Dysphagia in patients undergoing esophageal atresia surgery: Assessment using a functional scale. / Disfagia en pacientes intervenidos de atresia de esófago: valoración con una escala funcional.

INTRODUCTION: Dysphagia is defined as difficulty swallowing. Up to 84% of patients undergoing esophageal atresia surgery have dysphagia beyond the neonatal period. MATERIALS AND METHODS: A retrospective study of patients undergoing esophageal atresia surgery from 2005 to 2021 was carried out. The Functional Oral Intake Scale (FOIS) was used to assess dysphagia in 4 age groups (< 1 year old, 1-4 years old, 5-11 years old, and > 11 years old). FOIS scores < 7 or symptoms of choking, impaction, or food aversion were regarded as dysphagia. RESULTS: 63 patients were analyzed. 74% (47/63) had dysphagia during follow-up. Prevalence was 50% in patients < 1 year old (FOIS mean 4.32), 77% in patients aged 1-4 (FOIS mean 5.61), 45% in patients aged 5-11 (FOIS mean 5.87), and 38% in patients > 11 years old (FOIS mean 6.8). The most frequent causes of dysphagia were stenosis, which occurred in 38% of the patients (n=24), and gastroesophageal reflux (n=18), which was present in 28% of the patients. Both conditions were associated with significantly lower mean FOIS scores (p< 0.05) in the patients under 11 years of age. Differences (p< 0.05) were found in the dysphagia-associated perinatal factors in the various age groups, with longer ventilation assistance times, parenteral nutrition, and hospital stays. CONCLUSIONS: Dysphagia is an extremely frequent symptom at any given age in patients undergoing esophageal atresia surgery. A standardized, cross-disciplinary follow-up is key to improve quality of life.

INTRODUCCION: La disfagia se define como dificultad en el proceso de alimentación. Hasta un 84% de pacientes intervenidos de atresia de esófago tienen disfagia más allá del periodo neonatal. MATERIAL Y METODOS: Estudio retrospectivo de serie de casos intervenidos por atresia de esófago 2005-2021. Se utilizó la escala FOIS (Functional Oral Intake Scale) para cuantificar la disfagia en 4 grupos de edad (menores de 1 año, 1-4 años, 5-11 años y mayores de 11 años). Se consideró disfagia cualquier valor de FOIS < 7 o síntomas de atragantamiento, impactación o aversión alimentaria. RESULTADOS: Se obtuvieron datos de 63 pacientes. El 74% (47/63) presentó disfagia durante el seguimiento. La prevalencia fue del 50% < 1 año (media FOIS 4.32), 77% 1-4 años (media FOIS 5.61), 45% 5-11 años (media FOIS 5.87) y 38% > 11 años (media FOIS 6.8). Las causas más frecuentes de disfagia fueron la estenosis, que presentó un 38% de los pacientes (n= 24) y el reflujo gastroesofágico (n= 18), que presentó a su vez un 28% de los pacientes. Ambas condiciones se asociaron con unos valores medios de FOIS significativamente menores (p< 0,05) en los pacientes menores de 11 años. Se encontraron diferencias (p< 0,05) en factores perinatales asociados a disfagia en los distintos periodos de edad, a destacar mayor tiempo medio de: asistencia ventilatoria, nutrición parenteral e ingreso hospitalario. CONCLUSIONES: La disfagia es un síntoma extremadamente frecuente a cualquier edad en los pacientes intervenidos de atresia de esófago. Un seguimiento estandarizado y multidisciplinar es esencial para mejorar la calidad de vida de estos pacientes.

Surgical neonates in a low-resource setting: Baseline nutrition and outcome assessment.

