A 3D printed esophageal atresia-tracheoesophageal fistula thorascopy simulator for young surgeons.

We developed a 3D-printed thoracoscopic surgery simulator for esophageal atresia with tracheoesophageal fistula (EA-TEF) and assessed its effectiveness in educating young pediatric surgeons. Prototype production and modifications were repeated five times before producing the 3-D printed final product based on a patient's preoperative chest computed tomography. A 24-item survey was used to rate the simulator, adapted from a previous report, with 16 young surgeons with an average of 6.2 years of experience in pediatric surgery for validation. Reusable parts of the thoracic cage were printed to combine with replaceable parts. Each structure was fabricated using diverse printing materials, and subsequently affixed to a frame. In evaluating the simulator, the scores for each factor were 4.33, 4.33, 4.27, 4.31, 4.63, and 4.75 out of 5, respectively, with the highest ratings in value and relevance. The global rating was 3.38 out of 4, with ten stating that it could be used with slight improvements. The most common comment from participants was that the esophageal anastomosis was close to the actual EA-TEF surgery. The 3D-printed thoracoscopic EA-TEF surgery simulator was developed and reflected the actual surgical environment. It could become an effective method of training young pediatric surgeons.

Imaging Features of Neonatal Bowel Obstruction.

Radiologic evaluation of neonatal bowel obstruction is challenging owing to the overlapping clinical features and imaging appearances of the most common differential diagnoses. The key to providing an appropriate differential diagnosis comes from a combination of the patient's gestational age, clinical features, and imaging findings. While assessment of radiographs can confirm bowel obstruction and indicate whether it is likely proximal or distal, additional findings at upper or lower gastrointestinal contrast study together with use of US are important in providing an appropriate differential diagnosis. The authors provide an in-depth assessment of the appearances of the most common differential diagnoses of proximal and distal neonatal bowel obstruction at abdominal radiography and upper and lower gastrointestinal contrast studies. These are divided into imaging patterns and their associated differential diagnoses on the basis of abdominal radiographic findings. These findings include esophageal atresia variants including the "single bubble," "double bubble," and "triple bubble" and distal bowel obstruction involving the small and large bowel. Entities discussed include esophageal atresia, hypertrophic pyloric stenosis, pyloric atresia, duodenal atresia, duodenal web, malrotation with midgut volvulus, jejunal atresia, ileal atresia, meconium ileus, segmental volvulus, internal hernia, colonic atresia, Hirschsprung disease, and functional immaturity of the large bowel. The authors include the advantages of abdominal US in this algorithm, particularly for hypertrophic pyloric stenosis, duodenal web, malrotation with midgut volvulus, and segmental volvulus. ©RSNA, 2023 Online supplemental material is available for this article. Quiz questions for this article are available through the Online Learning Center.

Usefulness of routine early œsophagogram after primary repair of œsophageal atresia: a multicenter study.

BACKGROUND: Advances in surgical and neonatal care have led to improved survival of patients with œsophageal atresia (OA) over time. Morbidity remains significant, with one-third of patients being affected by a postoperative complication. Several aspects of management are not consensual, such as the use of œsophagogram before starting oral feeding. METHODS: We conducted a multicenter retrospective study, including all children with OA that underwent a primary anastomosis in the first days of life, between 2012 and 2018 in five French centers, to determine the usefulness of postoperative œsophagogram during the 10 days after early primary repair of OA to diagnose the anastomotic leak and congenital œsophageal stenosis. RESULTS: Among 225 included children, 90 (40%) had a routine œsophagogram and 25 (11%) had an anastomotic leak, clinically diagnosed before the scheduled œsophagogram in 24/25 (96%) children at median postoperative day 4. Ten patients had associated congenital œsophageal stenosis diagnosed on the œsophagogram in only 30% of cases. CONCLUSION: Early œsophagogram is rarely useful in the diagnosis of an anastomotic leak, which is clinically diagnosed before performing an œsophagogram in the majority of cases. The need for a postoperative œsophagogram should be evaluated on a case-by-case basis. IMPACT: Early œsophagogram is not helpful in the diagnosis of an anastomotic leak in the majority of cases. An anastomotic leak is most often diagnosed clinically before performing an œsophagogram. Early postoperative œsophagogram could be helpful for the diagnosis of congenital œsophageal stenosis. However, dysphagia occurs later and early diagnosis of congenital œsophageal stenosis has no impact on the management and outcome of asymptomatic children. Indication of postoperative œsophagogram has to be evaluated on a case-by-case basis.

