bluuebunny/crossref_metadata_2025 huggingface dataset:
The nature of human IL-6
Authors: Puel Anne , Casanova Jean-Laurent
Published: June 2019

Countless functions have been attributed experimentally to IL-6. In this issue of JEM, Spencer et al. (https://doi.org/10.1084/jem.20190344) reveal essential, nonredundant functions of human IL-6. Patients with genetic deficiencies of the IL-6 receptor suffer from “hyper IgE syndrome.”
High fusion performance in Super H-mode experiments on Alcator C-Mod and DIII-D
Authors: Snyder P.B. , Hughes J.W. , Osborne T.H. , Paz-Soldan C. , Solomon W.M. , Knolker M. , Eldon D. , Evans T. , Golfinopoulos T. , Grierson B.A. , Groebner R.J. , Hubbard A.E. , Kolemen E. , LaBombard B. , Laggner F.M. , Meneghini O. , Mordijck S. , Petrie T. , Scott S. , Wang H.Q. , Wilson H.R. , Zhu Y.B.
Published: June 2019

The ‘Super H-Mode’ regime is predicted to enable pedestal height and fusion performance substantially higher than standard H-Mode operation. This regime exists due to a bifurcation of the pedestal pressure, as a function of density, that is predicted by the EPED model to occur in strongly shaped plasmas above a critical pedestal density. Experiments on Alcator C-Mod and DIII-D have achieved access to the Super H-Mode (and Near Super H) regime, and obtained very high pedestal pressure, including the highest achieved on a tokamak (p 
ped ~ 80 kPa) in C-Mod experiments operating near the ITER magnetic field. DIII-D Super H experiments have demonstrated strong performance, including the highest stored energy in the present configuration of DIII-D (W ~ 2.2–3.2 MJ), while utilizing only about half of the available heating power (P
heat ~ 7–12 MW). These DIII-D experiments have obtained the highest value of peak fusion gain, Q
DT,equiv ~ 0.5, achieved on a medium scale (R  <  2 m) tokamak. Sustained high performance operation (β
N ~ 2.9, H98 ~ 1.6) has been achieved utilizing n  =  3 magnetic perturbations for density and impurity control. Pedestal and global confinement has been maintained in the presence of deuterium and nitrogen gas puffing, which enables a more radiative divertor condition. A pair of simple performance metrics is developed to assess and compare regimes. Super H-Mode access is predicted for ITER and expected, based on both theoretical prediction and observed normalized performance, to allow ITER to achieve its goals (Q  =  10) at I
p  <  15 MA, and to potentially enable more compact, cost effective pilot plant and reactor designs.
DOZ047.03: The functional chewing training for chewing dysfunction in children with repaired esophageal atresia–tracheoesophageal fistula
Authors: Arslan S S , Demir N , Karaduman A , Tanyel F C , Soyer T
Published: June 2019

Introduction

Chewing disorders (CD) may cause restrictions in solid food intake and can be seen in 37% of children with esophageal atresia–tracheoesophageal fistula (EA-TEF).1 The Functional Chewing Training (FuCT) is a holistic approach to improve chewing function (CF) in children. The study aimed to evaluate the effects of FuCT on CF in children with EA-TEF.

Materials and Methods

Twenty children with CD were included. Patients received 12 weeks of FuCT, including impairment-based and adaptive components. Chewing performance level was scored with the Karaduman Chewing Performance Scale (KCPS), and tolerated food texture was determined by the International Dysphagia Diet Standardization Initiative (IDDSI). The baseline and final levels of KCPS and IDDSI were compared to evaluate the effects of FuCT on CF.

Results

45% of cases were isolated-EA and 55% were EA-distal TEF with a median age of 31 (min = 25, max = 84) months, of which 65% (n = 13) were male. Baseline evaluation showed that 12 cases were in level-1, 6 cases in level-3 and 2 cases in level-4 according to the KCPS. Eight children with CD (40%) had IDDSI level-3 and 12 (60%) had level-7. There was a significant improvement in KCPS scores and IDDSI scores after 12 weeks of training (P < 0.01, P = 0.005, respectively). KCPS scores showed level-0 in 15 cases, and level-1 in 5 cases. All children had IDSSI level-7.

Conclusions

The FuCT is an effective method to improve chewing function in children EA-TEF who had CD.
DOZ047.10: Acquired tracheoesophageal fistulas after esophageal atresia repair
Authors: Boybeyi-Turer O , Soyer T , Tanyel F C
Published: June 2019

Aim

Tracheoesophageal fistula (TEF) recurrence is a frequent but challenging complication after esophageal atresia (EA) repair. Although most recurrent TEFs are commonly seen at the original fistula site, long new fistulas localized differently from the congenital TEF sites are called acquired TEFs (acq-TEF). Acq-TEFs are long new fistulas with unusual locations, including fistulas from the esophagus to anywhere on the airway such as the bronchus, trachea, or lung parenchyma. Herein, we aimed to discuss diagnostic and management challenges in different localizations of acquired TEF.

Methods

The medical records of patients admitted with acq-TEF in the last 5 years were retrospectively evaluated. The demographic features, admission complaints, physical and radiological findings, TEF localization and management were recorded.

