bluuebunny/crossref_metadata_2025 huggingface dataset:
DOZ047.04: Upper gastrointestinal microbiome in esophageal atresia
Authors: Krishnan U , Singh H , Kaakoush N
Published: June 2019

Background

Esophageal atresia (EA) is a rare defect, resulting in the condition being understudied. Specifically, information on the upper gastrointestinal (GI) microbiome of EA patients is lacking.

Aims

This study aimed to examine the composition of the upper GI (oral, esophageal, and gastric fluid) microbiomes of EA patients as compared to controls.

Methods

A pediatric cohort was prospectively recruited at Sydney Children's Hospital in 2018, and comprised children with a history of EA and those without EA. The groups were subdivided into children with GERD, eosinophilic esophagitis (EoE), and those with no disease. Biopsies, saliva, blood, and both tracheal and gastric aspirates were collected. Histology was reviewed for changes related to reflux and peak eosinophil count in subjects with EoE. Ethics approval has been granted and informed consent obtained from all patients and/or their guardians. The bacterial component (16S rRNA gene) of the microbiome was amplified using the 515F–806R primers and sequenced with Illumina MiSeq 2 × 250 bp chemistry. Reads were analyzed using mothur and R.

Results

Within the cohort (n = 47), 29 (61.7%) had EA and 18 (39.3%) were non-EA patients. The mean age of the whole cohort was 8.36 years (SD 5.05) and 24 (44%) were male. Non-EA patients were having endoscopic assessment of GERD, EoE, suspected celiac or inflammatory bowel disease (IBD). Active GERD, defined as histological changes on biopsy, an abnormal acid reflux index, or retrograde bolus movement on pH/impedance testing, was present in 11 (23.4%; 5 with EA) children. The mean eosinophil count in patients with EoE was 11.1 (SD 14.74) in the EA cohort and 15.9 (SD 21) in the non-EA cohort. Proton pump inhibitors were used by 29 (61.7%; 16 with EA). EoE was present in 18 (44.4%; 8 with EA). Swallowed steroids were used in 13 (27.6%) patients with EoE. Microbiome changes were observed in patients with EA as compared to controls.

Conclusions

This study is the first to examine the upper GI microbiome of children with EA. Changes in the upper GI microbiomes of children with EA require validation in larger cohorts.
DOZ047.106: Internal stents and external splints for complex airway obstruction
Authors: Kamran A , Maschietto N , Baird C W , Jennings R W
Published: June 2019

Background

Life-threatening airway obstruction due to severe tracheobronchial stenosis or tracheobronchomalacia not amenable to tracheobronchopexy or resection is still a challenge, and warrants aggressive innovative strategies. In this study, our experience with placement of balloon-expandable stents and/or external bioabsorbable splints in children with severe tracheobronchial obstruction was reviewed.

Methods

All patients with symptomatic severe airway obstruction who underwent interaluminal stent and/or external splint placement (1997–2019) were retrospectively reviewed. The data collected included age, type of obstruction (stenosis or malacia), associated malformations, technical and clinical success, complications, and outcomes. Airway stents were placed under fluoroscopic guidance. Bioabsorbable splints (RapidSorb, Synthes CMF) were custom molded and then secured externally on the collapsing areas under simultaneous bronchoscopic and direct visualization.

Results

Seventeen patients underwent 14 airway stents (trachea = 5, bronchi = 9) and/or 11 external bioabsorbable splints (trachea = 5, bronchi = 6) at a median age of 5 months (IQR 3–9 months). Fourteen stents were placed in 8 patients, 10 external splints in 8 patients; one patient had two stents and one splint. Indications for stenting were stenotic airways in 11 patients (postoperative = 7, congenital = 4) and severe distal airway malacia in 3. All the external bioabsorbable splints were placed in patients with severe airway malacia secondary to lack of cartilaginous support and/or extrinsic compression. During follow-up 3/9 stented patients and 3/9 splinted patients did not experience any significant adverse events. Six of nine stented patients experienced serious adverse events: significant in-stent stenosis (n = 3), stent migration, and obstructive granulation tissue (n = 1), erosion through airway wall (n = 1), and death (n = 2). Of the nine splinted patients, one bronchial splint fractured, requiring replacement, and five patients required tracheostomies for continued respiratory failure from unilateral pulmonary hypoplasia and residual distal tracheal stenosis (n = 1), severe tracheomalacia (n = 2), subglottic stenosis (n = 1), and laryngeal cleft (n = 1). One patient died a week after surgery.

Conclusion

Airway internal stenting and external bioabsorbable splinting are effective therapeutic strategies for selected patients with complex airway abnormalities. Bioabsorbable splints are effective in maintaining airway patency, and being absorbable allow for age-proportional growth of the airways. Airway stents can be associated with significant adverse events and therefore should be reserved for patients who have failed other therapeutical strategies.
DOZ047.112: Myocardial strain in vascular anomalies with severe tracheomalacia: preliminary results
Authors: Massolo A C , Savignoni F , Monaco F , Giliberti P , Campanale M , Toscano A , Pasquini L , Dotta A , Bagolan P , Braguglia A
Published: June 2019

Background

Vascular anomalies (VA) may cause severe tracheomalacia in some cases. Chronic upper airway obstruction (UAO) is the most common symptom. Increased pulmonary pressure and cardiac dysfunction have been described in patients with chronic UAO, but not in infants with VA.

