Factors Associated With Long-term Survival in Children With Bronchial and Lung Carcinoid Tumors.

BACKGROUND AND AIMS: Bronchial carcinoids are rare in children and the treatment is based on tumor behavior in adults. The purpose of this study was to determine factors and management strategies associated with long-term survival in the pediatric population using a national cohort. METHODS: Patients aged ≤20 years with bronchial carcinoid tumors were identified in the 2004-2020 National Cancer Database using ICD-O-3 codes. Tumor characteristics and management were compared among typical (TC) and atypical (AC) histological subtypes using Chi-square and Fisher's exact tests. Kaplan-Meier and univariate Cox proportional hazards analyses were used to assess survival. RESULTS: Of 273 patients, 251 (92%) had TCs, and 22(8%) had ACs. The median (IQR) age was 18 (16,19) years. Most patients underwent lobectomy or bilobectomy (67%), followed by sublobar resection (17%), no resection or bronchoscopic excision or ablation (8%), and pneumonectomy (7.7%). Margins were negative in 96%. Lymph node (LN) assessment was performed in 216 patients (84%) with a median (IQR) of 7(3,13) LNs, and 50 (23%) had ≥1 positive LN. There was no difference in age, resection, margin status, LN assessment, or positivity between TC and AC (all p > 00.05). Detection of nodal metastasis did not increase beyond the resection of 1-3 LNs (p = 0.72). Ten-year survival was worse for AC than TC (79% (41, 100) vs 98% (95, 100), HR = 6.9 (95% CI: 1.2-38.3, p = 0.03). Ten-year survival among those with and without LN assessment was 97% (94, 100) vs 91% (81, 100), HR = 4.0, 95% CI: 0.8-19.9, p = 0.09). There were no deaths in those with negative LN while 10-year survival was 89% (72, 100) in those with ≥1 positive LN. CONCLUSION: Among children with bronchial carcinoids, survival is excellent with TC or negative LN. Atypical histology and positive LN have poor survival and should prompt close monitoring. These risk factors may be missed in the absence of surgical resection and lymph node sampling. LEVEL OF EVIDENCE: III. TYPE OF STUDY: Retrospective Study.

Endotracheal metallic stent removal: A novel ABC (airway balloon collapse) technique.

To illustrate a previously unreported method of tracheal stent removal that appears to cause less mucosal injury we present a case of a 9-year-old Down syndrome patient with a history of tracheoesophageal fistula, brought to our attention after recurrent bouts of exacerbating cough and tracheo-bronchitis. Endoscopic examination under general anesthesia noted the presence of severe tracheomalacia with inspiratory collapse, and a 10-mm balloon expandable metallic stent (BEMS) was deployed and symptomatic improvement was noted. The initial stent was then removed to consider a definitive procedure using the typical grasping fashion with an alligator forceps and expected mucosal excoriation was noted. Due to symptom recurrence, the patient underwent placement of a second BEMS stent. Initial improvement was noted followed by recurrent episodes of respiratory distress due to granulation tissue formation and stent compression and a decision to remove the stent was made. A new method of stent removal deemed ABC (airway balloon collapse) method was utilized where an expandable airway balloon is placed outside the stent between the stent and tracheal wall and then inflated to collapse the stent, facilitating easy removal.

Sequential Minimally Invasive Fetal Interventions for Two Life-Threatening Conditions: A Novel Approach.

INTRODUCTION: In utero interventions are performed in fetuses with "isolated" major congenital anomalies to improve neonatal outcomes and quality of life. Sequential in utero interventions to treat 2 anomalies in 1 fetus have not yet been described. CASE PRESENTATION: Here, we report a fetus with a large left-sided intralobar bronchopulmonary sequestration (BPS) causing mediastinal shift, a small extralobar BPS, and concomitant severe left-sided congenital diaphragmatic hernia (CDH). At 26-week gestation, the BPS was noted to be increasing in size with a significant reduction in right lung volume and progression to fetal hydrops. The fetus underwent ultrasound-guided ablation of the BPS feeding vessel leading to complete tumor regression. However, lung development remained poor (O/E-LHR: 0.22) due to the left-sided CDH, prompting fetal endoscopic tracheal occlusion therapy at 28-week gestation to allow increased lung growth. After vaginal delivery, the newborn underwent diaphragmatic repair with resection of the extralobar sequestration. He was discharged home with tracheostomy on room air at 9 months. DISCUSSION/CONCLUSION: Sequential in utero interventions to treat 2 severe major anomalies in the same fetus have not been previously described. This approach may be a useful alternative in select cases with otherwise high morbidity/mortality. Further studies are required to confirm our hypothesis.