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.

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.

Management of Congenital and Postoperative Chylothorax: Use of Thoracoscopic Lymphatic Leak Ligations with Intraoperative ICG Lymphangiography.

BACKGROUND: Congenital chylothorax (CCT) and postoperative chylothorax (POCT) are rare and difficult to treat. We report our treatment strategy and outcomes for chylothorax, including thoracoscopic surgery with indocyanine-green (ICG) near-infrared fluorescence lymphangiography. METHODS: A retrospective review of patients with CCT and POCT from 2014 to 2021 was performed. After definitive diagnosis, conservative treatments with octreotide, followed by intravenous steroids as needed, were performed. Patients who were refractory to conservative treatment were transferred to surgical treatment, consisting of thoracoscopic lymphatic leak ligations using ICG intraoperative lymphangiography. The effectiveness of conservative and surgical treatment was then examined. RESULTS: We included 19 cases of CCT and 31 cases of POCT. The 31 POCT patients included 23 of 84 postoperative patients with congenital diaphragmatic hernia (CDH), 7 of 54 postoperative patients with esophageal atresia (EA), and 1 of 3 postoperative patients with lymphatic malformation. The efficacy of conservative treatment was 12/19 for CCT, 22/23 for CDH, and 4/7 for EA. Surgical intervention was performed in 10 patients, and the rate of resolution of chylothorax within 3 weeks after surgery was 90%. CONCLUSION: Thoracoscopic lymphatic leak ligations with intraoperative ICG lymphangiography are feasible and useful in patients with chylothorax refractory to conservative treatment. LEVEL OF EVIDENCE: Level IV.

Phenotypic spectrum of patients with Poretti-Boltshauser syndrome: Patient report of antenatal ventriculomegaly and esophageal atresia.

Poretti-Boltshauser syndrome (PTBHS) is an autosomal recessive disorder characterized by cerebellar dysplasia with cysts and an abnormal shape of the fourth ventricle on neuroimaging, due to pathogenic variants in the LAMA1 gene. The clinical spectrum mainly consists of neurological and ophthalmological manifestations, including non-progressive cerebellar ataxia, oculomotor apraxia, language impairment, intellectual disability, high myopia, abnormal eye movements and retinal dystrophy. We report a patient presenting with ventriculomegaly on antenatal neuroimaging and a neonatal diagnosis of Type III esophageal atresia. She subsequently developed severe myopia and strabismus with retinal dystrophy, mild developmental delay, and cerebellar dysplasia. Genetic investigations confirmed PTBHS. This report confirms previous reports of antenatal ventriculomegaly in PTBHS patients and documents a so far unreported occurrence of esophageal atresia in PTBHS. We additionally gathered phenotype and genotype descriptions of published cases in an effort to better define the spectrum of PTBHS.

Application value of high-frequency ultrasound combined with ultrasonography in the diagnosis of neonatal esophageal atresia.

Objective: To explore the application value of high-frequency ultrasound combined with ultrasonography in the diagnosis of neonatal esophageal atresia (EA). Methods: Seventy neonates with suspected EA who received healing in our hospital from August 2019 to April 2022 were retrospectively selected as the study subjects and their preoperative esophageal high-frequency ultrasound and ultrasound hydrography data were analysed. The diagnostic value of high-frequency ultrasound, ultrasound hydrography and combined detection in neonatal EA was analysed using intraoperative findings as the gold standard. Results: (1) Among the 70 children with suspected EA, 62 were confirmed to be positive and 8 were negative; 59 were positive and 11 were negative by ultrasound hydrography alone; 61 were positive and 9 were negative by high-frequency ultrasound alone; 62 were positive and 8 were negative by combined detection.(2) The accuracy of combined detection was 97.14%, which was notably different from 92.86% by high-frequency ultrasound and 84.29% by ultrasound hydrography (P < 0.05).(3) The diagnostic AUC of ultrasound hydrography, high-frequency ultrasound, and combined detection for EA was 0.6125, 0.6500, and 0.6563, respectively (P < 0.05).(4) There was no significant variation in the distance between preoperative high-frequency ultrasound, ultrasound hydrography, and intraoperative measurements of distal and proximal blind ends of type IIIA and IIIB EA esophagus (P > 0.05). Conclusion: High frequency ultrasound and super fresh water injection angiography have good application value in the diagnosis of neonatal EA. There is no significant difference between the distance between the distal and proximal blind ends of the esophagus before and during the operation of type III EA and that during the operation of super fresh water injection angiography. However, in consideration of the risk of radiation and poisoning caused by esophagography, it is recommended that high-frequency ultrasound be selected first for diagnosis and if necessary, esophagography be supplemented for joint diagnosis.