Aim

The aim of this study was to evaluate myocardial strain in infants with VA.

Method

Demographics characteristics, respiratory symptoms, and the percentage of tracheal obstruction measured on CT were collected. Left and right ventricle (LV, RV) systolic function were measured with speckle tracking longitudinal strain (LS) analysis. Pulmonary artery pressure was evaluated on tricuspid regurgitation (TR) jet and quantified by end-systolic eccentricity index (EI).

Conclusion

Of 15 cases, 6 had tracheal obstruction < 50%, 9 > 50%. LS LV and RV was significantly reduced in cases with obstruction > 50% compared to those with < 50% (LV −15.9% vs −19.9%; RV −15.7% vs −20.5%, respectively) and respiratory symptoms were significantly more pronounced in cases with obstruction > 50%. There are no significant data for TR and EI.

Results

In cases with VA with severe tracheomalacia RV and LV myocardial strain is reduced, suggesting myocardial impairment. Further studies with larger sample size are needed to confirm these data and investigate cardiac function. Association with lung function test may be investigated too.
DOZ047.116: Reducing complications for tracheostomy patients
Authors: Valika T , Thompson D M , Ida J
Published: June 2019

Objective

The objective of this study was to review the utilization of a standardized tracheostomy handoff sheet to reduce tracheostomy-related complications.

Methods

A retrospective chart review was carried out of patients who underwent tracheostomy and had a dedicated tracheostomy handoff sheet utilized in the postoperative period. The handoff sheet contains trach size, backup trach size, airway findings, suction depth, location of maturation suture, location of stay sutures, and photograph of the wound bed on postoperative day 0. An anonymous survey of healthcare providers participating in the patient's care was undertaken regarding the utility of the dedicated trach handoff sheet.

Results

There were a total of 18 completed questionnaires. The majority of respondents felt the trach handoff sheet was helpful and provided vital information (4.61, scale 1–5). Hundred percent of respondents referenced the handoff sheet at some point during the hospital stay; 83% of respondents (15/18) felt the handoff sheet was more productive than a verbal handoff and 67% of respondents (12/18) preferred the sheet be available bedside rather than a digital format for reference in the electronic medical record. 89% of respondents (16/18) utilized the handoff sheet for wound care.

Conclusion

There are numerous aspects to proper tracheostomy care. From the immediate postoperative time period to several years out, proper care can make the difference for quality of life, respiratory standpoint, and airway safety. With a daily nursing handoff that at times can become redundant, utilization of a standardized tracheostomy handoff can lead to improve outcomes. These handoff sheets provide information deemed essential to optimal patient care and are always available bedside for review. Standardization and utilization of these tracheostomy sheets have led to decreased airway complications in emergency situations, reduced wound breakdown, and increased safety and efficiency in handoffs.
DOZ047.16: Rules are made to be broken: examining the rule of 3 in pediatric esophageal strictures
Authors: Clark S J , Ngo P D , Staffa S J , Yasuda J L , Manfredi M A
Published: June 2019

Background

The ‘rule of 3’ holds that bougie dilation of an esophageal stricture should not progress beyond 3 mm in a single session. Despite the existence of this ‘rule’ for at least 40 years, one recent study found that nonadherence to the rule for balloon and bougie dilations did not increase the risk of perforation. All these studies were done in adult populations; no studies have examined the rule of 3 in pediatric patients. In addition, no study has suggested a maximum dilation size that can be safely performed in one session.

Methods

A retrospective chart review of patients with esophageal strictures caused by surgery (esophageal atresia or congenital esophageal stricture repair), foreign body or caustic ingestion, or extrinsic compression was performed. Between January 2016 and May 2018, 275 patients underwent 1581 balloon dilations. Delta diameter increase was calculated for 1453 endoscopies by subtracting the initial stricture diameter as determined by the endoscopist prior to dilation from the diameter of the largest balloon used. Perforations were defined as any contrast extravastion outside of the esophagus or hospital readmission for delayed onset of perforation. Perforation rate by delta diameter increase was analyzed using logistic regression modeling, receiver operating characteristic (ROC) curve analysis, and Fisher's exact test.

Results

There were 8 perforations in 1093 dilations with delta diameter <6 mm (0.7%) and 18 perforations in 360 dilations with delta diameter ≥6 mm (5.0%). ROC analysis demonstrated good discriminatory ability of delta dilation in predicting perforation (area under ROC = 0.753; 95% CI: 0.665–0.841; P < 0.001). The optimal cutoff as determined by Youden's J index was a delta dilation ≥6 mm (sensitivity = 68%, specificity = 76%). The initial starting diameter at the time of dilation did not influence the perforation rate (P = 0.126).

Conclusion

Nonadherence to the rule of 3 with dilation up to 5.5 mm in a single balloon dilation session appears safe in pediatric patients.
DOZ047.27: Esophageal atresia and polyhydramnios
Authors: Arntzen T , Mikkelsen A , Haugen G , Emblem R
Published: June 2019

Background

Prenatal diagnosis of esophageal atresia (EA) is difficult and the detection rate is only 20–35%. Ultrasound features of EA are nonspecific with polyhydramnios as the most reported finding. Polyhydramnios is reported in approximately 2% of all pregnancies and thus have low specificity. The aim of our retrospective study is to explore perinatal characteristics of EA patients with prenatal suspicion of EA.

