Tracheobronchopexy to Avoid Tracheostomy in Esophageal Atresia Patients With Severe Life-Threatening Tracheobronchomalacia.

Background: Esophageal atresia (EA) is associated with tracheobronchomalacia (TBM), which in its most severe form, causes blue spells, brief resolved unexplained events (BRUEs) that can require cardiopulmonary resuscitation (CPR), and positive pressure ventilation (PPV) or ventilator dependence, often requiring tracheostomy. We study the role of tracheobronchopexy, as an alternative to tracheostomy, in EA patients with severe life-threatening TBM.

Methods: We reviewed EA patients who underwent tracheobronchopexy for blue spells, BRUEs, and failure to wean PPV or extubate from February 2013 to September 2021 at two institutions.

Management Strategies and Outcomes of Distal Congenital Esophageal Strictures in the Setting of Long-gap Esophageal Atresia.

Background: The management of neonates with long-gap esophageal atresia (LGEA) combined with distal congenital esophageal strictures (CES) is challenging. We sought to review our approach for this rare set of anomalies.

Methods: We reviewed children with LGEA + CES surgically treated at two institutions (2018-2024).

Botulinum Toxin Enhanced Foker Process for Long Gap Esophageal Atresia.

Background: The traction-induced esophageal growth (Foker) process for the treatment of long gap esophageal atresia (LGEA) relies on applying progressive tension to the esophagus to induce growth. Due to its anti-fibrotic and muscle-relaxing properties, we hypothesize that Botulinum Toxin A (BTX) can enhance traction-induced esophageal growth.

Methods: A retrospective two-center cohort study was conducted on children who underwent a BTX-enhanced Foker process for LGEA repair from 2021 to 2023.

Negative pressure suction test: An intraoperative airway maneuver to assess effectiveness of surgical correction of tracheobronchomalacia.

Background: Surgical correction of tracheobronchomalacia (TBM) has evolved greatly over the past decade, with select pediatric institutions establishing dedicated surgery and anesthesia teams to navigate the complexities and challenges of surgical airway repairs. Although anesthetic techniques have evolved internally over many years to improve patient safety and outcomes, many of these methods remain undescribed in literature.

Technique: In this article, we describe the intraoperative negative pressure suction test.

Primary Posterior Tracheopexy at Time of Esophageal Atresia Repair Significantly Reduces Respiratory Morbidity.

Purpose: Esophageal atresia with tracheoesophageal fistula (EA/TEF) is often associated with tracheobronchomalacia (TBM), which contributes to respiratory morbidity. Posterior tracheopexy (PT) is an established technique to treat TBM that develops after EA/TEF repair. This study evaluates the impact of primary PT at the time of initial EA/TEF repair.

Surgical Treatment of Esophageal Anastomotic Stricture After Repair of Esophageal Atresia.

Background: Anastomotic strictures (AS) after esophageal atresia (EA) repair are common. While most respond to endoscopic therapy, some become refractory and require surgical intervention, for which the outcomes are not well established.

Methods: All EA children with AS who were treated surgically at two institutions (2011-2022) were retrospectively reviewed.

Treatment of Esophageal Perforation: Endoscopic Vacuum-Assisted Closure.

Surgical repair of type C esophageal atresia (EA) with distal tracheoesophageal fistula (TEF) is complicated by an anastomotic leak in 10%-30% of cases with associated morbidity. A novel procedure in the pediatric population, endoscopic vacuum-assisted closure (EVAC), accelerates the healing of esophageal leaks by using the effects of VAC therapy, including fluid removal and stimulation of granulation tissue formation. We report 2 additional cases of chronic esophageal leak treated with EVAC in EA patients.

Esophageal Surveillance Practices in Esophageal Atresia Patients: A Survey by the Eastern Pediatric Surgery Network.

Introduction: Endoscopic surveillance guidelines for patients with repaired esophageal atresia (EA) rely primarily on expert opinion. Prior to embarking on a prospective EA surveillance registry, we sought to understand EA surveillance practices within the Eastern Pediatric Surgery Network (EPSN).

Methods: An anonymous, 23-question Qualtrics survey was emailed to 181 physicians (surgeons and gastroenterologists) at 19 member institutions.

Evolution of left-sided thoracoscopic approach for long gap esophageal atresia repair.

