Amelia F Drake

• MD
• Head of Faculty at University of North Carolina at Chapel Hill

Purpose: Craniofacial microsomia (CFM) is a congenital condition that can be associated with feeding challenges in infants. As part of the larger ‘Craniofacial microsomia: Accelerating Research and Education (CARE)’ program, this study described caregivers’ early feeding experiences. Design and Materials: US-based caregivers of children with CFM (...

Objective: Children with craniofacial microsomia (CFM) have complex healthcare needs, resulting in evaluations and interventions from infancy onward. Yet, little is understood about families’ treatment experiences or the impact of CFM on caregivers’ well-being. To address this gap, the NIH-funded ‘Craniofacial microsomia: Accelerating Research and...

Purpose This article reviews the literature focused on the psychological effects of craniofacial care for patients and their families. It provides an overview of pediatric medical traumatic stress (PMTS) associated with craniofacial conditions and related care, along with a review of its risk and protective factors. Findings from studies of PMTS in...

Objective Individuals with cleft lip and palate (CLP) are at a high risk of developing obstructive sleep apnea (OSA). Hypothetically, the severity of OSA might be associated with the morphology of the upper airway (UAW) and the characteristics of the airflow. Thus, the present study aimed to assess and compare, in adults with CLP and skeletal class...

Objective: Craniofacial microsomia (CFM) is a broad clinical term used to describe a congenital condition most commonly involving the underdevelopment of the external ear, mandible, soft tissues, and facial nerve. Despite medical advances, understanding of the psychological health and healthcare experiences of individuals with CFM and their caregi...

As healthcare systems become more complex, medical education needs to adapt in many ways. There is a growing need for more formal leadership learning for healthcare providers, including greater attention to health disparities. An important challenge in addressing health disparities is ensuring inclusive excellence in the leadership of healthcare sy...

As the nation seeks to recruit and retain physician-scientists, gaps remain in understanding and addressing mitigatable challenges to the success of faculty from underrepresented minority (URM) backgrounds. The Doris Duke Charitable Foundation Fund to Retain Clinical Scientists (FRCS) program, implemented in 2015 at 10 academic medical centers in t...

Background: Insufficient support for balancing career and family responsibilities hinders retention of physician-scientists. Programs to improve retention of this important group of faculty are crucial. Understanding the experiences of program implementers is key to refining and improving program offerings. Methods: We conducted an interpretive, de...

Craniofacial microsomia (CFM) is the second most common congenital facial anomaly, yet its genetic etiology remains unknown. We perform whole-exome or genome sequencing of 146 kindreds with sporadic (n = 138) or familial (n = 8) CFM, identifying a highly significant burden of loss of function variants in SF3B2 (P = 3.8 × 10⁻¹⁰), a component of the...

Children with craniofacial microsomia (CFM) are at increased risk for educational and social concerns. This study describes intervention services and frequency of teasing in a multinational population of children with CFM. Caregivers of children with CFM ages 3 to 18 years in the US and South America were administered a questionnaire. Additional in...

Objective To examine neurodevelopment in preschool-aged children with craniofacial microsomia (CFM) relative to unaffected peers. Design Multisite, longitudinal cohort study. Setting Tertiary care centers in the United States. Participants We included 92 children with CFM (“cases”) through craniofacial centers and clinics. Seventy-six children w...

Objective: To report brain magnetic resonance imaging (MRI) and ultrasonography findings in pediatric patients with congenital idiopathic bilateral vocal fold dysfunction and analyze factors associated with its etiology and resolution. Study design: Case series with retrospective review. Setting: Tertiary care multi-institutional setting: Nati...

Objective The study aim was to assess behavioral adjustment in preschool children with and without craniofacial microsomia (CFM). Design Multisite cohort study of preschoolers with CFM (“cases”) or without CFM (“controls”). Participants Mothers (89%), fathers (9%), and other caregivers (2%) of 161 preschoolers. Outcome Measure Child Behavior Che...

Objective An increasing number of patients use social media for health-related information and social support. This study’s objective was to describe the content posted on Facebook groups for individuals with microtia and/or craniofacial microsomia (CFM) and their families in order for providers to gain insight into patient and family needs and exp...

Background: Research is needed to improve understanding of work-life integration issues in academic medicine and to guide the implementation of the Doris Duke Charitable Foundation's Fund to Retain Clinical Scientists (FRCS), a national initiative offering financial support to physician-scientists facing caregiving challenges. Materials and Method...

