Development of Consensus-Based Best Practice Guidelines for the Peri- and Post-operative Care of Pediatric Patients with Spinal Deformity and Programmable Implanted Devices.

STUDY DESIGN: Modified Delphi consensus study. OBJECTIVE: To develop consensus-based best practices for the care of pediatric patients who have implanted programmable devices (IPDs) and require spinal deformity surgery. SUMMARY OF BACKGROUND DATA: Implanted programmable devices (IPDs) are often present in patients with neuromuscular or syndromic scoliosis who require spine surgery. Guidelines for monitoring and interrogating these devices during the peri-operative period are not available. METHODS: A panel was assembled consisting of 25 experts (i.e., spinal deformity surgeons, neurosurgeons, neuro-electrophysiologists, cardiologists, and otolaryngologists). Initial postulates were based on literature review and results from a prior survey. Postulates addressed the following IPDs: vagal nerve stimulators (VNS), programmable ventriculo-peritoneal shunts (VPS), intrathecal baclofen pumps (ITBP), cardiac pacemakers and implantable cardioverter-defibrillators (ICD), deep brain stimulators (DBS), and cochlear implants. Cardiologist and otolaryngologists participants responded only to postulates on cardiac pacemakers or cochlear implants, respectively. Consensus was defined as ≥80% agreement, items that did not reach consensus were revised and included in subsequent rounds. A total of three survey rounds and one virtual meeting were conducted. RESULTS: Consensus was reached on 39 total postulates across six IPD types. Postulates addressed general spine surgery considerations, use of intraoperative monitoring and cautery, use of magnetically-controlled growing rods (MCGRs), and use of an external remote controller to lengthen MCGRs. Across IPD types, consensus for the final postulates ranged from 94.4-100%. Overall, experts agreed that MCGRs can be surgically inserted and lengthened in patients with a variety of IPDs and provided guidance for the use of intraoperative monitoring and cautery, which varied between IPD types. CONCLUSION: Spinal deformity correction surgery often benefits from the use of intraoperative monitoring, monopolar and bipolar cautery, and MCGRs. Final postulates from this study can inform the peri- and post-operative practices of spinal deformity surgeons who treat patients with both scoliosis and IPDs. LEVEL OF EVIDENCE: V- Expert opinion.

The Delivery Room Resuscitation of Infants with Congenital Diaphragmatic Hernia Treated with Fetoscopic Endoluminal Tracheal Occlusion: Beyond the Balloon.

INTRODUCTION: Randomized controlled trials found that fetoscopic endoluminal tracheal occlusion (FETO) resulted in increased fetal lung volume and improved survival for infants with isolated, severe left-sided congenital diaphragmatic hernia (CDH). The delivery room resuscitation of these infants is particularly unique, and the specific delivery room events are largely unknown. The objective of this study was to compare the delivery room resuscitation of infants treated with FETO to standard of care (SOC) and describe lessons learned. METHODS: Retrospective single-center cohort study of infants treated with FETO compared to infants who met FETO criteria during the same period but who received SOC. RESULTS: FETO infants were more likely to be born prematurely with 8/12 infants born <35 weeks gestational age compared to 3/35 SOC infants. There were 5 infants who required emergent balloon removal (2 ex utero intrapartum treatment and 3 tracheoscopic removal on placental bypass with delayed cord clamping) and 7 with prenatal balloon removal. Surfactant was administered in 6/12 FETO (50%) infants compared to 2/35 (6%) in the SOC group. Extracorporeal membrane oxygenation use was lower at 25% and survival was higher at 92% compared to 60% and 71% in the SOC infants, respectively. CONCLUSION: The delivery room resuscitation of infants treated with FETO requires thoughtful preparation with an experienced multidisciplinary team. Given increased survival, FETO should be offered to infants with severe isolated left-sided CDH, but only in high-volume centers with the experience and capability of removing the balloon, emergently if needed. The neonatal clinical team must be skilled in managing the unique postnatal physiology inherent to FETO where effective interdisciplinary teamwork is essential. Empiric and immediate surfactant administration should be considered in all FETO infants to lavage thick airway secretions, particularly those delivered <48 h after balloon removal.

Immediate postdelivery airway management of neonates with prenatally diagnosed micrognathia: A retrospective observational study.