Results

From 16 TEF recurrences, 4 TEFs were acquired fistulas. Admission age ranged from 3 months to 8 years. The female/male ratio was 2/2. The complaints were recurrent respiratory tract infections, choking, and coughing in all cases. Three of the cases had proximal EA + distal TEF; the other case was isolated EA. Primary repair was performed in 3 cases and colon interposition was performed in 1 case. Anastomotic leak and mediastinitis after initial operation were seen in 3 cases. Three acq-TEFs were to the cervical part of the trachea, one was from the colon conduit to the trachea, one was to right bronchus by passing through the intrathoracic abscess cavity, and one was directly to the right bronchus. In all cases the TEFs were shown in sine-esophagography and confirmed with bronchoscopy during operation. TEF was repaired by thoracotomy incision in 3 cases and callor incision in 1 case. The second acq-TEF of Case 1 was closed spontaneously. Muscle flap or pleura was placed between suture lines in all cases. All TEFs were confirmed to be closed with esophagography in all cases at postoperative period.

Conclusion

Acq-TEF is mostly seen secondary to local or diffuse mediastinitis. Besides its classical location of TEF, acq-TEFs may be seen at unusual rare localizations such as esophagus to right bronchus, esophagus to abscess cavity, and conduit to trachea. They cause both diagnostic and surgical challenge. Clinicians should be aware of these different localizations of Acq-TEFs in order to evaluate and manage these patients more comprehensively.
DOZ047.113: Outcome of routine esophagogastroduodenoscopy, high-resolution manometry (HRM), and pH-(impedance) measurement in esophageal atresia patients according to international guidelines
Authors: van Lennep M , Krishnan U , van Wijk M P
Published: June 2019

Background

Evidence-based guideline recommendations for follow-up of patients with esophageal atresia (EA) include regular surveillance esophagogastroduodenoscopy (EGD) with biopsies and a pH(-impedance) measurement (pH-MII) to evaluate gastroesophageal reflux disease (GERD) in asymptomatic patients. In addition, high-resolution manometry (HRM) is considered useful to assess esophageal dysmotility. Additional testing is recommended when symptoms are present. Our aim was to evaluate the diagnostic and clinical yield of these tests.

Methods

All EA patients who underwent EGD, pH-MII, and HRM according to EA guideline recommendations in 2017 and 2018 were included.

Results

Seventy-eight patients (53% male, 78% type C, median age 5 (0–17) years) were included. Thirty-one patients had EGD (n = 30), pH-MII (n = 11), and/or HRM (n = 7) as part of a routine surveillance program. Forty-seven patients had EGD (n = 47), pH-MII (n = 26), and HRM (n = 9) for symptom evaluation. Symptoms at the time of testing included dysphagia (51%), regurgitation (41%), and heartburn (24%). Results and consequences of the surveillance and clinically indicated tests are shown in Tables 1 and 2, respectively. Macroscopic EGD findings included stricture (21%), signs of eosinophilic esophagitis (EoE, 3%), and signs of reflux esophagitis (3%). Histological findings included EoE (13%) and reflux esophagitis (17%). Clinical implications of EGD results included medication alteration (GERD/EoE), esophageal dilation, antireflux surgery, or diet change. MII-pH was performed in 37 patients; 15 (41%) were on acid suppressive medication during measurement. Clinical implications of MII-pH results were: start or adjustment of acid suppressive therapy (30%), start or increase of other medication (22%), ceasing of acid suppressive therapy (14%), and referral for fundoplication (3%). HRM was performed in 16 patients (21%, n = 2 uninterpretable due to continuous crying) and showed absent peristalsis in 8/14 (50%), ineffective peristalsis in 4/14 (25%), and fragmented peristalsis in 2/14 patients (12.5%). Clinical implications of manometry results included referral for contrast esophagram (n = 1), increased gastrostomy feeding (n = 1), and gastrostomy placement (n = 1).

Conclusion

EGD, MII-pH, and HRM reveal multiple abnormalities that have clinical consequences when performed as per recommended surveillance guidelines, which highlights their importance. In most symptomatic patients, additional testing demonstrates underlying pathology. Abnormal diagnostic tests can help guide clinical management in asymptomatic and symptomatic EA patients.
DOZ047.119: Five year experience of a tertiary-level referral center in children with anastomotic strictures after esophageal atresia repair
Authors: Contini A C I , Rea F , Guerra L , Caldaro T , Torroni F , Tambucci R , Angelino G , De Angelis P , Balassone V , Romeo E , Faraci S , Federici di Abriola G , Dall’Oglio L
Published: June 2019

Background

Esophageal atresia (EA) is the most common congenital anomaly of the esophagus. Anastomotic strictures (AS) frequently occur in patients surgically treated for esophageal atresia (EA). The primary aim of this study is to determine the role of esophageal dilations in the management of AS in childhood after reconstruction of EA.

Methods

A retrospective chart review of patients treated with esophageal dilation for EA was conducted at our tertiary referral center from 2013 to 2017. We included patients treated at our Institution since diagnosis and patients referred from other Institutions. All dilations were performed with Savary-Gilliard polyvinyl bougies. Dilation was performed on an ‘as needed’ basis.

Results

Eighty-nine patients (68.5% males, 31.5% female) underwent 433 dilations overall in the study period (median: 3, range: 1–36). Type I of EA was 26.1%, Type II was 9.1%, Type III was 64.8%. Associated malformations in 52.8% cases. Six patients received 33 local applications of mitomycin C (MMC) and two of triamcinolone acetate for recurrent stenosis. Four patients developed a preanastomotic diverticulum-treated endoscopically. Nine patients (10.1%) underwent dynamic esophageal stent placement after a median of six dilations.

Antireflux surgery was performed in 28 patients (31.5%), Nissen fundoplication in 20 patients, Toupet in 6, and both in 2. Patients treated with antireflux surgery received a mean of 7.1 dilations before surgery, versus 3.9 in patients without antireflux surgery (P = 0.0285, unpaired t test).

