External airway splint placement for severe pediatric tracheobronchomalacia.

OBJECTIVE: To present external airway splinting with bioabsorbable airway supportive devices (ASD) for severe, life-threatening cases of pediatric tracheomalacia (TM) or tracheobronchomalacia (TBM). METHODS: A retrospective cohort was performed for 5 pediatric patients with severe TM or TBM who underwent ASD placement. Devices were designed and 3D-printed from a bioabsorbable material, polycaprolactone (PCL). Pre-operative planning included 3-dimensional airway modeling of tracheal collapse and tracheal suture placement using nonlinear finite element (FE) methods. Pre-operative modeling revealed that triads along the ASD open edges and center were the most effective suture locations for optimizing airway patency. Pediatric cardiothoracic surgery and otolaryngology applied the ASDs by suspending the trachea to the ASD with synchronous bronchoscopy. Respiratory needs were trended for all cases. Data from pediatric patients with tracheostomy and diagnosis of TM or TBM, but without ASD, were included for discussion. RESULTS: Five patients (2 Females, 3 Males, ages 2-9 months at time of ASD) were included. Three patients were unable to wean from respiratory support after vascular ring division; all three weaned to room air post-ASD. Two patients received tracheostomies prior to ASD placement, but continued to experience apparent life-threatening events (ALTE) and required ventilation with supraphysiologic ventilator settings. One patient weaned respiratory support successfully after ASD placement. The last patient died post-ASD due to significant respiratory co-morbidity. CONCLUSION: ASD can significantly benefit patients with severe, unrelenting tracheomalacia or tracheobronchomalacia. Proper multidisciplinary case deliberation and selection are key to success with ASD. Pre-operative airway modeling allows proper suture placement to optimally address the underlying airway collapse.

Sustaining standardized opioid prescribing practices after pediatric tonsillectomy.

INTRODUCTION: Opioid prescribing patterns after pediatric tonsillectomy are highly variable, and opioids may not improve pain control compared to over-the-counter pain relievers. We evaluated whether a standardized, opioid-sparing analgesic protocol effectively reduced opioid prescriptions without compromising patient outcomes. METHODS: A quality improvement project was initiated in July 2019 to standardize analgesic prescribing after hospital-based tonsillectomy with/without adenoidectomy. An electronic order set provided weight-based dosing and defaulted to non-opioid prescriptions (acetaminophen and ibuprofen). Patients ages 0-6 received non-opioid analgesics alone. Patients ages 7-18 received non-opioid analgesics as first-line pain control, and providers could manually add hydrocodone-acetaminophen for breakthrough pain. Opioid prescriptions and quantities were compared for 18 months of cases pre- versus post-standardization. Postoperative returns to the system were reviewed as a balancing measure. RESULTS: From 2018 through 2020, 1817 cases were reviewed. The frequency of opioid prescriptions decreased significantly post-standardization, from 64.9% to 33.5% of cases (P < .001). Opioid prescribing for young children steadily decreased from over 50% to 2.4%. Protocol adherence improved over time; outlier prescriptions were eliminated. Opioid quantities per prescription decreased by 16.3 doses on average (P < .001), and variance decreased significantly post-standardization (P < .001). The incidence of returns to the system did not change (P = .33), including returns for pain or decreased intake (P = .28). CONCLUSION: An age-based and weight-based analgesic protocol reduced post-tonsillectomy opioid prescriptions without a commensurate increase in returns for postoperative complaints. Standardized protocols can facilitate sustained changes in prescribing patterns and limit potentially unnecessary pediatric opioid exposure.

Congenital Central Hypoventilation Syndrome: Optimizing Care with a Multidisciplinary Approach.