INTRODUCTION: Micrognathic neonates are at risk for upper airway obstruction, and many require intubation in the delivery room. Ex-utero intrapartum treatment is one technique for managing airway obstruction but poses substantial maternal risks. Procedure requiring a second team in the operating room is an alternative approach to secure the obstructed airway while minimizing maternal risk. The aim of this study was to describe the patient characteristics, airway management, and outcomes for micrognathic neonates and their mothers undergoing a procedure requiring a second team in the operating room at a single quaternary care children's hospital. METHODS: This was a retrospective descriptive study. Subjects had prenatally diagnosed micrognathia and underwent procedure requiring a second team in the operating room between 2009 and 2021. Collected data included infant characteristics, delivery room airway management, critical events, and medications. Follow-up data included genetic testing and subsequent procedures within 90 days. Maternal data included type of anesthetic, blood loss, and incidence of transfusion. RESULTS: Fourteen deliveries were performed via procedure requiring a second team in the operating room during the study period. 85.7% were male, and 50% had a genetic syndrome. Spontaneous respiratory efforts were observed in 93%. Twelve patients (85.7%) required an endotracheal tube or tracheostomy. Management approaches varied. Medications were primarily a combination of atropine, ketamine, and dexmedetomidine. Oxygen desaturation was common, and three patients experienced bradycardia. There were no periprocedural deaths. Follow-up at 90 days revealed that 78% of patients underwent at least one additional procedure, and one patient died due to an unrelated cause. All mothers underwent cesarean deliveries under neuraxial anesthesia. Median blood loss was 700 mL [IQR 700 mL, 800 mL]. Only one mother required a blood transfusion for pre-procedural placental abruption. DISCUSSION: Procedure requiring a second team in the operating room is a safe and effective approach to manage airway obstruction in micrognathic neonates while minimizing maternal morbidity. CONCLUSIONS: Though shown to be safe and effective, more data are needed to support the use of procedure requiring a second team in the operating room as an alternative to ex-utero intrapartum treatment for micrognathia outside of highly specialized maternal-fetal centers.

Impact of Illustrated Postoperative Instructions on Knowledge and Retention During a Cleft Lip and Palate Surgical Mission.

OBJECTIVE: To determine the impact of illustrated postoperative instructions on patient-caregiver knowledge and retention. DESIGN: Prospective study with all participants receiving an educational intervention. SETTING: Pediatric plastic surgical missions in Guatemala City, Guatemala, between 2019 and 2020. PARTICIPANTS: A total of 63 majority-indigenous Guatemalan caregivers of patients receiving cleft lip and/or palate surgery. INTERVENTION: Illustrated culturally appropriate postoperative care instructions were iteratively developed and given to caregivers who were surveyed on illustration-based and text-based information at preoperative, postoperative, and four-week follow-up time points. MAIN OUTCOME MEASURE: Postoperative care knowledge of illustration-based versus text-based information as determined by the ability to answer 11 illustration- and 8 text-based all-or-nothing questions, as well as retention of knowledge as determined by the same survey given at four weeks follow-up. RESULTS: Scores for illustration-based and text-based information both significantly increased after caregivers received the postoperative instructions (+13.30 ± 3.78 % SE, + 11.26 ± 4.81 % SE; P < .05). At follow-up, scores were unchanged for illustration-based (-3.42 ± 4.49 % SE, P > .05), but significantly lower for text-based information (-28.46 ± 6.09 % SE, P < .01). Retention of text-based information at follow-up correlated positively with education level and Spanish literacy, but not for illustration-based. CONCLUSIONS: In the setting of language and cultural barriers on a surgical mission, understanding of illustration-based and text-based information both increased after verbal explanation of illustrated postoperative instructions. Illustration-based information was more likely to be retained by patient caregivers after four weeks than text-based information, the latter of which correlated with increased education and literacy.

International Pediatric Otolaryngology Group (IPOG) consensus recommendations: Evaluation and management of congenital tracheal stenosis.

OBJECTIVES: To outline an expert-based consensus of recommendations for the diagnosis and management of pediatric patients with congenital tracheal stenosis. METHODS: Expert opinions were sought from members of the International Pediatric Otolaryngology Group (IPOG) via completion of an 18-item survey utilizing an iterative Delphi method and review of the literature. RESULTS: Forty-three members completed the survey providing recommendations regarding the initial history, clinical evaluation, diagnostic evaluation, temporizing measures, definitive repair, and post-repair care of children with congenital tracheal stenosis. CONCLUSION: These recommendations are intended to be used to support clinical decision-making regarding the evaluation and management of children with congenital tracheal stenosis. Responses highlight the diverse management strategies and the importance of a multidisciplinary approach to care of these patients.

Systemic Bevacizumab Treatment for Recurrent Respiratory Papillomatosis: Long-Term Follow-Up.