Seven patients (7.9%), 5 referral, needed major esophageal surgery (4 esophageal reanastomosis and 3 esophagocolonplasty). Patients underwent major surgery received a mean of 19.3 dilations versus 3.6 dilations in no-surgery patients (P = 0.0002, Mann-Whitney U test). Six of 9 stented patients did not require surgery.

Perforation was present in 0.4% of 433 dilatations.

Conclusions

Esophageal dilatation remains the mainstay of treatment for AS after EA repair. Stricture resection with end-to-end anastomosis is the reconstructive option of choice; esophageal substitution is reserved only in cases of intractable stricture. The use of esophageal dynamic stent, MMC, or steroids could help in avoiding or delaying the need of operative stricture resection. Antireflux surgery is mandatory in case of severe pathological gastroesophageal reflux to prevent intractable strictures from developing.
DOZ047.121: Usefulness of conservative treatment in recurrent tracheoesophageal fistula in children
Authors: Isoldi S , Torroni F , Faraci S , Balassone V , Rea F , Tambucci R , Angelino G , Romeo E F , Guerra L , Caldaro T , Contini A C I , di Abriola G F , De Angelis P , Bottero S , Bagolan P , Trozzi M , Dall’Oglio L
Published: June 2019

Introduction

Recurrent tracheoesophageal fistula (TEF) is a challenging complication in children with repaired esophageal atresia (EA). Thoracotomy, with surgical repair, is the traditional therapeutic approach, however it is often associated with a significant morbidity and technical difficulty. Endoscopic techniques, from the tracheal and/or esophageal side, have recently been proposed as a primary treatment, in order to prevent reoperation especially in previously operated fields. Aim of this retrospective study is to present our experience as Tertiary Care Center and Referral Center for EA, on the endoscopic management of recurrent TEF in children with repaired EA.

Method

This is a retrospective study conducted on children with repaired EA referred at Bambino Gesù Children's Hospital from January 2009 to January 2019. Demographic and clinical details, including type of EA, presence of fistula, concomitant malformations, endoscopic evaluation, number and kind of treatments, performed were collected.

Results

We identified 171 patients with EA, 15 type 1 (9%), 7 type 2 (4%), 140 type 3 (82%), 6 type 4 (3%), 3 type 5 (2%). Among patients with TEF, 11/156 (7%- M:F/6:5) presented with a TEF recurrence. Among the latter, 5 (45.4%) were patients with concomitant malformation (2 VACTER, 2 anorectal malformation, 1 Down syndrome, cardiac abnormalities, and hypothyroidism). All patients with TEF recurrence presented with symptoms, such as chronic cough, vomit, bronchitis, recurrent pneumonia, and failure to thrive. All patients underwent endoscopic evaluation both from digestive and tracheal side. Six (6/11–54.5%) patients underwent surgery, 5 patients (5/11–45.4%) were treated with endoscopic transtracheal fibrin glue injection after brushing that was successful in two patients (2/5–40%), one patient needed a retreatment. These patients maintained successful closure after treatment confirmed at endoscopic follow-up. Remaining three patients needed surgical approach during follow-up.

Conclusion

Endoscopic repair of recurrent TEF has proved safe and effective in the literature as an alternative to a second open thoracotomy/surgical repair. In our series, although in two cases, it represented a valid alternative to surgery. We suggest that it is worthy to try a conservative approach before surgery.
DOZ047.22: FEED-EASY: feeding disorders in children with esophageal atresia study
Authors: Pham A , Dugelay E , Bonnard A , Gelas T , Rousseau V , Thomassin N , Cabon-Boudard I , Michaud L , Guinot A , Rebeuh J , Mandat A L E , Djeddi D , Fouquet V , Bourchany A , Irtan S , Comte A , Bridoux-Henno L , Dupont C , Dimitrov G , Turquet A , Borderon C , Pelatan C , Legault E C , Jung C , Willot S , Gottrand F , Bellaiche M
Published: June 2019

Introduction

With advances in surgical and neonatal care, survival of patients with esophageal atresia (EA) has improved over time. While a number of conditions associated with EA may have an impact on feeding development (delayed primary anastomosis, anastomotic leaks, recurrent tracheoesophageal fistula, anastomotic stricture, gastroesophageal reflux, esophageal dysmotility, etc.) and although children with EA experience a number of oral aversive events in their first year of life, feeding disorders (FD) are poorly described and frequently unrecognized. The primary aim of this study was to describe FD in children born with EA, with a standardized scale. The secondary aim was to describe conditions associated with FD.

Methods

FEED-EASY is a multicentric French study. Parents of children born with EA between 2013 and 2016 in one of the 22 participating centers were asked to participate and received the French version of the standardized and reproductive ‘Montreal Children's Hospital Feeding Scale (MCH-FS)’.

Results

One hundred and forty-five children were included; 61 (42%) had FD according to the MCH-FS. These children were characterized by disinterest in food, oral hypersensitivity, difficulty in touching some textures and food avoidance, with an influence in quality of life. Nineteen (13%) were tube-fed between 1 and 4 years of age. Birth weight and chronic respiratory difficulties were associated with FD in children with EA. Anastomotic stricture (present in 31% of the included children) was not associated with FD.

Conclusions

FD is frequent and unrecognized in children with EA, and can influence growth and quality of life. MCH-FS allows pediatricians to identify FD in children with EA within a couple of minutes.
DOZ047.23: How esophageal atresia affects the family life
Authors: Rozensztrauch A , Patkowski D , Śmigiel R
Published: June 2019

Background

Tracheoesophageal fistula (TEF) with esophageal atresia (EA) is seen in about 35,000 births. The impact of caring for children after surgical repair of EA is poorly understood and the literature regarding this area is scarce. Mortalities are now mostly attributable to any associated anomalies or prematurity, but morbidity and quality of life (QoL) are important to address.

