Magnetic Resonance Imaging-Based Evaluation of Anatomy and Outcome Prediction in Infants with Esophageal Atresia.

INTRODUCTION: There is currently no validated diagnostic modality to characterize the anatomy and predict outcomes of tracheal esophageal defects, such as esophageal atresia (EA) and tracheal esophageal fistulas (TEFs). We hypothesized that ultra-short echo-time MRI would provide enhanced anatomic information allowing for evaluation of specific EA/TEF anatomy and identification of risk factors that predict outcome in infants with EA/TEF. METHODS: In this observational study, 11 infants had pre-repair ultra-short echo-time MRI of the chest completed. Esophageal size was measured at the widest point distal to the epiglottis and proximal to the carina. Angle of tracheal deviation was measured by identifying the initial point of deviation and the farthest lateral point proximal to the carina. RESULTS: Infants without a proximal TEF had a larger proximal esophageal diameter (13.5 ± 5.1 mm vs. 6.8 ± 2.1 mm, p = 0.07) when compared to infants with a proximal TEF. The angle of tracheal deviation in infants without a proximal TEF was larger than infants with a proximal TEF (16.1 ± 6.1° vs. 8.2 ± 5.4°, p = 0.09) and controls (16.1 ± 6.1° vs. 8.0 ± 3.1°, p = 0.005). An increase in the angle of tracheal deviation was positively correlated with duration of post-operative mechanical ventilation (Pearson r = 0.83, p < 0.002) and total duration of post-operative respiratory support (Pearson r = 0.80, p = 0.004). DISCUSSION: These results demonstrate that infants without a proximal TEF have a larger proximal esophagus and a greater angle of tracheal deviation which is directly correlated with the need for longer post-operative respiratory support. Additionally, these results demonstrate that MRI is a useful tool to assess the anatomy of EA/TEF.

Spectrum of fetal limb anomalies.

PURPOSE: Antenatal detection of limb anomalies is not uncommon, and pregnancies are usually terminated in view of the expected physical handicap. The aim of this retrospective observational study is to delineate the spectrum of fetal limb anomalies and provide evidence in support of complete postnatal evaluation in establishing recurrence risk. METHODS: We present 54 cases of limb malformations detected antenatally and discuss the spectrum of abnormalities, the utility of fetal autopsy, and genetic testing to establish recurrence risk in subsequent pregnancies. RESULTS: 16/54 cases were isolated radial ray anomalies. There were five cases of amniotic band syndrome, five limb body wall complex cases, three VACTERL (vertebral defects, anal atresia, cardiac defects, tracheo-esophageal fistula, renal anomalies, and limb abnormalities) associations, one case of sirenomelia, two cases of limb pelvis hypoplasia, and one case of OEIS (Omphalocele Exstrophy Imperforate anus and spinal defects). Four fetuses with non-isolated radial ray anomaly had trisomy 18. One case with bilateral radial ray defect had a mutation in the FANC-E gene confirming fanconi anemia. Twelve cases were unclassified. CONCLUSION: Autopsy is the most important investigation in fetuses with limb anomalies. We suggest chromosomal microarray (CMA) as a first-tier test after autopsy. However, in cases of bilaterally symmetrical limb anomalies, in case of previous similarly affected child, or history of consanguinity, whole exome sequencing (WES) can be offered as the primary investigation, followed by CMA if WES is normal.

Acquired Benign Tracheoesophageal Fistula: An Alternative Tracheoplastic Technique.

We present a case of surgical management of a tracheoesophageal fistula (TEF) following prolonged intubation. After transverse tracheal division and retraction of the distal stump, direct closure of the esophageal defect and repair of the membranous tracheal defect using a synthetic bioabsorbable patch were performed, followed by interposition of muscle flap between the suture lines and tracheal reconstruction. Large TEFs, without tracheal stenosis or circumferential airway defect, associated with marked peritracheal inflammation, may be treated with this alternative tracheoplastic technique in patients deemed not suitable for tracheal resection and anastomosis.

Indocyanine green fluorescence imaging localization-assisted thoracoscopy revision surgery after repair of esophageal atresia.

Revision surgery for the complications after repair of esophageal atresia is often complex because of previous surgeries and chest infections and thus requires surgical expertise. This study describes surgical experiences with the use of indocyanine green (ICG) fluorescence imaging localization-assisted thoracoscopy during revision surgery, including recurrent tracheoesophageal fistula (rTEF) (8 cases, one of which was esophageal-pulmonary fistula) and delayed esophageal closure (1 case). We performed fistula repair and esophageal reconstruction according to the indications of ICG. The application of this method avoids the excessive trauma caused by freeing the trachea and esophagus. Contrast imaging taken one week and one month after surgery indicated no spillover of the contrast agent from the esophagus, except in 1 case. Indocyanine green fluorescence imaging localization-assisted thoracoscopy is worth promoting for revision surgery after esophageal atresia repair.

