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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.

Infants affected by Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) may require non-invasive ventilation (NIV) in the post-operative period after elective extubation, especially if born preterm. The aim of the paper is to evaluate the role of different ventilation strategies on anastomotic complications, specifically on anastomotic leak (AL).Retrospective single Institution study, including all consecutive neonates affected by EA with or without TEF in a 5-year period study (from 2014 to 2018). Only infants with a primary anastomosis were included in the study. All infants were mechanically ventilated after surgery and electively extubated after 6-7 days. The duration of invasive ventilation was decided on a case-by-case basis after surgery, based on the pre-operative esophageal gap and intraoperative findings. The need for non-invasive ventilation (NCPAP, NIPPV, and HHHFNC) after extubation and extubation failure with the need for mechanical ventilation in the post-operative period were assessed. The primary outcome evaluated was the rate of anastomotic leak.102 EA/TEF infants were managed in the study period. Sixty-seven underwent primary anastomosis. Of these, 29 (43.3%) were born preterm. Patients who required ventilation (n = 32) had a significantly lower gestational age as well as birthweight (respectively p = 0.007 and p = 0.041). 4/67 patients had an AL after surgical repair, with no statistical differences among post-operative ventilation strategies.We found no significant differences in the rate of anastomotic leak (AL) according to post-operative ventilation strategies in neonates operated on for EA/TEF.

Feeding difficulties are widely acknowledged following oesophageal atresia/tracheo-oesophageal fistula repair. However, little is understood about the nature and severity of these difficulties. This study explored feeding in children with oesophageal atresia/tracheo-oesophageal fistula from the parent perspective.In collaboration with a patient support group, data were collected using a research-specific online discussion forum. Thematic analysis was used to code the data and identify themes.One hundred and twenty-seven parents registered for the online forum, of whom 83 (65%) provided demographic data. Seventy-four (89%) of responders were mothers, 75 (90%) were of white ethnicity, 65 (78%) were from the UK. Six key themes were identified: feeding is a traumatic experience, feeding my child is scary, feeding is isolating and filled with uncertainty, feeding outside of the home is difficult, feeding associated emotions, developing coping strategies. Parents described features of medical, nutritional, feeding skill and psychosocial dysfunction across all stages of eating/drinking development. They described how their child's feeding difficulties had an impacted their own well-being. An interactional model of feeding difficulties in OA/TOF is proposed.Exploring parent experiences provides rich data from which to expand understanding of the complex nature of feeding difficulties in OA/TOF. Feeding should be viewed as a dyadic process, occurring within a family system. Intervention for feeding difficulties should be family-centred, addressing parental anxiety, trauma and uncertainty, as well as the child's underlying medical/surgical needs to optimise outcome. Further study of clinical correlates with parental experience is required.IV (non-experimental, qualitative).

A 7-year-old girl was referred from a local hospital with a history of recurrent chest infections, chronic cough since birth and occasional wheeze. A key symptom reported by parents was the frequent passing of flatus. Her cough persisted during sleep, worsened with eating and drinking, and parents

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.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.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.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.

Thoracoscopically placed internal traction sutures (ITS) for the initial management of long gap oesophageal atresia (LGOA), not amenable to primary anastomosis, was first described in 2015. Here we describe our experience using ITS both thoracoscopically and at thoracotomy where the gap between upper and lower oesophagus is too wide for primary anastomosis.The case notes of all infants treated with ITS for oesophageal atresia (01/10/2015 to 01/12/2019) were reviewed. Gaps considered too wide for an anastomosis had ITS placed between the two pouches as described by Patkowski in 2015. All patients were gastrostomy fed. Patients returned to theatre with an expectation to complete the anastomosis or re-tighten the traction sutures.Seven patients (4 OA, 1 OA with proximal fistula, 2 OA/distal TOF) median birthweight 2.28 kg (1.2-3.6 kg) were managed using ITS. Median gap length 4.5 (3-9) vertebral bodies. ITS were placed thoracoscopically in 5 cases and at thoracotomy in 2 at median 46 days (1-120) old. In all cases, ITS was associated with significant intra-thoracic adhesions. Five patients leaked from the traction sutures. Four patients had a delayed primary anastomosis performed at thoracotomy and 3 required a cervical oesophagostomy. The median length of stay was 159 days (98-282). All patients started thoracoscopically eventually required thoracotomy.The use of ITS in our department was associated with significant complications, particularly intra-thoracic leaks and adhesions. In our hands ITS did not improve the feasibility of thoracoscopic repair for LGOA and has been abandoned by us.Level IV Case Series.

Tracheoesophageal fistula (TEF) constitutes a rare, but serious complication in laryngectomized patients, usually occurring after radiotherapy. TEF may occur spontaneously or may be due to enlargement of the TEF created for placement of a voice prosthesis. Surgical treatment of TEF can be complex, especially in the presence of a concomitant pharyngoesophageal stenosis (PES), and is associated with a high failure rate. In this article, we describe the surgical reconstruction technique for TEF associated with PES using a double skin paddle fasciocutaneous radial forearm free flap. The key points of this technique consist of correct positioning of the 2 skin paddles in order to reconstruct the anterior pharyngoesophageal wall and posterior tracheal wall, as well as de-epidermization of the intermediate part of the flap, which is then placed in the tracheoesophageal space.

To assess mid-/long-term postoperative quality of life (QOL) of esophageal atresia (EA) patients.Modified gastrointestinal quality-of-life index surveys were administered to postoperative EA patients who were at least 7 years old at evaluation to assess three topics about general lifestyle (GL), five topics about EA, and four topics about mental health (MH). For MH, caregivers were also interviewed, but separately. Subjects were divided according to age: children (7-12 years old), teenagers (13-19), and adults (20 and over) and compared according to Foker or Kimura elongation (FK) or bougienage stretching (BS).There were 22 patients evaluated. Responses for GL, EA, and MH did not differ significantly between age groups, but MH responses by caregivers for subjects who were children or teenagers scored significantly lower than responses they made themselves. For primary esophageal elongation technique (PET), age at esophagoesophagostomy was significantly higher in FK. Despite FK scoring 15.1 versus 12.4 for BS during EA evaluation, this difference was not statistically significant.Changes in QOL responses according to age were unremarkable. However, discrepancies in MH indicate that subjects felt better than their caregivers thought. PET did not appear to influence QOL.

Anastomotic stricture is the most common complication after esophageal atresia (EA) repair. We sought to determine if postoperative acid suppression is associated with reduced stricture formation.A prospective, multi-institutional cohort study of infants undergoing primary EA repair from 2016 to 2020 was performed. Landmark analysis and multivariate Cox regression were used to explore if initial duration of acid suppression was associated with stricture formation at hospital discharge (DC), 3-, 6-, and 9-months postoperatively.Of 156 patients, 79 (51%) developed strictures and 60 (76%) strictures occurred within three months following repair. Acid suppression was used in 141 patients (90%). Landmark analysis showed acid suppression was not associated with reduction in initial stricture formation at DC, 3-, 6- and 9-months, respectively (p = 0.19-0.95). Multivariate regression demonstrated use of a transanastomotic tube was significantly associated with stricture formation at DC (Hazard Ratio (HR) = 2.21 (95% CI 1.24-3.95, p0.01) and 3-months (HR 5.31, 95% CI 1.65-17.16, p0.01). There was no association between acid suppression duration and stricture formation.No association between the duration of postoperative acid suppression and anastomotic stricture was observed. Transanastomotic tube use increased the risk of anastomotic strictures at hospital discharge and 3 months after repair.