Objective

This study aimed to investigate whether syndromic EA, feeding problems, and prematurity have a significant influence on the family of a child after surgical repair of EA.

Methods

Ethical approval was obtained. This cross-sectional study consisted of 73 participants (mean mothers’ age = 28, SD = 5 years; mean fathers’ age = 31, SD = 6 years) who were parents of children after surgery of EA. The impact of EA on families was assessed using an author-designed questionnaire (ADQ) to collect medical and sociodemographic background data as well as a standardized questionnaire, the PedsQLTM Family Impact Scale (PedsQL-FIM).

Results

Cardiac malformations associated with EA significantly (P = 0.037) affect the functioning of the family in the emotional domain. Skeletal malformations have the greatest impact on the family functioning. Three statistically significant correlations have been demonstrated: (P = 0.021) in the social domain; (P = 0.009) in the cognitive domain; and (P = 0.023) in the domain of communication. The families of patients with TEF had the statistically lower (P < 0.05) score of functioning in the emotional domain than those without TEF. Nonhealth factors frequently included changes in daily activities and deterioration of social position affected caregivers.

Conclusions

Difficulties with feeding and the presence of associated anomalies significantly affect the functioning of the family of the child with EA.
DOZ047.24: Predictors of early and late mortality in esophageal atresia patients
Authors: Tanny S P T , Fearon E , Hawley A , Brooks J , Comella A , Hutson J M , Teague W J , Pellicano A , King S K
Published: June 2019

Background

Esophageal atresia (EA) is associated with mortality, despite significant advances in neonatal care. Predictors of early mortality (EM) are well described in the literature. However, little is known about late mortality (LM). This study aimed to describe EM and LM rates and their associations in EA patients.

Methods

Patients with EA, managed at The Royal Children's Hospital, Melbourne (1980–2017), who subsequently died, were identified from the Nate Myers Esophageal Atresia database. Data collected included patient demographics, VACTERL associations, mortality risk factors, and preoperative, operative, and postoperative findings. Early mortality was defined as death during initial admission.

Results

A total of 90/628 (14.3%) patients died during the study period—EM occurred in 68/90 (75.6%), whilst LM occurred in 22/90 (24.4%). Causes of EM were most often palliation for respiratory anomalies (17/68, 25.0%), followed by associated syndromes (11/68. 16.2%) and neurological anomalies (10/68, 14.7%). The most commonly associated syndrome leading to palliation was trisomy 18 (7/68, 10.3%). Causes of LM were most often respiratory compromise (6/22, 27.3%), sudden unexplained deaths (6/22, 27.3%), and Fanconi's anemia (2/22, 9.1%). Of patients discharged from hospital, 22/560 (3.9%) subsequently died. There was no difference in VACTERL association between EM (32/63, 50.8%) and LM (11/20, 55%) (P = 0.80), and no difference in the incidence of twins between EM (8/58, 13.8%) and LM (2/18, 11.1%) (P = 1).

Conclusion

We identified predictors of early and late mortality in esophageal atresia in a large cohort. Parents of children with esophageal atresia must be counseled appropriately as to the likelihood of death following discharge from hospital.
DOZ047.29: Pleural flap for partial esophageal substitution in case of difficult anastomosis
Authors: De Napoli Cocci S , Faraj S , Guinot A , Joseph S , Caldari D , Leclair M-D
Published: June 2019

Esophageal reconstruction in long-gap atresia remains challenging for the surgeon.

Complications of surgery, regardless of choices and methods proposed, are frequents, sometimes serious, and can significantly delay baby's feeding, with increased disruption of orality.

The ideal is a reconstruction with only esophageal tissue available.

There is renewed interest for technical of elongation described initially by Foker, especially modified by Van Der Zee.1

But, elongation is not always so simple that provided.

We report an observation of esophageal atresia (type 1 Ladd), treated by rapid elongation. On the sixth day of procedure, right pleurisy reveals a rip at the apex of inferior esophagus.

Anastomosis was possible on the posterior plane, but impossible on the anterior plane. The anterior plane has been reconstituted with a flap of parietal pleura. The operative course was simple without any fistula. Oral feeding started at fifteenth day postanastomosis was quickly complete. Gastrostomy left by security has not been used.

Three endoscopic dilations have been necessary at six weeks, two and five months after anastomosis.

At the age of eleven months, the orality was perfect, and diet was diversified with pieces.

Use a pleural flap to bury or isolate sutures is well known technic.2 But use only pleura to replace the esophageal wall is not described.

Partial reconstruction of a hemiesophageal wall with only pleura is possible, despite difficult conditions. It is a trick easily achievable in case of difficulty, with a good result that is maintained over time.
DOZ047.31: Screening and surveillance in adults after esophageal atresia repair: yield of follow-up endoscopies
Authors: Ten Kate C A , IJsselstijn H , Bruno M J , Wijnen R M H , Spaander M C W
Published: June 2019

Aim of the Study

Because of the high prevalence of Barrett's esophagus (BE) and esophageal carcinoma, it is internationally recommended to perform routine endoscopies in adult esophageal atresia (EA) patients. This study aimed to evaluate the yield of follow-up after the first screening endoscopy.

