Pulmonary torsion as an atypical complication of congenital esophageal atresia repair-a case report and review of literature.
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ABSTRACT:
SUBMITTER: Koziarkiewicz M
PROVIDER: S-EPMC4336054 | biostudies-other | 2014 Jun
REPOSITORIES: biostudies-other
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Project description:Background?:Tetralogy of Fallot is a congenital heart defect characterized by pulmonary valve stenosis, ventricular septal defect (VSD), overriding aorta, and right ventricular hypertrophy. In its' extreme form, the pulmonary valve orifice does not develop during organogenesis, resulting in pulmonary atresia. We report a case of catheter ablation of symptomatic atrial fibrillation (AF) in a 37-year-old patient with congenital pulmonary atresia. Case summary?:The young man described paroxysmal tachycardia correlating to AF episodes in the previously implanted event recorder. Computed tomography scan described the complex anatomy with congenital pulmonary atresia, VSD, and major aortopulmonary collateral arteries. Electroanatomical mapping revealed typical pulmonary vein electrograms in a hypotrophic left atrium. Modified pulmonary vein isolation was successfully performed and non-excitability of the ablation line was reached. The patient recovered uneventfully and event recorder interrogation showed no AF recurrence after 3 months. Discussion?:Incidence of pulmonary atresia is low. Untreated survival rate is 50% after 1 year and 8% after 10?years. Tachycardia is a major cause of increased morbidity and mortality in patients with cyanotic congenital heart defects and pulmonary vein foci are described as driver for AF. Considerations preceding catheter ablation included pathophysiological mechanism, complex anatomy, atypical left atrium access, and reduced pulmonary perfusion resulting in a hypotrophic left atrium. Pulmonary veins showed typical electrograms, and isolation of pulmonary veins was feasible without adverse events.
| S-EPMC6764579 | biostudies-literature
Project description:Iatrogenic perforation of the neonate's pharynx and esophagus with normal anatomy was first described by Eklöf et al in 1968. It typically occurs in severely premature neonates who have undergone repeated traumatic attempts at endotracheal intubation or passage of orogastric tubes. It may also mimic esophageal atresia (EA). Perforation of upper pouch in tracheoesophageal fistula with EA was rarely reported. We report a 1,400 g (32 weeks) neonate with pure EA and iatrogenic perforation of upper pouch due to use of catheter for diagnostic radiography.
| S-EPMC4335949 | biostudies-other
Project description:RationaleHemoptysis is an uncommon chief complaint but a distressing symptom in pediatric patients. Due to the recurrence and mortality in minor patients, an accurate diagnosis of the underlying cause is quite essential for treatment. The etiologies causing hemoptysis in children are similar to that in adults. Isolated unilateral pulmonary vein atresia (PVA), as an unusual cardiovascular anomaly, has rarely been reported to be an etiology of hemoptysis in children.Patient concernsA 2-year and 11-month-old boy was admitted into our hospital with a complaint of recurrent hemoptysis for 2 months and the symptom became more aggravated in recent 4 days before admission. Physical examination was only remarkable for slightly diminished breath sounds over the left lung field, pale face, and colorless lip. Series of targeted laboratory evaluation were negative expect for anemia. Due to the identification of asymmetrical transparency of bilateral lung, slight emphysema of right lung, less volume of left lung with ground-glass opacity and reticular opacity, and ipsilateral mediastinal shift on chest CT, and varices of submucosal vessels in the left bronchial tree on the fiber-optic bronchoscope.DiagnosesIt more likely indicated a congenital cardiovascular disease. The diagnosis of left isolated unilateral PVA was ultimately confirmed through chest CT angiography (CTA) with three-dimensional (3D) reconstruction.InterventionsSince the boy did not complain with hemoptysis after admission, respiratory tract infections seldom occurred and no pulmonary hypertension was detected, a conservative approach was chosen with periodic clinical follow-up after discussing with the cardiac surgeons and in accordance to his parents' own wishes.OutcomesFortunately, he was doing well after 3 months of clinical observation.LessonsWe firstly reported a rare case of hemoptysis in children secondary to isolated unilateral PVA with no associated congenital heart disease in Chinese population. It is significant to improve the recognition and prompt diagnosis of this rare condition for pediatric clinicians, and widen the etiology spectrum of hemoptysis in children. The diagnosis of unilateral PVA should be considered for a patient with recurrent hemoptysis and imaging findings that indicate hypoplastic lung, ipsilateral mediastinal shift, and smooth margins of left atrium without evidence of rudimentary pulmonary veins.
