality databases do not track tracheoesophageal fistula/esophageal atresia (TEF/EA)-specific data points such as TEF-type and incidence of postoperative complications such as gastroesophageal reflux disease (GERD) and esophageal stricture.

Methods
Patients diagnosed with esophageal atresia and trachesophageal fistula between January 2005 and January 2019 were identified using the appropriate ICD 9 or ICD 10 codes. Data for 35 variables including patient demographics and type of esophageal atresia were abstracted. SPSS 16.0 software was used for descriptive statistics.

Results
A total of 43 patients were identified, 64.3% female and 35.7% male. 77.5% were type C, 12.5% type A, 7.5% type H, and 2.5% type D. 15% were long-gap type. 53.8% had postoperative esophageal strictures, and 66.7% had postoperative gastroesophageal reflux disease. Of those who had strictures, 77.2% of patients required at least three postoperative dilations for stricture, with an overall mean six dilations. 26.2% of patients had tracheomalacia; of these, two patients underwent thoracoscopic aortopexy. Four patients were not included in subgroup analysis: two patients had missing variables, one patient passed away before surgical intervention, and one patient has not yet undergone surgical intervention for EA/TEF.

Conclusions
We conclude that, although a rare congenital anomaly, a database dedicated to tracheoesophageal fistula and esophageal atresia at a pediatric hospital can aid in reviewing trends in outcomes and inform standardization of management.
DOZ047.76: Eosinophilic esophagitis and esophageal atresia. Is there an association?
Authors: García L , Giné C , Laín A , Martín M , Molino J A , Bordón E , Alvarez M , Segarra O , López M 
Published: June 2019

Abstract

Aim of the Study
Eosinophilic esophagitis (EoE) is a recently diagnosed entity and seems to be more prevalent in patients with esophageal atresia (EA). It can mimic dysphagia and gastroesophageal reflux (GER) symptoms frequently observed in EA, but treatment is different.

Methods
Retrospective review (2002–2019) of patients with EA who underwent esophagogastroduodenoscopy (EGD) and esophageal biopsy was performed. EGD was performed in symptomatic patients and in all patients at 12–15 years. Diagnostic criteria for EoE included >15 eosinophils for a high-power field in the esophageal mucosa.

Main Results
From a total of 110 patients with EA, 27 lost follow-up. In the remaining 83, 56 patients (67, 5%) underwent EGD because of dysphagia or gastroesophageal reflux (GER) studies. Esophageal biopsies were performed in 35 patients and the diagnostic criteria for EoE were achieved in 5 (14,3%). The mean age at diagnosis of EoE was 10 + 2 years and the indication for EGD was GER symptoms (3) or dysphagia (2). In one case an antireflux surgery was previously performed but symptoms slightly persisted, while in 4 cases contrast studies and pH-metry showed no or minimal GER. EGD demonstrated light distal esophagitis in 2 cases, white exudates in 2, and was completely normal in the remaining one. On follow-up, 4 patients remain asymptomatic with proton pump inhibitor medication, and periodic EGD shows macroscopic improvement with a decreased eosinophilic peak on esophageal biopsies.

Conclusions
Patients with EA seem to have a higher risk of developing EoE at early puberty. EGD on follow-up should be focused not only on studying GER or Barrett, but also on actively search for EoE with an esophageal biopsy, even when the macroscopic appearance is normal.
DOZ047.77: Extraluminal airway stabilization for localized tracheomalacia: a case report
Authors: Laín A , Pumarola F , Giné C , García L , Barila P , López M 
Published: June 2019

Abstract

Aim
In the last years several treatments for tracheomalacia have been reported. However, management of this entity is still a complex matter. This study presents a case diagnosed of severe proximal tracheomalacia managed by external tracheal resorbable plates.