The prenatal detection of distal tracheoesophageal fistulas in fetuses diagnosed with esophageal atresia.

BACKGROUND: Esophageal atresia is a major anomaly of varying severity. The complexity of surgical correction depends on the presence of a distal fistula. OBJECTIVE: This study aimed to determine the feasibility and accuracy of prenatal ultrasound detection of the distal fistula in fetuses diagnosed with esophageal atresia. STUDY DESIGN: This was an observational study conducted at a single tertiary care center between 2019 and 2021. Included were pregnant patients carrying a fetus prenatally diagnosed with esophageal atresia that was confirmed postnatally during corrective surgery or at postmortem autopsy. During the scan, the performing investigator determined the presence or absence of a distal fistula by scanning the location of the lower esophagus during fetal breathing. Cases in which the lower esophagus was observed distending with amniotic fluid during breathing were deemed "fistula present," and the remaining cases "fistula absent." Test feasibility and performance indices, including sensitivity, specificity, and positive and negative predictive value were calculated. The offline clips and images were reviewed by 2 investigators for the assessment of interoperator agreement using Cohen's Kappa formula. RESULTS: Included were 16 fetuses with esophageal atresia scanned between 2019 and 2021. All fetuses were successfully scanned with sufficient resolution of the area of interest during at least 3 cycles of breathing. It took a median of 8.5 minutes to determine the presence or absence of a distal fistula. The feasibility of the test was 100% (16/16). The test's sensitivity, specificity, and positive and negative predictive values were 80% (95% confidence interval, 55-100), 100% (95% confidence interval, 60-100), 100% (95% confidence interval, 65-100), and 75% (95% confidence interval, 45-100), respectively. The Cohen's Kappa for interoperator agreement was calculated to be 1, P<.001, corresponding to a "perfect" level of agreement. CONCLUSION: Distal fistulas in esophageal atresia can be demonstrated prenatally by targeted scanning using appropriate technique. The method provided is feasible, reproducible, and has excellent performance indices. This novel technique and observations may improve the prenatal diagnosis and counseling of esophageal atresia.

Successful visualisation of foetal tracheo-oesophageal fistula in oesophageal atresia with prenatal ultrasonography.

This is the first report describing successful visualisation of a tracheo-oesophageal fistula (TEF) based on fluid flow on prenatal foetal ultrasonography. At 36 weeks of gestation, an anechoic tubular structure with a strong blue-coloured stream of fluid flow was observed extending from the trachea towards the gastric bubble on prenatal ultrasonography. TEF can be visualised prenatally under certain conditions.

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.

An Infant With Esophageal Atresia and Tracheoesophageal Fistula: What Does the X-Ray Reveal?

This case study presents an infant with non-specific symptoms for esophageal atresia and tracheoesophageal fistula shortly after birth. A detailed examination of the infant provides clues to the potential diagnosis while the radiograph provides confirmation. This column provides the clinician with a detailed evaluation of the x-ray findings specific to the infant with esophageal atresia with and without tracheo esophageal fistula. Additionally, a description of the different types of this congenital anomaly, embryology, pathophysiology, testing, treatment, and nursing considerations is presented.

Manejo endoscópico de la fístula traqueoesofágica recurrente con la aplicación de ácido tricloroacético, en el paciente pediátrico / Endoscopic management of recurrent tracheoesophageal fistula with trichloroacetic acid in pediatric patients