Methods

Prospective cohort study in a tertiary referral center since November 2011 was considered. All patients with EA ≥ 17 years old were invited to participate in the screenings and surveillance program. All patients underwent upper endoscopies with random biopsies of the distal esophagus at the level of the gastroesophageal junction and standard four-quadrant biopsies in case of BE. From the age of 25 years old chromoendoscopy with Lugol's staining was performed for detection of superficial squamous cell carcinoma. Depending on their age and the histologic results patients were recommended follow-up endoscopy every 3–5 years. Ethical approval had been obtained.

Results

In February 2019, 190 patients (58% male, 83% type C, median age at time of first endoscopy 25.5) (range: 16.8–68.6 years) participated in the screenings and surveillance program. Forty-six patients underwent at least one follow-up endoscopy after median 4.7 (range: 1.0–5.9) years. Most recent surveillance endoscopy revealed a normal esophagus in 62%, esophagitis in 10%, and columnar-lined esophagus in 26% of the patients. Histology revealed inflammatory changes within normal limits in 49%, esophagitis in 22%, gastric metaplasia in 18%, and intestinal metaplasia without dysplasia in 6%. In 8 patients (6 males, median age: 25.9 (range: 22.5–34.4)) columnar-lined mucosa was a new finding at the second endoscopy, after median 5.0 (range: 3.6–5.9) years. In two of them histology revealed intestinal metaplasia without dysplasia.

Conclusions

In line with previous studies, we found a 4-times increased prevalence (6.3%) of BE in adults with EA. Follow-up endoscopies detected two new cases of BE. Future studies are needed to identify patients at risk and personalize screening and surveillance regimes.
DOZ047.39: Respiratory morbidity at the age of one year in children with esophageal atresia: data from the French National Esophageal Atresia Register
Authors: Lejeune S , Rousseau V , Bonnard A , Michaud L , Gelas T , Boubnova J , Jacquier C , Irtan S , Breton A , Fouquet V , Guinot A , Lamireau T , Habonimana E , Schneider A , Elbaz F , Ranke A , Poli-Merol M L , Allal H , Petit T , Michel J-L , Buisson P , Sapin Z , Lardy H , Levard G , Lopez M , Schmitt F , Borderon C , Jaby O , Pelatan C , De Vries P , Pouzac-Arnould M , Grosos C , Breaud J , Tolg C , Auber F , Geiss S , Laplace C , Sfeir R , Gottrand F , Drumez E , Mourad S El , Thumerelle C
Published: June 2019

Respiratory diseases are common in children with esophageal atresia (EA), leading to an increased morbidity and mortality in the first months of life.

Objective

Assess the prevalence of hospitalizations linked to a respiratory disease and of maintenance inhaled therapy at the age of 1 year in French children.

Methods

Population based-study using data from the French national EA register. We included all children born between 2010 and 2015 with data available at birth and at follow-up at one year of age.

Results

A total of 981 patients born with EA were included in the register, 75 of them (8%) being deceased at the age of 1 year. Data were missing for 60 patients, thus 846 children (86%) were retained for analysis. EAs were type III of Ladd classification in 89% and type I in 7%. Rate of prematurity was 37% while 51% presented associated malformations. At 1 year of age, 1297 hospitalizations were reported for 508 patients (60%), at least one hospitalization for a respiratory disease for 251 children (51%). Factors significantly associated with respiratory hospitalizations were longer median length of oxygen supplementation (P < 10−2) and noninvasive ventilation in the neonatal period (P = 0.02), gastrostomy tube (P < 10−2), esophageal anastomosis dilation (P < 10−2). At 1 year of age, 29% had an inhaled maintenance treatment, and 85% inhaled corticosteroids. Factors significantly associated with inhaled maintenance treatment were male gender (P = 0.04), advanced older median age at gastrostomy tube insertion (P < 10−3), enteral feeding at one year of age (P < 10−3).

Conclusion

This study shows a high rate of respiratory problems responsive of frequent hospitalizations and inhaled maintenance treatment in children born with EA in the first year of life.
DOZ047.41: Tracheomalacia after esophageal repair: a systematic review
Authors: Aanen I P , Elders B B L J , Pullens B , Wijnen R M H
Published: June 2019

This study was performed to obtain an overview of literature on diagnosing tracheomalacia (TM) in patients with esophageal atresia (EA). The aim of this study is to find answers in the literature on the following questions: (1) How often is a TM seen on preoperative scopy? (2) What is the predictive value of preoperative scopy findings on postoperative TM outcome? (3) How often is tracheomalacia seen in postoperative scopy in EA? (4) How often is tracheomalacia severe?

Methods

A systematic literature search to identify studies on pre-, per-, and postoperative endoscopy to identify TM in patients with EA was performed. TM was classified into the following categories: mild, moderate, and severe, based on percentage of collapse of the trachea seen on tracheobronchoscopy (TBS) and/or clinical symptoms. The following databases were searched: Embase, Medline Epub (Ovid), Cochrane Central, Web of Science, Google Scholar. The search strategy contained ‘oesophageal atresia’ and ‘tracheo-oesophageal fistula’ and ‘tracheomalacia’ and ‘pediatric.’

Main Results

A total of 43 articles were included. Of the studies who conducted initial TBS pre- or peroperative, 6 described TM incidences, ranging from 7% to 65%. One study reported a sensitivity of dynamic fiberoptic bronchoscopy at time of initial repair as a test for symptomatic TM of 86% and a specificity of 54%. The positive and negative predictive values were 71% and 75%, respectively. Thirty-three studies described postoperative TM incidences ranging from 0% to 89% overall. Of the patients who were identified with TM 8–53% were classified as severe, e.g., in need of surgical intervention or according to institute guidelines on collapse percentage at endoscopy.

