Brianne Barnett Roby

Sinus surgery in cystic fibrosis patients: comparison of sinus and lower airway cultures.

OBJECTIVE The aim of this study is to determine whether cross-infection occurs between infections in the sinuses and lower airways in Cystic Fibrosis patients, and to determine whether the infections begin in the sinuses before spreading to the lungs. METHODS Retrospective study of pediatric Cystic Fibrosis patients who underwent simultaneous sinus surgery and bronchial washings. The results of the cultures were reviewed to determine if the same organisms colonized both the sinuses and lower airways. RESULTS Staphylococcus aureus (Staph. Aureus) was found in 40.7% of the sinuses but only 33.3% of the lower airways. One patient had Staph. Aureus in the lower airway but not the sinuses. Pseudomonas, Hemophilus Influenza, and Moraxella showed similar patterns: multiple instances of positive sinus cultures and negative bronchial cultures, but only a small number of cases with positive lower airway cultures and negative sinus cultures. CONCLUSION The data showed that as patients age, they are more likely to have infections in both upper and lower airways, but infections start in the upper airways at a younger age. In many cases, organisms were cultured from sinuses in patients who had negative lower airway cultures. In only a few instances, organisms grew in the lower airways and not the sinuses. In most cases, the bacteria that caused sinus infections at a young age caused the lung infections in older patients. This is the paper to show that bacteria spread from the sinuses to the lungs to cause infections in Cystic Fibrosis patients, and not vice-versa.

Pediatric tracheal and endobronchial tumors: an institutional experience.

OBJECTIVES To report the pathologic findings in cases involving endotracheal and endobronchial tumors in the pediatric population and to describe the presenting symptoms and treatment modalities for endotracheal and endobronchial tumors. DESIGN Retrospective chart review. SETTING Tertiary care childrens hospital. PATIENTS The study included 14 patients with endotracheal and endobronchial tumors. MAIN OUTCOME MEASURES Patients were selected if bronchoscopy was performed to obtain biopsy specimens from the trachea or bronchus. RESULTS There were 14 cases that met the inclusion criteria between 1993 and 2009. The patients ranged in age from 4 to 18 years. The most common presenting symptom was recurrent pneumonia (n = 6), followed by wheezing or asthma that was unresponsive to treatment (n = 4). Nine lesions (64%) were malignant and 5 (36%) were benign. Of the malignant tumors, 5 (55%) were carcinoid, 3 (33%) were mucoepidermoid carcinoma, and 1 was adenoid cystic carcinoma. There were 1 or 2 cases of each of the following benign to intermediate malignant potential lesions: histoplasmosis nodules, chondroid hamartoma, pulmonary chondroma, and inflammatory myofibroblastic tumor. In 12 cases, definitive treatment included surgical resection. Three of these cases required postoperative chemotherapy and radiotherapy. CONCLUSIONS The results of this series suggest that in the pediatric population tracheal and endobronchial tumors are most likely to be carcinoid tumors or mucoepidermoid carcinomas, both malignant processes. For patients with recurrent pneumonias or chronic wheezing, an occult tumor is a diagnostic consideration that may require additional studies.

Prevalence of Positional Plagiocephaly in Teens Born after the "Back to Sleep" Campaign

Objectives. To determine the prevalence of positional plagiocephaly and brachycephaly in teenagers born after the “Back to Sleep” campaign but before orthotic helmet treatment became widely available and to provide long-term outcomes data on those children with plagiocephaly who were not treated with remolding therapy. Study Design. Cross-sectional analysis. Setting. Local high schools in the Minneapolis–St Paul Metro area. Subjects and Methods. Subjects were selected if they attended participating high schools and were born after the Back to Sleep campaign began. Skull measurements, including transcranial diameter, length, and width, were taken. Overall facial appearance was inspected for any apparent abnormal characteristics. Cranial vault asymmetry and cephalic index were calculated for each participant. Plagiocephaly was diagnosed if cranial vault asymmetry was >1 cm. Brachycephaly was diagnosed if the cephalic index was >0.90. Results. There were 1045 participants, ranging from 12 to 17 years old, with an average age of 15.7 years. The prevalence of plagiocephaly was 1.1%. The prevalence of brachycephaly was 1.0%. The overall prevalence of a deformational cranial abnormality was 2.0%. Of those who met diagnostic criteria of plagiocephaly or brachycephaly, 38.1% were noted to have abnormal facial characteristics. Conclusions. The prevalence of plagiocephaly and brachycephaly in teenagers is significantly lower than the 20% to 48% prevalence found in infants in previous studies, suggesting most children will outgrow the condition without intervention. Additional studies are needed to determine which patients might benefit from treatment and which will likely have resolution without intervention, since treating all infants who meet criteria results in significant overtreatment.