Endoscopic Resection of a Pediatric Carcinoid Lung Tumor Presenting as Persistent Pneumonia.

Primary lung tumors are very rare in children and constitute only 0.2 percent of all pediatric malignancies. Carcinoids are the most common primary pediatric lung tumor and account for 80 percent of all primary malignant bronchial tumors. Carcinoid tumors can be histologically categorized as typical or atypical. They are derived from neuroendocrine cells in the bronchial epithelium and are locally infiltrative. Surgical resection of endobronchial carcinoid tumors is the mainstay of treatment with a five-year survival of 95 percent. Endoscopic resection has been reported in adult patients with typical carcinoid tumors (less than 20 mm) with no extrabronchial disease. We present the first pediatric bronchial carcinoid tumor treated with endoscopic resection.

Coordinated approach to spinal and tracheal reconstruction in a patient with morquio syndrome.

Morquio syndrome (Mucopolysaccharidosis IVA) is an autosomal recessive lysosomal storage disease with manifestations ranging from mild to severe phenotype. Mechanical spinal cord injury and airway insufficiency are major causes of mortality. A 17-year-old male patient with severe Morquio syndrome presented with cervical and upper thoracic spinal stenosis with spinal cord myelopathy, and progressive severe tracheal stenosis. Coordinated care among otolaryngology, orthopedic surgery, neurosurgery, anesthesiology, cardiovascular surgery, radiology, and pulmonology teams facilitated the successful planning and execution of two major surgical interventions in rapid succession. This is the first description of a successful coordinated spine and airway repair in the literature.

Outcome measures for pediatric laryngotracheal reconstruction: International consensus statement.

OBJECTIVES: Develop multidisciplinary and international consensus on patient, disease, procedural, and perioperative factors, as well as key outcome measures and complications, to be reported for pediatric airway reconstruction studies. METHODS: Standard Delphi methods were applied. Participants proposed items in three categories: 1) patient/disease characteristics, 2) procedural/intraoperative/perioperative factors, and 3) outcome measures and complications. Both general and anatomic site-specific measures were elicited. Participants also suggested specific operations to be encompassed by this project. We then used iterative ranking and review to develop consensus lists via a priori Delphi consensus criteria. RESULTS: Thirty-three pediatric airway experts from eight countries in North and South America, Europe, and Australia participated, representing otolaryngology (including International Pediatric Otolaryngology Group members), pulmonology, general surgery, and cardiothoracic surgery. Consensus led to inclusion of 19 operations comprising open expansion, resection, and slide procedures of the larynx, trachea, and bronchi as well as three endoscopic procedures. Consensus was achieved on multiple patient/comorbidity (10), disease/stenosis (7), perioperative-/intraoperative-/procedure-related (16) factors. Consensus was reached on multiple outcome and complication measures, both general and site-specific (8 general, 13 supraglottic, 15 glottic, 17 subglottic, 8 cervical tracheal, 12 thoracic tracheal). The group was able to clarify how each outcome should be measured, with specific instruments defined where applicable. CONCLUSION: This consensus statement provides a framework to communicate results consistently and reproducibly, facilitating meta-analyses, quality improvement, transfer of information, and surgeon self-assessment. It also clarifies expert opinion on which patient, disease, procedural, and outcome measures may be important to consider in any pediatric airway reconstruction patient. LEVEL OF EVIDENCE: 5 Laryngoscope, 129:244-255, 2019.

Subglottic Stenosis.

Subglottic stenosis refers to narrowing of the airway diameter below the vocal folds and may be congenital or acquired. Typical signs and symptoms range from recurrent croup and exertional stridor to complete airflow obstruction requiring tracheotomy. Management of moderate and severe subglottic stenosis often requires intricate surgical techniques. To optimize the success of these surgeries, a thorough assessment of the child's airway, lungs, reflux, and swallow needs to be evaluated. In order to provide concerted and coordinated care between typically otolaryngology (ENT), pulmonary, gastroenterology (GI), speech, swallow and language pathologists (SLP), "aerodigestive" teams have been developed and increasing in prevalence at children's medical hospitals. This article sets out to provide a brief overview of an aerodigestive program and evaluation, review a few of the more common laryngotracheal conditions, and the surgical techniques involved to augment the airway.

Indirect management of full-thickness tracheal erosion in a complex pediatric patient.