Case Report
A seven-year-old child was tracheostomized in the neonatal period in relation to severe generalized tracheomalacia in association with swallowing incoordination and recurrent respiratory infections. Data to point out of his personal history were prematurity, a mosaic type genetic anomaly, cognitive and development impairment, and bilateral hypoacusia.
After improvement of the neurological status and his feeding and swallow problems he was evaluated by the Airway Unit of our centre. Dynamic transnasal flexible laryngotracheobronchoscopy showed severe tracheomalacia. The collapse was 100% in the proximal airway (from the subglotis to the middle part of the trachea) and decannulation was not possible. Given the extent, localization, and proximity of the tracheomalacia to the vocal cords a surgical approach with external bioresorbable plates was indicated. By a transcervical approach, dissection of the larynx and cervical trachea was carried out. Two 2.5 cm multiperforated plates of ‘poly [L-lactide-co-glycolide] copolymer’ were modeled and fixed to the anteromedial laryngotracheal surface. Direct intraoperative endoscopy defined the proper location to fix the plates and showed significant improvement of the collapse thanks to the rigidification of the airway. Three weeks after the procedure endoscopic control was realized and the patient was successfully decannulated. He remains asymptomatic with a 6 months follow-up. No complications were reported.

Conclusion
Extraluminal stabilization with resorbable plates can be a safe and effective option for selected patients with localized tracheomalacias. It represents a valid alternative for the management of this complex entity.
DOZ047.79: Bronchoscopic treatment of plastic bronchitis in children
Authors: Erdas M Y , Valiyev N , Cerit K K , Gokdemir Y , Kiyan G 
Published: June 2019

Abstract

Purpose
Plastic bronchitis or cast bronchitis is a rare condition characterized by the formation of large gelatinous or rigid airway casts. Classification of plastic bronchitis is done by disease association and cast histology. This rare condition can cause airway obstruction and there is no standardized treatment. Here are presented two cases with plastic bronchitis treated by bronchoscopic removal of casts in our clinic.

Patients
Case 1: A 9-year-old boy presented with wheezing, cough, and respiratory distress. He expectorated the cast spontaneously and the cast was misdiagnosed as germinative membrane of a hydatid cyst at the previous center. Total collapse of the left hemithorax was seen on chest X-ray. Any underlying disease was not detected. The plastic-like solid structure was detected and easily extracted from the bronchial tree. Pathology results of the casts were eosinophilic mucoid casts. The patient experienced three bronchoscopic removals and had no recurrence thereafter for two years.
Case 2: A 7-year-old girl presented with persistent cough and respiratory distress. The patient was referred to our center with the diagnosis of foreign body aspiration. Total collapse of the right middle and lower segment and partial collapse of the upper segment were seen on chest X-ray. Previously, she was diagnosed with reactive airway disease. The plastic-like semisolid structure was detected during bronchoscopy its extraction from the bronchial tree was not easy but was possible. Pathology results were eosinophilic mucoid and fibrinotic casts. This patient still needs bronchoscopy for cast removal after 12 bronchoscopic removals but her symptoms and bronchoscopy findings have regressed with time.

Conclusion
Bronchoscopic extraction of casts provides fast improvement of patients’ symptoms and postoperative chest X-rays, but recurrence of cast formation is common. Nevertheless, it is the mostly chosen therapy in the literature. Further researches in this area are required.
DOZ047.84: Use of a decannulation protocol in the pediatric population: decrease in the day of use
Authors: Urquizo M , Canepari A , Ricciardelli M , Sanchez M 
Published: June 2019

Abstract

Objective
This study aimed to compare days of use of a tracheostomy cannula in two groups of patients: one group used a guided decannulation protocol carried out by an interdisciplinary team and the other group did not apply any protocol.

Design and Population
A comparative retrospective cohort study of 40 patients was carried out, of whom 20 were decannulated without a protocol (WOP) between 2008 and 2011 and in the other 20 patients, the decannulation protocol (WP) was applied by an interdisciplinary team between 2015 and 2017.

Method
In 2015 a guided decannulation evaluation protocol was implemented in patients successfully weaned from prolonged mechanical ventilation that consisted of three parts: (1) daily evaluation tracheotomy requirement; (2) measuring subglottic pressure, decreasing the internal diameter of the cannula and usage of phonation valve; and (3) endoscopic evaluation, occlusion test according to age, and decannulation. The main variable was days of use of tracheostomy cannula in both groups, from the day of the surgery to the day of decannulation.

