Advanced practice providers and children's hospital-based pediatric otolarynology practices.

Introduction: Advanced practice providers (APPs), including nurse practitioners and physician assistants, have been deployed in children's hospital-based academic pediatric otolaryngology practices for many years. However, this relationship in terms of prevalence, roles, financial consequences and satisfaction has not been examined. The objective of this study is to explore how APPs impact healthcare delivery in this setting.

Complete Tracheal Ring Deformity. A Translational Genomics Approach to Pathogenesis.

Complete tracheal ring deformity (CTRD) is a rare congenital abnormality of unknown etiology characterized by circumferentially continuous or nearly continuous cartilaginous tracheal rings, variable degrees of tracheal stenosis and/or shortening, and/or pulmonary arterial sling anomaly. To test the hypothesis that CTRD is caused by inherited or mutations in genes required for normal tracheal development. CTRD and normal tracheal tissues were examined microscopically to define the tracheal abnormalities present in CTRD.

Efficacy and Safety of Sirolimus in the Treatment of Complicated Vascular Anomalies.

Background And Objectives: Complicated vascular anomalies have limited therapeutic options and cause significant morbidity and mortality. This Phase II trial enrolled patients with complicated vascular anomalies to determine the efficacy and safety of treatment with sirolimus for 12 courses; each course was defined as 28 days.

Methods: Treatment consisted of a continuous dosing schedule of oral sirolimus starting at 0.

Multicenter Evaluation of the Effectiveness of Systemic Propranolol in the Treatment of Airway Hemangiomas.

Objective: To evaluate the effectiveness of systemic propranolol in airway infantile hemangioma (AIH) treatment.

Design: Case series with chart review.

Participants: Patients with AIH treated with propranolol between 2009 and 2012 with at least a 1-year follow-up.

Standardized outcome and reporting measures in pediatric head and neck lymphatic malformations.

Objective: To develop general and site-specific treatment effect and outcome measures to standardize the reporting of head and neck lymphatic malformation (HNLM) treatments.

Study Design: Consensus statement/expert opinion.

Setting: Multiple tertiary academic institutions.

Lymphatic malformations: diagnosis and management.

Lymphatic malformations are benign vascular lesions that arise from embryological disturbances in the development of the lymphatic system. They encompass a wide spectrum of related abnormalities, including cystic lymphatic lesions, angiokeratoma, lymphatic malformations that occur in bones (Gorham-Stout Syndrome), lymphatic and chylous leak conditions, and lymphedema. This article will focus only on lymphatic malformation mass lesions, whereas other related disease entities will be covered elsewhere in this journal issue.

Congenital salivary gland anlage tumor - in utero and postnatal imaging.

We present a case of an infant with congenital salivary gland anlage tumor, with fetal and postnatal imaging. To the best of our knowledge, this is the first case describing the in utero imaging findings of salivary gland anlage tumor. A fetal MRI was performed secondary to the clinical finding of polyhydramnios, which identified a nasopharyngeal mass.

Management of manifestations of epidermolysis bullosa.

Purpose Of Review: Children with epidermolysis bullosa can present with disease(s) of the ears, nose, and throat, often related directly to the pathophysiology of their epidermolysis bullosa. Otolaryngologic diseases in children with epidermolysis bullosa have to be managed having a proper understanding of the diagnosis and pathophysiology of epidermolysis bullosa. The purpose of this review is to describe the current nomenclature and diagnostic algorithms for epidermolysis bullosa, and methods for the management of cutaneous and mucosal lesions.

Tracheal agenesis with tracheoesophageal fistulae: fetal MRI diagnosis with confirmation by ultrasound during an ex utero intrapartum therapy (EXIT) delivery and postdelivery MRI.

Tracheal agenesis is a rare and essentially lethal anomaly with divergent prenatal imaging findings depending on the presence or absence of a tracheoesophageal fistula. All prenatally diagnosed cases of tracheal agenesis reported to date have not had a tracheoesophageal fistula and presented with thoracoabdominal findings similar to congenital high airway obstruction syndrome. We present the case of a 32-week gestation fetus with rapid onset of polyhydramnios and no persistent findings of congenital high airway obstruction syndrome that was ultimately diagnosed with tracheal agenesis plus tracheoesophageal fistula by fetal MRI.

Cutaneous vascular lesions.

In 1982, vascular anomalies were classified as either vascular tumors or vascular malformations. Hemangiomas were identified as benign tumors that undergo a phase of active growth characterized by endothelial proliferation and hypercellularity, followed by gradual tumor regression over the first decade. Vascular malformations were described as structural congenital anomalies derived from capillaries, veins, lymphatic vessels, arteries, or a combination of these.

Upper airway complications of junctional epidermolysis bullosa.

Objective: To assess the incidence of upper airway pathology in patients with junctional epidermolysis bullosa (JEB).

Study Design: We conducted a retrospective chart review of all patients with JEB who came to an interdisciplinary epidermolysis bullosa center at a tertiary care institution between 2004 and 2010.

Results: Twenty-five patients with JEB were identified, and 12 patients were seen in the otolaryngology clinic (age range, 2 months-15 years; 8 male, 4 female).

