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.

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.

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.

Assessing Pediatric Feeding Disorders by Domain in Complex Aerodigestive Patients.

OBJECTIVE: Pediatric feeding disorder (PFD) is defined as impaired oral intake, associated with dysfunction in at least one of four domains: medical, nutritional, feeding skill, and/or psychosocial. The pediatric aerodigestive patient presents with conditions impacting airway, breathing, feeding, swallowing, or growth. The objective of the study was to determine the prevalence of PFD and dysfunctional domain, in the aerodigestive patient presenting to a tertiary aerodigestive clinic. METHODS:  Twenty-five charts from patients enrolled in Mayo Clinic Children's Center Aerodigestive Program were retrospectively reviewed for documentation of dysfunction within the four feeding disorder domains. Results from the aerodigestive triple scope, functional endoscopic evaluation of swallow (FEES), and videofluoroscopic swallow study (VFSS) were recorded. Height and weight z-scores were compared between the initial assessment and 6-12 months later. RESULTS: Median age was 20 months (range 2-81 months). Of the patients, 100% (n = 25) had dysfunction in at least one PFD domain. The domain identified most frequently was medical dysfunction (96%; n = 24). Feeding dysfunction was observed in 76% (n = 19). Psychosocial dysfunction was observed in 76% (n = 19). Nutritional dysfunction was observed in 60% (n = 15). Dysfunction in three or greater domains was seen in 80% (n = 20). Weight z-score increased in 76% (n = 19) of patients 6 to 12 months after the initial aerodigestive evaluation. CONCLUSION:  Aerodigestive patients frequently have PFD and utilizing the consensus definition of PFD at intake may enhance clinical assessment and therapeutic evaluation, and provide a framework to measure outcomes in this heterogeneous patient population.

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.

The Multidisciplinary Approach to Pediatric Aerodigestive Disorders.

Multidisciplinary programs for the care of children with upper and lower respiratory and gastrointestinal tract disorders have emerged across the United States and become known as aerodigestive centers. This model is designed to improve clinical outcomes and healthcare value through a coordinated approach by a team that appreciates the inter-relatedness of these disorders. The primary elements include: (1) Interdisciplinary medical and surgical team, (2) Care coordination, (3) Team meeting, and (4) Combined endoscopic procedures. This article will describe the origin and current trends in the multidisciplinary approach to pediatric aerodigestive disorders.

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.

Interdisciplinary aerodigestive care model improves risk, cost, and efficiency.

OBJECTIVE: This study sought to evaluate the impact of an interdisciplinary care model for pediatric aerodigestive patients in terms of efficiency, risk exposure, and cost. METHODS: Patients meeting a standard clinical inclusion definition were studied before and after implementation of the aerodigestive program. RESULTS: Aerodigestive patients seen in the interdisciplinary clinic structure achieved a reduction in time to diagnosis (6 vs 150 days) with fewer required specialist consultations (5 vs 11) as compared to those seen in the same institution prior. Post-implementation patients also experienced a significant reduction in risk, with fewer radiation exposures (2 vs 4) and fewer anesthetic episodes (1 vs 2). Total cost associated with the diagnostic evaluation was significantly reduced from a median of $10,374 to $6055. CONCLUSION: This is the first study to utilize a pre-post cohort to evaluate the reduction in diagnostic time, risk exposure, and cost attributable to the reorganization of existing resources into an interdisciplinary care model. This suggests that such a model yields improvements in care quality and value for aerodigestive patients, and likely for other pediatric patients with chronic complex conditions.

Structure and Functions of Pediatric Aerodigestive Programs: A Consensus Statement.

Aerodigestive programs provide coordinated interdisciplinary care to pediatric patients with complex congenital or acquired conditions affecting breathing, swallowing, and growth. Although there has been a proliferation of programs, as well as national meetings, interest groups and early research activity, there is, as of yet, no consensus definition of an aerodigestive patient, standardized structure, and functions of an aerodigestive program or a blueprint for research prioritization. The Delphi method was used by a multidisciplinary and multi-institutional panel of aerodigestive providers to obtain consensus on 4 broad content areas related to aerodigestive care: (1) definition of an aerodigestive patient, (2) essential construct and functions of an aerodigestive program, (3) identification of aerodigestive research priorities, and (4) evaluation and recognition of aerodigestive programs and future directions. After 3 iterations of survey, consensus was obtained by either a supermajority of 75% or stability in median ranking on 33 of 36 items. This included a standard definition of an aerodigestive patient, level of participation of specific pediatric disciplines in a program, essential components of the care cycle and functions of the program, feeding and swallowing assessment and therapy, procedural scope and volume, research priorities and outcome measures, certification, coding, and funding. We propose the first consensus definition of the aerodigestive care model with specific recommendations regarding associated personnel, infrastructure, research, and outcome measures. We hope that this may provide an initial framework to further standardize care, develop clinical guidelines, and improve outcomes for aerodigestive patients.

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.

Prevention of tracheostomy-related pressure ulcers in children.

BACKGROUND AND OBJECTIVES: Pressure ulcers are commonly acquired in pediatric institutions, and they are a key indicator of the standard and effectiveness of care. We recognized a high rate of tracheostomy-related pressure ulcers (TRPUs) in our ventilator unit and instituted a quality improvement program to develop and test potential interventions for TRPU prevention, condensed them into a clinical bundle, and then implemented the bundle into our standard practice. METHODS: The intervention model used a rapid-cycle, Plan-Do-Study-Act (PDSA), framework for improvement research. All tracheostomy-dependent patients admitted to our 18-bed ventilator unit from July 2008 through December 2010 were included. TRPU stage and description, number of days each TRPU persisted, and bundle compliance were recorded in real time. All TRPUs were staged by a wound-care expert within 24 hours. The interventions incorporated into the TRPU-prevention bundle included frequent skin and device assessments, moisture-reducing device interface, and pressure-free device interface. RESULTS: There was a significant decrease in the rate of patients who developed a TRPU from 8.1% during the preintervention period, to 2.6% during bundle development, to 0.3% after bundle implementation. There was a marked difference between standard and extended tracheostomy tubes in TRPU occurrence (3.4% vs 0%, P = .007) and days affected by a TRPU (5.2% vs 0.1%, P < .0001). CONCLUSIONS: Education and ongoing assessment of skin integrity and the use of devices that minimize pressure at the tracheostomy-skin interface effectively reduce TRPU even among a population of children at high risk. These interventions can be integrated into daily workflow and result in sustained effect.