INTRODUCTION: Congenital anomalies necessitating prolonged fasting have a high mortality in low-income settings, partially due to malnutrition and electrolyte disturbances in the absence of parenteral nutrition (PN). Interventions to address these problems require an accurate baseline quantification of the morbidity and mortality of this population. This prospective study aimed to determine peri-operative morbidity, fluid and electrolyte disturbance, growth, and mortality in neonates with gastroschisis (GS), intestinal atresia (IA), and esophageal atresia (EA) in Uganda. METHODS: Standardized patient care of 45 neonates treated in Uganda from Oct 2021 to March 2022 with protocolized fluid and nutrition, vital signs, and routine laboratory measurements. Patient demographics, admission and hospital characteristics are described with mean ± SD. Characteristics of survivors and non-survivors were compared with Fischer's exact tests, logrank tests, and CoX Ph model. RESULTS: Twenty-eight (62.2%) patients had GS, 4 (8.9%) EA, and 13 (28.9%) IA. Thirty-six percent (16/44) of patients survived to discharge (26% GS, 50% EA, 54% IA) with an average length of stay of 17.3 days ( ± 2.2) (survivors) and 9 days ( ± 1.7) (non-survivors). Average weight was 2.21 kg ( ± 0.62) at presentation, with no significant weight change during the study. Abnormal serum sodium in 64%, phosphate 36.5%, and magnesium 20.8% of measurements. Mortality did not correlate with diagnosis (p = 0.47), electrolyte derangement, or weight change. CONCLUSION: Mortality of neonates born with GS, EA, and IA is high in Uganda. Malnutrition and fluid/electrolyte derangements are common and may affect mortality. This study provides a comparison group for studying interventions to improve outcomes for these populations. TYPE OF STUDY: Prospective cohort. LEVEL OF EVIDENCE: Level 3.

Gastric Mucosal Viability Assessment Using 99mTcO4 in a Case of Pure Esophageal Atresia After Isoperistaltic Gastric Tube Replacement.

ABSTRACT: Gastric surface mucosal cells are responsible for the uptake and secretion of 99mTcO4, a feature that has been used in imaging heterotopic gastric mucosa. We used the same principle to look for gastric mucosal viability in this case of pure esophageal atresia admitted for cervical stomal closure after a previous isoperistaltic gastric tube (IGT) replacement procedure. 99mTcO4 scintigraphy was done after encountering a failure to maneuver the esophagoscope through the IGT. The study was helpful in assessing the loss of gastric mucosal viability in an intervening segment of the IGT.

Assessment of MRI and Ultrasound Screening for Tethered Cord Syndrome in Patients Diagnosed With Esophageal Atresia/Tracheoesophageal Fistula.

INTRODUCTION: Infants with esophageal atresia and/or tracheoesophageal fistula (EA/TEF) undergo screening for tethered cord syndrome (TCS) via ultrasound and magnetic resonance imaging. Existing literature lacks data to guide optimal timing of screening and magnetic resonance imaging (MRI) is often delayed until 3-6 mo of age, when it is frequently forgotten. Detethering surgery has a high rate of success in patients with TCS and is often performed prophylactically due to potential irreversible deficits. This study aims to improve screening procedure for infants with EA/TEF. METHODS: A retrospective chart review was done of all EA/TEF patients treated over 6 y (n = 79). The study examined how often each imaging modality was performed and identified a TCS lesion, as well as age of screening/surgical intervention. RESULTS: Screening for TCS was done with MRI 58% of the time and US 15% of the time. However, 38% of patients did not undergo any screening. Out of the patients with TCS on MRI (n = 19, 41.3%), 73.7% had neurosurgery. Of patients who underwent ultrasound (US) (n = 12), nine patients also had MRI later: two reported TCS lesions and subsequently had neurosurgery. Surgical infection rates and complications were 0/14. CONCLUSIONS: MRI demonstrated a higher rate of detecting TCS lesions than US, and patients with TCS frequently had detethering. Patients with ≥3 VACTERL or vertebral anomalies had a higher incidence of TCS on MRI. Patients with vertebral anomalies reported false negative ultrasounds in two cases, suggesting the potential superiority of MRI screening in this subgroup. A third of children did not undergo any imaging and this will require a process improvement.

Functional, nutritional, and developmental assessment of gastric transposition and colonic interposition: Long-term follow-up outcome analysis.