Methods

Patients with EA born in the periods 1996–2002 and 2011–2017 were included after consent of the parents. Data regarding the pregnancy, birth, and perinatal treatment were obtained from medical records.

Results

We registered a total of 124 EA patients: 68 from 1996 to 2002, and 56 from 2011 to 2017. Among the 124 patients, 5(4%) had type Gross A or B, 108(87%) type C, 6(5%) type D, and 5(4%) type E. 73(59%) patients had an associated anomaly. 20/124(16%) patients had a prenatal suspicion of EA, and there were no significant differences between neonates with and without prenatal suspicion of EA in terms of birth weight, gestational age, and prematurity. Patients with prenatal suspicion of EA had more caesarean sections, were more frequently born at regional hospitals, and had more associated anomalies.

Polyhydramnios was registered in 70 patients; in 18 of these (90% of all with prenatal diagnosis) a prenatal suspicion of EA had been raised. In 52 EA patients with polyhydramnios, no prenatal statement about anomaly was registered.

Four of 5 patients (90%) with type A and B had a prenatal suspicion of EA. Corresponding number for type C was 15/108(14%) and 1/5(20%) with type E.

Conclusion

The detection rate for EA is low, and prenatal suspicion of EA implicates a more serious condition with higher morbidity than EA patients born without prenatal suspicion of malformation. Four of five patients without fistula to lower esophagus (type A and B) had a prenatal suspicion of EA, and polyhydramnios is the ‘signal sign’ for the possible presence of esophageal atresia.
DOZ047.30: First year risk factors of mortality and morbidity in esophageal atresia with tracheo-esophageal fistula: data from a population-based national register
Authors: Sfeir R , Rousseau V , Bonnard A , Gelas T , Michaud L , Boubnova J , Piolat C , Irtan S , Fouquet V , Mandat A Le , De Napoli S , Habonimana E , Lamireau T , Lemelle J L , Elbaz F , Schneider A , Polimerol M L , Allal H , Buisson P , Petit T , Sapin E , Lardy H , Podevin G , Levard G , Varlet F , Michel J L , Jaby O , Pelatan C , De Vries P , Borderon C , Fourcade L , Breaud J , Pouzac M , Tolg C , Auber F , Geiss S , Laplace C , Drumez E , Gottrand F
Published: June 2019

Objectives and Study

The aim of our study is to assess the risk factor of mortality and morbidity in a large population-based registry in a population of type III/C esophageal atresia (EA).

Methods

Under the umbrella of the national plan for rare disease, a population based register was set up in 2008 recording the data of all the live newborns with EA in France. Based on the registry data, survival and morbidity at 1 year were studied. Morbidity was approached by calculating the rate of full oral autonomy and the hospital length of stay during the first year. Multivariate analysis was performed via multinomial logistic regression to evaluate independent predictors of overall survival and morbidity items among all significant category variables identified on univariate analysis.

Results

A total of 1008 patients with type III EA were extracted from the national database born from January first 2008 till 31 December 2014. Mean birth weight was 2610 g (2060 to 3075). Right lateral thoracotomy was used in 93% of cases and primary anastomosis was possible in 95% of cases. Associated abnormalities were present in 53% of patients, VACTERL in 19%, and CHARGE syndrome in 3%. Mortality at 3 months was at 5% and at one year at 6% and was correlated to prenatal diagnosis (odd ratio (OR):2.96 (1.08 to 8.08)), low birth weight (OR: 0.52 (0.38 to 0.72)), and heart defect (OR: 6.09 (1.96 to 18.89)). Length of hospitalization was correlated to birth weight (OR: 0.83 (0.61–1.13)), the difficulty of the anastomosis (OR: 1.59 (0.71–3.55)) and associated abnormalities (OR: 1.93(0.65–5.68)), the prenatal diagnosis is correlated only to the rate of full oral autonomy and not with the length of hospitalization.

Conclusions

Surgical procedure or difficulties did not seem to affect survival in this group of patients. In addition to the continuous need to improve care of low weight neonates, our results clearly identify that efforts should be focused on the small group of EA with prenatal diagnosis and/or severe cardiac malformation to reduce even more mortality and morbidity in EA patients.
DOZ047.32: Infantile hypertrophic pyloric stenosis in patients with esophageal atresia: proposal for a causative seesaw model
Authors: Ten Kate C A , Brouwer R W W , van Bever Y , Martens V K , Brands T , van Beelen N W G , Brooks A S , Huigh D , Eussen H , van IJcken W F J , IJsselstijn H , Tibboel D , Wijnen R M H , de Klein A , Hofstra R M W , Brosens E
Published: June 2019

Aim of the Study

Patients born with esophageal atresia (EA) appear to have a 30 times higher prevalence of infantile hypertrophic pyloric stenosis (IHPS). This makes sense from a developmental perspective as both the esophagus and the pyloric sphincter are foregut derived structures. We hypothesized that genetic defects, disturbing foregut morphogenesis, are responsible for the specific combination of EA and IHPS.

Methods

Patients with both EA and IHPS born between 1970 and 2017 and where possible their parents were included. This study was approved by the internal review board of the Erasmus MC. After parental written informed consent, we determined genetic profiles with whole exome sequencing and SNP-array-based copy number variation analysis. We focused on (1) genetic variation in known EA and IHPS disease genes, (2) pathways important for foregut morphogenesis, (3) shared rare genetic variation, and (4) ultrarare variants in variant-intolerant genes, which have a high chance of being de novo. Segregation analysis of possible candidate variants was performed.