| S-EPMC6112952 | biostudies-literature
Project description:BackgroundA high prevalence of eosinophilic esophagitis (EoE) has been preliminarily reported in patients after repair of esophageal atresia (EA), but the basis of this association is unknown.ObjectivesWe aimed to (1) characterize the EoE transcriptome in patients with EA, (2) compare the EoE transcriptome in patients with EoE and EA with that in patients with EoE alone, and (3) identify transcripts that could predispose patients with EA to EoE.MethodsThis single-center, population-based, retrospective study identified 4 EoE study cohorts: healthy control subjects, patients with EA and EoE (EA+EoE+), patients with EA without EoE (EA+EoE-), and patients with EoE without EA (EA-EoE+). Molecular signatures were assessed by using the EoE diagnostic panel, a 94-gene expression quantitative PCR array.ResultsIn a cohort of 110 pediatric patients with surgically repaired EA, 20 (18%) patients were given a diagnosis of EoE, representing a 364-fold enrichment of EoE in patients with EA compared with the general pediatric population. EoE diagnostic panel analyses revealed a major overlap between the EA+EoE+ and EA-EoE+ cohorts. A proportion (approximately 25%) of EoE signature genes were dysregulated in patients with EA+EoE- compared with healthy control subjects, including those involved in epithelial barrier function and type 2-associated inflammatory responses. Patients with EA+EoE+ exhibit a more severe EoE clinical phenotype than those with EA-EoE+ in terms of dysphagia and dilation need.ConclusionsPatients with EA have increased risk of EoE. Patients with EoE with EA have a similar molecular profile compared with that of patients with EoE without EA. Dysregulated baseline epithelial barrier and type 2-associated genes in EA monomorbidity might explain the higher EoE prevalence in patients with EA.
| S-EPMC6461044 | biostudies-literature
Project description:ObjectiveThis study aims to investigate and analyze the connection between PITX2 polymorphisms and the susceptibility of congenital esophageal atresia.MethodsFrom January 2015 to June 2020, 46 children with congenital esophageal atresia undergoing surgery were recruited for the study and placed in an observation group, and 40 neonates born in pediatrics during the same period were also recruited for the study and placed in a control group. The alleles and distribution frequencies of the polymorphisms of PITX2 gene rs2200733 were analyzed, and the odds ratio (OR) of esophageal atresia caused by the rs2200733 polymorphism were calculated using a logistic analysis.ResultsIn the observation group, there were 23 patients (50.00%) with the TT genotype of rs2200733, 21 patients with the TC genotype (45.65%), and 2 patients with the CC genotype (4.35%). In the control group, there were 13 patients with the TT genotype (32.50%), 17 patients with the TC genotype (42.50%), and 10 patients with the CC genotype (25.00%), and the differences in the genotypes between the two groups were statistically significant (P<0.05). The frequencies of the T-alleles and C-alleles of rs2200733 in the observation group were 72.83% and 27.17% respectively, while the frequencies of the control group were 53.75% and 46.25% respectively, and the differences in the rs2200733 allele frequencies were statistically significant (P>0.05). Taking the CC genotype as a reference, the neonates with the TC genotype (OR=2.978, 95% CI=1.084~7.952, P=0.042) or the neonates with the TT genotype (OR=4.778, 95% CI=1.208~13.492, P=0.009) had an increased risk of esophageal atresia, of which the TT genotype indicated a higher risk.ConclusionThe polymorphic site rs2200733 (T/C) of the PITX2 gene is connected to the incidence of congenital esophageal atresia. The T-allele is a risk factor for congenital esophageal atresia, and compared with the CC genotype, the TT genotype has an increased risk of esophageal atresia.