To assess the growth status of children with recurrent tracheoesophageal fistula (rTEF), and determine the possible risk factors of growth retardation (GR).The medical records of 83 patients with rTEF who underwent surgical repair were retrospectively analyzed. The patients were retrospectively divided into two groups according to whether they had GR. The clinical variables were compared between the GR and non-GR groups. Univariate and multivariable logistic regression analysis were performed to identify the risk factors for GR.Eighty-three children diagnosed with rTEF were included in this study. After a median follow-up of 31.4 (19.8, 48.7) months, GR occurred in 28 patients (33.7%). Among them, six patients with only weight for age Z score (WAZ) -2SD, five patients with only height for age Z score (HAZ) -2SD, and six patients with only BMI for age Z score (BAZ) -2SD, while seven patients with both WAZ and HAZ -2SD and four patients with both WAZ, HAZ and BAZ -2SD. Multivariate logistic regression analysis showed that birth weight, anastomotic stricture and dysphagia after rTEF repair were independent risk factors with OR of 0.325 (0.119, 0.891), 4.396 (1.451, 13.324) and 5.341 (1.153, 24.752) for GR, respectively.GR is a common complication after rTEF repair. Birth weight, anastomotic stricture and dysphagia after rTEF repair are independent risk factors affecting growth.

Social media has become a means to allow individuals affected by rare diseases to connect with others. This study aimed to characterize the use of three popular social media platforms by individuals affected by common pediatric surgery index conditions.A systematic search of Instagram, Facebook, and Twitter was performed using standardized terms for the following pediatric surgical conditions: anorectal malformation, biliary atresia, bronchopulmonary sequestration, congenital diaphragmatic hernia (CDH), congenital pulmonary airway malformation, duodenal atresia, esophageal atresia/tracheoesophageal fistula (EA/TEF), gastroschisis, Hirschsprung disease (HD), and omphalocele. Accounts active within the last year were analyzed and assessed.A total of 666 accounts were identified. Instagram was the most common platform, but accounts dedicated to support and story sharing were most common on Facebook. Biliary atresia and CDH had the largest communities identified. Support groups were most common among those dedicated to EA/TEF (43.3%, p 0.001). Most accounts were created by parents, but accounts dedicated to HD had the greatest proportion founded by patients (22.4%, p = 0.04).Social media use is common among patients and families affected by congenital surgical conditions. Certain diseases have larger communities and support networks. Knowledge about their existence may allow surgeons to direct patients towards supportive communities and resources.IV.

It is unclear if musculoskeletal deformities observed in patients with congenital diaphragmatic hernia (CDH), congenital lung lesion (CLL) and esophageal atresia/tracheoesophageal fistula (EA/TEF) are associated with the anomaly or are a result of the surgery required to treat the anomaly. This study compared the prevalence of musculoskeletal deformities for: (1) children with congenital thoracic anomalies to controls; (2) CLL to EA/TEF both repaired via thoracotomy; and (3) CLL and EA/TEF to CDH repaired via laparotomy.We performed a retrospective study of children with CLL, CDH or EA/TEF between 1990 and 2016. Date-of-birth-matched control groups were generated from a population-based dataset. International Classification of Disease codes were used to identify scoliosis and pectus anomalies. We determined Hazard ratios (HR) for cases versus controls.We included 167 cases (CDH n = 82; CLL n = 29; EA/TEF n = 56) and 1670 controls. EA/TEF had a greater risk of scoliosis (HR 5.52, 95%CI 1.49,13.73) and pectus deformities (HR 4.07, 95%CI 1.96,8.45). CDH showed more scoliosis (HR 5.03, 95%CI 1.99,12.74) but not pectus anomalies. Musculoskeletal deformities were not more common in CLL.Children born with CDH or EA/TEF, but not CLL, had more musculoskeletal deformities than controls. The inconsistent association between musculoskeletal deformities and the surgical approach suggested a congenital predisposition.

Advances in neonatal intensive care have meant improved survival of the extremely low birth weight neonate. We report the successful primary repair of a preterm neonate born with an oesophageal atresia and tracheo-oesophageal fistula with a birth weight of just 525 g, on day 1 of life, the smallest survivor so far reported in the literature, now followed up for 5 years.

Congenital isolated ‘H-type’ tracheoesophageal fistula (TOF) is a rare disorder which is difficult to diagnose. Clinical presentation is characterised by a triad consisting of paroxysmal coughing and cyanosis during feeds; recurrent chest infections and failure to thrive; and abdominal distention secondary to gaseous loading of the bowel. It is often difficult to diagnose ‘H-type’ TOF because the continuity of the oesophagus is not interrupted. The diagnosis is often missed or delayed, leading to complications such as chronic lung disease and failure to thrive.

Ingestion of a foreign body (FB) is a common condition with a few potentially life-threatening complications, including esophageal perforation (EP), aortoesophageal fistula (AEF), mediastinal infection, and tracheoesophageal fistula (TEF). In this case, a patient who accidentally ingested a duck bone gradually experienced all of the above complications. To resolve the symptom of difficulty swallowing, the patient underwent emergency treatment for removal of the esophageal FB via endoscopic surgery. Under endoscopy, esophageal mucosal injuries were present, but no other abnormalities, such as active bleeding, were observed. However, the patient returned to our hospital a week later with symptoms of vomiting and black stool and received the diagnosis of EP, AEF and mediastinal infection. Two days later, he vomited 1,000-2,000 mL of blood after experiencing sudden severe chest pain. Then, thoracic endovascular aortic repair (TEVAR) and mediastinal drainage with video-assisted thoracoscopic surgery (VATS) were performed under emergency general anesthesia. Additionally, the patient underwent esophageal stent implantation when TEF was confirmed by tracheal computed tomography (CT). The patient was treated with anti-infective therapy throughout the treatment process. Finally, he recovered and was able to tolerate a liquid diet. Comprehensive evaluation and multidisciplinary cooperation are all very important for the treatment of esophageal foreign bodies and complications.

A 46-year-old woman with a sore throat was diagnosed with anaplastic lymphoma kinase-negative anaplastic large cell lymphoma that had spread from the piriform fossa to the cervical esophagus. Tracheoesophageal fistula developed after induction chemotherapy. Because conservative repair was not applicable, intensified chemotherapy, including autologous stem cell transplantation was performed with the fistula remaining open. Laryngopharyngectomy and cervical esophagectomy were performed 6 months after transplantation. The optimal strategy for refractory lymphoma with active tracheoesophageal fistula remains undetermined. To the best of our knowledge, this is the first report describing the successful treatment of a patient with tracheoesophageal fistula who received high-dose chemotherapy supported by autologous stem cell transplantation.