Results

Twenty-seven out of 664 patients (4.1%) born with EA during the study period developed IHPS, of which 15 cases were analyzed. As none of the parents were affected, we considered dominant (de novo) or recessive and X-linked inheritance models. Unfortunately, we could neither identify rare de novo mutations, nor variants fitting a recessive model. We did however identify inherited putative deleterious heterozygous variants in genes either known to be involved in EA or IHPS (e.g., COL7A1, TNXB, WDR11, GDF6) or important in foregut morphogenesis (e.g., GLI3, NKX2.1) in all patients. Moreover, seven genes were affected by rare variation in ≥2 patients (ADAMTSL4, ANKRD26, CNTN2, HSPG2, KCNN3, LDB3, SEC16B) and expressed in the developing foregut, esophagus or pyloric sphincter in mice between E8.25 and E18.5. However, burden analysis did not show a significant difference with unaffected controls.

Conclusions

Although the presence of genetic variation in likely candidate genes suggests a genetic component, genetic factors alone could not explain the abnormalities seen in these patients. Therefore, we propose a multifactorial model in which the combination of high impact genetic, mechanical, and environmental factors together can shift the balance from normal to abnormal development.
DOZ047.33: Neonatal airway emergency: report of two cases
Authors: Grazioli P , Bosio R , Baronchelli C , Santoro A , Chirico G , Redaelli de Zinis L O
Published: June 2019

Objective

This paper discusses two cases of congenital airway malformations that presented in NICU in a four-month period. The aim is to present extremely rare evidences that inevitably lead to the death of the patient if not correctly identified during pregnancy.

Case 1: male twin born at 34 weeks by emergency caesarean section to a 37-year-old mother. Antenatal history was notable for in vitro fertilization and renal dysplasia. Immediately after delivery, there was respiratory distress, cyanosis, with a 1- and 5-min Apgar score of 0 and 1, respectively. He required ventilation and was supposed intubated orally with significant difficulty with a 2.0 mm ETT. For persistent ventilation problems and severe combined acidosis, the neonatologists tried without success to reintubate the patient. Some hours later, the otolaryngologist was called and was again unable to intubate with flexible laryngoscopy due to an obstruction that prevented advancement of the endotracheal tube past the vocal folds, but since the baby general conditions had been already deteriorated a decision to withdraw the treatment was made. He died after few hours. Postmortem revealed a polymalformative syndrome with subglottic complete diaphragm, a tracheoesophageal fistula 1 cm caudally to the diaphragm and unilateral multicystic renal dysplasia.

Case 2: male vaginally delivery at 35 weeks to a 43-year-old mother with gestational diabetes. Antenatally, ‘VACTERL’ association was suspected on the basis of the US and a MRI was planned but not performed because of the early delivery. Following the delivery, there was severe respiratory distress, no audible cry, and ventilation was not effective in relieving the respiratory distress. Subsequent intubation was unsuccessful. An emergency tracheostomy was attempted: the larynx was identified, but only a tracheal stump was present on neck exploration. Postmortem confirmed type II (according to Floyd) tracheal agenesis with the esophagus connect to the main bronchus, renal dysplasia, anal atresia, and single umbilical artery.

Conclusion

Clinicians need to be aware of congenital airway malformation and subsequent difficulties upon endotracheal intubation and they have to be prepared to plan a multidisciplinary management at the delivery including emergency intubation through esophageal fistula.
DOZ047.34: Oral feeding attainment
Authors: Hernandez K , Davidson K , Dargie J , Jennings R , Manfredi M
Published: June 2019

Aim

A primary goal of esophageal atresia repair is to establish esophageal continuity to allow for swallowing of secretions, liquid, and food boluses. The transition to oral feeding and acquisition of oral sensorimotor skills following repair of long-gap esophageal atresia (LGEA) can be challenging. The timing of attaining full PO status (F-PO), without need for enteral tube feeding support, can vary greatly. A retrospective study was performed to identify predictors of oral feeding success in children with LGEA.

Methods

A retrospective case series was conducted with chart review of patients with a diagnosis of LGEA who underwent Foker process for staged repair from 2012 to 2017. Children with previous failed attempts at esophageal repair or other significant surgeries were excluded. Comparison was made between patients who achieved full PO status (F-PO) within the study follow-up period (minimum of one year postrepair) and those who did not.

Results

Twenty-three patients were included: twelve male and eleven female. Eight patients (35%) had an accompanying genetic diagnosis. Thirteen patients (57%) were born prematurely. Six patients (26%) were F-PO at 6 month post-repair; four of which were on an age appropriate diet without restrictions/modifications. Thirteen patients (57%) achieved F-PO by end of the study follow-up period while 43% required supplemental nutrition. Gestational age ≥ 37 weeks (P = 0.03), younger age at first PO trial (P = 0.013), shorter time between Foker 2 and first PO trial (P = 0.011), consistent PO intake at 6 months post-repair (P = 0.02), and fewer total number of airway/esophageal procedures within 1 year post-repair (P = 0.018) were found to be significantly associated with achieving F-PO. Total number of esophageal dilations within 2 years of esophageal repair and presence of a genetic syndrome were not significantly different between groups.