Objective: To three-dimensionally evaluate the upper airway of individuals with cleft lip and palate (CLP) and Class III malocclusion and the occurrence of obstructive sleep apnea (OSA). Methods: Twenty-one CLP individuals with Class III malocclusion, 20–29 years of age, who underwent computed tomography for orthognathic surgery planning, were pros...

Craniofacial interventions are common and the surgical options continue to grow. The issues encountered include micrognathia, macroglossia, midface hypoplasia, hearing loss, facial nerve palsy, hemifacial microsomia, and microtia. In addition, a unifying theme is complex upper airway obstruction. Throughout a child's life the focus of interventions...

Purpose: To enhance understanding of challenges related to work-life integration in academic medicine and to inform the ongoing implementation of an existing program and the development of other interventions to promote success of physician-scientists. Method: This study is part of a prospective analysis of the effects of the Fund to Retain Clin...

Objectives Craniofacial microsomia (CFM) is a congenital condition that typically involves hypoplasia of the ear and jaw. It is often associated with adverse effects such as hearing loss and sleep‐disordered breathing. There is little research on its etiology. Methods We conducted a case–control study from maternal interview data collected from mo...

Objective: The Craniofacial microsomia: Longitudinal Outcomes in Children pre-Kindergarten (CLOCK) study is a longitudinal cohort study of neurobehavioral outcomes in infants and toddlers with craniofacial microsomia (CFM). In this article, we review the data collection and methods used to characterize this complex condition and describe the demog...

Objectives Neonates weighing < 2.5 kg have known higher rates of surgical mortality and morbidity, but this remains unexamined specifically for tracheostomy. We present outcomes of neonates undergoing tracheostomy stratified by weight. Methods Retrospective review of the American College of Surgeons National Surgical Quality Improvement Program–Pe...

The Facial Asymmetry Collaborative for Interdisciplinary Assessment and Learning (FACIAL) network applies key principles of established team science while using equity-based approaches that advance career development and accelerating collaborative research in craniofacial microsomia. Team science is an effort to leverage a challenge in science by u...

Obstruction of the upper airway commonly contributes to respiratory distress in the premature neonate. It is a multifactorial problem that can present heterogeneously and requires accurate anatomical and pathophysiological diagnosis for effective treatment. In this chapter, the diagnosis and treatment options, as well as outcomes, of upper airway o...

In the 30 years since Leonard Furlow published his landmark article on the double-opposing Z-plasty technique to repair cleft palate, this procedure has been used with great enthusiasm.¹ Initially employed in the repair of primary cleft palate, it has been reported in submucous cleft palate and in secondary palate repair with excellent results.² St...

Objective: To describe medical malpractice trends in patients with cleft and/or craniofacial abnormalities. Methods and materials: A modified Delphi approach was used to gather search terms. Search settings included "all jury verdicts and settlements", with jurisdiction of "all states" and "all federal courts" (by court and circuit). A retrospec...

Objectives/hypothesis: The purpose of this study is to present one of the largest case series of pediatric transoral robotic surgery (TORS) in the upper airway demonstrating a wide range of ages and indications. Study design: A retrospective case series at an academic tertiary referral center from August 2010 to September 2014. Methods: The da...

Background: Children with complex airway problems see multiple specialists. To improve outcomes and coordinate care, we developed a multidisciplinary Children's Airway Center. For children with tracheostomies, aspects of care targeted for improvement included optimizing initial hospital discharge, promoting effective communication between provider...

OBJECTIVES/HYPOTHESIS: Children with cleft lip and palate (CLP) often suffer from nasal obstruction that may be related to effects on nasal volume. The objective of this study was to compare side:side volume ratios and nasal volume in patients with unilateral (UCLP) and bilateral (BCLP) clefts with age-matched controls. STUDY DESIGN: Retrospective...

Objectives/HypothesisThe aim of this study was to compare the volume of nasopharyngeal airways of patients with unilateral cleft lip and palate (UCLP) with a control (noncleft) group of subjects.Study DesignRetrospective case-control study.Methods The sample consisted of 45 subjects, 30 with UCLP who underwent cone beam computed tomography (CBCT) e...

To examine the characteristics of sleep in patients with Goldenhar Syndrome. Retrospective review of all polysomnography studies conducted at the University of North Carolina Hospitals between 2003 and 2013 on patients carrying the diagnosis of Goldenhar's Syndrome. A preponderance of patients demonstrated severe obstructive sleep apnea and hyperca...

Limited quality of life data exist for pediatric chronic rhinosinusitis (CRS) patients undergoing endoscopic sinus surgery (ESS). Further exploration of the following areas will enhance understanding and support clinical decision-making: baseline and post-ESS general and disease-specific quality of life, parent vs. child report, and correlation of...

