Outcomes of surgical treatment of tracheobronchomalacia in children.

BACKGROUND: Tracheobronchomalacia (TBM) is characterized by excessive dynamic airway collapse. Severe TBM can be associated with substantial morbidity. Children with secondary TBM associated with esophageal atresia/tracheoesophageal fistula (EA/TEF) and vascular-related airway compression (VRAC) demonstrate clinical improvement following airway pexy surgery. It is unclear if children with severe primary TBM, without secondary etiologies (EA/TEF, vascular ring, intrinsic pulmonary pathology, or complex cardiac disease) demonstrate clinical improvement following airway pexy surgery. MATERIALS AND METHODS: The study cohort consisted of 73 children with severe primary TBM who underwent airway pexy surgery between 2013 and 2020 at Boston Children's Hospital. Pre- and postoperative symptoms as well as bronchoscopic findings were compared with Fisher exact test for categorical data and Student's t-test for continuous data. RESULTS: Statistically significant improvements in clinical symptoms were observed, including cough, noisy breathing, prolonged respiratory infections, pneumonias, exercise intolerance, cyanotic spells, brief resolved unexplained events (BRUE), and noninvasive positive pressure ventilation (NIPPV) dependence. No significant differences were seen regarding oxygen dependence, ventilator dependence, or respiratory distress requiring NIPPV. Comparison of pre- and postoperative dynamic bronchoscopy findings revealed statistically significant improvement in the percent of airway collapse in all anatomic locations except at the level of the upper trachea (usually not malacic). Despite some initial improvements, 21 (29%) patients remained symptomatic and underwent additional airway pexies with improvement in symptoms. CONCLUSION: Airway pexy surgery resulted in significant improvement in clinical symptoms and bronchoscopic findings for children with severe primary TBM; however, future prospective and long-term studies are needed to confirm this benefit.

Pulling instead of pushing: A case report of gastrostomy-assisted pull technique as an alternative method for endoluminal sponge placement in EVAC therapy.

Endoscopic vacuum-assisted therapy offers an easier and safer alternative to thoracic surgery, self-expanding stents, or esophageal clips and has been shown to be a promising technique for management of pediatric esophageal perforations. In this report, we present a novel application of a percutaneous endoscopic gastrostomy-assisted pull technique, wherein a preexisting gastrostomy is reaccessed to allow safe placement of the vacuum sponge with a more comfortable and effective endoscopic vacuum-assisted closure therapy compared to transnasal or transoral options. A 7-year-old male with a history of type C esophageal atresia with distal tracheoesophageal fistula complicated by leak and refractory esophageal stricture, severe tracheomalacia, and prior esophageal stricture resection presented for posterior tracheoplasty and tracheopexy complicated by esophageal perforation. A preexisting gastrostomy site was re-accessed to allow for a novel approach for endoluminal sponge placement in endoscopic vacuum-assisted closure (EVAC) therapy by gastrostomy-assisted pull technique. The patient had appropriate healing without further leak 1 month after repair. This case highlights the use of EVAC as a minimally invasive option for repair of esophageal perforation using a pull-through method at the percutaneous endoscopic gastrostomy tube site as gastric access. This method may improve control of placement and reduce sponge migration, reduce intraluminal distance of sponge placement, and reduce morbidity by avoiding thoracotomy.

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. METHODS: Review of all newborn primary EA/TEF repairs (2016-2021) at two institutions. Long-gap EA and reoperative cases were excluded. Based on surgeon preference and preoperative bronchoscopy, neonates underwent primary PT (EA + PT Group) or not (EA Group). Perioperative, respiratory and nutritional outcomes within the first year of life were evaluated. RESULTS: Among 63 neonates, 21 (33%) underwent PT during EA/TEF repair. Groups were similar in terms of demographics, approach, and complications. Neonates in the EA + PT Group were significantly less likely to have respiratory infections requiring hospitalization within the first year of life (0% vs 26%, p = 0.01) or blue spells (0% vs 19%, p = 0.04). Also, they demonstrated improved weight-for-age z scores at 12 months of age (0.24 vs -1.02, p < 0.001). Of the infants who did not undergo primary PT, 10 (24%) developed severe TBM symptoms and underwent tracheopexy during the first year of life, whereas no infant in the EA + PT Group needed additional airway surgery (p = 0.01). CONCLUSION: Incorporation of posterior tracheopexy during newborn EA/TEF repair is associated with significantly reduced respiratory morbidity within the first year of life. LEVEL OF EVIDENCE: Level III.