PURPOSE: The choice of Esophageal replacement (ER) depends on surgeons' preference and patients' anatomical condition. A cross-sectional study was done to compare the long-term outcomes of two methods of ER, Gastric transposition (GT) and Colonic interposition (CI). METHODS: Children who had undergone ER from January 1997 to December 2017 with a minimum of two-year post-ER follow-up were evaluated by anthropometry, hepatobiliary scintigraphy, gastroesophageal reflux study, gastric emptying test, pulmonary function test and blood tests. RESULTS: Twenty-six (Male:female=17:9) children were recruited. The median age at ER was 13 months (interquartile range 9-40 months) and mean follow-up post-ER was 116.7 ± 76.4 months (range 24-247 months). GT:CI was done in 15(57.7%):11(42.3%) cases. A greater number of abnormal oral contrast studies (p = 0.02) and re-operations (p = 0.05) were documented as baseline characteristics with CI group. The presence of gastroesophageal reflux 9/23(39.1%), duodenogastric reflux 6/24(25%), delayed gastric emptying 6/25(24%), abnormal pulmonary function test 14/22(63.6%) were documented during the study period. However, there was no significant(p>0.05) difference in nutritional, developmental and functional outcomes of both operative methods of ER in the study. CONCLUSION: Assessment of nutritional, developmental and functional parameters in children after ER reveals good long-term results. There was no significant difference in CI and GT. LEVEL OF EVIDENCE: Comparative study; II.

Radiographic assessment of traction-induced esophageal growth and traction-related complications of the Foker process for treatment of long-gap esophageal atresia.

BACKGROUND: Radiographic assessment of esophageal growth in long-gap esophageal atresia while on traction and associated traction-related complications have not been described. OBJECTIVE: To demonstrate how chest radiography can estimate esophageal position while on traction and to evaluate radiography's utility in diagnosing certain traction system complications. MATERIALS AND METHODS: In this retrospective evaluation of portable chest radiographs obtained in infants with long-gap esophageal atresia who underwent the Foker process between 2014 and 2020, we assessed distances between the opposing trailing clips (esophageal gap) and the leading and trailing clips for each esophageal segment on serial radiographs. Growth during traction was estimated using longitudinal random-effects regression analysis to account for multiple chest radiograph measurements from the same child. RESULTS: Forty-three infants (25 male) had a median esophageal gap of 4.5 cm. Median traction time was 14 days. Median daily radiographic esophageal growth rate for both segments was 2.2 mm and median cumulative growth was 23.6 mm. Traction-related complications occurred in 13 (30%) children with median time of 8 days from traction initiation. Daily change >12% in leading-to trailing clip distance demonstrated 86% sensitivity and 92% specificity for indicating traction-related complications (area under the curve [AUC] 0.853). Cumulative change >30% in leading- to trailing-clip distance during traction was 85% sensitive and 85% specific for indicating traction complications (AUC 0.874). CONCLUSION: Portable chest radiograph measurements can serve as a quantitative surrogate for esophageal segment position in long-gap esophageal atresia. An increase of >12% between two sequential chest radiographs or >30% increase over the traction period in leading- to trailing-clip distance is highly associated with traction system complications.

The Financial Burden of Surgery for Congenital Malformations-The Austrian Perspective.

Neonatal "surgical" malformations are associated with higher costs than major "non-surgical" birth defects. We aimed to analyze the financial burden on the Austrian health system of five congenital malformations requiring timely postnatal surgery. The database of the Austrian National Public Health Institute for the period from 2002 to 2014 was reviewed. Diagnosis-related group (DRG) points assigned to hospital admissions containing five congenital malformations coded as principal diagnosis (esophageal atresia, duodenal atresia, congenital diaphragmatic hernia, gastroschisis, and omphalocele) were collected and compared to all hospitalizations for other reasons. Out of 3,518,625 total hospitalizations, there were 1664 admissions of patients with the selected malformations. The annual mean number was 128 (SD 17, range 110-175). The mean cost of the congenital malformations per hospital admission expressed in DRG points was 26,588 (range 0-465,772, SD 40,702) and was significantly higher compared to the other hospitalizations (n = 3,516,961; mean DRG 2194, range 0-834,997; SD 6161; p < 0.05). Surgical conditions requiring timely postnatal surgery place a significant financial burden on the healthcare system. The creation of a dedicated national register could allow for better planning of resource allocation, for improving the outcome of affected children, and for optimizing costs.

