Neurostimulation for Pediatric Obstructive Sleep Apnea.

Up to 80% of children with Down syndrome (DS) are affected by obstructive sleep apnea (OSA), and only 16% to 30% will have resolution of their OSA with adenotonsillectomy. Hypoglossal nerve stimulation is a well-established therapy for adults with OSA and was recently approved by the Food and Drug Administration for use in children with DS and residual OSA. There is robust experience with this therapy in adults that has led to well-established care pathways. However, given the challenges inherent to caring for a complex pediatric population, these pathways are not directly transferrable to children with DS.

Pediatric Down Syndrome Upper Airway Stimulation: Patient Selection and Post-Implantation Optimization.

OBJECTIVE: The Food and Drug Administration recently approved upper airway stimulation (UAS) for children with Down Syndrome and persistent obstructive sleep apnea who meet certain inclusion and exclusion criteria. Although there is a robust experience with this therapy in the adult population, established protocols used in adults are not directly transferrable to a complex pediatric population. This review aims to combine the protocols from several institutions for patient selection and postimplantation optimization, including a protocol for Drug-Induced Sleep Endoscopy in children with Down Syndrome, preactivation threshold measurements, device titration, and follow-up sleep studies. STUDY DESIGN: Expert panel development of best Practice algorithm. SETTING: Multi-institutional investigator review. METHODS: An expert panel was assembled of pediatric otolaryngologists with extensive experience in hypoglossal nerve stimulation in children with Down Syndrome. Thirty statements were created during an initial drafting session. A modified Delphi method was used assess consensus among the panel. RESULTS: After 2 rounds of Delphi surveys, 29 statements met criteria for consensus. One statement did not meet consensus. The statements were grouped into several categories to facilitate presentation. CONCLUSIONS: A standardized approach to UAS for children with Down Syndrome must take into account the unique challenges inherent to treating a complex pediatric population with a high rate of sensory processing disorders. This expert panel has met consensus on several statements that will guide clinicians as this novel therapy is adopted.

Challenges and adverse events in pediatric hypoglossal nerve stimulation.

INTRODUCTION: Hypoglossal nerve stimulation was recently FDA approved for use in children with Down Syndrome and persistent obstructive sleep apnea. Although there is a robust experience in hypoglossal nerve stimulation in adults, we observed several challenges that are unique to providing this therapy to a complex pediatric population with a high rate of sensory processing disorders. We sought to review the adverse events and challenges to inform clinicians as hypoglossal nerve stimulation becomes a more accessible option for this complex population. METHODS: Retrospective case series of children with Down Syndrome and persistent OSA who underwent hypoglossal nerve stimulation. Inclusion and exclusion criteria included Down Syndrome, age 10-22 years, persistent severe OSA after adenotonsillectomy (AHI>10 with <25 % central or mixed events), inability to tolerate positive airway pressure, and absence of concentric palatal collapse on sleep endoscopy. Patients were identified and their charts were reviewed. Adverse events and their subsequent management were recorded. The major outcome variable was the total number of adverse events. RESULTS: A total of 53 patients underwent implantation of a hypoglossal nerve stimulator; 35 (66 %) patients were male and the average age at implantation was 15.1 years (standard deviation 3.0y). A total of 30 adverse events were noted, including 17 nonserious and 13 serious. The most common nonserious complications included temporary tongue discomfort, rash at the surgical site, and cellulitis. Serious complications included readmission (for cellulitis, pain, and device extrusion), reoperation (most commonly for battery depletion) and pressure ulcer formation. CONCLUSION: Hypoglossal nerve stimulation provides a much-needed therapy for children with DS and persistent OSA after adenotonsillectomy. Although there is a robust experience in providing this treatment to adults, many considerations must be made when adapting this technology to a pediatric population with a high rate of sensory processing disorders.

Blue light laser recontouring for pediatric benign fibrovascular vocal fold lesions.

INTRODUCTION: Up to 40% of children with hoarseness due to benign fibrovascular vocal fold lesions do not respond to voice therapy and could benefit from further intervention to improve their communication abilities. Currently there are limited surgical options for children too young to comply with post-operative voice rest. We have begun using the 445 nm laser to recontour benign fibrovascular vocal fold lesions without post-operative voice rest in young children with hoarseness due to vocal fold nodules that is unresponsive to voice therapy. This report presents pilot data on the effect of this treatment on pediatric voice-related quality of life. METHODS: Pediatric patients with benign fibrovascular lesions and severe subjective dysphonia (per parents or teachers) were given the option of undergoing surgery or continuing voice therapy. Those that opted for surgery underwent 445 nm laser recontouring of their vocal folds. They were discharged home the same day with no voice rest required. Pre- and post-demographic, acoustic measures, auditory perceptual assessments, and validated patient quality of life measures were collected. RESULTS: Four patients (ages 3-8y, all male) were selected for surgery. The mean pre- and post-op PVRQOL scores were 59.4 (range: 22.5-80) and 98.1 (range: 97.5-100), respectively (low scores suggest lower voice-related QOL), and a mean change of 38.8 (previously established minimum clinically important difference: 12). Pre- and post-acoustic and aerodynamic measures similarly reflected this improvement. CONCLUSION: This pilot case series addresses a commonly seen population (children with benign fibrovascular lesions and significant dysphonia despite voice therapy) where the best practice for timing and types of surgical intervention is unclear. Photoangiolytic lasers (including the 445 nm laser) are gaining popularity for the treatment of benign laryngeal pathologies, and their use may expand treatment possibilities for children with severe dysphonia due to benign vocal fold lesions that do not respond to voice therapy. Further longitudinal investigations are necessary to confirm the safety and efficacy.

Endoscopic Management of Primary Acquired Cholesteatoma.

Endoscopic ear surgery is increasingly accepted as a primary modality for cholesteatoma surgery. A major advantage is the enhanced visualization of the middle ear in traditionally poorly accessible locations by the microscope. We discuss novel techniques for selective mastoid obliteration when a canal wall down mastoidectomy is necessary. Postoperatively, indications for non-echo planar diffusion-weighted imaging MRI versus second-look surgery are discussed. Finally, outcome data for endoscopic versus microscopic ear surgery are reviewed, which show equivalent outcomes regarding residual and recurrent disease, similar rates of complications, decreased pain, and shorter healing time.

Squamous Suture Synostosis: A Review With Emphasis on Cranial Morphology and Involvement of Other Cranial Sutures.

Squamosal suture synostosis has received little attention, potentially due to its rare nature. The authors present here a clinical report of isolated unilateral squamosal suture synostosis and a literature review, which produced 6 articles describing 33 patients of squamosal synostosis.Of the reported patients, 15 were associated with a craniofacial syndrome, 10 were nonsyndromic, and 8 were not specified. The cranial morphology varied greatly and only 1 patient was consistent with the morphology predicted by Virchow law-decreased vertical growth with compensatory ipsilateral longitudinal growth (manifesting as occipital and possibly frontal zygomatic bulging). Additional suture synostoses were observed in 36.3% of nonsyndromic and 80% of syndromic patients, suggesting that either squamosal synostosis may have an effect on other sutures, or more likely, only the most severe patients are recognized and reported.Surgical and nonsurgical interventions have found limited utility due to the subtle nature of the cranial defects and a lack of increased intracranial pressure, with a conservative follow-up course being the preferred treatment.