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. This simulates the negative pressure seen in awake and spontaneously breathing patients, including the higher pressures seen during coughing which induce airway collapse in patients with TBM. Also known as the Munoz maneuver in surgical literature, this test has been performed on over 300 patients since 2015. DISCUSSION: The negative pressure suction test allows for controlled intraoperative assessment of surgical airway repairs, replaces the need for risky intraoperative wake-up tests, increases the chances of a successful surgical repair, and improves anesthetic management for emergence and extubation. We provide a guide on how to perform the test and videos demonstrating its efficacy in intraoperative airway evaluation. CONCLUSIONS: As surgeries to repair TBM become more prevalent in other pediatric institutions, we believe that pediatric patients and anesthesia providers will benefit from the insights and methods described here.

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. Surgical repair was performed for those with AS that were either refractory to endoscopic therapy or clinically symptomatic and undergoing surgery for another indication. Anastomotic leak, need for repeat stricture resection, and esophageal replacement were considered poor outcomes. RESULTS: 139 patients (median age: 12 months, range 1.5 months-20 years; median weight: 8.1 kg) underwent 148 anastomotic stricture repairs (100 refractory, 48 non-refractory) in the form of stricturoplasty (n = 43), segmental stricture resection with primary anastomosis (n = 96), or stricture resection with a delayed anastomosis after traction-induced lengthening (n = 9). With a median follow-up of 38 months, most children (92%) preserved their esophagus, and the majority (83%) of stricture repairs were free of poor outcomes. Only one anastomotic leak occurred in a non-refractory stricture. Of the refractory stricture repairs (n = 100), 10% developed a leak, 9% required repeat stricture resection, and 13% required esophageal replacement. On multivariable analysis, significant risk factors for any type of poor outcome included anastomotic leak, stricture length, hiatal hernia, and patient's weight. CONCLUSIONS: Surgery for refractory AS is associated with inherent yet low morbidity and high rates of esophageal preservation. Surgical repair of non-refractory symptomatic AS at the time of another thoracic operation is associated with excellent outcomes. LEVEL OF EVIDENCE: Level III.

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. Likert scale questions gauged agreement with international EA surveillance guideline-derived statements. Multiple-choice questions assessed individual and institutional practices. RESULTS: The response rate was 77%. Most respondents (80%) strongly agree or agree that EA surveillance endoscopy should follow a set schedule, while only 36% claimed to perform routine upper GI endoscopy regardless of symptoms. Many institutions (77%) have an aerodigestive clinic, even if some lack a multi-disciplinary EA team. Most physicians (72%) expressed strong interest in helping develop evidence-based guidelines. CONCLUSIONS: Our survey reveals physician agreement with current guidelines but weak adherence. Surveillance methods vary greatly, underscoring the lack of evidence-based data to guide EA care. Aerodigestive clinics may help implement surveillance schedules. Respondents support evidence-based protocols, which bodes well for care standardization. Results will inform the first multi-institutional EA databases in the United States (US), which will be essential for evidence-based care. LEVEL OF EVIDENCE: This is a prognosis study with level 4 evidence.

30-Day Outcomes Following Esophageal Replacement in Children: A National Surgical Quality Improvement Project Pediatric Analysis.