Background: Limited quality of life data exist for pediatric chronic rhinosinusitis (CRS) patients undergoing endoscopic sinus surgery (ESS). Further exploration of the following areas will enhance understanding and support clinical decision-making: baseline and post-ESS general and disease-specific quality of life, parent vs. child report, and cor...

Craniofacial microsomia (CFM) is a complex condition associated with microtia, mandibular hypoplasia, and preauricular tags. It is the second most common congenital facial condition treated in many craniofacial centers and requires longitudinal multidisciplinary patient care. The purpose of this article is to summarize current recommendations for c...

Objective: To identify the presenting features of a laryngeal cleft in children. To compare rigid and flexible endoscopic methods available for identifying laryngeal cleft and determine their utility. Methods: The charts of all patients diagnosed with laryngeal cleft in a tertiary care institution between 2009 and 2010 were evaluated retrospectivel...

Children with cleft palate and other craniofacial anomalies are at risk for a variety of otopathologies including otitis media and structural deformities of the outer and middle ear. These conditions are usually associated with conductive hearing impairment; however, when hearing loss occurs as part of a syndrome, there may be sensorineural or mixe...

Abstract An otolaryngology evaluation is an important aspect of care for any craniofacial patient. Otolaryngologists are commonly involved in the care of these patients to treat chronic otitis media, frequently requiring placement of pressure equalization tubes; however, there are many other otorhinolaryngologic issues that are addressed. Craniofac...

Introduction: Few educational programs exist for medical students that address professionalism in surgery, even though this core competency is required for graduate medical education and maintenance of board certification. Lapses in professional behavior occur commonly in surgical disciplines, with a negative effect on the operative team and patie...

This review describes important aspects of the most commonly encountered craniofacial syndromes. The goal is to provide otolaryngologists and other health care providers with critical information necessary to manage these patients appropriately. The algorithm provided in this article should be helpful in guiding the treatment of craniofacial patien...

Sleep disordered breathing syndromes in pediatric patients can lead to adverse effects in the cardiovascular system, neurocognitive function, growth, and behavior. These syndromes occur more frequently in patients with craniofacial disorders. A high index of suspicion as well as early recognition, detection, and treatment of these syndromes are con...

Craniofacial microsomia (CFM) is a congenital condition associated with orbital, mandibular, ear, nerve, and soft tissue anomalies. We present a standardized, two-dimensional, digital photographic protocol designed to capture the common craniofacial features associated with CFM.

Objective: 1) Identify the signs and symptoms of a laryngeal cleft in children. 2) Compare diagnostic methods available for identifying laryngeal cleft and determine their utility. Method: The charts of all patients diagnosed with laryngeal cleft in a tertiary care institution between 2009 and 2010 were evaluated retrospectively for age, gender, co...

Objective: Understand the complex management and prognosis of patients with congenital oral and pharyngeal masses that cause cleft palate. To understand the multifaceted role of the pediatric otolaryngologist in the care of these patients. Method: Retrospective study of all patients with oral or pharyngeal masses and cleft palate or velopharyngeal...

Objective: Social networking sites such as Facebook are popular avenues of social discourse among adolescents and young adults, allowing for communication through photographs, videos, and chat features. These websites are also emerging as portals for health-centered support groups, bringing together people with common medical conditions. Adolescen...

Objective: The greater palatine canal (GPC) local injection is used to limit posterior bleeding during sinus surgery in adults. Given the potential for causing iatrogenic damage to the intra-orbital contents, this procedure is not commonly utilized in the pediatric population. No studies have described the anatomic development of the GPC during fac...

The greater palatine canal (GPC) local injection is used to limit posterior bleeding during sinus surgery in adults. Given the potential for causing iatrogenic damage to the intraorbital contents, this procedure is not commonly used in the pediatric population. No studies have described the anatomic development of the GPC during facial growth. By u...

Little is known about the school experience of children with tracheostomy tubes. These children may represent a population that qualifies for special services in school. Understanding how tracheostomy affects school-aged children may provide information needed to develop programs that provide these children with invaluable experiences. To understan...

To characterize the ultrasonographic appearance of laryngeal papillomatosis and to compare ultrasound with direct laryngoscopy and bronchoscopy, the criterion standard, for airway evaluation. Prospective, nonrandomized analysis of preoperative and postoperative airway ultrasound images. Tertiary, university-based medical center. Eight patients (4 f...