Conclusion

A majority of patients (57%) who undergo repair of LGEA via Foker process will progress to oral feeding; however, the timing of this progression is variable. Predictors of oral feeding success can be used to guide prognosis and identify patients at greatest need of therapeutic services.
DOZ047.36: Thoracoscopic esophageal internal axial traction for long- gap esophageal atresia
Authors: Juricic M , Panait N , Podevin G , Bonnard A , Lopez P , Grossos C , Fourcade L , Merrot T , Boubnova J , Dariel A
Published: June 2019

Aim of the Study

Long-gap esophageal atresia (LGEA) remains a surgical challenge. This study aimed to report the results of thoracoscopic esophageal axial internal traction in LGEA.

Methods

This multicenter observational study included retrospectively neonates who underwent primary thoracoscopic esophageal axial internal traction for LGEA between June 2017 and July 2018. LGEA was defined as the technical impossibility to perform a primary esophageal anastomosis. The Ethical Review Board of our institution approved the study.

Main Results

Eight neonates were included with a median gestational age at birth of 35 weeks [25; 37] and a median birth weight of 2266 g [890; 3800], 6 types I and 2 types II according to Ladd's classification. Initial median gap between 2 esophageal ends was 5 vertebral bodies [4.5; 7]. Internal traction was performed at a median age of 5 weeks of life [1; 17] with a median operative time of 87 minutes. Four patients required at least 2 internal traction procedures. After a mean traction time of 1.5 weeks [1; 13.5], esophageal anastomosis was successfully performed in 7 patients (5 thoracoscopies, 2 thoracotomies) with a median operative time of 165 minutes. One patient needed a colonic interposition. Five of these 7 patients required an esophageal endoscopic dilatation (median number: 4 [2; 6]). Median follow-up was 9.75 months [3; 16].

Conclusions

Thoracoscopic esophageal axial internal traction for LGEA was a safe and feasible procedure that allowed an esophageal anastomosis in 7 of the 8 patients. Improvement of the procedure requires setting a common protocol concerning the timing of the first internal axial traction and the duration of traction before considering esophageal anastomosis.
DOZ047.48: Postoperative anastomotic stricture after the operation for esophageal atresia
Authors: Wolski M , Jasińska A , Kamiński 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. Predisposing factors are the leakage of the anastomosis, undertension anastomosis, elongation, and gastroesophageal reflux. A few dilation techniques are being utilized.

Material and Methods

Between 2006 and 2018, 79 patients with esophageal atresia were treated in the clinic. Primary or delayed anastomosis was possible in 69 patients. Five patients died in the early postoperative period. Clinical data of the remaining 64 patient were analyzed retrospectively.

Results

All anastomoses were calibrated with either bougie or balloon dilators. The anticipated diameter of the anastomosis was acquired during calibration in 24 patients (seven of these with bougie and 17 with balloon dilations). The total number of bougie dilations was 29 and balloon dilations 35. The stricture was present in 40 patients. In this group under—tension anastomoses were present in 25 patients, anastomotic leakage in 13 (81% of all leaks), gastroesophageal reflux in 14 (66% of all refluxes). The stricture was dilated with the balloon in 22 cases, bougie in 18 cases, stent placement in 3 cases. The number of required dilations was most often 2 or 3. In 4 patients multiple dilations were necessary. Dilating procedures were finished with a satisfactory result before the age of 6 months in 26 patients, 12 months in 13 and 24 months in 5 patients. Rupture of the anastomotic site during dilation occurred in 4 patients.

Conclusions

Anastomotic stricture occurred in 63% of patients. The majority of the undertension and leaking anastomoses were complicated with stricture. A single calibration with balloon was successful in 49% of cases compared to 24% bougie calibrations. In 59% of dilated patients, the procedure was finished before the age of 6 months, which enabled physiological feeding pattern.
DOZ047.50: Patterns of sensory processing in children with esophageal atresia
Authors: Huri M , Günal R , Soyer T
Published: June 2019

Esophageal atresia (EA) is a congenital anomaly requiring neonatal surgery, medical treatment, extended hospitalization, and rehabilitation. Occupational therapists offer a unique perspective on the sensory aspects of behavior. From a sensory processing and integrative perspective, the behaviors are related either to generate or avoid sensory stimulation. Determining the child's threshold for tolerating sensory stimuli helps families and professionals to understand the child's reaction to life experiences additionally help to develop skills such as learning, attention, and somatosensory awareness of the body. The aim of this study is to assess and compare the sensory responses of children with EA and typically developing children (TDC).

Sensory Profile is a parent-reporting measure, which assesses children's sensory responses in daily life. Parents response the frequency of their child responds to 125 commonly occurring experiences. Items assess eight categories: auditory, visual, activity level, taste/smell, body position, movement, touch, and emotional/social and nine factors as sensory seeking, emotional reactive, low endurance/tone, oral sensory sensitivity, inattention/distractibility, poor registration, sedentary, fine motor/perceptual. The measure is determined by a Likert scale.

Parents of 12 children (5.60 ± 2.07 years/min: 3; max: 8) with EA and parents of 12 TDC (4.23 ± 1.14 years/min: 3; max: 8) matched by age and gender completed the Sensory Profile. The results were analyzed with the SPSS 18.