Gastrointestinal (GI) emergencies in neonates and infants encompass from the beginning to the end of the GI tract. Both congenital and acquired conditions can cause various GI emergencies in neonates and infants. Given the overlapping or nonspecific clinical findings of many different neonatal and infantile GI emergencies and the unique characteristics of this age group, appropriate imaging is key to accurate and timely diagnosis while avoiding unnecessary radiation hazard and medical costs. In this paper, we discuss the radiological findings of essential neonatal and infantile GI emergencies, including esophageal atresia and tracheoesophageal fistula, hypertrophic pyloric stenosis, duodenal atresia, malrotation, midgut volvulus for upper GI emergencies, and jejunoileal atresia, meconium ileus, meconium plug syndrome, meconium peritonitis, Hirschsprung disease, anorectal malformation, necrotizing enterocolitis, and intussusception for lower GI emergencies.

Purpose Recent studies have identified transanastomotic tubes (TATs) as a risk factor for the development of anastomotic strictures after repair of esophageal atresia with tracheoesophageal fistula (EATEF). We further investigated these findings in a multicenter study. Methods We conducted a retrospective cohort study at three university-affiliated hospitals in the province of Quebec. All patients with types C and D EATEF who underwent primary repair between January 1993 and August 2018 were included. Anastomotic stricture was defined as clinical symptoms of stricture with confirmation on esophagram or endoscopy. Multivariate logistic regression and the Wilcoxon Rank-Sum test were used to evaluate the primary outcome of stricture within one year of surgery and secondary outcome of duration of postoperative total parenteral nutrition (TPN). Results 244 patients were included, of which 234 (96%) were type C and 10 (4%) were type D. The anastomotic stricture rate at 1 year was 30%. TATs were utilized in 61% of patients. Thirty-six percent of patients with TATs developed a stricture within one year, as compared to 19% of patients without TATs (p = 0.005). TATs were associated with stricture on univariate analysis (OR 2.49, p = 0.004, 95% CI: 1.37-4.69). On multivariate analysis, after adjusting for gestational age, birth weight, leak, long gap, anastomotic tension, and daily acid suppression, patients with TATs had 2.72 times higher odds of developing a stricture as compared to patients without TATs (p = 0.006, 95% CI: 1.35-5.74). The median duration of TPN was 9 days in both groups (p = 0.139, IQR 6-14 in patients with TATs versus IQR 7-16 in patients without). Conclusion Transanastomotic tubes are associated with a significantly higher risk of postoperative stricture following repair of esophageal atresia with tracheoesophageal fistula and do not shorten the duration of total parenteral nutrition. Level of evidence III.

Surgical repair of recurrent tracheoesophageal fistula has a high risk of complications. Therefore, various endoscopic techniques have been used to avoid complications.To understand the usefulness of trichloroacetic acid endoscopic application for the treatment of recurrent tracheoesophageal fistula.An observational, descriptive, retrospective, case-series-based study was carried out in a tertiary pediatric hospital. Records of patients with recurrent tracheoesophageal fistula from 2015 to 2021 were reviewed. All patients within this period underwent brushing and trichloroacetic acid application.Mean time of recurrent tracheoesophageal fistula occurrence 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 two patients had a large fistula (more than 4 mm). Mean sessions for fistula closure were 2.2 (range: 1-4). Mean time between procedures was 22 days (range: 14-30). Mean follow-up since fistula closure confirmation was 33 months (range: 9-72), during which no recurrences were noted.Endoscopic management of recurrent transesophageal fistula with trichloroacetic acid is a safe and effective procedure. Brushing and trichloroacetic acid combined improve success rates. Fistulas over 4 mm in diameter require more procedures. However, a larger patient cohort and a longer follow-up period are needed to confirm this.La reparación quirúrgica de la fístula traqueoesofágica recurrente tiene alto riesgo de complicaciones por lo que se han utilizado diversas técnicas endoscópicas para evitarlas.Conocer la utilidad de la aplicación endoscópica de ácido tricloroacético para el tratamiento de la fístula traqueoesofágica recurrente.Estudio observacional, descriptivo y retrospectivo, tipo de serie de casos en un hospital pediátrico de tercer nivel. Se revisaron los expedientes de pacientes con fístula traqueoesofágica recurrente de 2015 a 2021. En todos los pacientes durante este periodo se realizó cepillado y aplicación de ácido tricloroacético.El tiempo medio de aparición de la fístula traqueoesofágica 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 fue 2,2 veces (rango 1-4). El intervalo medio entre procedimientos fue 22 días (rango 14-30). El tiempo medio de seguimiento desde la confirmación de cierre de la fistula fue de 33 meses (rango 9-72), periodo de tiempo donde no se observó recurrencia del cuadro.El manejo endoscópico de la fístula traqueoesofágica recurrente con la aplicación de ácido tricloroacético es un procedimiento seguro y efectivo. La técnica de cepillado y aplicación de ácido tricloroacético mejora la efectividad de éxito. La fístula mayor de 4 mm de diámetro requiere mayor número de procedimientos; sin embargo, se requiere mayor número de pacientes y mayor tiempo de seguimiento para poder aseverar esta opinión.