Conclusions

Although most medical centers carry out airway endoscopy pre- or peroperative in EA-repair patients, reports on TM incidences at this time point are still poor. Postoperative TM occurs highly in symptomatic patients with a substantial number of airway interventions.
DOZ047.55: Midterm follow-up of long-gap esophageal atresia national cohort: study of feeding difficulties and morbidity
Authors: Thomas J , Gottrand F , Levard G , Lamireau T , Michaud L , Sfeir R , Michel J-L , Carcauzon V , Pouzac M , Branchereau S , Petit T , Sapin E , Piolat C , Allal H , Lemelle J-L , De Napoli Cocci S , Ricard J , Merol M L P , Elbaz F , Lardy H , Schneider A , Mure P-Y , Audry G , Auber F , Rousseau V , Bonnard A , Varlet F , Parmentier B
Published: June 2019

Introduction

Long-gap esophageal atresia (LGEA) leads to multifactorial morbidity. The aim of this study is to analyze midterm gastrointestinal and respiratory morbidities with special assessment of feeding difficulties. Secondary goals were to determine risk factors of malnutrition and the impact of surgical techniques on morbidity.

Methods

We conducted a prospective observational national study. Using French national database, we reviewed medical charts of every patient treated for LGEA between 2008 and 2010 in France. Phone contact was proposed to assess orality disorders using Functional Oral Intake Scale (FOIS). Patients with complete data were included. We compared conservative management with esophageal replacement.

Results

We included 31 cases with a median age of follow-up of 9 years [7–10]. Median z-score for weight was −0.97 [−3.52–2.50], <to −2DS in eight patients (26%). Eleven patients (35%) had a medical treatment for gastroesophageal reflux (GER), 10 children (35%) had required at least one dilatation for stricture (median number of 2 [0–10]) and 17 patients (55%) complained of food impaction. Enteral feeding was required in three patients (10%). Orality disorders were present in 16 cases (52%). Ten children (30%) required a daily treatment for asthma and 3 (10%) had recurrent pulmonary infections. Low birth weight, preterm birth, congenital malformations, GER, dysphagia, abnormal FOIS and asthma were not associated with malnutrition. Conservative strategy was significantly associated with orality disorders (P = 0.04).

Conclusion

Midterm morbidity in LGEA concerns 80% of our population. Gastrointestinal morbidity includes mainly dysphagia, GER, and orality disorders. This study suggests that conservative management provides more orality disorders. Other studies are mandatory to confirm it.
DOZ047.56: Esophageal stent placement for the treatment of severe stricture after the operation for esophageal atresia—preliminary report
Authors: Jasinskaa A , Wolskia M , Banaszkiewiczb A , Kaminskia A
Published: June 2019

Introduction

Anastomotic stricture is the most common complication after the treatment of esophageal atresia. It occurs in 20%–80% of operated patients. The stricture can be usually effectively managed by repeated bougie or balloon dilations. Stent placement therapy is reserved for severe strictures.

Material and Methods

Between 2006 and 2018, 73 patients with esophageal atresia were admitted to the clinic. The esophagus was anastomosed in 63 of those patients. Clinical history of three patients with severe stricture treated with stent placement was analyzed retrospectively.

Results

Two patients were treated for esophageal atresia with lower tracheoesophageal fistula and one without the fistula (type ‘A’). In two patients, the undertension primary anastomosis was possible. The third patient underwent esophageal reconstruction with the large curvature of the stomach after upper esophagus elongation (Collis procedure). Anastomotic leakage and severe anastomotic stricture occurred in all three patients. Severe stricture was the indication for stent placement in two cases and a perforation during the dilation procedure in one. All stents were removed after the period of one week. The removal was accelerated by the aggravation of tracheomalacia symptoms in one case and by the onset of pulmonary tract infection due to gastroesophageal reflux in another. The third stent migrated to the stomach and had to be removed during endoscopy. One patient, qualified for stent therapy due to the perforation, did not require any other dilations after stent removal. In one patient with severe stricture, the stent therapy accelerated the healing—the narrowing never got tighter than the stent diameter and he required only three more dilations. In the third patient, no positive impact of the stent therapy was observed.

Conclusions

Stent placement for severe esophageal stricture after anastomosis was the definite therapy in one patient and substantially reduced the number of required dilations in one. Complications of the stent placement appeared in two out of three patients.
DOZ047.71: Long-gap vs. non-long-gap esophageal atresia: a prospective two-year follow-up study
Authors: Valfre L , Conforti A , Scuglia M , Aite L , Bevilacqua F , Iacobelli B D , Braguglia A , Bagolan P
Published: June 2019

Aim of the Study

Long-gap esophageal atresia (LGEA) represents the most challenging spectrum of esophageal atresia (EA). This condition is poorly defined, particularly regarding long-term sequelae. The aim of this study was to evaluate mid-term outcomes of patients with LGEA.

Methods

A longitudinal prospective study was performed of all EA patients treated at our institution from January 2008 to December 2016. Patients were followed up in a dedicated multidisciplinary outpatient clinic. LGEA was defined as a gap wider than 3 vertebral bodies at preoperative gap measurement. Data were collected with specific attention to auxological outcomes, esophageal dilations, antireflux procedures, redo-esophageal surgery, readmission, and dysphagic problems. Chi-squared test and Mann–Whitney test were used as appropriate; P < 0.05 was considered significant.

