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.

Innovative management of severe tracheobronchomalacia using anterior and posterior tracheobronchopexy.

OBJECTIVES/HYPOTHESIS: Combined anterior and posterior tracheobronchopexy is a novel surgical approach for the management of severe tracheobronchomalacia (TBM). We present our institutional experience with this procedure. Our objective was to determine the utility and safety of anterior and posterior tracheopexy in the treatment of severe TBM. STUDY DESIGN: Retrospective chart review. METHODS: All patients who underwent anterior and posterior tracheopexy from January 2013 to July 2017 were retrospectively reviewed. Charts were reviewed for indications, preoperative work-up, tracheobronchomalacia classification and severity, procedure, associated syndromes, synchronous upper aerodigestive tract lesions, and aberrant thoracic vessels. Main outcomes measured included improvement in respiratory symptoms, successful extubation and/or decannulation, vocal fold immobility, and new tracheotomy placement. RESULTS: Twenty-five patients underwent anterior and posterior tracheopexy at a mean age of 15.8 months (range, 2-209 months; mean, 31 months if 2 outliers of 206 and 209 months included). Mean length of follow-up was 26.8 months (range, 13-52 months). Indications for surgery included apneic events, ventilator dependence, need for positive pressure ventilation, tracheotomy dependence secondary to TBM, recurrent pneumonia, and exercise intolerance. Many patients had other underlying syndromes and synchronous upper aerodigestive tract lesions (8 VACTERL, 2 CHARGE, 1 trisomy 21, 1 Feingold syndrome, 17 esophageal atresia/tracheoesophageal fistula, 20 cardiac/great vessel anomalies, 1 subglottic stenosis, 1 laryngomalacia, 7 laryngeal cleft). At preoperative bronchoscopy, 21 of 25 patients had >90% collapse of at least one segment of their trachea, and the remaining four had 70% to 90% collapse. Following anterior and posterior tracheopexy, one patient developed new bilateral vocal-fold immobility; one patient with a preoperative left cord paralysis had a new right vocal-fold immobility. Postoperatively, most patients had significant improvement in their respiratory symptoms (21 of 25, 84%) at most recent follow-up. Three patients with preexisting tracheotomy were decannulated; two patients still had a tracheotomy at last follow-up. Two patients required new tracheotomy for bilateral vocal-fold immobility. CONCLUSIONS: Combined anterior and posterior tracheopexy is a promising new technique for the surgical management of severe TBM. Further experience and longer follow-up are needed to validate this contemporary approach and to minimize the risk of recurrent laryngeal nerve injury. LEVEL OF EVIDENCE: 4 Laryngoscope, 130:E65-E74, 2020.

Esophagitis in Pediatric Esophageal Atresia: Acid May Not Always Be the Issue.

OBJECTIVE: Esophagitis is highly prevalent in patients with esophageal atresia (EA). Peptic esophagitis has long been assumed to be the primary cause of esophagitis in this population, and prolonged acid suppressive medication usage is common; such treatment is of unknown benefit and carries potential risk. METHODS: To better understand the role of commonly used antireflux treatments in EA, we analyzed all patients with repaired EA who underwent endoscopy with biopsies at our institution between January 2016 and August 2018. Macroscopic erosive and histologic esophagitis on biopsy was graded per predefined criteria. Clinical characteristics including acid suppressive medication usage, type of EA and repair, presence of hiatal hernia, and history of fundoplication were reviewed. RESULTS: There were 310 unique patients (33.5% long gap EA) who underwent 576 endoscopies with biopsies during the study period. Median age at endoscopy was 3.7 years (interquartile range 21-78 months). Erosive esophagitis was found in 8.7% of patients (6.1% of endoscopies); any degree of histologic eosinophilia (≥1 eosinophil/high power field [HPF]) was seen in 56.8% of patients (48.8% of endoscopies), with >15 eosinophils/HPF seen in 15.2% of patients (12.3% of endoscopies). Acid suppression was common; 86.9% of endoscopies were preceded by acid suppressive medication use. Fundoplication had been performed in 78 patients (25.2%). Proton pump inhibitor (PPI) and/or H2 receptor antagonist (H2RA) use were the only significant predictors of reduced odds for abnormal esophageal biopsy (P = 0.011 for PPI, P = 0.048 for H2RA, and P = 0.001 for PPI combined with H2RA therapy). However, change in intensity of acid suppressive therapy by either dosage or frequency was not significantly associated with change in macroscopic erosive or histologic esophagitis (P > 0.437 and P > 0.13, respectively). Presence or integrity of a fundoplication was not significantly associated with esophagitis (P = 0.236). CONCLUSIONS: In EA patients, acid suppressive medication therapy is associated with reduced odds of abnormal esophageal biopsy, though histologic esophagitis is highly prevalent even with high rates of acid suppressive medication use. Esophagitis is likely multifactorial in EA patients, with peptic esophagitis as only one of multiple possible etiologies for esophageal inflammation. The clinical significance of histologic eosinophilia in this population warrants further investigation.