Infantile myofibromatosis is a rare proliferative mesenchymal disorder that has a potential for rapid growth and recurrence. Approximately 30% of infantile myofibromatosis lesions present in the head and neck, and some of these can cause significant morbidity. We report a case of recurrent infantile myofibromatosis that persisted after surgery and...

Evaluate parental perceptions associated with tracheostomy morbidity and quality of life in the management of Pierre Robin Sequence (PRS). Retrospective review/survey. 42 Pierre Robin patients were identified, records were reviewed and airway assessments evaluated relative to airway compromise. Twenty patients had undergone tracheostomy. Perception...

To determine the effect of chronic (recurrent) otitis media with effusion (OME) on frequency weighting in the perception of speech in noise. It was hypothesized that children with a history of OME weight speech information in the mid frequency region higher than control children. This is a matched cohort study looking at differences in frequency we...

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. Multicenter blinded R (inter) and R (intra) study. Eight academic tertiary care centers. Sixteen otolaryngologists from 8 centers. Raters esti...

The choice of antibiotics for sinusitis in children with cystic fibrosis (CF) is empirical or based on lower airway cultures, because sinus cultures are difficult to obtain. The aim of this study was to identify the main organisms cultured from CF children with chronic sinusitis, and to evaluate the concordance of concomitant sinus, oropharyngeal s...

Obstruction of tympanostomy tubes is a potentially significant complication, sometimes requiring replacement of the nonfunctioning tube. Early blockage can occur secondary to bleeding during the tube placement procedure. Delayed obstruction is usually caused by inspissated secretions or epithelial casts. We briefly report our treatment of 9 cases o...

The goal of this review is to provide a synthesis of the current literature addressing the effects of sleep-disordered breathing on cognition and behavior in children aged 2-18. A computerized search was performed to include studies between 1966 and 2001. Studies were assessed based on research question, sampling, intervention, outcomes, confoundin...

Children with syndromic craniosynostosis have an increased risk of airway obstruction. Addressing this issue early in their clinical course helps ensure a safe result. Because of their abnormal airways and a higher risk of sleep-related breathing disorders, regular evaluation of the airway is recommended. An algorithm for evaluation, including slee...

Prevention and early recognition remain critical factors in the treatment of foreign body inhalation in children. Accidental inhalation of both organic and nonorganic foreign body material continues to be a cause of childhood morbidity and mortality. The University of North Carolina Department of Otolaryngology has collected foreign bodies acquired...

To determine the effect of otitis media with effusion (OME) on perceptual masking (a phenomenon in which spondee threshold for a 2-talker masker is poorer than for a speech-shaped noise masker). Longitudinal testing over a 1-year period following insertion of tympanostomy tubes, using clinical and normal-hearing control groups. Forty-seven children...

Twenty-four nasal mucosa specimens were obtained from the inferior or middle turbinates of 6 normal subjects and 18 patients with chronic sinusitis, inflammatory polyp formation, or sinus allergies. Reverse transcription—polymerase chain reaction analysis was used to identify the non-neuronal nicotinic cholinergic receptor (nAChR) subunits that wer...

To explore how rigid and flexible bronchoscopy are used in pediatric otolaryngologic practice. Survey. Members of the American Society of Pediatric Otolaryngology who practice in the United States and Canada and were listed in the membership directory were eligible. Of the 206 members, 24 practicing outside the United States or Canada and 11 withou...

A choristoma is a tumor-like mass consisting of tissues that are foreign to the site at which they are located. A hamartoma, on the other hand, is a tumor-like mass consisting of tissues that are native to the site of origin. We report a choristoma in the nasopharynx that consisted of normal skin tissue.

The possible occurrence of developmental effects associated with mild hearing loss remains a significant concern. Recent psychoacoustic results in animals and humans suggest that mild conductive hearing loss can have negative consequences for binaural hearing in young listeners. These results appear to have physiological analogues in animal studies...

The purpose of this study is to determine the effect of histamine-induced nasal congestion on nasal airflow and the perception of externally applied resistance to nasal breathing. Nasal cross-sectional area and nasal airflow during free breathing were measured in 15 adult subjects before and after histamine challenge. The threshold for perception o...

Despite enormous advances in the field of pediatric airway, stridor continues to be the hallmark sign of airway compromise. Recognition of the signs and symptoms of airway obstruction promotes early diagnosis. The diagnostic evaluation of stridor has progressed with newer techniques being studied. When appropriate, these are added to the time-honor...

Otolaryngologists have long been involved in influencing social policy. Through his extensive efforts, Chevalier Jackson, MD, helped to enact the Federal Caustic Act of 1927.1 Despite such changes in federal law, Jackson spent much of his career treating asphyxiation due to foreign body aspiration. Today, foreign body aspiration remains the most im...