COMPARATIVE ASSESSMENT OF RISK-BENEFIT RATIO OF USE OF SILICONE BOUGIE VERSUS ALTERNATIVE METHODS IN POSTOPERATIVE MANAGEMENT OF ESOPHAGEAL ATRESIA.

The aim of the present research is to assess the beneficial and adverse effects of silicone bougie use in comparison to alternative methods of post-operative management of esophageal atresia. The study was carried on 28 patients treated at our healthcare institution for esophageal atresia during the period from May, 2017 to September, 2020. As an alternative to the use of esophageal bougie these patients were managed postoperatively, by utilizing the contrast imaging of the esophagus on the 7th and 14th days after surgery, and at the age of 1, 3, 6 months and one year. Unless the esophageal stricture has developed, the procedures are repeated annually until the age of 5. The aim of utilizing the contrast imaging technique is to prevent the stricture of the esophagus and recurrence of trachea esophageal fistula.

Dynamic esophageal patency assessment: an effective method for prenatally diagnosing esophageal atresia.

BACKGROUND: Esophageal atresia is a major anomaly with a low prenatal detection rate. We propose a sonographic method termed dynamic esophageal patency assessment. OBJECTIVE: This study aimed to assess the feasibility and performance of the dynamic esophageal patency assessment in a high-risk population. STUDY DESIGN: A prospective study was conducted in a single tertiary fetal ultrasound unit for 12 months. The study group included pregnant women referred for a targeted scan because of one or more of the following: (1) polyhydramnios; (2) small or absent stomach; (3) vertebral, anal atresia, cardiac, tracheoesophageal fistula, renal, and limb abnormalities; (4) first-degree relative with esophageal atresia; and (5) genetic mutation associated with esophageal atresia. In addition to dynamic esophageal patency assessment, a comprehensive anomaly scan was carried out. The fetal esophagus was observed during swallowing. Cases that demonstrated uninterrupted fluid propagation through the esophagus were classified as normal. Cases that demonstrated interrupted fluid propagation, with the formation of a pouch, were classified as abnormal. Cases with unclear visualization of the esophagus or cases that failed to demonstrate either fluid propagation or a pouch were classified as undetermined. Dynamic esophageal patency assessment results were compared with postnatal findings, considered "gold standard." Test performance indices and intra- and interobserver agreements were calculated. RESULTS: For 12 months, 130 patients were recruited, and 132 fetuses were scanned. The median gestational age (interquartile range) at the time of scan was 31.4 weeks (29.0-35.3). Of 132 fetuses enrolled, 123 (93.2%) were normal, 8 (6%) were abnormal, and 1 (0.8%) was undetermined. Excluded from test performance analysis were 3 cases that were terminated without postmortem autopsy (1 was abnormal and 2 were normal), and a fourth case was excluded as it was classified as undetermined. The detection rate of esophageal atresia was 100%, with no false-positive or false-negative case. Sensitivity, specificity, and positive and negative predictive values of the dynamic esophageal patency assessment were 100%. The Kappa coefficient was 1 for both inter- and intraobserver agreements (P<.0001). The median time (interquartile range) required to complete the dynamic esophageal patency assessment was 6.00 minutes (3.00-13.25). CONCLUSION: The dynamic esophageal patency assessment is a feasible and highly effective method of ascertaining an intact esophagus and detecting esophageal atresia in suspected cases.

Validation of low-cost models for minimal invasive surgery training of congenital diaphragmatic hernia and esophageal atresia.