Technique and perioperative management of left pneumonectomy in neonatal piglets.

BACKGROUND: Although commonly performed in adult swine, unilateral pneumonectomy in piglets requires significant modifications in the surgical approach and perioperative care because of their smaller size and limited physiological reserve. METHODS: Nineteen neonatal piglets underwent a left pneumonectomy. They were allowed 5-7 d of preoperative acclimation and nutritional optimization. Preoperative weight gain and laboratory values were obtained before the time of surgery. A "ventro-cranial" approach is adopted where components of the pulmonary hilum were sequentially identified and ligated, starting from the most ventral and cranial structure, the superior pulmonary vein. The principle of gentle ventilation was followed throughout the entire operation. RESULTS: The median age of the piglets at the time of surgery was 12 (10-12) d. The median preoperative weight gain and albumin level were 20% (16-26%) and 2.3 (2.1-2.4) g/dL, respectively. The median operative time was 59 (50-70) min. Five of the first nine piglets died from complications, two from poor preoperative nutritional optimization (both with <10% weight gain and 2 g/dL for albumin), one from an intubation complication, one from intra-operative bleeding, and one in the postoperative period from a ruptured bulla. No mortality occurred for the next 10 cases. CONCLUSIONS: Successful outcomes for unilateral pneumonectomy in piglets require special attention to preoperative nutritional optimization, gentle ventilation, and meticulous surgical dissection. Preoperative weight gain and albumin levels should be used to identify appropriate surgical candidates. The "ventro-cranial" approach allows for a technically straightforward completion of the procedure.

Jejunal Interposition after Failed Esophageal Atresia Repair.

BACKGROUND: The early outcomes of using jejunal interpositions to establish esophageal continuity in patients who have had a failed repair of esophageal atresia (EA) were determined. STUDY DESIGN: This was a retrospective review of all patients treated at our institution with a jejunal interposition after a failed EA repair from 2010 to 2015. Demographics, anatomy encountered, operative techniques, requirement for microvascular support, and length of stay were analyzed. Outcomes measures included conduit survival, as well as feeding status at last follow-up. RESULTS: Ten patients were reviewed. Median age at time of interposition operation was 48 months (range 8 to 276 months) and median weight was 14.2 kg (range 7.2 to 49.7 kg). Preoperative anatomy, operative techniques, and outcomes are presented. Four patients had microvascular "supercharging" for a long jejunal graft. Median follow-up was 1.5 years (range 0.5 to 5 years) with no long-term loss of graft or deaths. Six patients are eating by mouth completely, 1 by mouth primarily with supplemental night-time feeds, 1 is transitioning from tube to oral feeds, and 2 with functional grafts are fed mostly enterally due to severe oral aversion in 1 and aspiration in 1. CONCLUSIONS: Jejunal interpositions have been used for the past 5 years to establish esophageal continuity after a failed EA repair. All jejunal conduits survived and were joined to the upper esophageal segment. For shorter gaps with a longer upper esophageal pouch, a thoracic esophageal anastomosis was possible without additional vascular support. For longer interpositions into the neck, upper conduit survival might benefit from additional vascular anastomoses (ie, supercharging). To provide adequate space in the mediastinum, the first rib can be removed, as well as a portion of the manubrium to enlarge the pathway into the neck.

