Oral Losartan After Limited Mandibulectomy for Treatment of Desmoid-Type Fibromatosis.

Desmoid-type fibromatosis (DF) is a rare soft tissue lesion with an annual incidence of 2 to 4 per million population and peak incidence occurring at approximately 4.5 years of age. While benign, the tumor has a locally aggressive infiltrative growth pattern and a high rate of recurrence. Given the functional and aesthetic implications of excision and reconstruction in the facial skeleton, novel medical treatment options are highly desirable. We describe the case of a 3-year-old boy who presented with an enlarging, asymptomatic mass involving the left mandible. Biopsy revealed an immunohistochemical profile consistent with DF. Despite the high likelihood of recurrence, conservative, mandible-sparing en bloc resection and limited mandibulectomy were performed. Pathological and immunohistochemical analysis of the resection specimen revealed DF with grossly positive margins and elevated expression of angiotensin II type 1 receptor. Postoperative medical treatment with the angiotensin receptor blocker losartan was initiated. The patient remains medically stable and disease progression-free on repeat imaging at 20 months post-resection. We describe for the first time the successful use of the angiotensin blocker losartan following conservative surgery for management of DF.

Choosing Wisely Canada - pediatric otolaryngology recommendations.

The Choosing Wisely Canada campaign raises awareness amongst physicians and patients regarding unnecessary or inappropriate tests and treatments. Using an online survey, members of the Pediatric Otolaryngology Subspecialty Group within the Canadian Society of Otolaryngology - Head & Neck Surgery developed a list of nine evidence based recommendations to help physicians and patients make treatment decisions regarding common pediatric otolaryngology presentations: (1) Don't routinely order a plain film x-ray in the evaluation of nasal fractures; (2) Don't order imaging to distinguish acute bacterial sinusitis from an upper respiratory infection; (3) Don't place tympanostomy tubes in most children for a single episode of otitis media with effusion of less than 3 months duration; (4) Don't routinely prescribe intranasal/systemic steroids, antihistamines or decongestants for children with uncomplicated otitis media with effusion; (5) Don't prescribe oral antibiotics for children with uncomplicated tympanostomy tube otorrhea or uncomplicated acute otitis externa; (6) Don't prescribe codeine for post-tonsillectomy/adenoidectomy pain relief in children; (7) Don't administer perioperative antibiotics for elective tonsillectomy in children; (8) Don't perform tonsillectomy for children with uncomplicated recurrent throat infections if there have been fewer than 7 episodes in the past year, 5 episodes in each of the past 2 years, or 3 episodes in each of the last 3 years; and (9) Don't perform endoscopic sinus surgery for uncomplicated pediatric chronic rhinosinusitis prior to failure of maximal medical therapy and adenoidectomy.

Systemic Bevacizumab for Treatment of Respiratory Papillomatosis: International Consensus Statement.

OBJECTIVES/HYPOTHESIS: The purpose of this study is to develop consensus on key points that would support the use of systemic bevacizumab for the treatment of recurrent respiratory papillomatosis (RRP), and to provide preliminary guidance surrounding the use of this treatment modality. STUDY DESIGN: Delphi method-based survey series. METHODS: A multidisciplinary, multi-institutional panel of physicians with experience using systemic bevacizumab for the treatment of RRP was established. The Delphi method was used to identify and obtain consensus on characteristics associated with systemic bevacizumab use across five domains: 1) patient characteristics; 2) disease characteristics; 3) treating center characteristics; 4) prior treatment characteristics; and 5) prior work-up. RESULTS: The international panel was composed of 70 experts from 12 countries, representing pediatric and adult otolaryngology, hematology/oncology, infectious diseases, pediatric surgery, family medicine, and epidemiology. A total of 189 items were identified, of which consensus was achieved on Patient Characteristics (9), Disease Characteristics (10), Treatment Center Characteristics (22), and Prior Workup Characteristics (18). CONCLUSION: This consensus statement provides a useful starting point for clinicians and centers hoping to offer systemic bevacizumab for RRP and may serve as a framework to assess the components of practices and centers currently using this therapy. We hope to provide a strategy to offer the treatment and also to provide a springboard for bevacizumab's use in combination with other RRP treatment protocols. Standardized delivery systems may facilitate research efforts and provide dosing regimens to help shape best-practice applications of systemic bevacizumab for patients with early-onset or less-severe disease phenotypes. LEVEL OF EVIDENCE: 5 Laryngoscope, 131:E1941-E1949, 2021.