The combination of duodenal atresia (DA) with esophageal atresia with tracheoesophageal fistula (EA-TEF) is a rare pathology, the frequency of which ranges from 1% to 6% of all cases of EA. Surgical treatment of the DA+EA-TEF combination always causes significant difficulties, primarily in determining the timing and stages of surgical correction. Objective of the study: to evaluate the results of treatment with a combination of DA+EA-TEF to determine the effective tactics of surgical treatment. Materials and methods of research: a retrospective, nonrandomized, uncontrolled, multicenter study was carried out. The work is based on the results of treatment of 15 newborns – 6 (40%) boys, 9 (60%) girls with a combination of DA+EA-TEF, who were treated in clinics of 6 university centers for pediatric surgery in the Russian Federation in 2015–2021. Simultaneous operations (SIMOPs) were performed in 10 (60%) patients, two-stage operations (TO) – in 5 (40%) newborns. The following criteria were taken into account: the period of antenatal (weeks) and postnatal (days) of establishing the diagnosis of obstruction of the gastrointestinal tract (GIT), gestational age (weeks), birth weight (g), weight at the time of surgery (g), type of concomitant pathology , sequence and methods of surgical treatment, terms of complete enteral feeding (days), outcomes of operations and reasons for unsatisfactory outcomes. The average gestational age of children who underwent SIMOPs was 35.1 weeks. (Q1 – 31.5, Q3 – 39; Me – 37; SD – 5.1; min/max – 25–40; 95% CI: 31.1–39.0) versus 29.8 weeks. at DO (Q1 – 29, Q3 – 30.5; Me – 30; SD – 1.0; min/max – 28–31; 95% CI: 28.4–31.6) did not differ statistically significantly (р=0.083). The mean body mass values did not have statistically significant differences (p=0.081) and amounted to 2224 g (Q1 – 1410, Q3 – 2930; Me – 2665; SD – 890.8; min/max – 760–3260; 95% CI: 1556–2926) for SIMOPs, versus 1322 g (Q1 – 1165, Q3 – 1450; Me – 1380; SD – 196; min/max – 980–1450; 95% CI: 1078.4–16565) in the TO group. Results: the average duration of SIMOPs was on average 144.4 min (Q1 – 125, Q3 – 155; Me – 147.5; SD – 22; min/max – 120–190; 95% CI: 1321–159.3), TO – 147.0 (Q1 – 125, Q3 – 172; Me – 140; SD – 33.4; min/max – 120–205; 95% CI: 126–178.6). The sequence of surgical correction of defects in SIMOPs in 8 (53%) patients consisted of thoracotomy, ligation of the TEF, direct anastomosis of the esophagus and the imposition of duodeno-duodenoanstomosis (DDA). In one case, DDA was selected as the first operation, which was supplemented with Kader gastrostomy followed by thoracotomy, ligation of the TEF and anastomosis of the esophagus after elongation according to I. Livaditis. In one patient, after thoracotomy and ligation of the TEF in connection with an insurmountable diastasis of the esophagus, a cervical esophagostomy (CE), duodenojejunoanastomosis (DEA) and a gastrostomy according to Kader were applied. In a two-stage correction (TO), the first operation in 3 patients was DDA (20%), supplemented in one case (7%) with Kader gastrostomy, and the second stage after 2 days performed thoracotomy with the elimination of TEF and EA. In 2 (13%) newborns, the first stage was thoracotomy, elimination of TEF and EA, followed by imposition of DDA 2 days later. In one case, due to an insurmountable diastasis of the esophagus after thoracotomy and ligation of the TEF, intrathoracic elongation of the esophagus according to Foker with delayed anastomosis of the esophagus (on the 7th day) and laparoscopic fundoplication according to Nissen (at 5 months) were performed. The duration of hospitalization did not statistically significantly depend on the chosen method for correcting the combination of DA+EA-TEF (p=0.79) and averaged 28.4 days for SIMOPs (Q1 – 16, Q3 –34.5; Me – 26; SD – 21.4; min/max – 5.0–79; 95% CI: 17.6–42.4), and for TOs – 27,2 days (Q1 – 21, Q3 – 33; Me – 28; SD – 7.1; min/max – 19–38; 95% CI: 27.2–33). In the group of patients with SIMOPs, 2 deaths (13%) were recorded on the 5th and 7th days after surgery due to progressive multiple organ failure and intractable pulmonary hypertension. In one case (7%), a lethal outcome was recorded 8 months after primary surgery due to progressive cardiovascular failure. Early postoperative mortality after SIMOPs was 20%, overall mortality was 30%. In the TO group, 3 (20%) deaths were recorded: 2 in the early postoperative period (on day 3 and day 19) and one at the age of 3 months of life. Early postoperative mortality after TO was 40%, overall mortality – 60%. Conclusion: it is preferable to choose the ligation of the TEF as the first operation and, if the child's condition allows, to impose an esophageal anastomosis and restore duodenal patency, followed by a nasogastric tube through the esophageal anastomosis into the stomach. If, after ligation of the TEF, the patient's cardiorespiratory status does not stabilize, it is possible to restore the patency of the esophagus and pass the probe into the stomach without imposing a gastrostomy, which will allow the patient to be further treated as an isolated DA, and the operation to restore the patency of the duodenum is delayed. In the presence of insurmountable diastasis, the use of esophageal elongation technology with subsequent delayed EA is justified.

We sought to evaluate the impact of thoracoscopic repair on perioperative outcomes in infants with esophageal atresia and tracheoesophageal fistula (EA/TEF).The American College of Surgeons National Surgical Quality Improvement Program pediatric database from 2014 to 2018 was queried for all neonates who underwent operative repair of EA/TEF. Operative approach based on intention to treat was correlated with perioperative outcomes, including 30-day postoperative adverse events, in logistic regression models.Among 855 neonates, initial thoracoscopic repair was performed in 133 (15.6%) cases. Seventy (53%) of these cases were converted to open. Those who underwent thoracoscopic repair were more likely to be full-term (p = 0.03) when compared to those in the open repair group. There were no significant differences in perioperative outcome measures based on surgical approach except for operative time (thoracoscopic: 217 min vs. open: 180 min, p0.001). A major cardiac comorbidity (OR 1.6, 95% CI 1.2-2.1; p = 0.003) and preoperative ventilator requirement (OR 1.4, 95% CI 1.0-1.9; p = 0.034) were the only risk factors associated with adverse events.Thoracoscopic neonatal repair of EA/TEF continues to be used sparingly, is associated with high conversion rates, and has similar perioperative outcomes when compared to open repair.III.

Background : Children born with esophageal atresia (EA) face comorbidities and complications often requiring surgery and anesthesia. We aimed to assess all procedures performed under general anesthesia during their first 12 years of life. Methods : We performed a retrospective cohort study about subsequent surgeries and procedures requiring general anesthesia in children born with type C EA between January 2007 and December 2017, with follow-up to March 2019. Results : Of 102 eligible patients, 63 were diagnosed with comorbidities, of whom 18 had VACTERL association. Follow-up time for all patients varied between 14 months and 12 years (median 7 years). The patients underwent total 637 procedures, median 4 [IQR2-7] per patient. In the first year of life, 464 procedures were performed, in the second year 69 and in the third year 29. Thirteen patients underwent no other procedures than primary EA repair. In 57 patients, 228 dilatations were performed. Other frequently performed procedures were esophagoscopy (n=52), urologic procedures (n=44) and abdominal procedures (n=33). Conclusions : Patients with EA frequently require multiple anesthetics for a variety of procedures related to the EA, complications and comorbidities. This study can help care providers when counselling parents of a patient with an EA by giving them more insight into possible procedures they can be confronted with during childhood.

SummaryInsertion of voice rehabilitation devices (VRD) via tracheoesophageal punctures (TEP) post laryngectomy is considered the standard of care. The management of periprosthetic leakage from the puncture site in a zone of previous neck dissection and or radiotherapy is problematic when it results in a tracheoesophageal fistula (TOF), as simple surgical closure and use of tissue for flaps carries a high risk of failure. We share our clinical experience in 3 of 42 patients who had a laryngectomy and VDR, who developed an expanding TEP with failure of free flaps in two, and the utility of a narrow pectoralis major myofascial flaps (PMMF) as a universally successful salvage or primary procedure.

In the COVID-19 era the tracheal complications due to prolonged mechanical ventilation have significantly increased. Acquired tracheoesophageal fistula is one of those in ventilated COVID-19 patients. Thus, the knowledge of their management in such fragile patient is crucial. We report a case of tracheoesophageal fistula in a 56-year-old female under prolonged mechanical ventilation for COVID-19 bilateral pneumonia and discuss its management. A surgical approach was proposed. By a collar-shaped transverse cervicotomic access, we transected the trachea at level of fistula en-bloc with the tracheostoma. The esophageal lesion was longitudinally repaired in two-layers. Protective left strap muscle was sandwiched between esophagus and trachea. The tracheal end-to-end anastomosis was completed without a re-tracheostoma. Even if surgical approach of tracheoesophageal fistula in COVID-19 patients has not been tested before, surgery remains the treatment of choice according to the multidisciplinary board.