Children with EA differed significantly from TDC children in their sensory responsiveness based on Sensory Profile results in the areas of auditory, tactile, vestibuler, taste and smell processing (P < 0.05) and sensory seeking, emotional reactive, low endurance/tone, oral sensory sensitivity, inattention/distractibility, poor registration factors (P < 0.05). The Sensory Profile can contribute to diagnostic and rehabilitation planning processes and increase understanding of the nature of sensory problems of children with EA. Further studies should be designed with extended groups to generate the results and the effects of interdisciplinary rehabilitation programmes including sensory- based occupational therapy must be analyzed on children diagnosed with EA.
DOZ047.53: Esophageal balloon dilatation for strictures developing after esophageal atresia surgery
Authors: Durakbasa C U , Aksu B , Uzun E , Ugurlu D , Aydoner S , Erkoc G , Ozel K
Published: June 2019

Aim

The aim of this study is to evaluate the results of esophageal balloon dilatation (BD) for strictures after esophageal atresia (EA) surgery.

Methods

Flexible endoscopic BD was done under fluoroscopic and manometric control. The balloon placed in the stricture was inflated by contrast. The target pressure and the diameter were decided in compliance with the manufacturer's directions as well as fluoroscopic guidance. The balloon was kept inflated for 3 minutes. Prospectively collected data over 10 years were retrospectively evaluated. Cure was defined as no need for dilatation during the last 12 months.

Results

A total of 79 patients with variable diagnoses underwent 481 BD. Forty (51%) had strictures which developed after EA surgery. They underwent 175 (36%) BD (P < 0.05). There were 21 males and 19 females. The atresia was distal fistula type in 31 (77.5%) patients and isolated EA in nine (22.5%). The BD was done for primary esophago-esophageal anastomosis site in 37 patients. The remaining three patients with long gap EA had undergone previous replacement surgery and the BD was done for the proximal esophago-colonic anastomosis. The median age at the time of the first BD was 14 months with 17 (43%) patients below the age of 1 year. The maximal inflation diameter varied between 5–20 mm. Esophageal BD catheters were used in all except two occasions where 5 mm ureteral balloons were used. The median number of BD was four (n = 1–15). Thirty-four (85%) patients underwent more than one BD. A transmural perforation was encountered in one occasion (0.6%) and the patient eventually underwent esophageal replacement surgery with an uneventful outcome. There was no mortality. Fundoplication was done in nine patients. Thirty-three patients (82.5%) were regarded as ‘cure’.

Conclusion

Esophageal BD is employed for strictures caused by a variety of reasons. Patients with EA comprise a substantial number of cases needing BD and have strictures less resistant to dilatation. The technique employed in this study is advantageous because it enables direct endoscopic visualization of the stricture and both gradual and controlled increase of the dilatation pressure. BD is safe and efficient yet there is a risk of esophageal perforation as in the other dilatation techniques.
DOZ047.61: Elective tracheostomy in critically ill children: to do or not to do? Ten year experience from an academic center in Pakistan
Authors: Ishaque S , Karim F , Qazi S H , Abbas Q
Published: June 2019

Background

Tracheostomy is one of the oldest and most commonly performed procedures among critically ill patients. The advantages of an elective tracheostomy in pediatric intensive care unit are improved patient comfort, lesser need for sedative drugs, early weaning from mechanical ventilation support eventually leading to reduced cost of care.

Objective

This study describes the frequency, indications, complications, and outcome of elective pediatric tracheostomies in critically ill children from a single pediatric intensive care unit of a tertiary care center.

Design

This is a retrospective cohort study of patients undergoing tracheostomy.

Setting

This is a pediatric intensive care unit (PICU) of a tertiary-care hospital.

Patients

All patients underwent tracheostomy in our PICU over the ten-year period.

Main Results

A total of 48 children underwent a tracheostomy, corresponding to a 1.5% of the total PICU admissions during the study period. 34/48 (71%) patients were male. A 25% of our patients undergoing a tracheostomy had an underlying CNS condition, followed closely by a respiratory problem (11/48 patients).The main indication for tracheostomy in children was prolonged mechanical ventilation secondary to respiratory 35/48 (73%), that included upper airway obstruction, foreign body aspiration or pneumonia and neurological or neuromuscular illness (6.3%) including traumatic brain injury, meningitis/encephalitis, Gullain Barre’ syndrome, and neurodegenerative disorders. Two patients died from tracheostomy-related complications, making it an overall mortality rate of 4%.

Conclusion

Tracheostomy in children is a relatively frequent procedure at our hospital. The commonest indication was prolonged mechanical ventilation. Early tracheostomy is associated with better patient outcomes in terms of morbidity and length of stay.
DOZ047.62: Fixed the gap, solved the problem? Children operated on of esophageal atresia: what happen 3 years later
Authors: Bevilacqua F , Ragni B , Conforti A , Morini F , Dotta A , Bagolan P , Aite L
Published: June 2019

Background

With improvements in surgical care long-term morbidity of patients with esophageal atresia (EA) has been recently taken into account (Lees et al., 2018). Morbidities include gastrointestinal and respiratory complication, problems of nutrition and growth, feeding difficulties, oral aversion or food refusal (Menzies et al., 2016).

Aim

Purpose of this study is to explore eating problems and its implications at 3 years in infants born with esophageal atresia.

Methods

A retrospective study including all infants operated for EA at our Institution in the period ranging from January 2012 and January 2016 who attended our follow-up program. A specific interview was set up for the study. The interview was delivered by telephone by a trained psychologist.