BACKGROUND: Minimal invasive surgery (MIS) is increasingly used for the correction of congenital diaphragmatic hernia (CDH) and esophageal atresia (EA). It is important to master these complex procedures, preferably preclinically, to avoid complications. The aim of this study was to validate recently developed models to train these MIS procedures preclinically. METHODS: Two low cost, reproducible models (one for CDH and one for EA) were validated during several pediatric surgical conferences and training sessions (January 2017-December 2018), used in either the LaparoscopyBoxx or EoSim simulator. Participants used one or both models and completed a questionnaire regarding their opinion on realism (face validity) and didactic value (content validity), rated on a five-point-Likert scale. RESULTS: Of all 60 participants enrolled, 44 evaluated the EA model. All items were evaluated as significantly better than neutral, with means ranging from 3.7 to 4.1 (p < 0.001). The CDH model was evaluated by 48 participants. All items scored significantly better than neutral (means 3.5-3.9, p < 0.001), with exception of the haptics of the simulated diaphragm (mean 3.3, p = 0.054). Both models were considered a potent training tool (means 3.9). CONCLUSION: These readily available and low budget models are considered a valid and potent training tool by both experts and target group participants. TYPE OF STUDY: Prospective study. LEVEL OF EVIDENCE: Level II.

"The Hopkins Mongol Case": The Dawn of the Bioethics Movement.

One of the earliest controversies in the modern history of bioethics was known at the time as "the Hopkins Mongol case," involving an infant with Trisomy 21 and duodenal atresia whose parents declined to consent to surgery. Fluids and feeding were withheld, and the infant died of dehydration after 15 days. The child's short life had a profound impact on the author's career and that of several others and ultimately led to changes in the care of children and adults with disabilities and the way difficult end-of-life decisions are made in US hospitals today. It also contributed to the growth of the modern bioethics movement and scholarship focused on pediatric bioethics issues.

Using multidimensional scaling in model choice for congenital oesophageal atresia: similarity analysis of human autopsy organ weights with those from a comparative assessment of Aachen Minipig and Pietrain piglets.

Swine models had been popular in paediatric oesophageal surgery in the past. Although being largely replaced by rodent models, swine experienced a revival with the establishment of minipig models. However, none of them has ever been investigated for similarity to humans. We conducted a pilot study to determine whether three-week old Pietrain piglets and three-month old Aachen Minipigs are suitable for experimental paediatric oesophageal atresia surgery. We tested the operation's feasibility, performed a necropsy, weighed organs, measured organ length and calculated relative weights and lengths, and measured laboratory parameters. We used multidimensional scaling to assess the similarity of the swine breeds with previously published human data. Pietrain piglets had a higher a priori bodyweight than Aachen Minipigs (Δ = 1.31 kg, 95% confidence interval (CI): 0.37-2.23, p = 0.015), while snout-to-tail length was similar. Pietrain piglets had higher absolute and relative oesophageal lengths (Δ = 5.43 cm, 95% CI: 2.2-8.6; p = 0.0062, q1* = 0.0083 and Δ = 11.4%, 95% CI: 5.1-17.6; p = 0.0025, q3* = 0.0053). Likewise, absolute and relative small intestinal lengths were higher in Pietrains, but all other parameters did not differ, with the exception of minor differences in laboratory parameters. Multidimensional scaling revealed three-week old Pietrain piglets to be similar to two-month old humans based on their thoracoabdominal organ weights. This result indicates three-week old Pietrain piglets are a suitable model of paediatric oesophageal atresia surgery, because clinically many procedures are performed at around eight weeks age. Three-month old Aachen Minipigs were more dissimilar to eight-week old humans than three-week old Pietrain piglets.

Ultrashort Echo-Time MRI for the Assessment of Tracheomalacia in Neonates.