Oesophageal atresia (EA) is associated with life-long gastrointestinal and respiratory morbidity and other associated malformations. The aim of this study is to compare physical activity (PA) levels of children and adolescents with and without EA. A validated questionnaire (MoMo-PAQ) was used to evaluate PA in EA patients EA (4–17 years), who were randomly matched for gender and age (1:5) with a representative sample of the Motorik-Modul Longitudinal Study (n = 6233). Sports activity per week (sports index) and minutes of moderate to vigorous physical activity per week (MVPA minutes) were calculated. Correlations between PA and medical factors were analysed. In total, 104 patients and 520 controls were included. Children with EA were significantly less active at higher intensities (mean MPVA minutes 462; 95% confidence interval (CI): 370–554) compared to controls (626; 95% CI: 576–676), although there was no statistically significant difference in the sports index (187; 95% CI: 156–220 versus 220; 95% CI: 203–237). A lower mean weight-for-age and height-for-age, additional urogenital (r = − 0.20, p = 0.04) or anorectal malformation (r = − 0.24, p = 0.01) were associated with fewer MVPA minutes. For other medical factors (prematurity, type of repair, congenital heart disease, skeletal malformation or symptom load), no statistically significant association with PA was found. Conclusion: EA patients participated in PA at a similar level but lower intensities compared to the reference cohort. PA in EA patients was largely independent of medical factors.Trial registration: German Clinical Trials Register (ID: DRKS00025276) on September 6, 2021. What is Known: • Oesophageal atresia is associated with a low body weight and height, delayed development of motor skills and impaired lung function and exercise capacity. What is New: • Patients with oesophageal atresia have a similar amount of sports activity per week but engage significantly less in moderate to vigorous physical activities compared to peers. • Physical activity was associated with weight-for-age and height-for-age, but largely independent of symptom load and other medical factors.

Background. Congenital malformations of the gastrointestinal (GI) tract are common birth defects detected in the neonatal period and usually present with signs of GI obstruction which at times can be life threatening. Anorectal malformations are among the more frequent congenital anomalies. The co-occurrence of congenital heart defect (CHD) along with GI malformation can significantly affect the natural history of either defect. Purpose - to study the prevalence of GI malformations in children with CHD and study the risk factors. Materials and methods. A total 100 patients of GI malformations were enrolled out of which 66 (66%) were males and 34 (34%) were females. All patients with any GI malformations (anorectal malformation, tracheoesophageal fistulae, anterior abdominal wall defects) presenting to Neonatal Intensive Care Unit (NICU), Paediatric Cardiac unit, Pediatric and Pediatric surgery outpatient department (OPD) as well as In-patient department (IPD) between October 2019 to October 2021 were included in study. A detailed history and examination was done followed by echocardiography and the prevalence of CHD among GI malformation was observed and risk factors were studied. Results. Most common GI anomaly was anorectal malformation (71%) followed by tracheoesophageal fistula (17%), CHD was seen in 14 children. Most common heart defect was ventricular septal defect (VSD) (43%) followed by patent ductus arteriosus (PDA) (36%) and atrial septal defect (ASD) (14%). Among cases with anorectal malformation, CHDs was seen in 10% and the most common defect was VSD (43%). Forty one percent of cases with tracheoesophageal fistula had CHDs with PDA (57%) being the most common underlying defect. Functional heart defects were present in 28 children of GI malformations. Conclusions. The coexistence and severity of CHD in patients with GI malformation can have prognostic implications. Thus, early cardiac evaluation should be performed in every case of GI malformation, preferably with echocardiography. This is likely to help in the risk stratification as well as management of such children. The study was performed in accordance with the principles of the Declaration of Helsinki. Study is approved by Ethical Committee of the Institution. Informed consent of patients was obtained for the study. No conflict of interests was declared by the authors., Введение. Врожденные пороки развития желудочно-кишечного тракта (ЖКТ) являются распространенными врожденными дефектами, выявляемыми в неонатальном периоде и обычно сопровождающимися признаками желудочно-кишечной непроходимости, которые иногда могут быть опасными для жизни. Аноректальные пороки развития относятся к наиболее частым врожденным аномалиям. Сочетание врожденного порока сердца (ВПС) с пороком развития ЖКТ может существенно повлиять на естественное течение любого порока. Цель - изучить распространенность пороков развития ЖКТ у детей с ВПС и выявить факторы риска. Материалы и методы. Всего включено 100 пациентов с пороками развития ЖКТ, из них - 66 (66%) мальчиков и 34 (34%) девочки. Все пациенты с любыми пороками развития ЖКТ (аноректальные пороки развития, трахеопищеводные свищи, дефекты передней брюшной стенки), поступающие в отделение интенсивной терапии новорожденных, детское кардиологическое отделение, амбулаторное отделение детской и детской хирургии, а также стационарное отделение в период с октября 2019 г. по октябрь 2021 г., были включены в исследование. Проведен подробный анамнез и обследование с последующей эхокардиографией, выявлена распространенность ВПС среди пороков развития ЖКТ, изучены факторы риска. Результаты. Наиболее распространенной аномалией ЖКТ была аноректальная мальформация (71%), за которой следовал трахеопищеводный свищ (17%), ВПС наблюдались у 14 детей. Наиболее частым пороком сердца был дефект межжелудочковой перегородки (43%), за которым следовали открытый артериальный проток (36%) и дефект межпредсердной перегородки (14%). Среди случаев аноректальной мальформации ВПС встречались в 10%, а наиболее частым дефектом был дефект межжелудочковой перегородки (43%). 41% случаев с трахеопищеводной фистулой имели ВПС, с открытой артериальной протокой - 57%, являющимся наиболее частым основным дефектом. Функциональные пороки сердца выявлены у 28 детей с пороками развития ЖКТ. Выводы. Сосуществование и тяжесть ВПС у пациентов с пороками развития ЖКТ могут иметь прогностическое значение. Таким образом, в каждом случае порока развития ЖКТ следует проводить раннюю кардиологическую оценку, предпочтительно с помощью эхокардиографии. Это может помочь в стратификации риска, а также в ведении таких детей. Исследование проведено в соответствии с принципами Хельсинкской декларации. Протокол исследования одобрен Локальным этическим комитетом учреждения. На проведение исследования получено информированное согласие пациентов. Авторы заявляют об отсутствии конфликта интересов., Вступ. Вроджені вади розвитку шлунково-кишкового тракту (ШКТ) є поширеними вродженими дефектами, які виявляються в неонатальному періоді та зазвичай супроводжуються ознаками шлунково-кишкової непрохідності, які інколи можуть бути небезпечними для життя. Аноректальні вади розвитку належать до найчастіших вроджених аномалій. Поєднання вродженої вади серця (ВВС) із вадою розвитку ШКТ може істотно вплинути на природний перебіг будь-якої вади. Мета - вивчити поширеність вад розвитку ШКТ в дітей з ВВС та виявити фактори ризику. Матеріали та методи. Усього залучено 100 пацієнтів із вадами розвитку ШКТ, із них - 66 (66%) хлопчиків і 34 (34%) дівчинки. Усіх пацієнтів із будь-якими вадами розвитку ШКТ (аноректальні вади розвитку, трахеостравохідні нориці, дефекти передньої черевної стінки), які надходили до відділення інтенсивної терапії новонароджених, дитячого кардіологічного відділення, амбулаторного відділення дитячої та дитячої хірургії, а також стаціонарного відділення в період із жовтня 2019 року до жовтня 2021 року, включили в дослідження. Проведено детальний анамнез і обстеження з подальшою ехокардіографією, виявлено поширеність ВПС серед вад розвитку ШКТ, вивчено фактори ризику. Результати. Найпоширенішою аномалією ШКТ була аноректальна мальформація (71%), за якою слідувала трахеостравохідна нориця (17%), ВПС спостерігалися в 14 дітей. Найчастішою вадою серця був дефект міжшлуночкової перегородки (43%), за яким слідували відкрита артеріальна протока (36%) і дефект міжпередсердної перегородки (14%). Серед випадків аноректальної мальформації ВПС зустрічалися в 10%, а найчастішим дефектом був дефект міжшлуночкової перегородки (43%). 41% випадків із трахеостравохідною фістулою мали ВПС, із відкритою артеріальною протокою - 57%, що є найчастішим основним дефектом. Функціональні вади серця виявлені у 28 дітей із вадами розвитку ШКТ. Висновки. Співіснування і тяжкість ВПС у пацієнтів із вадами розвитку ШКТ можуть мати прогностичне значення. Отже, у кожному випадку вади розвитку ШКТ слід проводити ранню кардіологічну оцінку, переважно за допомогою ехокардіографії. Це може допомогти в стратифікації ризику, а також у веденні таких дітей. Дослідження проведено відповідно до принципів Гельсінської декларації. Дослідження схвалено Локальним етичним комітетом установи. На проведення дослідження отримано інформовану згоду пацієнтів. Автори заявляють про відсутність конфлікту інтересів.