Introducción: La reparación quirúrgica de la fístula traqueoeso-fágica recurrente tiene alto riesgo de complicaciones por lo que se hanutilizado diversas técnicas endoscópicas para evitarlas. Objetivo: Conocer la utilidad de la aplicación endoscópica deácido tricloroacético para el tratamiento de la fístula traqueoesofágicarecurrente. Material y método: Estudio observacional, descriptivo y retros-pectivo, tipo de serie de casos en un hospital pediátrico de tercer nivel.Se revisaron los expedientes de pacientes con fístula traqueoesofágicarecurrente de 2015 a 2021. En todos los pacientes durante este periodose realizó cepillado y aplicación de ácido tricloroacético. Resultado: El tiempo medio de aparición de la fístula traqueoesofá-gica recurrente fue de 4,8 meses (rango 1-19,2). Dos con fístula pequeña(menor a 4 mm), tres con fístula mediana (4 mm) y dos con fístula grande(mayor a 4 mm). El número medio de sesiones para cerrar la fístula fue2,2 veces (rango 1-4). El intervalo medio entre procedimientos fue 22días (rango 14-30). El tiempo medio de seguimiento desde la confir-mación de cierre de la fistula fue de 33 meses (rango 9-72), periodo detiempo donde no se observó recurrencia del cuadro. Conclusión: El manejo endoscópico de la fístula traqueoesofágica recurrente con la aplicación de ácido tricloroacético es un procedimientoseguro y efectivo. La técnica de cepillado y aplicación de ácido triclo-roacético mejora la efectividad de éxito. La fístula mayor de 4 mm dediámetro requiere mayor número de procedimientos; sin embargo, serequiere mayor número de pacientes y mayor tiempo de seguimientopara poder aseverar esta opinión.(AU)

Introduction: Surgical repair of recurrent tracheoesophageal fistulahas a high risk of complications. Therefore, various endoscopic tech-niques have been used to avoid complications. Objective: To understand the usefulness of trichloroacetic acid en-doscopic application for the treatment of recurrent tracheoesophageal fistula. Materials and methods: An observational, descriptive, retrospec-tive, case-series-based study was carried out in a tertiary pediatric hos-pital. Records of patients with recurrent tracheoesophageal fistula from2015 to 2021 were reviewed. All patients within this period underwentbrushing and trichloroacetic acid application. Results: Mean time of recurrent tracheoesophageal fistula occur-rence was 4.8 months (range: 1-19.2). Two patients had a small fistula(less than 4 mm), three patients had a medium fistula (4 mm), and twopatients had a large fistula (more than 4 mm). Mean sessions for fistulaclosure were 2.2 (range: 1-4). Mean time between procedures was 22days (range: 14-30). Mean follow-up since fistula closure confirmationwas 33 months (range: 9-72), during which no recurrences were noted. Conclusion: Endoscopic management of recurrent transesophagealfistula with trichloroacetic acid is a safe and effective procedure. Brush-ing and trichloroacetic acid combined improve success rates. Fistulasover 4 mm in diameter require more procedures. However, a largerpatient cohort and a longer follow-up period are needed to confirm this.(AU)

Tailored endoscopic treatment of tracheo-oesophageal fistula using preoperative holographic assessment and a cardiac septal occluder.

Devices originally designed for closure of cardiac septal defects have also been proposed for the treatment of acquired tracheo-oesophageal fistula (TOF). Choosing the right occluder device to match TOF size and shape is essential for a tailored treatment. We report the successful endoscopic closure of a post-radiotherapy TOF using preprocedural CT scan with holographic three-dimensional reconstruction and an Amplatzer atrial septal device. Complete TOF sealing was achieved with resolution of respiratory symptoms, and the patient was maintaining his ability to eat at 4-month follow-up.

Detection of H-type bronchoesophageal fistula in a newborn: A case report and literature review.

RATIONALE: Congenital tracheoesophageal fistula (TEF) is a rare developmental malformation. The H subtype accounts for approximately 4% of TEFs. Unlike other TEFs, the H-type is not accompanied by esophageal atresia and has nonspecific clinical symptoms, and its specific anatomical abnormalities are not always readily apparent. Furthermore, none of the currently available diagnostic methods for H-type TEF have absolute sensitivity, resulting in misdiagnoses, and accurate diagnoses are often delayed even until adulthood; in our case, we detected a congenital bronchoesophageal fistula, which is even more rare than regular H-type TEF, through a technique that was not previously reported for newborns, involving bronchoscopy, with methylene blue injected through an esophagoscope. We believe that we have provided this kind of case first in newborns.Furthermore, because there is not one literature summarizing the clinical symptoms and the effective methods up to now, we still are not clear which detective method is more efficient or accurate, especially in newborns, so it is very necessary to summarize and compare for improving the early diagnosis of TEFs; our study makes a significant contribution to the literature because we collated previously reported cases, including the clinical features and the usefulness and success rates of major tests, which will be very helpful for the early diagnosis of TEFs. PATIENT CONCERNS: A newborn male presented with an array of nonspecific clinical symptoms from birth, leading to pneumonia and mechanical ventilation. Oral feeding led to an improvement in most but not all symptoms, which returned when oral feeding was resumed. A second round of confirmatory tests was still unable to detect the cause. DIAGNOSIS: The diagnosis of H-type bronchoesophageal fistula was established through a technique that was not previously reported for newborns, involving bronchoscopy, with methylene blue injected through an esophagoscope. INTERVENTIONS: The surgery was performed after diagnosis, and the bronchoesophageal fistula was successfully repaired. OUTCOMES: The patient was discharged on postoperative day 7, and his status was reported to be normal at a follow-up visit 8 months after surgery. LESSONS: H-type TEF is a rare congenital abnormality, and its early diagnosis is highly difficult, especially bronchoesophageal fistula. Increased oral saliva and air-filled stomachs are characteristic manifestations. Bronchoscopy combined with esophagoscopy can improve the rate of early diagnosis. A combination of tests can improve the detection rate.

