bluuebunny/crossref_metadata_2025_split huggingface dataset:
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

Abstract

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

Abstract

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

Abstract

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

Abstract

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

Abstract

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

Abstract

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

Abstract

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

Abstract

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

Abstract
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

Abstract

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.