Results

During the study period, 183 EA patients were treated, 52 with LGEA. Of those, 151 reached a minimum of 1-year follow-up and were enrolled into this study. Long-gap vs non-long-gap: weight 1st year, gr; 6800 vs 8800 P < 0.0001; weight 2nd year 9500 gr vs 11000 p < 0.0001. BMI 1st year, median 14,75 vs 15,64 p 0.04; BMI 2nd year, median 15,18 vs 15 p 0.9; N° dilations 1st year, median 3 vs 1 P < 0.0001, N° dilations 2nd year, median 1 vs 0 P < 0.0001; Nissen 1st year, n (%) 9 vs 3 p 0.0019; Nissen 2nd year, n (%) 12 vs 4 p 0.0002. Redo-esophageal surgery 1st year, n (%) 7 vs 7 p 0.14. Redo-esophageal surgery 2nd year, n (%) 8 vs 0 < 0.0001. Readmission 1st year, median 4 vs 2 P < 0.0001; readmission 2nd year, median 3 vs 0 P < 0.0001. Oral aversion 1st year, n (%) 27 vs 10 P < 0.0001; oral aversion 2nd year, n (%) 17 vs 6 P < 0.0001. Dysphagia 1st year, n (%) 25 vs 18 P < 0.0001; dysphagia 2nd year, n (%) 14 vs 19 p 0.13.

Conclusions

LGEA patients underwent a more challenging course at follow-up, experiencing late auxological, more esophageal and GERD-related problems, and increased feeding and swallowing disorders in comparison with non-LGEA patients. A longer follow-up study is warranted to describe late and/or persistent problems.
DOZ047.72: Three-dimensional printing for preoperative planning of open laryngotracheal surgery
Authors: Fishman G , Wasserzug O , Berman P , Golden E , DeRow A
Published: June 2019

Background

Three-dimensional (3D) printing is being employed in a variety of surgical specialties to improve patient care. These models enable preoperative in vitro planning, advanced resident training, and better patient education. 3D models of the tracheobronchial tree that can simulate bronchoscopy and 3D printed cricoid cartilage models for balloon dilation training have been reported. A 3D model for preoperative planning of open laryngotracheal surgery has not been reported.

Objectives

The objective of this study was to report preliminary results with the employment of 3D printing technology for preoperative planning of laryngotracheoplasty (LTP) and cricotracheal resection (CTR).

Materials and Methods

Actual-size 3D models of the upper airway, from the level of the base of tongue to the level of the carina, have been created by the surgical 3D printing lab in the medical center. The models were based on computed tomography of two patients who were scheduled for LTP and CTR. The models were composed of several elements: the framework of the larynx and the trachea, the air column, the cannula, and the peri-stomal region.

Results

Two models were created, a model of a patient with grade III subglottic stenosis who subsequently underwent LTP and a model of a patient with grade IV subglottic stenosis who subsequently underwent CTR and end to end anastomosis. The 3D models were found to be useful for preoperative planning of the incision site in the trachea, the status of the tracheal and laryngeal framework, the length of the diseased segment, and the length of the rib cartilage graft to be harvested.

Conclusions

The preliminary results of this study imply that 3D models can be useful for preoperative planning of open laryngotracheal surgery. Further experience is required to establish its efficacy, the optimal model design, and cost effectiveness.
DOZ047.78: Endoscopic management of symptomatic tracheal pouches with trichloroacetic acid chemocauterization
Authors: Laín A , Giné C , García L , Barila P , López M
Published: June 2019

Introduction

Trichloroacetic acid (TCA) has been used in different conditions such as recurrent and congenital tracheoesophageal fistula and branchial sinus of the pyriform fossa. Nevertheless, it has never been described as a treatment for symptomatic tracheal pouches after an esophageal atresia repair. This study reports two cases of endoscopic treatment with TCA of symptomatic residual tracheal pouches in 2 children with repaired type III esophageal atresia.

Case Reports

Two cases of 7 and 8 years with esophageal atresia type III repaired in the neonatal period. Both were diagnosed with severe tracheomalacia and presented recurrent pulmonary infections complicated with pulmonary bronchiectasis. One of the patients benefited from a Nissen fundoplication due to severe gastroesophageal reflux at 2 years of life. Due to persistent respiratory infections bronchoscopy was indicated detecting a blind 2 cm tracheal pouch at the level of the carina in both patients.

Endoscopic treatment by the use of TCA was performed. By rigid bronchoscopy chemo-cauterization of the pouch with 50% TCA followed by a brush abrasion of the mucosa was done. Postoperatively one of the patients developed a slight and limited subcutaneous and mediastinal emphysema due to postoperative cough. No other complications were reported. Endoscopic control at 3 months (and also 12 in 1 case) after procedure showed complete obliteration of the tracheal pouches. After a medium follow-up of 17.5 months (14 and 21) a significant decrease in the number of respiratory infections and hospital admissions has been observed in both cases.

Conclusion

Endoscopic treatment by the use of 50% TCA in symptomatic tracheal pouches seems safe and effective. The complex surgical corrections of these conditions make it a perfect indication, representing a minimally invasive alternative.
DOZ047.80: Whole lung lavage for pulmonary alveolar proteinosis in children
Authors: Valiyev N , Cerit K K , Erdas M Y , Gokdemir Y , Erdem E , Kiyan G
Published: June 2019

Purpose

Pulmonary alveolar proteinosis (PAP) is a rare cause of chronic interstitial lung disease in children. PAP is characterized by the accumulation of lipoproteins in alveoli. PAP is a heterogeneous disease and occurs in three clinically distinct forms: congenital, secondary, and acquired. Whole lung lavage (WLL) is currently the gold standard for therapy of PAP. This study aimed to describe the technique and results of WLL in two patients with PAP.