INTRODUCTION: The optimal method of esophageal replacement remains controversial. The aim of this study was to evaluate 30-d outcomes of children in the National Surgical Quality Improvement Project Pediatric (NSQIP-P) database who underwent esophageal replacement from 2012 to 2018. METHODS: Demographics, comorbidities, and procedural technique was identified in NSQIP-P and reviewed. Thirty-day outcomes were assessed and stratified by gastric pull-up or tube interposition versus small bowel or colonic interposition. Categorical and continuous variables were assessed by Pearson's chi-square, Fisher's exact, and Wilcoxon rank-sum tests, respectively. Multivariate logistic regression was performed to estimate the effects of procedure technique and clinical risk factors on patient outcomes. RESULTS: Of the 99 cases of esophageal replacement included, 52 (52.5%) utilized a gastric conduit, whereas 47 (47.5%) involved small bowel/colonic esophageal interposition. Overall risk of complications was 52.5%, the most common of which were perioperative transfusion (30.3%), surgical site infection (11.1%), and sepsis (9.1%). Risk of unplanned reoperation was 17.2%, and risk of mortality was 3.0%. Risk for complications, reoperation, and readmission did not differ significantly between those who underwent gastric esophageal replacement and those who underwent small bowel or colonic interposition. Median operative time was shorter in the gastric esophageal replacement group (5.2 versus 8.1 h, P = 0.009). CONCLUSIONS: Among children in NSQIP-P who underwent esophageal replacement from 2012 to 2018, the risk of 30-d complications, unplanned reoperation, and mortality was relatively frequent and was similar across operative techniques. Opportunities exist to improve preoperative optimization, utilization of blood transfusion services, and infectious complications in the perioperative period irrespective of operative technique. LEVEL OF EVIDENCE: Level III, retrospective comparative study.

When to consider a posterolateral descending aortopexy in addition to a posterior tracheopexy for the surgical treatment of symptomatic tracheobronchomalacia.

PURPOSES: The descending thoracic aorta typically crosses posterior to the left mainstem bronchus (LMSB). We sought to evaluate patient factors that may lead one to consider a posterolateral descending thoracic aortopexy (PLDA) in addition to a posterior tracheopexy (PT) in the surgical treatment of symptomatic tracheobronchomalacia (TBM) that involves the LMSB. METHODS: Retrospective review of patients who underwent PT with or without PLDA between 2012 and 2017. Severity and extent of TBM were assessed using dynamic tracheobronchoscopy. Aortic positioning compared to the anterior border of the spine (ABS) at the level of the left mainstem bronchus was identified on computed tomography (CT). Factors associated with performing a PLDA were evaluated with logistic regression. RESULTS: Of 188 patients who underwent a PT, 70 (37%) also had a PLDA performed. On multivariate analysis, >50% LMSB compression on bronchoscopy (OR 8.06, p < 0.001), >50% of the aortic diameter anterior to the ABS (OR 2.06, p = 0.05), and more recent year of surgery (OR 1.61, p = 0.003) were associated with performing a PLDA. CONCLUSION: When performing a PT, a PLDA should be considered for patients who have >50% LMSB compression on dynamic bronchoscopy, and in those with a descending thoracic aorta located >50% anterior to the ABS. LEVEL OF EVIDENCE: III TYPE OF STUDY: Retrospective comparative study.

Great vessel anomalies and their impact on the surgical treatment of tracheobronchomalacia.

BACKGROUND: Tracheobronchial compression (TBC) from great vessel anomalies (GVA) can contribute to tracheobronchomalacia (TBM) symptoms. The frequency, impact on symptoms and optimal management of GVA in these patients, with or without a history of esophageal atresia (EA), are still unclear. STUDY DESIGN: Patients who underwent surgery for TBM/ TBC between 2001 and 2017 were reviewed. Demographics, type of GVA, and operative interventions were collected. The frequency and treatment modalities of GVA between EA and non-EA patients were compared. RESULTS: Overall, 209 patients met criteria; 120 (57.4%) patients had at least one GVA, including double aortic arches (n = 4, 1.9%), right aortic arches (n = 14, 6.7%), aberrant right subclavian arteries (n = 15, 7.2%), and innominate artery compression (n = 71, 34.0%). Non-EA patients were more likely to have surgery later in life (29.5 months versus 16 months, p = 0.0002), double aortic arch (p = 0.0174), right aortic arch (p < 0.0001), and undergo vascular reconstruction concurrently with their airway procedure (25% vs 8.4%, p = 0.002). Vessel reconstruction was performed in 25 patients; six required cardiac bypass. CONCLUSION: The frequency of GVA in patients with symptomatic airway collapse is substantial. Multidisciplinary evaluation is imperative for operative planning as many require complex reconstruction and collaboration with cardiac surgery, particularly patients without a history of EA. LEVEL OF EVIDENCE: Level III.

