tracheoesophageal fistula
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2022 ◽  
Vol 4 (1) ◽  
Author(s):  
Michael Stevens ◽  
Amer Heider ◽  
Caroline T. Simon ◽  
Folafoluwa O. Odetola

Author(s):  
Alice King ◽  
Joshua R. Bedwell ◽  
Deepak K. Mehta ◽  
Gary E. Stapleton ◽  
Henri Justino ◽  
...  

Introduction: Without fetal or perinatal intervention, congenital high airway obstruction syndrome (CHAOS) is a fatal anomaly. The ex utero intrapartum treatment (EXIT) procedure has been used to secure the fetal airway and minimize neonatal hypoxia, but is associated with increased maternal morbidity. Case Presentation: A 16-year-old woman (gravida 1, para 0) was referred to our hospital at 31 weeks gestation with fetal anomalies, including echogenic lungs, tracheobronchial dilation and flattened diaphragms. At 32 weeks, fetoscopic evaluation identified laryngeal stenosis, which was subsequently treated with balloon dilation and stent placement. The patient developed symptomatic and regular preterm contractions at post-operative day 7 with persistent sonographic signs of CHAOS, which prompted a repeat fetoscopy with confirmation of a patent fetal airway followed by Cesarean delivery under neuraxial anesthesia. Attempts to intubate through the tracheal stent were limited and resulted in removal of the stent. A neonatal airway was successfully established with rigid bronchoscopy. Direct laryngoscopy and bronchoscopy confirmed laryngeal stenosis with a small tracheoesophageal fistula immediately inferior to the laryngeal stenosis and significant tracheomalacia. A tracheostomy was then immediately performed for anticipated long term airway and pulmonary management. The procedures were well tolerated by both mom and baby. The baby demonstrated spontaneous healing of the tracheoesophageal fistula by day of life 7 with discharge home with ventilator support at three months of life. Conclusion: Use of repeated fetoscopy in order to relieve fetal upper airway obstruction offers the potential to minimize neonatal hypoxia, while concurrently decreasing maternal morbidity by avoiding an EXIT procedure. Use of the tracheal stent in CHAOS requires further investigation. The long-term reconstruction and respiratory support of children with CHAOS remain challenging


NeoReviews ◽  
2022 ◽  
Vol 23 (1) ◽  
pp. e56-e59
Author(s):  
Kumar Ankur ◽  
Aparna Prasad ◽  
Ankit Parakh ◽  
Sanjeev Chetry ◽  
Prashant Jain ◽  
...  

Author(s):  
A.P. Akhter ◽  
S.M. Donn

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.


2021 ◽  
Vol 100 (6) ◽  
pp. 45-53
Author(s):  
I.N. Khvorostov ◽  
◽  
N.K. Barova ◽  
S.V. Minaev ◽  
M.A. Akselrov ◽  
...  

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.


2021 ◽  
Vol 9 ◽  
Author(s):  
Magdalena Klaniewska ◽  
Krystian Toczewski ◽  
Anna Rozensztrauch ◽  
Michal Bloch ◽  
Agata Dzielendziak ◽  
...  

The MYCN oncogene encodes a transcription factor belonging to the MYC family. It is primarily expressed in normal developing embryos and is thought to be critical in brain and other neural development. Loss-of-function variants resulting in haploinsufficiency of MYCN, which encodes a protein with a basic helix–loop–helix domain causes Feingold syndrome (OMIM 164280, ORPHA 391641). We present an occurrence of esophageal atresia (EA) with tracheoesophageal fistula in siblings from a three-generation family affected by variable expressivity of MYCN mutation p.(Ser90GlnfsTer176) as a diagnostic effect of searching the cause of familial esophageal atresia using NGS-based whole-exome sequencing (WES). All of our affected patients showed microcephaly and toe syndactyly, which were frequently reported in the literature. Just one patient exhibited clinodactyly. None of the patients exhibited brachymesophalangy or hypoplastic thumbs. The latest report noted that patients with EA and Feingold syndrome were also those with the more complex and severe phenotype. However, following a thorough review of the present literature, the same association was not found, which is also confirmed by the case we described. The variable phenotypic expression of the patients we described and the data from the literature guide a careful differential diagnosis of Feingold syndrome even in cases of poorly expressed and non-specific symptoms.


2021 ◽  
Vol 9 (12) ◽  
Author(s):  
Mohammad Negaresh ◽  
Saeed Hoseininia ◽  
Ali Hossein Samadi Takaldani ◽  
Iraj Feyzi‐Khankandi ◽  
Bahman Mohammadzadeh Germi ◽  
...  

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Jiawei Zhao ◽  
Shen Yang ◽  
Siqi Li ◽  
Peize Wang ◽  
Yanan Zhang ◽  
...  

Abstract Background To analyze the possible causes, treatment and outcomes of postoperative pneumothorax in patients with Gross type C esophageal atresia/tracheoesophageal fistula (EA/TEF). Methods Medical records of patients with Gross type C EA/TEF who were diagnosed and treated in Beijing Children’s Hospital from January 2007 to January 2020 were retrospectively collected. They were divided into 2 groups according to whether postoperative pneumothorax occurred. Univariate and multivariate logistic regression analysis were performed to identify risk factors for pneumothorax. Results A total of 188 patients were included, including 85 (45 %) in the pneumothorax group and 103 (55 %) in the non-pneumothorax group. Multivariate logistic regression analysis showed that postoperative anastomotic leakage [P < 0.001, OR 3.516 (1.859, 6.648)] and mechanical ventilation [P = 0.012, OR 2.399 (1.210, 4.758)] were independent risk factors for pneumothorax after EA/TEF repair. Further analysis of main parameters of mechanical ventilation after surgery showed that none of them were clearly related to the occurrence of pneumothorax. Among the 85 patients with pneumothorax, 33 gave up after surgery and 52 received further treatment [conservative observation (n = 20), pleural puncture (n = 11), pleural closed drainage (n = 9), both pleural puncture and closed drainage (n = 12)]. All of the 52 patients were cured of pneumothorax at discharge. Conclusions Anastomotic leakage and postoperative mechanical ventilation were risk factors for pneumothorax after repair of Gross type C EA/TEF, but the main parameters of mechanical ventilation had no clear correlation with pneumothorax. After symptomatic treatment, the prognosis of pneumothorax was good.


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