Esophageal dysmotility in esophageal atresia (EA) relates to abnormal development of esophageal innervation and musculature and to the esophageal repair. Few studies have investigated the preexisting dysmotility in EA, present prior to surgery. This systematic review aims to summarize the literature on neuronal studies in EA, to understand the causative factors for esophageal dysmotility. We performed a systematic review (PubMed, EMBASE, EBM, CINAHL databases; January 1947-February 2021) in accordance with PRISMA (PROSPERO number CRD42020171014). Fourteen studies were identified (eleven human, 187 EA patients; three animal, 64 EA rat specimens). Neural factors affecting esophageal dysmotility in human and animal studies included proteins, enzymes, growth factors, and genes, which play a role in the nervous system or neuroendocrine system, some of which have functions as neuromodulators or neurotransmitters. This systematic review has identified neural factors that affect esophageal dysmotility and contributes toward our understanding of the underlying dysmotility in patients with EA. The studies identified are important and essential for successful translation of basic science knowledge to impact clinical practice and understanding. Level of evidence: III.

A 4-month-old girl visited our hospital’s emergency room reporting fever and coughing. She had been conservatively followed for prenatally diagnosed tricuspid atresia, complicated with a supernumerary rib and a radial defect after she was born at 38 weeks’ gestation with a birth weight of 2662

A preterm female presented with severe respiratory distress in the delivery room and was found to have tracheal agenesis with a tracheoesophageal fistula and a congenital heart defect. Tracheal agenesis is uncommon and is often associated with other congenital abnormalities. Although there are surgical options for repair, mortality remains high.

Tracheoesophageal fistula results in persistent leakage of saliva into the trachea, prevents oral feeding, and predisposes to aspiration pneumonia. Large fistula closure may require a free flap to cover the defect. When the defect involves the tracheal area between the neck and the mediastinum, a tubeless field for optimal exposure can be advantageous. This article reports the use of veno-venous extracorporeal lung support, a known safe and efficient technique to support the patient's respiratory function, for this purpose. The typical veno-venous extracorporeal lung support setting includes a femoro-jugular bypass. The patient cases reported here had characteristics that precluded the use of the jugular vein, such as neck radiation dermatitis, previous radical neck dissection, and poor accessibility. Therefore a more rarely described femoro-femoral approach was used. The cases of three patients with persistent tracheoesophageal fistula who had free flap surgeries (two bi-paddled radial forearm free flap and one latissimus dorsi muscle free flap) assisted by femoro-femoral veno-venous extracorporeal lung support are reported.

BACKGROUND Total pharyngo-laryngo-esophagectomy with a reconstruction of gastric pull-up is the most common treatment method for patients with multiple primary upper digestive tract carcinomas, such as hypopharyngeal carcinoma with thoracic esophageal carcinoma. However, neck circumferential defect and tracheoesophageal fistula after gastric necrosis are still challenging problems for surgeons and patients. CASE SUMMARY This case report presents 2 patients who underwent reconstructive surgeries using 4 local random flaps with a split thickness skin graft in the first case, and 6 local random flaps in the second case to close the circumferential defect and tracheoesophageal fistula after failed gastric pull-up. Both patients achieved good swallowing function and could take solid diet without dysphagia postoperatively. CONCLUSION For selected patients, local random flaps (with a split thickness skin graft) can be a simple and reliable solution for reconstructing tracheoesophageal fistula or cervical circumferential defect after gastric necrosis, especially when the necrosis extends below the thoracic inlet.

BACKGROUND: Adults with esophageal atresia (EA) require a multidisciplinary follow-up approach, taking into account gastroesophageal problems, respiratory problems and psychosocial wellbeing. Too little is known about the full scope of these individuals' healthcare needs. We aimed to map all medical and psychosocial needs of adults with EA and their family members, and to formulate healthcare recommendations for daily practice.METHODS: A qualitative study was performed, using data from recorded semi-structured interviews with two focus groups, one consisting of adult patients with EA (n = 15) and one of their family members (n = 13). After verbatim transcription and computerized thematic analysis, results were organized according to the International Classification of Functioning, Disability and Health. Ethical approval had been obtained.RESULTS: Healthcare needs were described through 74 codes, classified into 20 themes. Most important findings for patients included the impact of gastrointestinal and pulmonary problems on daily life, long-term emotional distress of patients and parents and the need of a standardized multidisciplinary follow-up program during both child- and adulthood.CONCLUSION: The focus groups revealed numerous physical and mental health problems, as well as social difficulties, that require attention from different healthcare providers. We have formulated several healthcare recommendations that physicians may use in long-term follow-up.

Background: Although trachea-esophageal fistula (TEF) and esophageal atresia (EA) is rare, it is one of the most common congenital anomaly. There is paucity of data due to rarity of condition. More and more data on clinical profile, complications, and outcome are needed to guide the future research. Objective: To study clinical profile of newborns with TEF and EA and factors associated with outcome. Materials and Methods: Hospital based prospective study was carried out among 21 newborns with TEF and EA. Diagnosis was confirmed by investigations like X-ray chest after passing nasogastric catheter. Depending upon final diagnosis type of surgery required was determined and procedure was carried out as per the standard guidelines and operating techniques. All cases were followed till the complete post-operative period and the complications and outcome was assessed. Outcome was classified as death or discharge. Results: Males outnumbered females giving a male to female ratio of 2.5:1. 57.1% did not require ventilator pre-operatively. 61.9% of the cases had no associated abnormality. The most common type of surgery required and performed was tracheoesophageal repair in 52.4% of the cases. Two cases (9.5%) died during the post-operative period. 14 cases (66.7%) recovered and were discharged. Most common complication was anastomotic leak (57.1%). Term status and associated abnormalities were not found to be associated with outcome like death (p>0.05). Conclusion: Trachea-esophageal fistula and esophageal atresia was more common in males. Tracheoesophageal repair was commonly required surgery. Recovery rate was good. Anastomotic leak was most common complication. Outcome like death was not associated with term status and associated abnormalities. Keywords: Clinical profile, Newborns, Tracheoesophageal fistula, Atresia, Outcome

