Survey of Pediatric Otolaryngology Frenotomy Practice Patterns.

Objectives: Assess current frenotomy practice patterns of pediatric otolaryngologists via a cross-sectional survey.

Study Design: Survey study.

Methods: A 31-question electronic survey assessing frenotomy practice patterns was distributed to all American Society of Pediatric Otolaryngology (ASPO) members.

Rubella Virus Infected Macrophages and Neutrophils Define Patterns of Granulomatous Inflammation in Inborn and Acquired Errors of Immunity.

Rubella virus (RuV) has recently been found in association with granulomatous inflammation of the skin and several internal organs in patients with inborn errors of immunity (IEI). The cellular tropism and molecular mechanisms of RuV persistence and pathogenesis in select immunocompromised hosts are not clear. We provide clinical, immunological, virological, and histological data on a cohort of 28 patients with a broad spectrum of IEI and RuV-associated granulomas in skin and nine extracutaneous tissues to further delineate this relationship.

Racial and ethnic disparities in diagnostic efficacy of comprehensive genetic testing for sensorineural hearing loss.

Understanding racial and ethnic disparities in diagnostic rates of genetic testing is critical for health equity. We sought to understand the extent and cause of racial and ethnic disparities in diagnostic efficacy of comprehensive genetic testing (CGT) for sensorineural hearing loss (SNHL). We performed a retrospective cohort study at two tertiary children's hospitals on a diverse cohort of 240 consecutive pediatric patients (76% publicly insured, 82% non-White) with SNHL of unknown etiology who underwent CGT.

Access Challenge Index: A Novel Disparity Measure Predictive of Language Outcomes in Children Who Are Deaf/Hard of Hearing.

Objective: To evaluate the effect of demographic disparities on language outcomes in a diverse group of children who are deaf or hard of hearing.

Study Design: Retrospective cohort study.

Setting: UCSF Benioff Children's Hospital (a tertiary care center).

Primary Immunodeficiency in Children With Autoimmune Cytopenias: Retrospective 154-Patient Cohort.

Background: Primary immunodeficiency is common among patients with autoimmune cytopenia.

Objective: The purpose of this study is to retrospectively identify key clinical features and biomarkers of primary immunodeficiency (PID) in pediatric patients with autoimmune cytopenias (AIC) so as to facilitate early diagnosis and targeted therapy.

Methods: Electronic medical records at a pediatric tertiary care center were reviewed.

Genetic Testing Leading to Early Identification of Childhood Ocular Manifestations of Usher Syndrome.

Objectives: Hearing-loss gene panel testing (HLGPT) is increasingly accessible as a first-line test in determining the etiology of sensorineural hearing loss (SNHL) in children. A major advantage of HLGPT is early identification of syndromic forms of SNHL, especially Usher syndrome, prior to the development of overt syndromic phenotype, which may impact management and counseling. Here, we describe early ocular findings in children with clinically non-syndromic SNHL identified by HLGPT as having two variants associated with Usher Syndrome.

Clinical Consensus Statement: Ankyloglossia in Children.

Objective: To identify and seek consensus on issues and controversies related to ankyloglossia and upper lip tie in children by using established methodology for American Academy of Otolaryngology-Head and Neck Surgery clinical consensus statements.

Methods: An expert panel of pediatric otolaryngologists was assembled with nominated representatives of otolaryngology organizations. The target population was children aged 0 to 18 years, including breastfeeding infants.

Vasculitis in a Child With the Hyper-IgM Variant of Ataxia-Telangiectasia.

A subset of patients with Ataxia-Telangiectasia (A-T) have dramatically reduced levels of IgG, IgA, and IgE with retained or elevated IgM levels. Several reports suggest that these A-T patients with a "hyper-IgM phenotype" (HIgM) suffer more clinical immunologic consequences than other A-T patients. The immunopathologic mechanism driving this phenomenon is unknown, making it difficult to predict response to immunomodulatory therapy.