OBJECTIVES/HYPOTHESIS: Systemic bevacizumab is a new adjuvant therapy for recurrent respiratory papillomatosis (RRP) that has shown promising preliminary results in children. The objective of this study was to report the largest series to date that includes long-term follow-up data on bevacizumab treatment. STUDY DESIGN: Retrospective chart review. METHODS: Retrospective review of seven pediatric patients treated within the past 6 years with systemic bevacizumab for RRP refractory to traditional debridement. RESULTS: All seven patients had a significant reduction in disease burden after initiation of systemic bevacizumab. There have been no major complications associated with systemic therapy so far. Median duration of bevacizumab treatment was 2.13 years. Three of the seven patients have been on treatment for over 3 years with the longest duration of treatment in our first patient now at 5.5 years. One patient experienced significant disease recurrence on two occasions when therapy was temporarily discontinued and was recently diagnosed with squamous cell carcinoma of the lung. CONCLUSION: Systemic bevacizumab is an effective therapy for cases of severe RRP with promising results both in short-term and long-term follow-up. Side effects are minimal. Patients must be followed closely to determine appropriate dosing intervals to control disease and to screen for disease progression. LEVEL OF EVIDENCE: 4 Laryngoscope, 132:2071-2075, 2022.

Risk factor analysis and outcomes of airway management in antenatally diagnosed cervical masses.

PURPOSE: To investigate antenatally-determined imaging characteristics associated with invasive airway management at birth in patients with cervical masses, as well as to describe postnatal management and outcomes. STUDY DESIGN: A retrospective analysis of 52 patients with antenatally diagnosed neck masses was performed using single-center data from January 2008 to January 2019. Antenatal imaging, method of delivery, management, and outcomes data were abstracted from the medical record and analyzed. RESULTS: Antenatal diagnosis of neck masses in this cohort consisted of 41 lymphatic malformations (78.8%), 6 teratomas (11.5%), 3 hemangiomas (5.8%), 1 hemangioendothelioma (1.9%), and 1 giant foregut duplication cyst (1.9%). Mean gestational age at time of diagnostic imaging was 29 weeks 3 days (range: 19w4d - 37w). Overall, 22 patients (42.3%) required invasive airway management at birth, specifically 18 patients (34.6%) required endotracheal intubation and 4 (7.7%) required tracheostomy. 15 patients (28.8%) underwent ex-utero intrapartum treatment (EXIT) for the purposes of securing an airway. Polyhydramnios, tracheal deviation and compression, and anterior mass location on antenatal imaging were significantly associated with incidence of invasive airway intervention at birth, EXIT procedure, and tracheostomy during the neonatal hospitalization (p < 0.025; Fisher's exact test). Logistic regression analysis demonstrated statistically significant association between increasing antenatally-estimated mass volume and incidence of invasive airway management at birth (p = 0.02). Post-natal cervical mass management involved surgical excision (32.7%), sclerotherapy (50%), and adjuvant therapy with rapamycin (17.3%). Demise in the neonatal period occurred in 4 (7.7%) patients. CONCLUSION: This series documents the largest single-center experience of airway management in antenatally diagnosed cervical masses. Fetal imaging characteristics may help inform the appropriate method of delivery, airway management strategy at birth, and prenatal counseling.

Laryngeal Manifestations of Crohn's Disease in a Toddler with Very Early Onset-IBD.

OBJECTIVES: We report obstructing laryngeal manifestations of Crohn's disease in a toddler with very early onset-IBD (VEO-IBD) who required tracheotomy tube placement at 27 months of age for relief of recalcitrant airway obstruction unresponsive to maximal medical therapy. We review the literature for the frequency of extra-intestinal laryngeal manifestations of Crohn's disease in adults and children. METHODS: Case report and literature review of laryngeal manifestations of Crohn's disease. RESULTS: Laryngeal involvement of Crohn's disease is very rare with only 14 other cases reported. Most cases appear in adults, with the supraglottis most commonly affected. This case marks the youngest report and only the second report of a patient requiring a tracheotomy for supraglottic obstruction when intensive medical management, including use of steroids and biologics, failed to relieve obstructing laryngeal inflammation. Despite ongoing Crohn's disease, laryngeal manifestations improved permitting decannulation the following year. CONCLUSIONS: Laryngeal manifestations of Crohn's disease are rare and usually affect adults. Most cases are managed with medical therapy, however surgical excision of obstructing lesions or tracheotomy placement is sometimes required for temporary relief of airway obstruction.

Tracheocutaneous Fistula After Pediatric Open Airway Reconstruction.