Purpose To report our experience with endoscopic Thulium LASER for treatment of recurrent TEF after EA surgery, and for H-Type fistulas. Methods A retrospective chart review of consecutive patients undergoing standardized endoscopic closure as first line therapy of recurrent tracheoesophageal fistula (RTEF) and H-type fistula using Thulium LASER, from 2013 to 2019, in a pediatric tertiary care center. Control endoscopic procedure was systematically performed. If persistence of the TEF was noted an external approach was performed. Patient demographics, medical history, symptoms, TEF type, treatment modalities, complications and outcomes were collected. Results Eleven patients with tracheoesophageal fistula were included: six RTEF after primary repair of esophageal atresia and five H-type fistulas. The average age at endoscopic treatment was 19 months (SD 23 months, range 13 days–63 months). Closure of the fistula after single endoscopic procedure with Thulium LASER was obtained in 3 RTEF (50%) and 1 H-type fistula (20%). Six patients with failure of endoscopic treatment were cured after a single external procedure without any complications. One child, treated for H-type fistula, presented a severe complication of Thulium LASER treatment. Median follow-up after last repair was 24 months (range: 14–72 months). All fistulas were successfully treated. Conclusions In H-Type fistula, success rate of Thulium LASER is only 20% and thus should not be used. In contrast, in RTEF, success rate of 50% is achieved, avoiding as many open procedures, and Thulium LASER could be considered as first line treatment. In any case, open surgery is safe and efficient and can be considered as a first-line treatment for H-type fistulas, and as a salvage treatment for endoscopic treatment failures. Levels of Evidence Level IV.

Background: Congenital heart defect (CHD) is one of the leading causes of neonatal death. Although the majority of CHDs are isolated, a significant number of them are associated with noncardiac anomalies. Esophageal Atresia (EA)/ Tracheoesophageal Fistula (TEF) is the most common congenital disorder of the upper GI tract. It is estimated that up to 70% of EA/TEF infants have other associated congenital anomalies such as CHD. This study determined the proportion of heart anomalies among the diseases of the upper GI tract in Imam Reza Hospital of Mashhad.Methods: The records of 38 infants with upper GI obstruction who were referred to the Pediatric Cardiology Clinic of Imam Reza Hospital in Mashhad between 2001 and 2017 were evaluated in this retrospective study. Data were coded and entered into SPSS software (version 16) and analyzed using Chi-square and T-test.Results: In this study, 38 babies with upper GI obstruction were evaluated (20 patients were female, 52.6%), and the average birth weight was 2.390 +-0.870 gr. Among the parents, 13 patients (34.2%) were relative (third-degree or more) and 25 patients (65.8%) were nonrelative. The initial and final diagnosis was different at 14 pt (36.8%) that was confirmed with echocardiographic findings. CHDs were divided into two groups in this study. Malformations such as PFO (patent foramen ovale) or FMV without MR (floppy mitral valve without mitral regurgitation) considered as non-important congenital heart diseases. Other malformations that require interventional or medical management such as VSD, ASD, TOF, or other CHDs are considered important CHDs. Nineteen pt (50%) had important CHD and 16 pt (42.1%) had non-important CHD and 3 pt (7.9%) had normal echocardiographic findings.Conclusion: The heart defect is the most common associated anomaly in children with EA/TEF, which is divided into two subgroups. The first important one is CHD, which is effective in gastric surgery and management, and VSD is the most common type. The other group is non important CHD such as PFO or FMV without MR that are not effective in their management. The patients with EA/TEF are at risk for low birth weight and preterm delivery.

Endoscopy for revealing the orifice of congenital H-type tracheoesophageal fistula (cTEF) is important for diagnostics and therapeutics. To facilitate the identification and catheterization of cTEF, we developed a new modified flexible endoscopy technique using a laryngeal mask with intermittent airflow. A retrospective case series study was conducted from April 2016 to July 2019 at a national regional children's medical center. Twelve infants with cTEF underwent this flexible endoscopy technique. The intermittent positive pressure airflow through laryngeal mask was able to reveal the orifice of cTEF easily in tracheal lumen. Under the visual flexible endoscope, cannulation with a 3-French ureteral catheter in fistula was successfully used in all cases. There were no immediate or delayed complications. This case series shows that the flexible endoscopy technique is a safe, easy, and technically efficient approach for diagnosis and cannulation of cTEF.

Background: In the 21st century, the majority of trache-ostomies are now inserted by the intensivists in the intensive care unit (ICU). It is one of the most frequent procedures performed in critically ill patients. It has been advocated for those requiring prolonged mechanical ventilation because it facilitates weaning by decreasing the work of breathing, decreases the requirement for sedation and may allow for earlier patient mobilization, feeding and physical and occu-pational therapy. Aim of Study: To evaluate ultrasound guided percutaneous tracheostomy and conventional tracheostomy in critically ill patients regarding effect on outcome (weaning from mechanical ventilation and ICU stay), duration of the technique, success rate and to evaluate incidence of perioperative, early and late complications. Patients and Methods: Our study is a randomized con-trolled clinical trial conducted on 40 critically ill patients admitted to the Intensive Care Unit at Ain Shams University Hospitals, from the period from September 2020 until March 2021 they were intubated and mechanically ventilated and required elective percutaneous dilatational tracheotomy. Results: US-guided group showed fewer procedural com-plications compared to conventional group. We had faced procedural complications in conventional group in form of 2 (10%) of patients suffer from hypoxemia, Pneumothorax, decannulation and post. Tracheal wall injury. 3 (15%) of patients had transient hypotension and false passage. And 5 (25%) cases of perforation of ETT cuff during insertion, one case (5%) of subcutaneous emphysema and 7 (35%) cases of minor bleeding compered to three cases of minor bleeding in US-guided group, one case of decannulation and three case of transient hypotension. No early complications were detected in both study groups; except one case of tube obstruction or displacement in conventional group. According to late com-plications our analysis illustrates decrease in late complication in US-guided group 2 (10%) versus 4 (20%) in conventional group. In US-guided group only two cases of Stoma site infection resolved by antibiotic and local care. In conventional group there were two case of Tracheoesophageal fistula, one case of Stoma site infection and one case of Tracheoinnominate fistula. Conclusion: Percutaneous dilatational tracheostomy could be a safer procedure when performed by using peri- and preoperative US assistance. The use of US guidance for percutaneous dilatational tracheostomy could reduce the complication rates of the procedure. The ultrasound-guided percutaneous dilatational tracheostomy seems to reduce the late and early complications when compared to the anatomical landmark guided Percutaneous dilatational tracheostomy. Pre-procedural US-guided percutaneous dilatational tracheostomy can be considered as a reliable tool to increase safety and improve outcomes of elective tracheostomy.