Competency-Based Assessment Tool for Pediatric Tracheotomy: International Modified Delphi Consensus.

OBJECTIVES/HYPOTHESIS: Create a competency-based assessment tool for pediatric tracheotomy. STUDY DESIGN: Blinded, modified, Delphi consensus process. METHODS: Using the REDCap database, a list of 31 potential items was circulated to 65 expert surgeons who perform pediatric tracheotomy. In the first round, items were rated as "keep" or "remove," and comments were incorporated. In the second round, experts were asked to rate the importance of each item on a seven-point Likert scale. Consensus criteria were determined a priori with a goal of 7 to 25 final items. RESULTS: The first round achieved a response rate of 39/65 (60.0%), and returned questionnaires were 99.5% complete. All items were rated as "keep," and 137 comments were incorporated. In the second round, 30 task-specific and seven previously validated global rating items were distributed, and the response rate was 44/65 (67.7%), with returned questionnaires being 99.3% complete. Of the Task-Specific Items, 13 reached consensus, 10 were near consensus, and 7 did not achieve consensus. For the 7 previously validated global rating items, 5 reached consensus and two were near consensus. CONCLUSIONS: It is feasible to reach consensus on the important steps involved in pediatric tracheotomy using a modified Delphi consensus process. These items can now be considered to create a competency-based assessment tool for pediatric tracheotomy. Such a tool will hopefully allow trainees to focus on the important aspects of this procedure and help teaching programs standardize how they evaluate trainees during this procedure. LEVEL OF EVIDENCE: 5 Laryngoscope, 130:2700-2707, 2020.

Evaluating short and long term outcomes following pediatric Myringoplasty with Gelfoam graft for tympanic membrane perforation following ventilation tube insertion.

BACKGROUND: Myringotomy with ventilation tube (VT) insertion to treat recurrent acute otitis media and chronic secretory otitis media has become one of the most common surgical procedures performed in children. Although contemporary literature has detailed the various patient and perioperative factors that affect successful pediatric myringoplasty, there is still limited evidence surrounding the increasing number of graft material options. In particular, gelfoam patching has arisen as a simple and efficient modality for perforation closure, but has a paucity of evidence particularly in pediatric cohorts. Our study aims to evaluate the clinical and audiometric outcomes following gelfoam myringoplasty for TMP following prolonged VT insertion in an urban pediatric population. METHODS: A retrospective review of pediatric patients who underwent myringoplasty between 2013 and 2018 following ventilation tube insertion. Patient demographics, comorbidities, and graft material were correlated with audiometric and clinical outcomes on follow up examination. RESULTS: One hundred twenty patients underwent myringoplasty, with 61 (50.8%) males with a mean age of 8.9 years old. 101 (84.2%) of patients eventually underwent successful tympanic membrane (TM) closure, with 93 (77.5%) demonstrating closure at initial follow up. In the gelfoam cohort, 77 (90.6%) of patients demonstrated successful TM closure at initial follow up. Overall mean time to closure was 5.6 (standard error (SE) 0.9) months. A multivariate Cox proportional hazards model demonstrated none of the covariates including graft material significantly affected TM closure. Mean change in air conduction threshold were comparable between graft materials. CONCLUSIONS: Pediatric myringoplasty with gelfoam graft material is a safe and viable alternative with favorable short and long term clinical and audiometric outcomes.

Severe cryopyrin-associated periodic syndrome first characterized by early childhood-onset sensorineural hearing loss - Case report and literature review.