BACKGROUND: Bronchoscopy is the gold standard for evaluating tracheomalacia; however, reliance on an invasive procedure limits understanding of normal airway dynamics. Self-gated ultrashort echo-time MRI (UTE MRI) can assess tracheal dynamics but has not been rigorously evaluated. METHODS: This study was a validation of UTE MRI diagnosis of tracheomalacia in neonates using bronchoscopy as the gold standard. Bronchoscopies were reviewed for the severity and location of tracheomalacia based on standardized criteria. The percent change in cross-sectional area (CSA) of the trachea between end-inspiration and end-expiration was determined by UTE MRI, and receiver-operating curves were used to determine the optimal cutoff values to predict tracheomalacia and determine positive and negative predictive values. RESULTS: Airway segments with tracheomalacia based on bronchoscopy had a more than threefold change in CSA measured from UTE MRI (54.4 ± 56.1% vs 14.8 ± 19.5%; P < .0001). UTE MRI correlated moderately with bronchoscopy for tracheomalacia severity (ρ = 0.39; P = .0001). Receiver-operating curves, however, showed very good ability of UTE MRI to identify tracheomalacia (area under the curve, 0.78). A "loose" definition (> 20% change in CSA) of tracheomalacia had good sensitivity (80%) but low specificity (64%) for identifying tracheomalacia based on UTE MRI, whereas a "strict" definition (> 40% change in CSA) was poorly sensitive (48%) but highly specific (93%). CONCLUSIONS: Self-gated UTE MRI can noninvasively assess tracheomalacia in neonates without sedation, ionizing radiation, or increased risk. This technique overcomes major limitations of other diagnostic modalities and may be suitable for longitudinal population studies of tracheal dynamics.

Assessment of the Concerns of Caregivers of Children with Repaired Esophageal Atresia-Tracheoesophageal Fistula Related to Feeding-Swallowing Difficulties.

The study aimed to assess concerns of caregivers of children with EA-TEF related to feeding-swallowing difficulties, compare the concerns according to type of atresia and repair time, and investigate its relationship with time to start oral feeding. Caregivers accompanying 64 children with EA-TEF were included. Age, sex, type of atresia, repair time, and time to start oral feeding were noted. Parents completed the Turkish version of the Feeding/Swallowing Impact Survey (T-FS-IS) to assess the concerns of caregivers related to feeding-swallowing difficulties. The T-FS-IS has three subscales including daily activities, worry, and feeding difficulties. The median age of patients was 3 (min = 1, max = 12) years, of which 57.8% were male. 43.8% of cases were isolated-EA, and 56.3% were EA-distal TEF. 57.8% of cases received early repair, and 42.2% had delayed repair. The median time to start oral feeding was 4 weeks (min = 1, max = 128). The mean scores of daily activities, worry, feeding difficulties, and total score from the T-FS-IS were 2.43 ± 1.18, 2.73 ± 1.28, 2.10 ± 0.97, and 2.44 ± 1.09, respectively. Caregivers of children with isolated-EA reported more problems in total score and all subscales of the T-FS-IS than EA-distal TEF (p < 0.01). Caregivers of children who received delayed repair reported more problems in total score and all subscales of the T-FS-IS than children with early repair (p < 0.05). Moderate to strong correlations were found between the T-FS-IS and time to start oral feeding (p < 0.01, r = 0.55-0.65). This study suggests that caregivers of children with isolated-EA and/or delayed repair and/or delay in oral intake may have higher concerns related to feeding-swallowing difficulties.

Quality of life assessment in esophageal atresia patients: a systematic review focusing on long-gap esophageal atresia.

BACKGROUND: Children born with esophageal atresia (EA) have inherent abnormalities in esophageal motility which may impact upon patient and family Quality of Life (QoL). Currently, paucity of data exists for long-term outcomes of long-gap EA. We aimed to: (1) summarize QoL tools reported in the literature, focusing upon studies involving long-gap EA patients, and (2) compare QoL for long-gap versus non-long-gap EA patients. METHOD: We performed a systematic review of Cochrane Register of Controlled Trials, PubMed, EMBASE, and Ovid databases (January 1980-May 2018) in accordance with the PRISMA protocol. RESULT: Six studies were identified (536 patients total), and 419/536 (78%) patients completed QoL assessment. Response rates ranged from 29% to 100%. Median study size was 86 (range 8-159). Esophageal atresia type was described in 477 patients, and 74/477 (16%) were long-gap. Common assessment tools were Gastrointestinal Quality of Life Index and 36-Item Short-Form Health Survey. Compared with healthy individuals, long-gap EA patients suffered more gastrointestinal symptoms. There were no significant differences in QoL outcomes between long-gap and non-long-gap EA patients. CONCLUSION: Current literature suggests no significant difference in QoL outcomes between long-gap and non-long-gap EA patients. However, due to questionnaire variability and range of response rates, the data should be interpreted with care. LEVEL OF EVIDENCE: Level II.