| S-EPMC8430062 | biostudies-literature
Project description:BackgroundElongation and repair of long gap esophageal atresia (LGEA) can be performed thoracoscopically, even directly after birth. The effect of thoracoscopic CO2-insufflation on cerebral oxygenation (rScO2) during the consecutive thoracoscopic procedures in repair of LGEA was evaluated.MethodsProspective case series of five infants, with in total 16 repetitive thoracoscopic procedures. A CO2-pneumothorax was installed with a pressure of maximum 5 mmHg and flow of 1 L/min. Parameters influencing rScO2 were monitored. For analysis 10 time periods of 10' during surgery and in the perioperative period were selected.ResultsMedian gestational age was 35+3 [range 33+4 to 39+6] weeks; postnatal age at time of first procedure 4 [2-53] days and time of insufflation 127[22-425] min. Median rScO2 varied between 55 and 90%. Transient outliers in cerebral oxygenation were observed in three patients. In Patient 2 oxygenation values below 55% occurred during a low MABP and Hb < 6 mmol/L. The rScO2 increased after erythrocytes transfusion. Patient 5 also showed a rScO2 of 50% with a Hb <6 mmol/L during all procedures, except for a substantial increase during a high paCO2 of 60 mmHg. Patient 4 had a rScO2 > 85% during the first procedure with a concomitant high FiO2 > 45%. All parameters recovered during the surgical course.ConclusionsThis prospective case series of NIRS during consecutive thoracoscopic repair of LGEA showed that cerebral oxygenation remained stable. Transient outliers in rScO2 occurred during changes in hemodynamic or respiratory parameters and normalized after interventions of the anesthesiologist. This study underlines the importance of perioperative neuromonitoring and the close collaboration between pediatric surgeon, anesthesiologist and neonatologist.
| S-EPMC5394154 | biostudies-literature
Project description:Congenital left main coronary artery atresia is an exceedingly rare condition with potentially fatal consequences if not diagnosed in a timely fashion. We present a case series in children and adolescents, including surgical repair and outcomes. We describe the presenting symptoms and subsequent management of each patient, including surgical repair and outcomes. (Level of Difficulty: Advanced.).
| S-EPMC8310967 | biostudies-literature
Project description:BackgroundTo analyze the possible causes, treatment and outcomes of postoperative pneumothorax in patients with Gross type C esophageal atresia/tracheoesophageal fistula (EA/TEF).MethodsMedical 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.ResultsA 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.ConclusionsAnastomotic 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.
| S-EPMC8641193 | biostudies-literature
Project description:BackgroundThis study aimed to evaluate the effectiveness of primary posterior tracheopexy (PPT) in reducing ventilator dependence after repair of esophageal atresia (EA), and the risk of respiratory tract infections (RTI) requiring readmissions within one year.MethodsThis retrospective cohort study recruited patients with EA admitted to our hospital between June 2020 and December 2021.ResultsIn the PPT group (n = 17), the time to extubation after surgery was 86.7 h for 12 patients, with one patient (8.3%) requiring repeated postoperation intubation; six-in-sixteen patients (37.5%) experience at least one RTI requiring hospitalization in one year. In the non-PPT group (n = 17), the time to extubation was 127.0 h for 14 patients, with six-in-fourteen patients (42.9%) requiring repeated intubation; twelve-in-seventeen patients (70.6%) experienced at least one RTI requiring hospitalization in one year.ConclusionsAlthough the differences did not reach statistical significance due to limited number of participants, patients underwent PPT during EA repair had lower chance of repeated intubation and decreased risk of RTI requiring admissions within one year.
| S-EPMC10195884 | biostudies-literature
Project description:Congenital coronary-pulmonary artery fistula is a rare condition and is usually associated with pulmonary atresia. We present a 10-year-old girl with circumflex coronary artery to pulmonary artery (PA) fistula with a giant dilated circumflex coronary artery, ventricular septal defect (VSD), pulmonary atresia, and anomalous originofleft anterior descending (LAD) artery from right coronary sinus. The patient underwent surgical correction. We report the investigation, treatment, and review of literatureof this rare congenital anomaly associated with dilated circumflex coronary artery.
| S-EPMC4070206 | biostudies-other