Methods

There is no established standard technique in the management of lung lavage in children in the literature. We isolated one lung with an inflated Fogarty balloon and inserted a suction catheter of appropriate size and washed that lung while ventilating the contralateral lung via rigid bronchoscopy.

Case 1: A 2-year-old boy presented with dispnoe, cough and required continuous oxygen supplementation. The patient was diagnosed with congenital PAP confirmed pathologically and genetically. This patient underwent 9 times left-sided and 7 times right-sided WLL during the 39-month period.

Case 2: A 13-year-old boy presented with respiratory distress and required continuous oxygen supplementation. The patient was diagnosed with congenital PAP and confirmed pathologically and genetically. This patient underwent WLL 3 times for left and 3 times for right side during the 13-month period.

Both cases showed significantly decreased oxygen support and radiological improvement after WLL. But WLL did not provide a definitive treatment for either patient. No significant acute or long-term complication was observed.

Conclusions

We suggest that repeated WLL is an effective but not definitive treatment of PAP. The technique we used is safe and effective because it enables direct visualization of both main bronchi, allowing early recognition of any possible catheter dislocation and eliminating the risk of contralateral lung contamination.
DOZ047.81: Neurodevelopmental outcome in infants with AE: developmental trajectories in the first year of life
Authors: Bevilacqua F , Ragni B , Valfrè L , Conforti A , Braguglia A , Bagolan P , Aite L
Published: June 2019

Background

Esophageal atresia (EA) prognosis have improved significantly over the past three decades. Research and clinical attention has shifted to neurodevelopmental outcomes and quality of life.

Aim

The aim of this study wasto examine neurodevelopmental outcomes and to identify clinical and sociodemographic risk factors in a cohort of infants with EA.

Methods

An observational prospective longitudinal study was conducted between 2009 and 2017. Neurodevelopment was assessed at 6 and 12 months by Bayley Scales of Infants and Toddler Development—3rd Edition. Clinical and sociodemographic variables included were gender, birthweight, gestational age, associated malformations, number of hospitalizations, surgeries and dilatations at 12 months, days of mechanical ventilation, parental age, education level, and socioeconomic status.

Results

Ninety-six infants were enrolled in the study at 6 months and 73 of them were evaluated also at 12 months. Analysis showed significant differences between motor development at 6 and 12 months (M6 = 95.39, SD = 15.71; M12 = 91.83, SD = 12.87; t = 0.245, P = 0.017); significant differences emerged also between cognitive development at 6 and 12 months (M6 = 91.80, SD = 11.70; M12 = 100.92, SD = 15.39; t = −5.10, p = .000). Infants with long-gap AE achieved the worst scores in cognitive (r = -.28, P < .01) and motor scales (r = -.36, P < .01) at 6 months and in motor scale at 12 months (r = −0.30, P < 0.05). More days of mechanical ventilation were related to a lower score in both the cognitive (6 months r = −0.26, P < 0.05; 12 months r = −0.26, P < 0.05) and motor scale (6 months: r = −0.38, P < 0.01; 12 months r = −0.42, P < 0.01). A major number of interventions in the first year of life were related to lower scores in the motor scale at 12 months (r = −0.43, P < 0.01).

Conclusions

Infants operated on for AE are at risk of neurodevelopmental impairment in the first year of life. Findings support the association between neurodevelopmental outcomes and clinical risk factors. Careful interdisciplinary follow-up is essential for early detection of neurodevelopmental delay.
DOZ047.82: Outcome of thoracoscopic repair of type C esophageal atresia: single-center experience from North Africa
Authors: Elbarbary M , Shalaby A , Elseoudi M , Ragab M , Fares A E , Khairy D , Wishahy A M K , Seleim H , Eltagy G , Bahaaeldin K
Published: June 2019

Background and Aim

Thoracoscopic repair of esophageal atresia is gaining popularity worldwide owing to the availability of and advances in minimally invasive instruments. This report presents our early experience of thoracoscopic esophageal atresia repair (EA/TEF) in a single institute.

Methods

A prospective study on short-gap type C EA/TEF was conducted at Cairo University Specialized Pediatric Hospital from April 2016. The technique was standardized in all cases: patients placed in the fully prone position, and using a 5 mm 30° scope with 3 mm instruments and carried out by four experts in minimally invasive surgery. Data was collected to include patient demographics and operative time. The main outcome was survival. Secondary outcomes were leak rate and postoperative stricture.

Results

Over a period of 24 months (April 2016–April 2018) 136 cases of EA/TEF were admitted. Thoracoscopy was attempted in 76 cases. Thirty cases with pure atresia or long-gap anomaly were excluded from this study for gap length, leaving 46 (60%) nonconsecutive neonates who underwent thoracoscopic repair of their short-gap EA/TEF. Five cases (10.8%) were converted to open surgery via right thoracotomy. A primary anastomosis was possible in all cases, leaving the azygos intact. The mean age at operation was 8.7 days (range 2–32), the mean weight was 2.6 kg (range 1.8–3.6), and the mean operative time was 108.3 minutes (range 80–180). In the fully thoracoscopic patients the survival was 85.4% (n = 35). An anastomotic leak occurred in 17% (n = 7); it was managed conservatively in 2 and with esophagostomy and gastrostomy in 5 cases. Followup was at one week, one and three months postdischarge. A stricture developed in 5 (16.6%) of the 30 surviving patients who kept their native esophagus.

Conclusions

Thoracoscopic repair is a feasible alternative even in a low-resource setting. The better outcome compared to traditional open repair in our center may be due to patient selection bias.