Neonatal surgery in low- vs. high-volume institutions: a KID inpatient database outcomes and cost study after repair of congenital diaphragmatic hernia, esophageal atresia, and gastroschisis.

BACKGROUND/PURPOSE: The volume-outcome relationship and optimal surgical volumes for repair of congenital anomalies in neonates is unknown. METHODS: A retrospective study of infants who underwent diaphragmatic hernia (CDH), gastroschisis (GS), and esophageal atresia/tracheoesophageal fistula (EA/TEF) repair at US hospitals using the Kids' Inpatient Database 2009-2012. Distribution of institutional volumes was calculated. Multi-level logistic/linear regressions were used to determine the association between volume and mortality, length of stay, and costs. RESULTS: Total surgical volumes were 1186 for CDH, 1280 for EA/TEF, and 3372 for GS. Median case volume per institution was three for CDH and EA/TEF, and four for GS. Hospitals with annual case volumes ≥ 75th percentile were considered high volume. Approximately, half of all surgeries were performed at low-volume hospitals. No clinically meaningful association between volume and outcomes was found for any procedure. Median cost was greater at high- vs. low-volume hospitals [CDH: $165,964 (p < 0.0001) vs. $104,107, EA/TEF: $85,791 vs. $67,487 (p < 0.006), GS: $83,156 vs. $72,710 (p < 0.0009)]. CONCLUSIONS: An association between volume and outcome was not identified in this study using robust outcome measures. The cost of care was higher in high-volume institutions compared to low-volume institutions. LEVEL OF EVIDENCE: III.

Management of esophageal atresia and early predictive factors of mortality and morbidity in a developing country.

The aim of this study is to investigate management and outcome in esophageal atresia (EA) and to identify early predictive factors of morbidity and mortality in a developing country. Charts of neonates with repaired EA from 2007 to 2016 were reviewed. Patients' characteristics, operative details, and postoperative outcomes were collected. Statistical analyses were performed to identify predictors of complicated evolution. Forty-two cases were collected. There were 14 girls and 28 boys. Only one patient had antenatal diagnosis (2.3%). The mean gestational age was 38 weeks. Nine patients (21.4%) weighed less than 2.5 kg. Seventeen (40.4%) patients had associated malformations most commonly cardiac (9/17). Thirteen patients had delayed diagnosis (30.9%). Thirty-nine (92.8%) patients underwent primary esophageal anastomosis. Overall survival was 76.2%. Nineteen patients (57% of survivals) had complicated evolution before the age of one year and 15 patients (46.8% of survivals) developed complications after the age of one year. Perinatal variables associated with mortality were prematurity (p = 0.004, OR = 5.4, IC95% = [1.13-25.80]), low birth weight (p = 0.023, OR = 7, IC95% = [1.38-35.47]), cardiac malformations (p = 0.006, OR = 10.5, IC95% = [2.03-54.27]) and delayed diagnosis (p = 0.005, OR = 10.11, IC95% = [2.005-50.980]). Variables associated with short-term and middle-term complications were duration of intubation (p = 0.019, OR = 0.118, IC95% = [0.019-0.713]) and the presence of short-term complications (p = 0.016, OR = 7.33, IC95% = [1.467-36.664]) respectively. These factors may be used to identify patients who will benefit from more intensive follow-up program.

Esophageal atresia, Europe, and the future: BAPS Journal of Pediatric Surgery Lecture.