Congenital central hypoventilation syndrome (CCHS) is a rare genetic disorder affecting respiratory control and autonomic nervous system function caused by variants in the paired-like homeobox 2B (PHOX2B) gene. Although most patients are diagnosed in the newborn period, an increasing number of patients are presenting later in childhood, adolescence, and adulthood. Despite hypoxemia and hypercapnia, patients do not manifest clinical features of respiratory distress during sleep and wakefulness. CCHS is a lifelong disorder. Patients require assisted ventilation throughout their life delivered by positive pressure ventilation via tracheostomy, noninvasive positive pressure ventilation, and/or diaphragm pacing. At different ages, patients may prefer to change their modality of assisted ventilation. This requires an individualized and coordinated multidisciplinary approach. Additional clinical features of CCHS that may present at different ages and require periodic evaluations or interventions include Hirschsprung's disease, gastrointestinal dysmotility, neural crest tumors, cardiac arrhythmias, and neurodevelopmental delays. Despite an established PHOX2B genotype and phenotype correlation, patients have variable and heterogeneous clinical manifestations requiring the formulation of an individualized plan of care based on collaboration between the pulmonologist, otolaryngologist, cardiologist, anesthesiologist, gastroenterologist, sleep medicine physician, geneticist, surgeon, oncologist, and respiratory therapist. A comprehensive multidisciplinary approach may optimize care and improve patient outcomes. With advances in CCHS management strategies, there is prolongation of survival necessitating high-quality multidisciplinary care for adults with CCHS.

Airway Anomalies.

This article reviews congenital anomalies involving the larynx and trachea, including congenital subglottic stenosis, laryngeal webs, laryngeal cleft, and tracheal stenosis. Presenting signs and symptoms, prevailing surgical repair techniques, and postoperative care are discussed.

Adenotonsillectomy in children with sickle cell disease and obstructive sleep apnea.

INTRODUCTION: Obstructive sleep apnea (OSA) is prevalent and may be more severe in children with Sickle Cell Disease (SCD) compared to the general pediatric population. OBJECTIVES: The objective of this study was to describe the therapeutic effects and complications of tonsillectomy and adenoidectomy (T&A) for treatment of OSA in children with SCD. METHODS: A comprehensive database of pediatric SCD patients was reviewed to identify all patients who underwent T&A between 2010 and 2016. An IRB-approved, retrospective review of laboratory values, perioperative course, pre- and post-T&A hospital utilization, and polysomnography was conducted. RESULTS: There were 132 SCD children (108 HbSS) who underwent T&A. Mean age was 7.6 ±â€¯4.6 years. The mean baseline hemoglobin of these patients was 9.3 ±â€¯1.4 g/dL; 72.7% of patients had pre-operative transfusion, such that the mean Hb at time of T&A was 11.4 ±â€¯1.0 g/dL. The average admission length surrounding T&A was 3.5 ±â€¯1.2 days. Complications were documented in 11.4% of operative cases. Polysomnography was available in 104 pre-T&A and 45 post-T&A. The Apnea-Hypopnea Index decreased on post-T&A polysomnogram (7.6 ±â€¯8.7 vs. 1.3 ±â€¯1.9, p = 0.0001). The O2 nadir improved on post-T&A polysomnogram (81.2 ±â€¯10.8 vs. 89.3 ±â€¯7, p = 0.0003). Emergency room visits (mean events per year) decreased post-operatively (2.6 ±â€¯2.8 vs. 1.8 ±â€¯2.2, p = 0.0002). CONCLUSIONS: T&A can be a safe and effective option to treat OSA in pediatric patients with SCD and was significantly associated with reduced AHI and fewer ER visits post-operatively.

Structural airway abnormalities contribute to dysphagia in children with esophageal atresia and tracheoesophageal fistula.