Results

Parents of 51 children (male = 67%) were interviewed. Twenty-two percent of children showed a growth <10th percentile; 30% presented oral aversion for a period of their life; 45% still have episodes of chock during meals and 18% of these had more than one episode a week; 45% suffer of GERD and 41% experienced recurrent respiratory problems; 37% of parents experience anxiety during meals. Fifty-seven percent attended kindergarten (8% with a dedicated educator during meal time), and 92% attended preschool (26% with dedicated educator during meal time). Currently 67% of the families benefit of social facilities.

Conclusions

Three years after surgical repair of the structural defect, a high percentage of family still experience physical and psychological problems related to eating behavior of their children. Efforts of the health care team as well as research should focus on how to prevent eating problems and improve quality of life both of children and families.
DOZ047.66: Thoracoscopic posterior tracheopexy during primary esophageal atresia repair
Authors: Tytgat S H A J , van der Zee D C , Verweij J W , van Tuyll van Serooskerken E S , Lindeboom M Y A
Published: June 2019

Background

Esophageal atresia (EA) is usually accompanied by some form of tracheomalacia. During the early phases in life, excessive dynamic collapse of the trachea can cause a wide spectrum of symptoms ranging from mild complaints to apparent life-threatening events (ALTE’s). Therapeutic strategies for severe tracheomalacia include aortopexy to lift the anterior weakened cartilaginous rings or posterior tracheopexy of the floppy membranous tracheal intrusion. Earlier we have introduced a new approach in which the posterior tracheopexy is performed directly during primary thoracoscopic correction of EA.

Methods

In the period 2017–2018, all consecutive EA patients (27) underwent a rigid tracheobronchoscopic evaluation during induction of anesthesia prior to the thoracoscopic EA repair. Tracheomalacia was diagnosed in 11 patients. During the subsequent thoracoscopic procedure, the posterior tracheal membrane was fixed to the anterior longitudinal spinal ligament with nonabsorbable sutures. The esophageal ends were then mobilized toward the right hemithorax and anastomosed.

Results

On preoperative RTB, six patients had a severe (66–99%) mid tracheal collapse and five patients had a moderate (33–66%) collapse. Thoracoscopic posterior tracheopexy with on to three sutures was possible in all 11 patients, prior to the formation of the esophageal anastomosis. Median time per suture was 6 minutes (range: 4–12 minutes). All operative procedures were uneventful. A median follow-up of 10 months (range: 2–22 months) revealed that eight patients recovered without any respiratory symptoms, one patient had respiratory symptoms caused by a suture granuloma that was removed by bronchoscopy, one patient had a respiratory syncytial virus bronchiolitis and one patient had a rhinovirus infection. None of the patients experienced any ALTE’s.

Conclusion

Eleven patients have been treated by thoracoscopic posterior tracheopexy during primary EA repair. This technique could prevent potentially deleterious sequelae of moderate to severe tracheomalacia that may complicate the lives of EA patients. Also, a second, sometimes complex surgical procedure can be prevented as the posterior tracheopexy is performed during the primary thoracoscopic EA correction.
DOZ047.73: Endoscopic treatment of esophageal atresia
Authors: Vogiatzoglou T , Arrigo S , Gandullia P
Published: June 2019

Aim of the Study

The aim of this study was to provide an overview of endoscopic treatment in children after reconstruction of esophageal atresia.

Methods

This study, conducted at a single tertiary center, reviewed patients requiring endoscopic treatment during a 15-year period, from 2004 to 2018. Collected data include number, frequency, complications, and effectiveness of esophageal anastomotic dilations.

Results

A total of 55 children with esophageal atresia (EA) underwent upper gastrointestinal endoscopy. Of those, 37 required therapeutic endoscopy with dilation procedures for anastomotic strictures. Thirty-five patients underwent dilations using only through-the-scope (TTS) balloon (BD),1 patient using only a Savary bougie (SB), and 1 using both. A total of 126 dilations were performed using BD in 36 patients and 6 dilations using SB in 2 patients. Specifically, in children treated only with BD were performed 1 dilation in 6 children (17.1%), 2 in 6 children (17.1%), 3 in 7 children (20%), 4 in 6 children (17.1%), 5 in 6 children (17.1%), 7 in 2 children (5.7%), and 8 in 2 children (5.7%). Balloon catheter sizes ranged from 6 mm to 15 mm. In 2 patients (5.4%) stents were placed, 1 and 6 stents, respectively. Intralesional triamcinolone was injected in 8 patients (21.6%) as additional therapy. Major complications included perforation in 4 patients (10,8%), resolved with conservative treatment. All patients had clinical improvement in the follow-up.

Conclusion

Currently, endoscopic treatment for esophageal anastomotic stricture is the first procedure adopted in clinical practice after the surgical approach, since it is regarded safe and effective. Balloon dilations are preferred in our overview as a treatment option for esophageal anastomotic strictures. Symptom relief is reported in all cases, while the rate of complications is very low. Sometimes, refractory strictures influence the patient's quality of life and therapeutic alternatives such as stent placement should be considered.
DOZ047.87: Longitudinal evaluation of lung function in infants with esophageal atresia
Authors: Landolfo F , Conforti A , Columbo C , Calzolari F , Savignoni F , Scuglia M , Braguglia A , Dotta A , Bagolan P
Published: June 2019

Introduction

Patients affected by esophageal atresia (EA) often faced airway problems, due to multifactorial dynamics (tracheomalacia, gastroesophageal reflex disorder, etc.). The aim of this study was to longitudinally evaluate the lung function test (LFT) in those infants to explore how the LFT modifies at mid-term follow-up.