Europe has changed remarkably over the past decades and so have concepts and outcomes of esophageal atresia repair. In this article, both the efforts to create a united Europe and the achievements in dealing with esophageal atresia from the 1950s on are outlined. Furthermore, this paper deals with the future of pediatric surgery and is focused on two aspects: the "Fourth Industrial Revolution" which builds on the digital revolution, artificial intelligence and robotics, and its potential impact on pediatric surgery and the life of patients. I suggest that pediatric surgeons should participate and lead in the development of machine learning, data control, assuring appropriate use of machines, control misuse, and in particular ensure appropriate maintenance of ethical standards. Changes in health care structures within Europe, in particular the effect of centralization, will affect the concept of treatment for patients with rare diseases.

The assessment of the esophageal motility of children with esophageal disorders by the detailed observation of the pH-multichannel intraluminal impedance waveform and baseline impedance: screening test potential.

BACKGROUND: The present study aimed to evaluate whether the detailed observation of pH/MII waveforms and the analysis of baseline impedance (BI) values could detect esophageal dysmotility in pediatric patients with esophageal disorders. PATIENTS AND METHODS: Eleven patients with congenital esophageal disorder in whom pH/MII was conducted from April 2011 to June 2015, were enrolled in this study. The diagnoses of the patients were as follows: postoperative esophageal atresia (EA), n = 6; esophageal achalasia (EAch), n = 4; and congenital esophageal stenosis (CES), n = 1. The characteristics of the pH/MII waveform, pathological GERD, BI value, and the average BI value of the 2 distal channels (distal BI; DBI) were analyzed in each disorder. RESULTS: Two EA (33%) patients and one EAch (25%) patient were diagnosed with GERD. The mean DBI values of the EA, EAch and CES patients was 912 ± 550, 2153 ± 915 and 1392 Ω, respectively. The EA patients showed consistently low DBI values. One CES patient and two infantile EAch patients showed postprandial prolonged low DBI values. Whereas, the pH/MII waveforms of the adolescent EAch patients were difficult to interpret due to their extremely low BI values. CONCLUSIONS: The present study demonstrated that the detailed observation of the pH/MII waveforms in all channels and the analysis of BI were useful for evaluating esophageal motility in children with congenital esophageal disorders. In particular, infantile patients with EAch showed DBI findings that were distinct from those of adult EAch patients. Considering the difficulty of performing esophageal manometry in young children, the detailed observation of the pH/MII waveform may help in the diagnosis of esophageal dysmotility in children.

Utilizing stricture indices to predict dilation of strictures after esophageal atresia repair.

BACKGROUND: Anastomotic stricture is the most common postoperative complication in infants undergoing repair of esophageal atresia with or without tracheoesophageal fistula (EA/TEF). Stricture indices (SIs) are used to predict infants at risk for stricture requiring dilation. We sought to determine the most accurate SI and optimal timing for predicting anastomotic dilation. MATERIALS AND METHODS: A retrospective study of infants undergoing repair of EA/TEF between 2008 and 2013 was performed. Esophagrams were used to calculate four SIs (upper pouch esophageal anastomotic stricture index [U-EASI], lower pouch esophageal anastomotic stricture index [L-EASI], lateral SI, and anterior/posterior SI). The best performing SI was identified. Logistic regression analysis was performed to determine if a first or second esophagram SI threshold was associated with dilation. A receiver operating characteristic curve measured the accuracy of the model using SIs to predict dilation. RESULTS: Of 45 EA/TEF infants included, 20 (44%) had postoperative strictures requiring dilation. As the best performing SI, logistic regression analysis showed that U-EASI as a continuous variable was predictive of dilation (P = 0.03) but was not significant at U-EASI ≤ 0.37. However, U-EASI ≤ 0.37 was associated with needing earlier dilation. On second esophagram (median, 38 days), U-EASI of ≤0.39 was significantly associated with dilation (OR: 7.8, 95% CI: 1.05-57.58, P = 0.04). The area under the receiver operating characteristic curve of the U-EASI model controlling for days to esophagram demonstrated improved predictive ability from first (AUC 0.73) to second esophagram (AUC 0.81). CONCLUSIONS: Calculation of the SI utilizing a U-EASI ≤ 0.39 on the delayed esophagram is associated with future anastomotic dilation. A multi-institutional study is necessary to confirm the predictive ability of the U-EASI.