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.

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.

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.

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.

Feasibility of delayed anastomosis for long gap esophageal atresia in the neonatal period using internal traction and indocyanine green-guided near-infrared fluorescence.

INTRODUCTION: Management of neonates with long gap esophageal atresia (LGEA) is one of the most challenging situations facing pediatric surgeons. Delayed anastomosis after internal traction for esophageal lengthening was reported as a useful technique for long gap cases. Additionally, the use of near-infrared (NIR) fluorescence imaging with indocyanine green (ICG) has gained popularity in pediatric surgery, especially for blood perfusion validation. We report a novel technique for safe and secure anastomosis for LGEA in the neonatal period using internal traction and ICG-guided NIR fluorescence. PATIENT AND SURGICAL TECHNIQUE: A pregnant woman with polyhydramnios was admitted to the department of obstetrics in our hospital. At 29 weeks of gestation, ultrasound showed mild polyhydramnios and absence of the fetal stomach. A male neonate was born at 38 weeks of gestation with 21 trisomy. EA (Gross type A) was diagnosed based on an X-ray study that showed the absence of gastric bubble with a nasogastric tube showing the "coil-up" sign. Thoracoscopic internal traction and laparoscopic gastrostomy were performed on day 4 after birth. We confirmed the distance between the upper pouch and lower pouch on X-ray. On day 16 after birth, thoracoscopic anastomosis was performed. We successfully performed esophageal anastomosis without tearing the esophageal wall. Blood perfusion of the upper and lower pouch was validated after anastomosis using ICG-guided NIR fluorescence. CONCLUSION: Delayed anastomosis for LGEA in the neonatal period using internal traction and ICG-guided NIR fluorescence is safe and feasible.

The dilemma after an unforeseen aortic arch anomalies at thoracoscopic repair of esophageal atresia: Is curtailing surgery still a necessity?

BACKGROUND AND OBJECTIVE: There are several forms of relevant epi-aortic branching anomalies, and perhaps that is why different views as to the best approach have been reported. To help resolve this dilemma, we examined the unforeseen arch anomalies found at thoracoscopic repair of esophageal atresia and the outcomes. METHODS: In a retrospective cohort, all consecutive patients who were thoracoscopically approached for esophageal atresia over a 5-year period with unforeseen aortic/epi-aortic branching were identified and grouped. Thoracoscopic views, operative interventions, and outcomes were studied. RESULTS: A total of 121 neonates were thoracoscopically approached for EA, of whom 18 cases with aberrant aortic architecture were selected. Four (3%) cases were diagnosed on a preoperative echocardiography as a right-sided aortic arch, whereas unforeseen anomalous anatomies were reported in 14 cases (11.6%): left aortic arch with an aberrant right subclavian artery (ARSA) (n = 10), right-sided aortic arch with an aberrant left subclavian artery (ALSA) (n = 3), and mirror-image right arch (n = 1). Single postoperative mortality was reported among the group with left arch and ARSA (10%), whereas all the cases with right arch and ALSA died. CONCLUSIONS: In all, 11.6% of the studied series exhibited unexpected aberrant aortic architecture, with higher complication rates in comparison to the typical thoracoscopic repairs. For EA with left aortic arch and ARSA, the primary esophageal surgery could safely be completed. Meanwhile, curtailing surgery-after ligating the TEF-to get advanced imaging is still advised for both groups with the right arch due to the significant existence of vascular rings.