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OBJECTIVE. This project aims to prospectively and objectively assess otolaryngological manifestations and quality of life of children with primary ciliary dyskinesia (PCD) and compare these findings with healthy pediatric controls. STUDY DESIGN. Cross-sectional. SETTING. Two high-volume pediatric PCD specialty centers. METHODS. Standardized clinical assessment; Sino-Nasal Outcome Test 22 (SNOT-22); Hearing Environment and Reflection Quality of Life (HEAR-QL); Reflux Symptom Index (RSI); standardized physical examination of the sinonasal, laryngeal, and otological systems; and investigations including pure-tone audiograms (PTAs) and sinonasal cultures were collected. RESULTS. Forty-seven children with PCD and 25 control participants were recruited. Children with PCD had more upper airway symptoms than healthy children. They had significantly higher scores in both SNOT-22 and RSI, indicating worse sinonasal and reflux symptoms, with worse quality of life on the HEAR-QL index compared to healthy children (P < .05). Fifty-two percent of children with PCD-related hearing loss were not aware of their hearing deficit that was present on audiological assessment, and only 23% of children who had ventilation tubes had chronic otorrhea, most of which was easily controlled with ototopic drops. Furthermore, although all children with PCD had chronic rhinosinusitis, only 36% of them were using topical nasal treatment. The most common bacteria cultured from the middle meatus were Staphylococcus aureus in 11 of 47 (23%), followed by Streptococcus pneumoniae in 10 of 47 (21%). CONCLUSION. This multisite cohort highlights the importance of otolaryngology involvement in the management of children with PCD. More rigorous otolaryngological management may lead to reductions in overall morbidity and improve quality of life for children with PCD.

Purpose The use of a vascularized free fibula graft (FFF) for the reconstruction of a mandible in a child with a mandibular tumor is infrequent. The purpose of this study is to report our protocol for resection of mandibular jaw tumors and immediate reconstruction using FFF in pediatric patients. Methods This was a retrospective case series of children with a mandibular tumor, which was resected and immediately reconstructed with FFF. All patients were treated via the same staged protocol: 1) presurgical digital planning, 2) surgical intervention (resection and immediate reconstruction), 3) postoperative care in the pediatric intensive unit, and 4) prosthodontic dental rehabilitation. Outcomes were complications and recurrence. Medical records were reviewed to document demographic information, tumor details, surgical interventions, postoperative course, and prosthodontic rehabilitation. Results Fifteen patients (10 males, average age of 13.7 years) met inclusion criteria. Ten patients had mandibular ameloblastoma. All patients were treated by a dedicated pediatric team and followed the same protocol. The average tumor size was 4.87 × 3.22 × 2.03 cm. Most fibulas (n = 12) had one osteotomy to reestablish mandibular continuity and create appropriate contour. The most common microvascular anastomosis was with a facial artery (n = 13) and the external jugular vein (n = 9). At an average follow-up of 15.5 months, there were only 3 minor donor site complications. Eight implants were placed without complications. No tumors recurred. Conclusions The results of this study suggest that pediatric mandibular tumors can be successfully treated using a specific protocol involving resection and immediate reconstruction using FFF with minimal complications and without recurrence.

Evidence-based recommendations are constantly being updated for various pediatric surgical procedures, including the role for tympanostomy tubes, as well as indications for adenoidectomy and tonsillectomy. With a growing body of research available on some of the most prevalent pediatric conditions, an update on the current concepts surrounding management is warranted.

Background Concha bullosa mucocele is a rare diagnosis that presents as a nasal mass. It impinges on surrounding structures and can easily be mistaken for a neoplasm. Objective The objective of this study was to shed light on this rare entity and report its diagnostic features and treatment outcomes. Methods A case series conducted in a tertiary health care center. Demographic data, clinical presentation, imaging, cultures, and treatments were recorded. Operative video illustration and key images were obtained. A review of the literature was also performed. Results A total of five cases were reviewed, four of which were concha bullosa mucoceles and one was a mucopyocele. Three of the patients had some form of previous nasal trauma. Headache and nasal obstruction were the most common symptoms with a nasal mass finding on physical examination. Computed tomography was used in all the patients, and magnetic resonance imaging was used in four of the five patients. Four patients had coexistent chronic rhinosinusitis, and three had positive bacterial cultures. All these patients were treated endoscopically either with middle turbinate marsupialization or subtotal resection. No recurrence has been noted thus far. Conclusion Concha bullosa mucocele is a rare diagnosis. Imaging characteristics are helpful in considering the diagnosis, although surgical intervention is often necessary to confirm the diagnosis and treat concha bullosa mucocele.