Results
In the cohort WOP of the 38 tracheotomized children, 20 achieved decannulation. In the group WP, the first 20 children who reached decannulation were consecutively enrolled, out of a total of 24 tracheotomized patients in that period. The median age was 58 months, with IQR (21.5–138); the diagnoses of admissions for both groups were of respiratory cause 20% CI 95% (10–35), cardiovascular 15% CI 95% (7–30), neurological 17.5% CI 95% (8–33), and other 47.5% CI 95% (32–63). The surgical procedure of tracheostomy, 52.5% were percutaneous with 95% CI (37-68) while 47.5% were surgical with 95% CI (32–63). The median use of the tracheostomy cannula was 46 days with an IQR (29–144). When analyzing the days of cannulation in both cohorts, a median of 94 days WP and CI 95% (51–261) in the group WOP and a median of 33 days WP and CI 95.5% (23–44) were found, obtaining a value of Wilcoxon–Mann–Whitney p 0.0012.

Conclusions
The use of a decannulation protocol applied by an interdisciplinary work team showed a significant decrease in days of use of tracheostomy cannulas in pediatric patients unrelated from the respiratory assistance.
DOZ047.90: An interesting and extremely rare case report of PCD and HLHS
Authors: Jaiswal A , Andrew N 
Published: June 2019

Abstract
An antenatally diagnosed hypoplastic left heart syndrome boy from Kuwait was transferred in utero for postnatal surgical management. He was born to a nonconsanguineously married couple by spontaneous delivery at 33 + 5 weeks of gestation with a birth weight of 2.4 kg. He was on prostaglandin infusion after birth and shortly underwent bilateral pulmonary artery banding with gore-tex shunts (aorta to pulmonary artery shunts) to let him grow and get over his prematurity for his cardiac surgical palliative procedures. He had some right upper lobe collapse, which was treated with physiotherapy and suctioning to proceed for his first stage cardiac surgical repair (Norwood I). Following this, he had a rough course owing to his cardiac condition but was extubated without many problems in due course. He was persistently tachypnoeic, had two episodes of acute decompensations during his stay due to his lung segment collapse needing invasive ventilation, and was very fragile, needing careful management. He needed noninvasive positive pressure ventilation at other times even when stable, to keep his airways from collapsing. He underwent a bronchoscopy and CT scan when stable enough to do it that showed a normal airway but with thick, tenacious, and clear secretions on bronchoscopy while a CT showed some areas of atelectasis and pulmonary interstitial emphysema. He was investigated for cystic fibrosis and primary ciliary dyskinesia as other causes that could lead to persistent and recurrent atelectasis were ruled out and his acute episodes dealt with appropriately. His genetic testing results have confirmed the clinical diagnosis of primary ciliary dyskinesia and he is being discussed for further management from a multidisciplinary point of view.
DOZ047.91: Bronchoscopic treatment of recurrent tracheoesophageal fistula: is it an effective option?
Authors: Valiyev N , Cerit K K , Erdas M Y , Ergenekon A P , Kiyan G 
Published: June 2019

Abstract

Purpose
Despite technical and surgical improvements, treatment of recurrent tracheoesophageal fistula (rTEF) still remains a challenging problem in pediatric patients. Bronchoscopic closure of rTEF using different agents is described with different success rates in the literature. This study aimed to report results of patients with rTEF treated with chemocauterization using trichloroacetic acid (TCA) and with laser cauterization (LC) followed by fibrin glue (FG) application.

Methods
Nine patients with rTEF were included in the study from 2014 to 2018. Four patients underwent fistula closure with LC followed by FG application, while another four patients had chemocauterization with TCA. In one case both agents were used. Patients were checked for the success of the treatment by bronchoscopy. All patients had the diagnosis of rTEF. Seven patients had EA with distal TEF and the remaining two patients had H-type TEF as primary pathologies. FG application was performed by LC followed by injection of FG into the fistula. Application of the TCA was made by a TCA-soaked sponge for 30 seconds and 3 times each trial.

Results
Nine children of the age range 4 months to 5 years (mean 1.5 year) were treated endoscopically. Follow-up ranged between 3 months and 5 years (mean 28 months). Among the nine patients only in one patient with only laser coagulation with FG application the treatment was successful. In the remaining eight patients all attempts failed. All these patients underwent open surgical closure of the fistula. There were no acute or late complications related to either endoscopic method.

Conclusion
The results of this study suggest that endoscopic occlusion of recurrent TEF has a low success rate. Surgical closure is still a standard method with a high success rate. Even though endoscopic options are less invasive, they prolong the period of aspiration and may cause increased pulmonary problems.