An experimental study on magnetic esophageal compression anastomosis in piglets.

INTRODUCTION: Fashioning a patent, watertight anastomosis in patients with esophageal atresia is a challenging task in pediatric surgery, particularly when performed under tension. A reproducible suture-less alternative would decrease operative time. We evaluated magnetic esophageal compression anastomoses in a novel bypass-loop swine model. METHODS: Eight-week-old piglets underwent thoracotomy to mobilize the esophagus at the carina to create a U-shaped loop. Custom-made 8 mm diameter Neodymium Magnets were inserted into the esophagus proximal and distal to the loop, then mated side-to-side at the future anastomosis site. Pigs were observed for 8 (n = 4), 10 (n = 6), and 12 (n = 2) days and then sacrificed. The magnetic compression anastomosis was evaluated macroscopically, by radiography, burst pressure testing, and histology. RESULTS: All 12 pigs survived until the endpoint. Separation of the magnets occurred at a median of 9 days. Contrast esophagrams showed patency and no leak. All anastomoses withstood pressures well over 13 kPa without leak. Histopathology showed epithelialized circular scar tissue. CONCLUSION: Magnetic compression anastomoses of the esophagus using our specially-designed magnets are formed between the 8th and 10th postoperative day, are patent and mechanically resistant to supraphysiologic intraluminal pressures. These data lay the basis for a potential clinical application in patients born with esophageal atresia. LEVEL OF EVIDENCE: Not applicable (experimental animal study).

Thoracoscopic esophagomyotomy for achalasia in the pediatric population: A retrospective cohort study.

BACKGROUND/PURPOSE: Achalasia is an extremely rare disease in children (0.1 per 100,000 individuals). Standard treatments for this include pneumatic dilation and esophagomyotomy. Minimally invasive esophagomyotomies have increasingly been used owing to improved postoperative pain and length of stay. We describe our experience with thoracoscopic esophagomyotomy in this population. METHODS: This is a retrospective cohort study of all patients at our institution who underwent thoracoscopic esophagomyotomy for achalasia from 1995 to 2016. We used endoscopic guidance during all procedures. No fundoplication was performed. RESULTS: Thirty-one patients were included in this study. Thirteen patients underwent pneumatic dilations prior to their operation with a median of 3 dilations. Two patients had a mucosal injury during the case. There were no conversions to an open procedure. Median length of stay was 2 days. After the procedure, 97% of patients had initial symptom relief. Eight patients (26%) required postoperative pneumatic dilations for recurrent symptoms; there was a greater chance of this (OR 8.5) if they had a preoperative dilation. No patients required a fundoplication for reflux postoperatively. CONCLUSIONS: Thoracoscopic esophagomyotomy is a safe and effective procedure for achalasia in the pediatric population. It should be considered as an alternative to the laparoscopic approach for these patients. LEVEL OF EVIDENCE: IV.

Vascular Endothelial Growth Factor Enhances Compensatory Lung Growth in Piglets.