Methods

Retrospective evaluation of lung functions in infants treated for EA (2010–2017) was performed at three time points: 6 months, 12 months, and 24 months.

Tidal volume (Vt), respiratory rate (RR), and time to peak tidal expiratory flow as a percentage of total expiratory time (tPTEF/te) were analyzed. ANOVA test was used as appropriate.

Results

During the study period 172 patients were treated for EA. Of those 50 infants (28%) underwent LFT at 6 months, 30 at 12 months, and 11 at 24 months. Tracheomalacia was present in 20 infants (42%). Both Vt (6,79 ml/kg vs 7,82 ml/kg vs 8,37 ml/kg—p = 0,001) and RR (49,7 a/min vs 40,6 a/min vs 34,0 a/min—p = 0,020) significantly improved, while there was no significant difference for tPTEF/te (0,25 vs 0,26 vs 0,29—p = 0,62) despite the data showing an improvement trend.

Conclusion

The preliminary data suggest that, although EA patients may present impaired LTF in early infancy, lung function seems to improve over time, showing normal pulmonary function test at 24 months. The presence of selection bias and the retrospective nature of the study limit our result. Nonetheless, evaluation of lung function is warranted in EA infants to early detect respiratory symptoms, ideally reducing the impact on short- and long-term pulmonary outcomes.
DOZ047.94: The thoracoscopic approach for esophageal atresia and tracheoesophageal fistula—lessons learnt from 178 cases
Authors: Patkowski D , Gerus S , Rasiewicz M , Polok M , Świątek K , Dzielendziak A , Palczewski M , Borselle D , Toczewski K , Śmigiel R
Published: June 2019

Purpose

The aim of this study was to present an operative technique based on personal experience with 178 cases of thoracoscopic repair with different types of EA/TOF.

Material and Methods

From 2005 178 patients (111 boys, 47 girls) underwent thoracoscopic repair in different hospitals. The first author was involved in all cases except two final procedures of staged repair.

Results

There were 30 cases of type A, 10 of type B, 129 of type C, 2 of type D, and 7 of H-type isolated fistulas. There was no contraindication to thoracoscopy if the surgical intervention was considered. The smallest operated patient weighed 1000 g. If the patient was respiratory unstable, only the fistula was closed, and the anastomosis was completed later (3 cases). All cases of type C, type D, and H-type fistula were completed thoracoscopically without conversion. Four cases of type A had primary thoracoscopic anastomosis. For the remaining long-gap EA (type A and B exclusively) stage internal traction suture technique was used. There were 16 deaths with 3 related to postoperative surgical complications. The operative technique evolved with growing experience. At the beginning the lateral position was used, which finally changed to a complete prone position. Three trocars 2 × 2,5–3,5 mm and 1 × 5 mm in diameter were placed around the scapula. The 5 mm telescope 25–30 degrees was preferred. A pneumothorax was maintained with 4–6 mmHg. The azygos vein was never divided, and the anastomosis was almost in each case made above the vein. In the majority of cases the vein separated the anastomosis and the fistula closure site. At the beginning the TEF was closed with 5 mm titanic clips. As the clips in a few cases disappeared on X-ray taken later ligation of the fistula was started. The esophageal anastomosis was made over the nasogastric tube using routinely sliding (slipping) 4–0 or 5–0 absorbable knots. If there was a considerable tension two or three sliding knots were placed and closed gradually. If the anastomosis was successful, the chest drainage was not routinely used. For the primary cases there was no need to use the electrocautery—the procedure was bloodless with blunt tissue dissection. The first procedure took almost 360 minutes; the last cases were completed within 60 to 80 minutes.

Conclusions

The thoracoscopic repair of EA/TOF is an effective method and based on our experience it is the procedure of choice if performed by an experienced endoscopic pediatric surgeon.
DOZ047.95: Crossing the disciplines: what airway surgeons can learn from interventional cardiology
Authors: Brigger M T , El-Said H
Published: June 2019

The intimate association of airway and cardiac anatomy has long led to frequent and collegial collaboration between airway surgeons and cardiovascular surgeons. While surgical colleagues tend to provide clear lines of interaction, communication between all members of the team is critical to long-term outcomes. Interventional cardiologists are integral to the care of children with combined cardiac and airway problems and have increasingly redefined the indications for open surgical repair of cardiac defects. In a similar vein, collaboration between airway surgeons and interventional cardiologists yields a blending of strategies, approaches, and instrumentation that allows for innovative approaches to primary airway problems. The nonsurgical and minimally invasive practice of interventional cardiologists can provide an invaluable viewpoint that has direct application to difficult problems of the airway. Using a case-based approach, representative strategies applying various principles of interventional cardiology will be used to illustrate the cross-discipline value. Specific cases will focus on the role of diagnostic approaches and strategies, imaging modalities including fluoroscopy as well as advanced computed tomography and magnetic resonance imaging techniques, instrumentation including wires, balloons, and stents that have evolved to manage pediatric cardiac anomalies and have been demonstrated to be useful in managing the pediatric airway. The cases will demonstrate how innovation and cross-fertilization of ideas cultivate advancement in both disciplines and advocate for institutional collaboration.