H-type congenital tracheo-oesophageal fistula associated with oesophageal stenosis: anatomical variant.

The goal of this paper was to report a new variant of oesophageal atresia: an H-type congenital tracheo-oesophageal fistula associated with oesophageal segmental stenosis distal to the fistula. Although symptoms were present from birth, we did not differentiate the new anatomical variant preoperatively. The patient was treated by fistula ligation, segmental resection of the distal oesophagus and end-to-end anastomosis of the oesophagus by thoracoscopic surgery. Here we describe the clinical history and management of the newborn infant, together with diagnostic recommendations to prevent misdiagnosis in the management of this condition.

Oesophageal atresia: sonographic signs may prenatally predict surgical complexity.

OBJECTIVE: Oesophageal atresia (OA) is a major anomaly of varying severity. The complexity of surgical correction highly depends on the gap length of missing oesophagus and the presence of a distal fistula. The aim of this study was to identify antenatal sonographic findings associated with presence of a distal fistula and type of surgical repair METHODS: Prenatal medical records of neonates postnatally diagnosed with OA were reviewed. Sonographic signs of OA (small/absent stomach, polyhydramnios, oesophageal pouch) and the trimester at sign detection were recorded and compared between (1) OA with and without a distal fistula and (2) early one-step versus delayed two-step anastomosis. Multivariate analysis was performed. RESULTS: Overall, 80 cases of OA were included. Absence of a distal fistula was significantly associated with higher rates of small/absent stomach (100% vs 28.6%, P<0.0001), oesophageal pouch (100% vs 24.3%, P<0.0001) and severe polyhydramnios (66.7% vs 22.9%, P=0.006), compared with OA with a distal fistula.Cases requiring a delayed two-step repair had higher rates of small/absent stomach (84.2% vs 16.7%, P>0.0001), severe polyhydramnios (47.4% vs 16.7%, P=0.008) and oesophageal pouch (73.7% vs 18.5%, P<0.0001), compared with those corrected in an early one-step anastomosis.Multivariate logistic regression found small/absent stomach and pouch to be significantly and independently associated with a delayed two-step anastomosis. CONCLUSION: OA without a distal fistula is associated with higher rates of prenatal sonographic signs. Both small/absent stomach and a pouch are independently associated with a delayed two-step anastomosis. These findings may help improve antenatal parental counselling regarding the anticipated surgical repair.

A rare congenital esophageal anomaly mimicking an isolated esophageal atresia: Kluth Type IV1a membranous esophageal atresia.

Congenital esophageal web is a very rare type of esophageal atresia (EA) and is considered as Type IV atresia in Kluth's classification. Type IV EA is further classified into subgroups according to the location of web and the presence of tracheoesophageal fistula (TEF). Herein, we present a newborn having Type IV1a EA who was initially suspected as isolated EA and has co-existing malrotation together with the review of the literature. A newborn having congenital esophageal web was admitted with antenatal ultrasonography revealing polyhydramnios, dilated stomach, and duodenum. Abdominal X-ray revealed a gasless abdomen suggesting isolated EA. The upper gastrointestinal series confirmed complete obstruction at the lower esophagus. Bronchoscopy performed excluding TEF. Laparotomy revealed intestinal malrotation and the absence of another GI webs. Gastrotomy was performed and mucosal web at GEJ was seen by pushing nasogastric catheter. Web excision and Ladd procedure were performed. Congenital esophageal web is an extremely rare type of esophageal anomaly that can be only classified in Kluth classification. A high index of suspicion is required for diagnosis especially in newborns with the gasless abdomen and esophageal obstruction at the level of GEJ. Preoperative bronchoscopy is mandatory to exclude an associating TEF. Surgical exploration via laparotomy is beneficial for exploring additional GIS anomalies.