To determine whether there is a relationship between the presumed complexity of auditory processing and the time course of recovery of auditory function in children with a history of otitis media with effusion (OME). Longitudinal testing over a 1-year period following insertion of tympanostomy tubes in clinical and control groups. A total of 34 chi...

Congenital nasal pyriform aperture stenosis (CNPAS) is a recently defined clinical entity that causes airway obstruction in the neonate as a result of narrowing of the nasal pyriform aperture. The first description of nasal pyriform aperture stenosis (NPAS) was in 1952 by Douglas,1 who reported a series of eight affected adults, most of whom also h...

In infants and children, cervical masses originate from a variety of pathologic, congenital and developmental conditions, and it is important to distinguish benign from serious lesions. Fibromatosis colli is a rare cervical lesion that typically presents in the neonatal period. It is the most common cause of neonatal torticollis and should be recog...

Nitric oxide (NO) production in the respiratory epithelium of the upper airways has recently been described. To better delineate the role of epithelial NO, the authors of this study attempted to identify the cell type responsible for the production of NO in rat tracheal epithelium and human nasal epithelium. They localized the activity of NO throug...

We reviewed cases of early childhood hoarseness caused by vocal nodules and functional hearing loss as being possible manifestations of child abuse. This is a retrospective review set in two urban referral centers. The patients consisted of four children with vocal nodules and known histories of abuse, and four children with functional hearing loss...

CT and MR revealed a case of nasopharyngeal atresia, a malformation in which the soft palate is not formed, and the hard palate extends posteriorly to fuse with the anterior surface of the clivus, resulting in complete isolation of the nasal and oral cavities and the absence of a nasopharynx. We believe this rare anomaly results from abnormal persi...

The hypothesis that upper airway breathing behaviors generally follow the rules of a physiologic regulating system implies the existence of sensors that monitor the airway environment. The purpose of this study was to assess the sensitivity of the monitoring system to sudden changes in airway patency in healthy, adult subjects. An instrument capabl...

Oral antibiotic therapy can alter the gastrointestinal microflora and result in troublesome gastrointestinal complaints. Patients who have experience with broad-spectrum antibiotics may be reluctant to start or to comply with antibiotic therapy due to the associated discomfort. In the field of otolaryngology, oral antibiotic therapy is commonplace,...

The hypothesis that upper airway breathing behaviors generally follow the rules of a physiologic regulating system implies the existence of sensors that monitor the airway environment. The purpose of this study was to assess the sensitivity of the monitoring system to sudden changes in airway patency in healthy, adult subjects. An instrument capabl...

The present study was undertaken to determine whether a modification to commercially available acoustic rhinometry (AR) instrumentation might allow equipment designed for use in adults to determine accurately the nasal cavity configuration of infants and children. The standard wave tube was replaced with a version having a narrow (0.312-cm2) intern...

The present study was undertaken to determine in model studies whether currently available acoustic rhinometry instrumentation might be used to analyze the nasal cavity configuration of infants and children. A simple nasal cavity model was constructed using eight Lucite inserts that were placed between standard nosepieces provided by the manufactur...

The present study was undertaken to determine whether a modification to commercially available acoustic rhinometry (AR) instrumentation might allow equipment designed for use in adults to accurately determine the nasal cavity configuration of infants and children. The standard wave tube was replaced with a version having a narrow (0.312 cm2) intern...

A technique that combines psychophysical measurements with continuous recording of nasal patency and respiratory behavior was used to study the psychophysical and respiratory responses of 10 subjects to well-controlled stimulation with three compounds differing in relative stimulatory effectiveness for nasal olfactory and trigeminal chemoreceptors....

A technique that combines psychophysical measurements with continuous recording of nasal patency and respiratory behavior was used to study the psychophysical and respiratory responses of 10 subjects to well-controlled stimulation with three compounds differing in relative stimulatory effectiveness for nasal olfactory and trigeminal chemoreceptors....

Seven patients who received pharyngeal flaps for velopharyngeal incompetence (VPI) were studied to assess the effect of the procedure on nasal airway size. The findings suggest that the pharyngeal flap does not significantly decrease the upper airway in all patients. The effect of the flap did not correlate with the type of cleft, and was most pron...

Clefts of the lip and palate frequently produce nasal deformities that tend to reduce the size of the nasal airway. Surgical correction of nasal, palatal, and pharyngeal structures may compromise breathing further. A significant number of individuals with cleft noses mouthbreathe to some extent because of the high prevalence of airway compromise.