Prolonged tracheostomy dependence in pediatric patients can be associated with significant complications, including damage to the tracheal wall requiring reconstruction. We present a case of an 8 year-old female with full-thickness tracheal erosion secondary to the presence of a tracheostomy tube combined with a narrow thoracic inlet. A direct tracheal reconstruction was considered but eliminated due to the poor tissue quality of the trachea. Instead, a multi-disciplinary surgical team conceived of a novel indirect approach to manage the patient's tracheal defect. To our knowledge the use of indirect repair of a full-thickness tracheal defect has not been reported in the literature.

Three-dimensional printed models in multidisciplinary planning of complex tracheal reconstruction.

Three-dimensional printed models are increasingly used in medicine and surgery, but applications of these models in the planning of operative procedures is not well described. In particular, their benefits have not been documented in complex, multiservice, high-risk operations. We describe five cases of complex pediatric tracheal reconstruction for which three-dimensional models had specific benefits in planning as well as in education of trainees, operating room staff, and patient families. We also describe our method for producing models so that others can adopt the technology if desired. Laryngoscope, 127:967-970, 2017.

Avoidance of a potential tracheoinnominate fistula by innominate artery re-implantation in a four year old girl with tracheostomy dependence and Pfeiffer syndrome.

A 4 year old tracheostomy dependent girl with Pfeiffer syndrome was noted on bronchoscopy to have a pulsatile tracheostomal mass. CT chest angiography was consistent with the innominate artery crossing anterior to the trachea and superior to the sternal notch. The patient underwent reimplantation of the innominate artery via a median sternotomy approach. Tracheoinnominate fistula is a potentially devastating complication of tracheostomy. We report discovery of a near tracheoinnominate fistula in order to highlight the importance of regular interval surveillance endoscopy in tracheostomy dependent children and to discuss a preventative surgical intervention employed in prevention of this potentially devastating complication.

Obstructive lung disease in children with idiopathic scoliosis.

OBJECTIVE: To measure the prevalence of obstructive lung disease (OLD) among patients undergoing preoperative pulmonary assessment for idiopathic scoliosis. STUDY DESIGN: This was a retrospective, descriptive review from clinical data in a tertiary care pediatric hospital in the US. Patients (n = 176) with idiopathic scoliosis with Cobb angles of ≥ 40 degrees who performed acceptable and repeatable preoperative pulmonary function testing were included. The primary outcome measure was the forced expiratory volume in the first second (FEV1)/forced vital capacity (FVC) ratio. RESULTS: The prevalence of OLD (low FEV1/FVC ratio) was 39% (68/176 patients). In multivariate modeling, radiographic measures were poor predictors of pulmonary function outcomes of FVC (r(2) 0.06), FEV1 (r(2) 0.05), FEV1/FVC ratio (r(2) 0.08), and total lung capacity (r(2) 0.06). CONCLUSIONS: OLD is common in patients with idiopathic scoliosis. We recommend preoperative pulmonary function testing for patients with idiopathic scoliosis under consideration for spinal fusion surgery.

Outcomes of fundoplication in children with cystic fibrosis.

PURPOSE: Children with cystic fibrosis (CF) have a high prevalence of gastroesophageal reflux disease (GERD). As GERD is associated with chronic respiratory symptoms and feeding problems, fundoplication is often performed in children with CF. Although the outcomes of fundoplication have been described across diverse pediatric groups, there is no published experience with CF. METHODS: The records of 25 children with CF who underwent fundoplication in our center were reviewed. Data on symptoms and diagnostic testing results as well as on complications related to fundoplication were collected. Nutritional parameters and pulmonary function were compared before and after fundoplication. RESULTS: There was no mortality associated with fundoplication, but 12% had complications that required a subsequent surgical procedure. Whereas 28% were able to discontinue their antireflux medications, 48% developed symptoms of recurrent GERD. Overall, there was no change in body mass index, body mass index percentile, or the slope of forced expiratory volume in 1 second (FEV1) after fundoplication. Children who had an FEV1 of less than 60% predicted at the time of fundoplication exhibited an improvement in FEV1 slope compared to those with FEV1 of 60% or more (+5.3% vs -8.6% per year, P = .004). CONCLUSION: The complication rate of fundoplication is similar to what has been reported in large series in children without CF. There is a high rate of recurrent GERD and little apparent benefit for either nutritional or pulmonary outcomes. The observed difference on FEV1 slope, in those with moderate-severe vs mild lung disease, highlights the need to thoroughly evaluate the role of fundoplication in children with CF.