OBJECTIVES: Tracheocutaneous fistula (TCF) is a common occurrence after pediatric tracheostomy decannulation. However, the persistence of TCF after staged reconstruction of the pediatric airway is not well-described. The primary objective was to determine the rate of persistent TCF after successful decannulation in children with staged open airway reconstruction. METHODS: A case series with chart review of children who underwent decannulation after double-stage laryngotracheal reconstruction between 2017 and 2019. RESULTS: A total of 26 children were included. The most common open airway procedure was anterior and posterior costal cartilage grafting (84.6%, 22/26). Median age at decannulation was 3.4 years (IQR: 2.8-4.3) and occurred 7.0 months (IQR: 4.3-10.4) after airway reconstruction. TCF persisted in 84.6% (22/26) of children while 15.4% (4/26) of stomas closed spontaneously. All closures were identified by the one-month follow-up visit. There was no difference in age at tracheostomy (P = .86), age at decannulation (P = .97), duration of tracheostomy (P = .43), or gestational age (P = .23) between stomas that persisted or closed. Median diameter of stent used at reconstruction was larger in TCFs that persisted (7.0 mm vs 6.5 mm, P = .03). Tracheostomy tube diameter (P = .02) and stent size (P < .01) correlated with persistence of TCF on multivariable logistic regression analysis. There were 16 surgical closure procedures, which occurred at a median of 14.4 months (IQR: 11.4-15.4) after decannulation. Techniques included 56.3% (9/16) by primary closure, 18.8% (3/16) by secondary intention and 25% (4/16) by cartilage tracheoplasty. The overall success of closure was 93.8% (15/16) at latest follow-up. CONCLUSIONS: Persistent TCF occurs in 85% of children who are successfully decannulated after staged open airway reconstruction. Spontaneous closure could be identified by 1 month after decannulation and was more likely when smaller stents and tracheostomy tubes were utilized. Surgeons should counsel families on the frequency of TCF and the potential for additional procedures needed for closure.

Pediatric laryngoscopy and bronchoscopy during the COVID-19 pandemic: A four-center collaborative protocol to improve safety with perioperative management strategies and creation of a surgical tent with disposable drapes.

Aerosolization procedures during the COVID-19 pandemic place all operating room personnel at risk for exposure. We offer detailed perioperative management strategies and present a specific protocol designed to improve safety during pediatric laryngoscopy and bronchoscopy. Several methods of using disposable drapes for various procedures are described, with the goal of constructing a tent around the patient to decrease widespread contamination of dispersed droplets and generated aerosol. The concepts presented herein are translatable to future situations where aerosol generating procedures increase risk for any pathogenic exposure. This protocol is a collaborative effort based on knowledge gleaned from clinical and simulation experience from Children's Hospital Colorado, Children's Hospital of Philadelphia, The Hospital for Sick Children in Toronto, and Boston Children's Hospital.

Management of neonatal difficult airway emergencies in the delivery room.

Neonatal airway emergencies in the delivery room are associated with significant morbidity and mortality. Etiologies vary, but often predispose the neonate to life threatening airway obstruction. With the recent expansion of fetal medicine programs, pediatric anesthesiologists are increasingly being asked to care for these patients. In this review, we discuss common etiologies of difficult airway at delivery, management tools and techniques, and surgical approaches.

Anesthetic Management for Pediatric Awake Tracheostomy.

Awake tracheostomy is indicated for acute upper airway obstruction, when other methods of securing the airway, such as intubation and cricothyrotomy, have failed or are inappropriate. This option is rarely considered in pediatrics because of the concerns of patient cooperation and safety and has not been described in the literature. We describe the anesthetic management of an awake tracheostomy performed on a 7-year-old girl, with a large supraglottic mass obstructing the laryngeal introitus.

Total Airway Reconstruction in the Neonate: Combined Mandibular Distraction and Slide Tracheoplasty for Multiple Level Airway Obstruction.

BACKGROUND: Long-segment congenital tracheal stenosis (CTS) is characterized by segmental tracheal stenosis, complete tracheal rings, and absent posterior pars membranosa for >50% of tracheal length. Slide tracheoplasty on cardiopulmonary bypass (CPB) has traditionally been the procedure of choice for airway reconstruction. Pierre Robin sequence (PRS) is characterized by the triad of micrognathia, glossoptosis, and airway obstruction. The authors and others, have demonstrated the efficacy of mandibular distraction osteogenesis (MDO) to avoid tracheostomy in severe cases of PRS. METHODS: The authors present a unique case of the multidisciplinary management of long-segment CTS and concomitant PRS via total airway reconstruction off CPB, involving our otolaryngology, cardiothoracic, and plastic surgery teams. RESULTS: This 36-week baby girl, prenatally diagnosed with PRS and polyhydramnios concerning for airway obstruction, was delivered via planned ex utero intrapartum treatment (EXIT). Tracheostomy was aborted because of long-segment CTS. A 2.5-French endotracheal tube (ETT) was temporarily sutured in before transfer to our facility for definitive airway management.Bilateral MDO was performed without complication at 2 weeks old (distraction to 20mm by postoperative day 25). At 6 weeks old, delayed slide tracheoplasty avoiding cardiopulmonary bypass was followed by an uneventful recovery. Most recent follow-up demonstrates airway patency without signs of obstruction. CONCLUSIONS: This patient's case is the first reporting combined MDO and slide tracheoplasty to relieve multilevel neonatal airway obstruction. Unique and challenging, it demonstrates the importance of multidisciplinary management of complex neonatal airway obstruction.