Voice outcomes following adult cricotracheal resection.

Objectives: Cricotracheal resection (CTR) is an effective treatment for moderate-to-severe laryngotracheal stenosis (LTS) in adults. However, one of the potentially significant sequela of this procedure is postoperative dysphonia and permanent voice alteration. The objective of this study is to characterize voice changes in adult patients with subglottic stenosis who have undergone CTR.

Neonates with tongue-based airway obstruction: a systematic review.

Objective: In this systematic review, the authors summarize the current evidence in the literature regarding diagnosis, treatment, and long-term outcomes in neonates with tongue-based airway obstruction (TBAO) and assess the level of evidence of included studies.

Data Sources: The terms Pierre Robin syndrome/sequence, micrognathia, retrognathia, and cleft palate were combined with airway obstruction, treatment, tongue-lip plication, and osteogenesis distraction to perform an Ovid literature search, yielding 341 references. The authors excluded references containing patients with isolated choanal/nasal obstruction, patients older than 12 months, and expert opinion papers, yielding 126 articles.

Antihypertensive drug-induced angioedema causing upper airway obstruction in children.

Objective: Angioedema is a well-described complication arising from the use of antihypertensive agents in the adult population. However, its occurrence and potential for upper airway compromise in pediatrics has only been sporadically reported in the literature. Our objective is to report and review the occurrence of antihypertensive-induced angioedema in the pediatric population and the potential for airway compromise.

Use of sentinel node biopsy for staging parameningeal rhabdomyosarcoma.

Identification of nodal involvement is important for treatment planning in patients with rhabdomyosarcoma, and is facilitated by sentinel node biopsy. Although it is employed primarily for extremity tumors, we report using sentinel node biopsy in a patient with parameningeal rhabdomyosarcoma arising in the ethmoid sinus. Lymphoscintigraphy with single photon emission computed tomography following injection of tracer at the tumor site helped identify contralateral cervical node involvement not previously recognized by physical exam, cross sectional imaging, or other functional imaging.

Comprehensive airway management of neonatal head and neck teratomas.

Objectives: To determine the success of initial airway management and to characterize late airway-related complications in patients with airway obstruction due to congenital head and neck teratomas.

Study Design: Case series with chart review.

Setting: Tertiary airway referral institution.

Severe micrognathia: indications for EXIT-to-Airway.

The ex utero intrapartum treatment (EXIT) procedure has become an important management option in cases of fetal airway obstruction. Select cases of severe micrognathia may be candidates for EXIT-to-Airway due to high-risk of airway obstruction at birth. Here we present three successful EXIT-to-Airway procedures for the management of congenital micrognathia in its most severe manifestations.

Developmental aspects of the upper airway: report from an NHLBI Workshop, March 5-6, 2009.

The upper airway serves three important functions: respiration, swallowing, and speech. During development it undergoes significant structural and functional changes that affect its size, shape, and mechanical properties. Abnormalities of the upper airway require prompt attention, because these often alter ventilatory patterns and gas exchange, particularly during sleep when upper airway motor tone and ventilatory drive are diminished.

A contemporary review of wound healing in otolaryngology: current state and future promise.

Over the last 15 years, a new paradigm shift has occurred in the way we approach and treat soft tissue defects to the face and neck. These changes are due to recent advances in wound healing research and new biological products that are now available to the otolaryngologist. This article summarizes some of the recent developments and newer concepts in soft tissue healing with skin, the subglottis and facial allotransplantation relevant to the otolaryngologist.

Laryngotracheoplasty as an alternative to tracheotomy in infants younger than 6 months.

Objective: To compare the success rates of laryngotracheoplasty (LTP) with those of anterior cricoid split (ACS) performed over the same period in infants younger than 6 months.

Design: Case-control study.

Setting: Tertiary children's hospital.

Fibroblast growth factor 18 gives growth and directional cues to airway cartilage.

Objectives/hypothesis: The majority of congenital airway anomalies arise from deficits in the respiratory tract cartilage, emphasizing the importance of this cartilage to the form and function of the upper airway. The primary objective of this study was to characterize molecular mechanisms that regulate rate and direction of chondrocyte growth in the larynx and trachea. Our hypothesis for this study was that fibroblast growth factor 18 (FGF18) provides proliferative and directional cues to the developing laryngeal and tracheal cartilage in the mouse by up-regulating the cartilage-specifying gene, Sox9.

A novel murine model for the examination of experimental subglottic stenosis.

Objective: To develop a novel mouse model of acquired subglottic stenosis (SGS) using heterotopic transplanted laryngotracheal complexes (LTCs).

Design: Pilot randomized controlled animal study.

Subjects: Forty-eight C57BL/6 mice.

Multicenter interrater and intrarater reliability in the endoscopic evaluation of velopharyngeal insufficiency.

Objective: To explore interrater and intrarater reliability (R (inter) and R (intra), respectively) of a standardized scale applied to nasoendoscopic assessment of velopharyngeal (VP) function, across multiple centers.

Design: Multicenter blinded R (inter) and R (intra) study.

Setting: Eight academic tertiary care centers.