BACKGROUND: Vascular endothelial growth factor has been found to accelerate compensatory lung growth after left pneumonectomy in mice. The aim of this study was to determine the natural history and the effects of vascular endothelial growth factor on compensatory lung growth in a large animal model. METHODS: To determine the natural history of compensatory lung growth, female Yorkshire piglets underwent a left pneumonectomy on days of life 10-11. Tissue harvest and volume measurement of the right lung were performed at baseline (n = 5) and on postoperative days 7 (n = 5), 14 (n = 4), and 21 (n = 5). For pharmacokinetic studies, vascular endothelial growth factor was infused via a central venous catheter, with plasma vascular endothelial growth factor levels measured at various time points. To test the effect of vascular endothelial growth factor on compensatory lung growth, 26 female Yorkshire piglets underwent a left pneumonectomy followed by daily infusion of vascular endothelial growth factor at 200 µg/kg or isovolumetric 0.9% NaCl (saline control). Lungs were harvested on postoperative day 7 for volume measurement and morphometric analyses. RESULTS: Compared with baseline, right lung volume after left pneumonectomy increased by factors of 2.1 ± 0.6, 3.3 ± 0.6, and 3.6 ± 0.4 on postoperative days 7, 14, and 21, respectively. The half-life of VEGF ranged from 89 to 144 minutes. Lesser doses of vascular endothelial growth factor resulted in better tolerance, volume of distribution, and clearance. Compared with the control group, piglets treated with vascular endothelial growth factor had greater lung volume (P < 0.0001), alveolar volume (P = 0.001), septal surface area (P = 0.007) and total alveolar count (P = 0.01). CONCLUSION: Vascular endothelial growth factor enhanced alveolar growth in neonatal piglets after unilateral pneumonectomy.

Risk factors for recurrence after thoracoscopic repair of congenital diaphragmatic hernia (CDH).

PURPOSE: To identify technical modifications concerning factors that may lower the risk of recurrence following thoracoscopic repair of congenital diaphragmatic hernia (CDH). METHODS: All CDH patients who underwent thoracoscopic repair from April 2003 to September 2017 were retrospectively reviewed. Some of the more recently treated patients underwent technically modified repairs with underlay and overlay buttresses. RESULTS: Sixty-eight patients underwent thoracoscopic repair of a diaphragmatic hernia that presented either neonatally (n = 52) or beyond the neonatal period (>1 month) (n = 16). At our institution, the minimally invasive surgical approach is considered for clinically stable CDH patients, who are likely to have type A or B defects. 21 patients had a sac-type defect. Forty-seven patients with type A defect had primary closure, buttressed in 6 cases. In 21 patients, the type B defect was repaired with a patch, buttressed in 11 patients. Median follow-up was 36 months (IQR 9-45). Recurrence occurred in 13 patients (overall 19% recurrence rate); all had a neonatally presented defect (25% vs. 0%, p = 0.03). Patients with a sac-type defect had a lower recurrence rate than patients with no hernia sac (5% vs. 26%, p = 0.05). Recurrence complicated 7 of 47 (15%) patients after primary closure and 6 of 21 (29%) patients with patch repair; none of the 17 cases with buttressed repairs had a recurrence. CONCLUSIONS: Due to a higher rate of recurrence following thoracoscopic CDH repair compared to the standard open approach, we suggest a sandwich-type buttress repair with underlay and overlay components for both primary and patch repairs. LEVEL OF EVIDENCE: Level III cohort study.

Minimally Invasive Surgical Approach for Posterior Tracheopexy to Treat Severe Tracheomalacia: Lessons Learned from Initial Case Series.