Laryngotracheal reconstruction with resorbable microplate buttressing.

OBJECTIVES/HYPOTHESIS: In patients undergoing laryngotracheal reconstruction (LTR), malacic segments of trachea can pose challenges to successful reconstruction. Malacic segments may inadequately support cartilage grafts used in augmentation surgery, sometimes requiring cricotracheal or tracheal resections. We describe a novel technique of LTR with resorbable microplate buttressing of malacic lateral tracheal segments. STUDY DESIGN: Retrospective case series. METHODS: Review of technique, treatment outcomes, and complications of seven children with subglottic stenosis and tracheomalacia requiring a microplate-augmented LTR technique. RESULTS: Seven infants ranging from 26 months to 9 years of age successfully underwent LTR for subglottic stenosis. Six children had a grade III subglottic stenosis. The seventh child had grade II subglottic stenosis, bilateral vocal fold paralysis, an elliptical cricoid, and an obstructing giant suprastomal fibroma. Five children underwent a double-stage LTR with resorbable microplates sutured bilaterally to support severely malacic lateral tracheal segments. A cricotracheal resection would not have been feasible in one child due to the resection length and inadequate tracheal mobilization. Two children underwent a single-stage LTR with unilateral application of a microplate. Six children were decannulated within 3 months and continue without airway symptoms or complications. One child, who is just over 2 months from reconstructive surgery, is being setup for decannulation. No complications were encountered. CONCLUSIONS: LTR with resorbable microplate buttressing of malacic lateral tracheal segments is technically feasible, safe, and can avoid more extensive surgery requiring tracheal resection. Further experience may support the use of this technique in challenging airway reconstructions.

Evolving treatments in the management of laryngotracheal hemangiomas: will propranolol supplant steroids and surgery?

There has been a dramatic evolution in the treatment of laryngotracheal hemangiomas during the past decade and recent accounts and case reports of propranolol treatment have been encouraging. The purpose of the study is to determine the clinical course and outcomes of treating laryngotracheal hemangiomas at The Children's Hospital of Philadelphia in the last 8 years with the various modalities. We review with contemporary surgical techniques, including propranolol, and determine the results, limitations and complications. The study was a retrospective review of all patients referred to the Otolaryngology service at The Children's Hospital of Philadelphia with symptomatic laryngotracheal hemangiomas between January 2002 and December 2010. The study consisted of 30 infants, ranging in age from 1 to 18 months at time of diagnosis. Surgical interventions included open surgical excision, laser surgery, microdebrider excision and/or propranolol therapy. The main outcome measures include improvement in symptoms, decannulation, number of required treatments and airway size. All but two patients underwent an initial trial of steroids. Thirteen patients underwent open surgical excision, 9 requiring cartilage grafts and 12 were done in a single stage. Twelve surgical patients remained asymptomatic. One patient with diffuse mediastinal disease experiencing postoperative airway symptoms despite a normal appearing airway improved on propranolol. Two patients underwent at least 2 laser ablations, 4 responded to systemic steroids alone, and 1 had microdebrider resection. In the last 14 months, 12 patients have had propranolol therapy at a dose of 2mg/kg divided every 8h. Eight patients improved clinically within 1 week of initiating treatment. Four patients failed to respond to propranolol therapy; 1 patient subsequently underwent open excision and the other continued with a tracheostomy for 18 months and finally was decannulated. A third patient had a partial response, but remains relatively asymptomatic. A fourth patient has had no response at all. There were no major complications from propranolol; minor complications included diarrhea and decreased appetite. This study gives an overview of the evolution of hemangioma treatment at our institution over the last 8 years. Surgical excision remains an effective treatment for subglottic hemangiomas. Carefully administered, propranolol may demonstrate efficacy as a first-line agent in most cases avoiding surgery, tracheostomy, prolonged steroids, or as treatment of diffuse and unresectable disease. However, some lesions may be resistant to propranolol and require surgery or long-term steroids.