Decorin (Dcn) is an extracellular matrix proteoglycan, which affects airway mechanics, airway-parenchymal interdependence, airway smooth muscle proliferation and apoptosis, and transforming growth factor-β bioavailability. As Dcn deposition is differentially altered in asthma, we questioned whether Dcn deficiency would impact the development of allergen-induced asthma in a mouse model. Dcn−/− and Dcn+/+ mice (C57Bl/6) were sensitized with ovalbumin (OA) and challenged intranasally 3 days/wk × 3 wk. After OA challenge, mice were anesthetized, and respiratory mechanics measured under baseline conditions and after delivery of increasing concentrations of methacholine aerosol. Complex impedance was partitioned into airway resistance and tissue elastance and damping. Bronchoalveolar lavage was performed. Lungs were excised, and tissue sections evaluated for inflammatory cell influx, α-smooth muscle actin, collagen, biglycan, and Dcn deposition. Changes in TH-2 cytokine mRNA and protein were also measured. Airway resistance was increased in OA-challenged Dcn+/+ mice only ( P < 0.05), whereas tissue elastance and damping were increased in both OA-challenged Dcn+/+ and Dcn−/−, but more so in Dcn+/+ mice ( P < 0.001). Inflammation and collagen staining within the airway wall were increased with OA in Dcn+/+ only ( P < 0.001 and P < 0.01, respectively, vs. saline). IL-5 and IL-13 mRNA were increased in lung tissue of OA-challenged Dcn+/+ mice. Dcn deficiency resulted in more modest OA-induced hyperresponsiveness, evident at the level of the central airways and distal lung. Differences in physiology were accompanied by differences in inflammation and remodeling. These findings may be, in part, due to the well-described ability of Dcn to bind transforming growth factor-β and render it less bioavailable.

Objectives To identify whether measurement of the prosthesis length is mandatory in patients requiring otosclerosis surgeries and to assess their postoperative audiometric outcomes. In addition, evaluation of prosthesis length used in revision compared to primary stapedectomy was carried out. Study Design Case series with chart review. Methods Chart review of 393 patients undergoing primary (321) versus revision stapedectomy (72) was performed in a tertiary referral center. The indication for surgery was the presence or persistence/recurrence of an air-bone gap (ABG) greater than 20 dB. Air and bone conduction thresholds (ACT and BCT, respectively), ABG as well as pure tone averages (PTAs) were determined for all patients, and the results were compared preoperatively and postoperatively. Results Prosthesis length used ranged from 3.0 to 6.0 mm without differences between primary and revision groups. Of the revision surgeries, 62.5% were stapedectomies versus stapedotomies (P < 0.001). Patients showed significant decrease in speech discrimination score, with increased air and bone conduction thresholds as well as mean ABG and PTA before the revision surgeries as a first procedure failure. Prosthesis length changes occurred in 73.5% of the cases, with an average absolute change of 0.55 mm. Prosthesis length did not affect postoperative audiometric results between primary and revision groups, in all surgeries combined. When grouping stapes surgery into accurately versus inaccurately measured incus-footplate distance, significant differences were observed in prosthesis length employed (P < 0.01). Hearing outcomes were also better in the group in which an accurately measured prosthesis was chosen, as opposed to “standard-length” prosthesis. Conclusions This study corroborates postoperative success rates of revision surgeries, which show smaller improvements in hearing compared to a primary intervention. Accurate intra-operative measurement of prosthesis length was correlated with better audiometric results postoperatively.

The purpose of this study is to identify the clinical characteristics of children above 6 years of age with chronic otitis media with effusion (COME) who have required pressure equalizing tube (PET) insertions.A retrospective chart review was performed of all children above the age of 6 years, who underwent PET insertion due to chronic OME, between 2000 and 2009 at a tertiary care institution. Children were also divided into those with a single PET and those with ≥ 2 PET. Comparison of various characteristics between the two groups was undertaken.290 children were identified as having at least one PET insertion after the age of 6 years. 45.5% of the children underwent a single PET insertion while 54.5% underwent 2 procedures or more. No significant gender-based difference was observed. In those patients requiring ≥ 2 PET, 65.2% also underwent tonsillectomy and/or adenoidectomy compared to only 53.8% of children with a single PET intervention (p0.05). Asthma, trisomy 21 and cleft palate, were more common in those children who had undergone ≥ 2 PET (p0.001, p0.01 and p0.05, respectively).Children above 6 years of age who have undergone ≥ 2 PET insertions for COME demonstrated an increased rate of tonsillectomy and adenoidectomy. Trisomy 21, cleft palate and particularly, asthma, were also found to be risk factors for COME necessitating ≥ 2 PET insertions.