Infant-onset bilateral sensorineural hearing loss is a key presenting symptom of the autoinflammatory cryopyrin-associated periodic syndrome. Other symptoms include periodic fever, cold-induced urticaria-like rash, chronic aseptic meningitis, polyarticular arthralgias, and renal AA amyloidosis. Early recognition and treatment with interleukin-1 blockade are critical for preventing disabling or fatal complications. We describe a patient with severe cryopyrin-associated periodic syndrome who presented at age 18 months with macrocephaly and moderate sensorineural hearing loss, later developing systemic sequelae. The pathogenic nature of the de novo NLRP3 gene variant identified was supported by a markedly elevated serum amyloid A level and sustained clinical response to anti-IL-1 therapy.

Secretory carcinoma: the eastern Canadian experience and literature review.

BACKGROUND: Secretory Carcinoma (SC) is a recently described malignancy affecting salivary glands of the head and neck, with a paucity of evidence regarding the natural history, morbidity, and mortality. This study aimed to investigate the current treatment options utilized for SC, as well as its presentation and outcomes. METHODS: This study is a retrospective case series and includes patients diagnosed with SC at four Maritime Canadian institutions. Literature review of patient outcomes following treatment of SC is also included. RESULTS: Thirteen patients were identified. Parotid was the most common subsite (69%), followed by minor salivary gland (23%) and submandibular gland (8%). All patients were S100 positive and had at least one additional positive confirmatory stain, including mammaglobin, CK7, or vimentin. Two patients had N2b disease. All patients were treated with primary surgery, and four were offered adjuvant radiotherapy. There was one instance of locoregional recurrence, and one of metastasis. Three patients displayed perineural invasion on pathology, and one patient displayed lymphovascular invasion. CONCLUSION: Secretory Carcinoma remains understudied regarding its natural history, presentation, and treatment options. This study is the largest single case series in Canada, and highlights the young age and possible aggressiveness of SC. As well, we provide the most comprehensive literature review to date, with a focus on treatment and outcomes for this disease entity.

Informed Consent in Pediatric Otolaryngology: What Risks and Benefits Do Parents Recall?

OBJECTIVE: To evaluate parental recall of surgical risks and benefits in pediatric otolaryngology and to assess for factors that may influence recall. STUDY DESIGN: Prospective cohort study. SETTING: Academic pediatric otolaryngology clinic. SUBJECTS AND METHODS: Eighty-four parents of children <6 years of age who underwent consultation for adeno/tonsillectomy and/or tympanostomy tube insertion were prospectively enrolled. Consultation visits were video recorded and the benefits and risks of surgery documented. Two weeks following the consultation, parents were contacted for assessment of recall of information discussed during the consultation. RESULTS: Overall, parents recalled only one-third of the risks of surgery mentioned by the surgeons. Parents were significantly more likely to recall the benefits of surgery as opposed to the risks (P < .001). Nine parents (10.7%) reported that no benefits were discussed during the consultation, and 10 (11.9%) reported no mention of any risks. Inconsistencies were present in which risks and benefits were mentioned by the providers. Parents who decided to proceed with surgery (58.3%) were significantly less likely to recall the surgical risks than those who did not (P < .001). The specific surgeon involved, the number of caregivers present, parental education level, and prior surgical history did not influence recall. CONCLUSION: Parental recall of benefits and risks associated with common pediatric otolaryngology procedures was poor. This information is important because a low rate of recall may influence parents' perspectives of the procedure and could alter their decision-making processes or expectations. Methods to improve parental recall should be further studied.

A randomized double-blind controlled trial of phosphorylcholine-coated tympanostomy tube versus standard tympanostomy tube in children with recurrent acute and chronic otitis media.

OBJECTIVE: To compare the postoperative complication rates of phosphorylcholine-coated fluoroplastic tympanostomy tubes versus uncoated fluoroplastic tympanostomy tubes. METHODS: A prospective randomized double-blind controlled trial in children with recurrent acute otitis media and chronic otitis media with effusion; 240 children were randomized to receive a phosphorylcholine-coated tube in one ear and an uncoated tube in the other. Postoperatively, patients were assessed at 2 weeks and 4, 8, 12, 18, and 24 months to ascertain the incidence of otorrhea, tube lumen blockage, and early extrusion. RESULTS: Out of 240 children, 5 withdrew and 16 were lost to early follow-up. The mean age was 43.8 months. There were no statistically significant differences in the incidence of postoperative otorrhea, tube blockage, and extrusion. CONCLUSIONS: Phosphorylcholine-coated fluoroplastic ventilation tubes do not offer any advantages over uncoated standard fluoroplastic tympanostomy tubes.