BACKGROUND: Long-term dysphagia occurs in up to 50% of repaired esophageal atresia and tracheoesophageal fistula (EA/TEF) patients. The underlying factors are unclear and may include stricture, esophageal dysmotility, or associated anomalies. Our purpose was to determine whether structural airway abnormalities (SAA) are associated with dysphagia in EA/TEF. METHODS: We conducted a retrospective chart review of children who underwent EA/TEF repair in our hospital system from 2007 to 2016. Children with identified SAA (oropharyngeal abnormalities, laryngeal clefts, laryngomalacia, vocal cord paralysis, and tracheomalacia) were compared to those without airway abnormalities. Dysphagia outcomes were determined by the need for tube feeding and the modified pediatric Functional Oral Intake Scale (FOIS) at 1 year. RESULTS: SAA was diagnosed in 55/145 (37.9%) patients with EA/TEF. Oropharyngeal aspiration was more common in children with SAA (58.3% vs. 36.4%, p=0.028). Children with SAA were more likely to require tube feeding both at discharge (79.6% vs. 48.3%, p<0.001) and at 1 year (52.7% vs. 13.6%, p<0.001) and had lower mean FOIS (4.18 vs. 6.21, p<0.001). In the logistic regression model adjusting for gestational age, long gap EA, and esophageal stricture, the presence of SAA remained a significant risk factor for dysphagia (OR 4.17 (95% CI 1.58-11.03)). CONCLUSION: SAA are common in children with EA/TEF and are associated with dysphagia, even after accounting for gestational age, esophageal gap and stricture. This study highlights the need for a multidisciplinary approach, including early laryngoscopy and bronchoscopy, in the evaluation of the EA/TEF child with dysphagia. LEVEL OF EVIDENCE: Level II retrospective prognostic study.

Open staple diverticulectomy for a large recurrent Zenker's diverticulum: still a valid procedure.

OBJECTIVES: We demonstrate indications for external mechanical stapler diverticulectomy in the modern era of endoscopic treatment. We review treatment of a large diverticulum and discuss considerations that should be made in deciding on the type of surgical treatment. METHODS: The index case was in a 75-year-old man who had undergone open cricopharyngeal myotomy with diverticulopexy 35 years earlier. He presented with 25 years of recurrent symptoms. A swallow study showed a 6.5 x 5.0-cm diverticulum. The diverticulum was deemed too large for standard endoscopic myotomy, so diverticulectomy was performed with a stapler. RESULTS: The patient was discharged on postoperative day 3 without complications. A swallow study on postoperative day 5 demonstrated no diverticulum or extravasation of barium. The patient resumed a normal diet with resolution of dysphagia. Two additional patients with large Zenker's diverticula that were managed similarly are also discussed. CONCLUSIONS: Although endoscopic laser cricopharyngeal myotomy and stapler diverticulostomy have become standard treatments for Zenker's diverticulum, this case of a large recurrent diverticulum illustrates a situation in which older techniques may be preferred. Use of the mechanical stapler allowed for a shorter surgery time than traditional suture techniques, and the potential for an earlier return to a normal diet.

Intraoral metal contact allergy as a possible risk factor for oral squamous cell carcinoma.

OBJECTIVES: Intraoral exposure to dental restorations can cause contact allergy that may induce carcinogenesis. We investigated the relationship of intraoral metal contact allergy to epithelial carcinogenesis. METHODS: The prevalence of positive patch test reactions to dental restoration metals in 65 prospectively enrolled patients with newly or previously diagnosed oral squamous cell carcinoma (SCC) was compared to that in 48 control patients. The relative risk of oral SCC was estimated by calculating odds ratios for exposure to dental metals resulting in allergy. RESULTS: Of the 65 patients with oral SCC, 34% were allergic to at least 1 adjacent metal. They were 1.57 times as likely as control patients to have metal contact allergy (odds ratio, 1.57; 95% confidence interval, 0.65 to 3.80) and more than 3 times as likely to react to mercury (odds ratio, 3.20; 95% confidence interval, 0.42 to 33.20). CONCLUSIONS: Patients with oral SCC who have metal dental restorations should undergo patch testing and possible removal of the restorations if their reactions are positive.