Cryoprobe retrieval of an airway foreign body: A case report and literature review.

We report a case of a 7-year-old boy with clinical and radiographic evidence of foreign body (FB) aspiration with a 2-week delay in diagnosis. The retrieval of the pushpin with traditional bronchoscopic instrumentation was made difficult by granulation tissue formation. A cryoprobe through a flexible bronchoscope was used to successfully remove the FB.

Sleep-disordered breathing in pediatric head and neck vascular malformations.

Objectives: To determine the prevalence of sleep-disordered breathing (SDB) symptoms among children with head and neck vascular malformations and to compare obstructive sleep apnea (OSA)-18 scores between children with head and neck vascular malformations and children with non-head and neck vascular malformations.

Study Design: Retrospective cohort and prospective cross-sectional studies METHODS: Forty-three pediatric subjects with head and neck vascular malformations evaluated at a tertiary-care multidisciplinary vascular anomalies center were included in a retrospective cohort study. Eighty-three consecutive pediatric subjects with vascular malformations evaluated at the same center were included in the prospective cross-sectional study.

Airway response to sirolimus therapy for the treatment of complex pediatric lymphatic malformations.

Head and neck lymphatic malformations can create airway management challenges requiring tracheotomy. Sirolimus, an inhibitor of mammalian target of rapamycin (mTOR), may inhibit growth of lymphatic malformations. We describe two patients born with large lymphatic malformations with improved airway symptoms following sirolimus therapy.

Pregnancy outcomes among African-American patients with systemic lupus erythematosus compared with controls.

Objective: In a study of Gullah African-Americans, we compared pregnancy outcomes before and after systemic lupus erythematosus (SLE) diagnosis to controls to test whether there is a predisease state that negativelyaffects pregnancy outcomes.

Design: Cases and controls reporting at least one pregnancy were included. Controls were all Gullah African-American females.

Airway hemangiomas in PHACE syndrome.

Objectives/hypothesis: To describe the clinical presentation and airway characteristics of infants with airway hemangiomas and concomitant PHACE syndrome and to determine the prevalence of airway hemangiomas in PHACE subjects at our institution.

Study Design: Case series.

Methods: Retrospective review including clinical presentation, airway findings, treatment measures, and outcomes.

Drug-induced sleep endoscopy in persistent pediatric sleep-disordered breathing after adenotonsillectomy.

Objectives: To demonstrate the feasibility of drug-induced sleep endoscopy (DISE) in the pediatric population and to examine DISE results in children with persistent sleep-disordered breathing (SDB) after tonsillectomy and adenoidectomy (T&A).

Design: Retrospective case series with medical chart review.

Setting: Tertiary pediatric medical center.

Comparison of imaging modalities in pediatric thyroglossal duct cysts.

Objectives/hypothesis: To determine and compare the accuracy of different imaging modalities including ultrasound (US), magnetic resonance imaging (MRI), and computed tomography (CT) in the diagnosis of thyroglossal duct cysts (TGDC) in children.

Study Design: Retrospective chart review.

Methods: A retrospective chart review was performed on patients under the age of 18 years who had undergone surgical excision of midline neck masses between January 2002 and June 2011.

Propranolol in the management of airway infantile hemangiomas.

Objective: To report our experience with propranolol in managing airway infantile hemangiomas.

Design: Case series of 3 consecutive patients who had extensive, symptomatic airway infantile hemangiomas treated with propranolol.

Setting: Tertiary academic medical center.

Extensive airway hemangiomas in two patients without beard hemangiomas.

Airway hemangiomas are most often seen in association with cutaneous hemangiomas involving the "beard area." We report two unusual cases of extensive airway hemangiomas developing in patients with facial hemangiomas predominantly involving the upper face, emphasizing the need to consider the possibility of airway hemangiomas even in the absence of "beard area" hemangiomas.