Background/Purpose: Posterior tracheopexy directly addresses membranous tracheal intrusion in severe tracheomalacia (TM). We have previously reported our experience of posterior tracheopexy through open approach in a large series of patients. This study aimed to review lessons learned from our initial series posterior tracheopexy through the video-assisted or robotic video-assisted thoracoscopic approach. Methods: We retrospectively reviewed our single-institution experience on all patients who underwent video-assisted or robotic video-assisted posterior tracheopexy for treatment of symptomatic TM between October 2016 and February 2018. Results: Ten patients underwent video-assisted (n = 4) or robotic video-assisted (n = 6) thoracoscopic posterior tracheopexy (age range: 8 months-19 years). One patient, the youngest in our cohort, had a type C esophageal atresia repair; none of the other patients had undergone prior thoracic operations. All had symptomatic TM; 1 patient with tracheostomy dependence also had severe distal bronchomalacia, including segmental airways. Postoperatively, patients were hospitalized 3-7 days, with 1-2 days for ICU observation. Improved respiratory symptoms were noted in 9/10 patients following the operation. The 1 patient with preexisting tracheostomy had reduced ventilator dependence but remained hospitalized with ongoing respiratory symptoms. Conclusions: The thoracoscopic approach for posterior tracheopexy, while challenging, can be applied to a select cohort of children with severe TM. Thoracoscopic surgery with robotic assistance can eliminate some technical limitations of the video-assisted approach by providing an easier platform for more complicated suturing angles.

Slide Esophagoplasty vs End-to-End Anastomosis for Recalcitrant Esophageal Stricture after Esophageal Atresia Repair.

BACKGROUND: Anastomotic stricture is a common complication after esophageal atresia (EA) repair. Patients with a recalcitrant stricture may require surgical intervention. The technique of reanastomosis after stricture resection can affect patient outcomes. STUDY DESIGN: Patients with EA who underwent anastomotic stricture resection, from July 2010 to February 2017, were reviewed. After stricture resection, patients who had slide esophagoplasty performed were compared with those having conventional end-to-end anastomosis. RESULTS: Fifty patients underwent stricture repair surgery by slide esophagoplasty (n = 12) or end-to-end (n = 38) anastomosis technique at a median age of 14 months (interquartile range [IQR] 6 to 23 months). Significantly fewer patients required dilation therapy after slide esophagoplasty: 6 of 12 (50%) compared with 32 of 38 (84%) in the end-to-end group (p = 0.02). The number of dilation sessions was significantly lower in the slide group vs the end-to-end (p = 0.004) group, with a risk ratio confirming the approximately half the number of dilations for the slide approach (risk ratio 0.57, 95% CI 0.38 to 0.86). Steroid injection was combined with dilation in 3 of 12 (25%) vs 22 of 38 (58%) in the slide and end-to-end groups, respectively (p = 0.10). Stent placement was used in none of slide cases vs 8 of 38 (21%) in the end-to-end group (p = 0.17). Stricture incision was performed in 1 of 12 (8%) in the slide group and 11 of 38 (29%) in the end-to-end group (p = 0.25). There were leak complications in fewer patients after slide esophagoplasty compared with end-to-end anastomosis: 1 of 12 (8%) vs 8 of 38 (21%) (p = 0.43). CONCLUSIONS: Slide esophagoplasty may be a useful technique of anastomotic configuration for selected patients with recalcitrant esophageal stricture, offering more favorable outcomes compared with end-to-end anastomosis.

Combined en bloc liver-double kidney transplantation in an infant with IVC thrombosis.

We report a case of a pediatric en bloc liver-double kidney transplant in a patient with IVC thrombosis below the renal veins. The patient is an 11-month-old girl diagnosed with congenital nephrotic syndrome at two months of age. Multifocal liver masses were identified on routine ultrasound at eight months of age. Alpha fetoprotein level was 55 319. Biopsy confirmed hepatoblastoma. CT scan confirmed multiple lesions in both lobes, which would require liver transplantation for resection. She was also found to have thrombosis of her infrarenal IVC secondary to multiple central lines. She was listed for combined liver-kidney transplant and began chemotherapy. After four cycles of chemotherapy, she underwent bilateral nephrectomies followed by a combined en bloc liver-double kidney transplant from a size matched donor. In order to provide adequate venous outflow from the kidneys in the absence of a recipient infrarenal IVC, the donor liver and kidneys were procured en bloc with a common arterial inflow via the infrarenal aorta and common outflow via the suprahepatic IVC. Kidney transplantation in the absence of adequate recipient venous drainage may require unusual vascular reconstruction techniques. This case demonstrates a novel approach in patients who may require combined liver-kidney transplantation.