Background Esophageal atresia (EA) is a life-threatening congenital condition, affecting one in 2600 newborns. Morbidity remains high, with many patients experiencing complications, including anastomotic leak/stricture, and gastro-esophageal reflux disease (GERD). Increased understanding of esophageal motility patterns may help explain the etiology of these complications. Aims We aimed to review knowledge regarding esophageal motility and related complications in children with EA, evaluate patients' symptomatology and relate this to esophageal motility. Methods We performed a systematic review (PROSPERO: CRD42018092277), according to the PRISMA protocol. Two investigators independently conducted search strategies (OvidMEDLINE, PubMed, Cochrane Review, BMJ BestPractice), identifying complications in patients following EA repair. Rates of esophageal dysmotility, GERD, dysphagia, anastomotic leak, anastomotic stricture, recurrent fistula formation, and esophagitis were sought. Results A total of 65 publications met selection criteria (n = 4882). Rates of morbidity were high: esophageal dysmotility (78%), GERD (43%), dysphagia (44%), anastomotic leak (19%), anastomotic stricture (26%), recurrent fistula formation (7%), and esophagitis (47%). No correlation appeared to exist with severity of symptoms. Conclusions This systematic review identified high rates of complications in children with EA, with esophageal dysmotility present in the majority of patients. Increasing survival, with resultant longer timeframes to develop morbidities, makes standardized follow-up regimens crucial. Type of study Prognosis study. Level of evidence Level 3.

Background There is a paucity of literature on the anesthetic management of pediatric esophageal substitution using the stomach. We did a retrospective analysis of all such cases done at our institution. We analyzed the patient’s demography, indication, and type of surgery, co-morbid conditions, anesthesia techniques, duration of postoperative ventilation, hospital stay, complications, and mortality. The use of beta-blockers and their effect on the incidence of intraoperative and postoperative tachycardia in gastric pull-up patients was also analyzed. Results Thirty-four cases of gastric substitution of the esophagus in children were done over 19-year period; gastric pull-up was done in 28 patients and a gastric tube was made in 6 patients. General anesthesia was given to all; a thoracic epidural for pain was sited in 25 patients. Twenty-eight patients were ventilated postoperatively; the mean duration of ventilation is 54 h. Significant intraoperative tachycardia was observed in 85.7% of patients without beta-blocker as compared to 23.8% patients with beta-blocker (p = 0.004). Postoperatively, tachycardia was absent in patients receiving beta-blocker and present in 71.4% of patients not receiving beta-blockers (p < 0.001). Overall mortality was 8.8% but mortality due to cardiac arrhythmia was 42.9% in the patients not receiving beta-blockers (p = 0.001). Conclusions A thorough preoperative preparation, control of tachyarrhythmias, postoperative ventilation, and pain management is recommended for a favorable outcome. In addition, our paper supports the preoperative use of beta-blockers in reducing the incidence of fatal tachyarrhythmias associated with gastric pull-up surgery without any serious adverse effects. Level of evidence Level III

© 2022 Wiley Periodicals LLC.Objectives: Postoperative ventilatory strategies in patients with esophageal atresia (EA) and tracheoesophageal fistula (TEF) may have an impact on early postoperative complications. Our national Esophageal Atresia Registry was evaluated to define a possible relationship between the type and duration of respiratory support on postoperative complications and outcome. Study Design: Among the data registered by 31 centers between 2015 and 2021, patients with esophago-esophageal anastomosis (EEA)/tracheoesophageal fistula (TEF) were divided into two groups; invasive ventilatory support (IV) and noninvasive ventilatory support and/or oxygen support (NIV-OS). The demographic findings, gestational age, type of atresia, associated anomalies, and genetic malformations were evaluated. We compared the type of repair, gap length, chest tube insertion, follow-up times, tensioned anastomosis, postoperative complications, esophageal dilatations, respiratory problems requiring treatment after the operation, and mortality rates. Results: Among 650 registered patients, 502 patients with EEA/TEF repair included the study. Four hundred and seventy of patients require IV and 32 of them had NIV-OS treatment. The IV group had lower mean birth weights and higher incidence of respiratory problems when compared to NIV-OS group. Also, NIV-OS group had significantly higher incidence of associated anomalies than IV groups. The rates of postoperative complications and mortality were not different between the IV and NIV-OS groups. Conclusion: We demonstrated that patients who required invasive ventilation had a higher incidence of low birth weight and respiratory morbidity. We found no relation between mode of postoperative ventilation and surgical complications. Randomized controlled trials and clinical guidelines are needed to define the best type of ventilation strategy in children with EA/TEF.

Esophageal atresia with or without tracheoesophageal fistula and tracheobronchomalacia encompass 2 of the most common complex congenital intrathoracic anomalies. Tailoring interventions to address the constellation of problems present in each patient is essential. Due to advances in neonatology, anesthesia, pulmonary, gastroenterology, nutrition and surgery care for patients with complex congenital tracheoesophageal disorders has improved dramatically. Treatment strategies tailored to the individual patient needs are best implimented under the aegis of a comprehensive longitudinal multidisciplinary care team.

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Esophageal atresia (EA) with tracheoesophageal fistula (TEF) is among the most common congenital anomalies requiring surgical intervention in infancy. General surgeons practicing in rural or austere environments may encounter emergency situations requiring their involvement. Respiratory emergencies can arise in the neonatal period; the recommended approaches are the ligation of the fistula through the chest or occlusion of the distal esophagus through the abdomen. As survivors of the condition reach late adulthood, general surgeons can anticipate encountering these patients. An understanding of risk factors, common symptoms, associated anomalies, and the appropriate diagnostic evaluation will facilitate care.

Congenital esophageal atresia, in which the proximal esophagus forms a blind end, results in respiratory failure due to aspiration of saliva. When accompanied by a distal tracheoesophageal fistula, increased intragastric pressure causes gastric juice to reflux into the trachea, resulting in severe pneumonia. Because of the above conditions, surgery should be performed early in life once the diagnosis is made. Conventionally, a two-stage radical operation was performed after gastrostomy. Recently, if the patient is in good general condition, a one-stage radical operation is preferred without gastrostomy. The patient is positioned in the left lateral recumbent position. The tracheoesophageal fistula is double ligatured near the tracheal junction by extra-pleural approach. After dissection of the upper esophageal blind end, the upper and lower esophagus are anastomosed using a single-layer intermittent suture. In cases of a long gap in which one-stage anastomosis is difficult, two-stage esophageal anastomosis is performed. As the long-term prognosis of esophageal reconstruction using the stomach, small intestine, or large intestine in children is not always good, it is desirable to reconstruct the esophagus using autologous esophagus. Recently, thoracoscopic repair has been performed for esophageal atresia, and its results have been reported to be comparable to those of open repair.

The mechanisms coupling fate specification of distinct tissues to their physical separation remain to be understood. The trachea and esophagus differentiate from a single tube of definitive endoderm, requiring the transcription factors SOX2 and NKX2-1, but how the dorsoventral site of tissue separation is defined to allocate tracheal and esophageal cell types is unknown. Here, we show that the EPH/EPHRIN signaling gene Efnb2 regulates tracheoesophageal separation by controlling the dorsoventral allocation of tracheal-fated cells. Ventral loss of NKX2-1 results in disruption of separation and expansion of Efnb2 expression in the trachea independent of SOX2. Through chromatin immunoprecipitation and reporter assays, we find that NKX2-1 likely represses Efnb2 directly. Lineage tracing shows that loss of NKX2-1 results in misallocation of ventral foregut cells into the esophagus, while mosaicism for Nkx2-1 generates ectopic NKX2-1/EPHRIN-B2 boundaries that organize ectopic tracheal separation. Together, these data demonstrate that NKX2-1 coordinates tracheal specification with tissue separation through the regulation of EPHRIN-B2 and tracheoesophageal cell sorting.