Foker process for the correction of long gap esophageal atresia: Primary treatment versus secondary treatment after prior esophageal surgery.

PURPOSE: The Foker process (FP) uses tension-induced growth for primary esophageal reconstruction in patients with long gap esophageal atresia (LGEA). It has been less well described in LGEA patients who have undergone prior esophageal reconstruction attempts. METHODS: All cases of LGEA treated at our institution from January 2005 to April 2014 were retrospectively reviewed. Patients who initially had esophageal surgery elsewhere were considered secondary FP cases. Demographics, esophageal evaluations, and complications were collected. Median time to esophageal anastomosis and full oral nutrition was estimated using the Kaplan-Meier method. Multivariate Cox-proportional hazards regression identified potential risk factors. RESULTS: Fifty-two patients were identified, including 27 primary versus 25 secondary FP patients. Median time to anastomosis was 14 days for primary and 35 days for secondary cases (p<0.001). Secondary cases (p=0.013) and number of thoracotomies (p<0.001) were identified as significant predictors for achieving anastomosis and the development of a leak. Predictors of progression to full oral feeding were primary FP cases (O.R.=17.0, 95% CI: 2.8-102, p<0.001) and patients with longer follow-up (O.R.=1.06/month, 95% CI: 1.01-1.11, p=0.005). CONCLUSIONS: The FP has been successful in repairing infants with primary LGEA, but the secondary LGEA patients proved to be more challenging to achieve a primary esophageal anastomosis. Early referral to a multidisciplinary esophageal center and a flexible approach to establish continuity in secondary patients is recommended. Given their complexity, larger studies are needed to evaluate long-term outcomes and discern optimal strategies for reconstruction.

Direct tracheobronchopexy to correct airway collapse due to severe tracheobronchomalacia: Short-term outcomes in a series of 20 patients.

PURPOSE: Tracheobronchomalacia (TBM) is associated with esophageal atresia, tracheoesophageal fistulas, and congenital heart disease. TBM results in chronic cough, poor mucous clearance, and recurrent pneumonias. Apparent life-threatening events or recurrent pneumonias may require surgery. TBM is commonly treated with an aortopexy, which indirectly elevates trachea's anterior wall. However, malformed tracheal cartilage and posterior tracheal membrane intrusion may limit its effectiveness. This study describes patient outcomes undergoing direct tracheobronchopexy for TBM. METHODS: The records of patients that underwent direct tracheobronchopexy at our institution from January 2011 to April 2014 were retrospectively reviewed. Primary outcomes included TBM recurrence and resolution of the primary symptoms. Data were analyzed by McNemar's test for matched binary pairs and logistic regression modeling to account for the endoscopic presence of luminal narrowing over multiple time points per patient. RESULTS: Twenty patients were identified. Preoperative evaluation guided the type of tracheobronchopexy. 30% had isolated anterior and 50% isolated posterior tracheobronchopexies, while 20% had both. Follow-up was 5 months (range, 0.5-38). No patients had postoperative ALTEs, and pneumonias were significantly decreased (p=0.0005). Fewer patients had tracheobronchial collapse at postoperative endoscopic exam in these anatomical regions: middle trachea (p=0.01), lower trachea (p<0.001), and right bronchus (p=0.04). CONCLUSION: The use of direct tracheobronchopexy resulted in ALTE resolution and reduction of recurrent pneumonias in our patients. TBM was also reduced in the middle and lower trachea and right mainstem bronchus. Given the heterogeneity of our population, further studies are needed to ascertain longer-term outcomes and a grading scale for TBM severity.

Restoring esophageal continuity following a failed colonic interposition for long-gap esophageal atresia.

The Foker process is a method of esophageal lengthening through axial tension-induced growth, allowing for subsequent primary reconstruction of the esophagus in esophageal atresia (EA). In this unique case, the Foker process was used to grow the remaining esophageal segment long enough to attain esophageal continuity following failed colonic interpositions for long-gap esophageal atresia (LGEA). Initially developed for the treatment of LGEA in neonates, this case demonstrates that (i) an active esophageal lengthening response may still be present beyond the neonate time-period; and, (ii) the Foker process can be used to restore esophageal continuity following a failed colonic interposition if the lower esophageal segment is still present.