Management of symptomatic tracheal pouches.

OBJECTIVE: Acquired tracheal pouches arise following tracheoesophageal fistula (TEF) repair, but are usually asymptomatic. Symptomatic tracheal pouches are rare, and the optimal management strategy debated. The evolution of our management to this challenging problem is presented. DESIGN: A case series over a 5-year period. SETTING: A tertiary care pediatric hospital. PATIENTS: Children with a past history of TEF repair in whom severity of pouch-related respiratory symptomatology warranted surgical intervention. MAIN OUTCOME MEASURES: Symptomatology, bronchoscopic findings, number and type of surgical interventions required, complications, and outcomes. RESULTS: Eleven patients required surgical intervention for a symptomatic tracheal pouch. Symptoms were due to stasis of secretions with associated pulmonary soiling in three children, severe tracheomalacia in six children, and respiratory obstruction related to the tip of a tracheotomy tube or endotracheal tube being displaced intermittently into the pouch in six children. Surgical management of the pouch included one fulguration with the CO(2) laser, one with microlaryngeal instruments, Bugbee cautery in two, open resection in one, and transcervical division in another. The final five children had endoscopic pouch division with Clickline biopsy forceps. All children have had resolution of their pouch-related symptoms, although two remain tracheotomy-dependent. CONCLUSIONS: Symptomatic tracheal pouches are rare. Surgical intervention to divide the common party wall between the trachea and the pouch may alleviate associated respiratory symptomatology. The Clickline biopsy forceps is a safe, rapid, and effective method of dividing a tracheal pouch.

Acquired subglottic cysts in preterm infants.

OBJECTIVES: Acquired subglottic cysts (SGCs) are a recognized complication of intubation in preterm infants. A brief window of our experience is reviewed to determine the demographics and outcomes of therapy. DESIGN: A retrospective review encompassing patients diagnosed and treated from 2001 to 2002 inclusively. SETTING/METHODS: A case series of 11 patients evaluated at a tertiary care pediatric institution over a 2-year period is presented. RESULTS: Eleven patients evaluated for symptoms of severe airway distress were diagnosed with SGC by microlaryngoscopy and bronchoscopy. All had a history of prematurity with intubation in the perinatal period. All but one became symptomatic many months following intubation. Two patients presented for assessment with tracheotomies, whereas a third received a tracheotomy during therapy owing to a large obstructive bilateral SGC. Large unilateral SGCs were the most common finding on endoscopy. Treatment consisted of cyst marsupialization or direct excision on laryngotracheoplasty. Twenty-six procedures were performed. Four patients required single procedures for complete resolution of symptoms. Two patients had staged procedures with the use of endoscopic diathermy. An element of subglottic stenosis was present in 10 of the 11 patients; however, only 3 patients required laryngotracheoplasty for definitive treatment. CONCLUSION: SGCs are associated with intubation in premature infants and may develop many months following extubation. The duration of intubation does not appear to be a predictive risk factor. Subglottic stenosis is commonly an underlying finding in patients with SGC. Staging procedures with endoscopic diathermy is a highly effective modality, minimizing the need for more invasive procedures.

Complications of adenotonsillectomy.

OBJECTIVE: To review the immediate, short-term, and long-term complications of adenotonsillectomy. STUDY DESIGN: Review. METHODS: Complications of adenotonsillectomy and methods for preventing and treating them were reviewed. RESULTS: The most common complications of adenotonsillectomy, such as bleeding, generally occur in the immediate perioperative period but can develop up to 2 weeks postoperatively. Long-term complications, such as nasopharyngeal stenosis, may appear months to years after surgery. These more unusual sequelae result from scar contracture and maturation. CONCLUSION: Although rare, complications associated with adenotonsillectomy can be taxing for patients and health care resources. The most common complications, namely, anesthesia risks, pain, otalgia, and bleeding, should be discussed with patients' caregivers.