Background: Left-sided repair for long gap esophageal atresia (LGEA) has been described for patients with a large leftward upper pouch, no thoracic tracheoesophageal fistula (TEF) nor tracheobronchomalacia (TBM), or as salvage plan after prior failed right-sided repair. We describe our experience with left-sided MIS traction induced growth process.

Methods: We retrospectively reviewed patients who underwent Foker process for LGEA at two institutions between December 2016 and November 2021.

Endobronchial mucoepidermoid carcinoma in a pediatric patient: A case report.

Mucoepidermoid carcinoma (MEC) is an uncommon type of salivary gland tumor that can present as an endobronchial neoplasm, most commonly in the adult population. Neuroendocrine carcinoid tumors comprise the majority of bronchial neoplasms in the pediatric population and are nearly indistinguishable from MEC on imaging. We present a rare case of MEC in a 3-year-old presenting with recurrent symptoms of lower airway obstruction and discuss its typical associated symptoms and imaging features.

Cautionary tales in the use of magnets for the treatment of long gap esophageal atresia.

Background: The use of magnets for the treatment of long gap esophageal atresia or "magnamosis" is associated with increased incidence of anastomotic strictures; however, little has been reported on other complications that may provide insight into refining selection criteria for appropriate use.

Methods: A single institution, retrospective review identified three cases referred for treatment after attempted magnamosis with significant complications. Their presentation, imaging, management, and outcomes were reviewed.

Non-operative management of extralobar pulmonary sequestration: a safe alternative to resection?

Purpose: This retrospective cohort study compares the natural history of patients with extralobar sequestrations (ELS) who do not undergo intervention with those who undergo resection to assess the safety of non-operative management.

Methods: 126 patients with pulmonary sequestrations or congenital pulmonary airway malformations born between 1999 and 2016 were identified. 49 patients had ELS on postnatal imaging, but two were excluded for associated congenital diaphragmatic hernia.

Prenatal imaging throughout gestation in Beckwith-Wiedemann syndrome.

Purpose: Prenatal occurrence and timing of appearance of associated features in Beckwith-Wiedemann syndrome (BWS) are unknown. We reviewed our BWS patients with serial fetal imaging and correlated these with postnatal findings.

Methods: All BWS patients with fetal ultrasound (US) or magnetic resonance imaging (MRI) from 2000 to 2016 were reviewed to determine the presence of polyhydramnios, placentamegaly, macrosomia, macroglossia, retrognathia, omphalocele, visceromegaly, and hemihypertrophy.

Transamniotic stem cell therapy (TRASCET) in a rabbit model of spina bifida.

Purpose: Transamniotic stem cell therapy (TRASCET) with select mesenchymal stem cells (MSCs) has been shown to induce partial or complete skin coverage of spina bifida in rodents. Clinical translation of this emerging therapy hinges on its efficacy in larger animal models. We sought to study TRASCET in a model requiring intra-amniotic injections 60 times larger than those performed in the rat.

Descending Aortopexy and Posterior Tracheopexy for Severe Tracheomalacia and Left Mainstem Bronchomalacia.

Posterior descending aortopexy can relieve posterior intrusion of the left mainstem bronchus that may limit the effectiveness of posterior tracheobronchopexy. We review outcomes of patients undergoing both descending aortopexy and posterior tracheopexy for severe tracheobronchomalacia with posterior intrusion and left mainstem compression to determine if there were resolution of clinical symptoms and bronchoscopic evidence of improvement in airway collapse. All patients who underwent both descending aortopexy and posterior tracheopexy from October 2012 to October 2016 were retrospectively reviewed.

Posterior Tracheopexy for Severe Tracheomalacia Associated with Esophageal Atresia (EA): Primary Treatment at the Time of Initial EA Repair versus Secondary Treatment.

Purpose: We review outcomes of posterior tracheopexy for tracheomalacia in esophageal atresia (EA) patients, comparing primary treatment at the time of initial EA repair versus secondary treatment.

Methods: All EA patients who underwent posterior tracheopexy from October 2012 to September 2016 were retrospectively reviewed. Clinical symptoms, tracheomalacia scores, and persistent airway intrusion were collected.

Long-gap esophageal atresia.

The management of long-gap esophageal atresia remains challenging with limited consensus on the definition, evaluation, and surgical approach to treatment. Efforts to preserve the native esophagus have been successful with delayed primary anastomosis and tension-based esophageal growth induction processes. Esophageal replacement is necessary in a minority of cases, with the conduit of choice and patient outcomes largely dependent on institutional expertise.