Symptom Timeline Preceding Cochlear Implant Failure An Institutional Experience

Objective. Review cochlear explants and determine the incidence of device and medical failures and develop a pattern of symptoms indicating probable implant failure. Study Design. Case series with chart review. Setting. Tertiary referral center. Subjects and Methods. Subjects were selected if they underwent cochlear explantation. Data were reviewed to determine initial symptom and symptom timeline preceding implant failure. Results. A total of 847 implants were performed from 1988 to 2008, with 128 devices explanted. Of total implants, 72% were Advanced Bionic devices, and 28% were Cochlear Nucleus devices. Overall failure rate was 128 of 847 (15%), with 51 (6%) medical failures and 77 (9%) device failures. Patients with Advanced Bionic devices underwent 102 explants (16.7% failure rate), with 35% medical failures and 65% device failures. Patients with Nucleus devices underwent 26 explants (11% failure rate), with 58% medical failures and 42% device failures. Medical failures included infected devices and wound dehiscence, with the pattern averaging 4.5 months to explantation. Hard device failures included sudden malfunction and slow decline in function, with pattern of failure over 4.2 months. Soft failures included tinnitus and discomfort, with failure over 8 months. Conclusions. There were common patterns when evaluating both medical and device failures. By recognizing patterns of symptoms that may indicate probable implant failure, otolaryngologists can better counsel patients on what to expect with their implants when they present with certain symptoms.

Neuroradiographic findings in 22q11.2 deletion syndrome

22q11.2 deletion syndrome (22q11.2DS) is a common genetic disorder with enormous phenotypic heterogeneity. Despite the established prevalence of developmental and neuropsychiatric issues in this syndrome, its neuroanatomical correlates are not as well understood. A retrospective chart review was performed on 111 patients diagnosed with 22q11.2DS. Of the 111 patients, 24 with genetically confirmed 22q11.2 deletion and brain MRI or MRA were included in this study. The most common indications for imaging were unexplained developmental delay (6/24), seizures of unknown etiology (5/24), and unilateral weakness (3/24). More than half (13/24) of the patients had significant radiographic findings, including persistent cavum septi pellucidi and/or cavum vergae (8/24), aberrant cortical veins (6/24), polymicrogyria or cortical dysplasia (4/24), inner ear deformities (3/24), hypoplastic internal carotid artery (2/24), and hypoplastic cerebellum (1/24). These findings reveal the types and frequencies of brain malformations in this case series, and suggest that the prevalence of neuroanatomical abnormalities in 22q11.2DS may be underestimated. Understanding indications for imaging and frequently encountered brain malformations will result in early diagnosis and intervention in an effort to optimize patient outcomes.

Complete peripartum airway management of a large epignathus teratoma: EXIT to resection

We present a case of a female infant that was diagnosed on prenatal ultrasound with an oral mass, and subsequently underwent a planned EXIT procedure to secure the airway. Ultimately the epignathus teratoma was excised at 3 days of age. The number of cases in which neonates have survived epignathus teratomas has increased in the past decade due to improved prenatal imaging and the development of the EXIT procedure. This case demonstrates the importance of a multidisciplinary approach that must be established when a prenatal diagnosis raises concern for airway compromise, and the thorough work-up required for definitive management.

Early Airway Intervention for Craniofacial Anomalies

This article reviews the presentation of children with craniofacial anomalies by the most common sites of airway obstruction. Major craniofacial anomalies may be categorized into those with midface hypoplasia, mandible hypoplasia, combined midface and mandible hypoplasia, and midline deformities. Algorithms of airway interventions are provided to guide the initial management of these complex patients.

Upper airway obstruction in neonates: Does sleep exacerbate symptoms?: Upper Airway Obstruction in Neonates

Describe the factors that exacerbate upper airway obstructions (UAOs) in neonates.

Congenital and iatrogenic laryngeal and vocal abnormalities in patients with 22q11.2 deletion

BACKGROUND Voice abnormalities often go unrecognized in patients with 22q11.2 deletion because speech abnormalities become the focus of evaluation. OBJECTIVE To analyze voice and vocal fold abnormalities in patients with 22q11.2 deletion by examining voice, not speech. METHODS This is a case series with chart review from 2009 to 2016. Records of both a velocardiofacial syndrome (VCF) clinic and pediatric otolaryngology clinic at a tertiary pediatric hospital were reviewed. All patients with confirmed 22q11.2 deletion were identified. Cardiac, otolaryngological, speech, and voice characteristics were recorded along with surgical history. Main outcomes included voice characteristics based on speech therapy assessment, along with vocal fold or laryngeal abnormalities. Co-morbidities and surgical history were also recorded. RESULTS 109 patients were identified with 22q11.2 deletion by genetic testing. Eighteen percent (n = 20) displayed a vocal fold or laryngeal abnormality, either congenital or iatrogenic. The most common congenital abnormalities were laryngeal web (n = 10) and subglottic stenosis (n = 7). The most common iatrogenic abnormality was paralyzed left vocal fold (n = 4). Perceptual analysis by speech therapy showed 65% (n = 71) with a voice within normal limits while 17% (n = 19) were not within normal limits. Of the 20 patients with a vocal fold abnormality, 15% (n = 3) presented a voice within normal limits, while 65% (n = 13) were not within normal limits. Of the 19 patients with a voice outside normal limits, 68% (n = 13) had a diagnosed vocal fold abnormality. Sixteen percent (n = 3) of these patients had an abnormal voice as a result of other issues. CONCLUSION Out of 109 patients with 22q11.2 deletion, 18% displayed a laryngeal abnormality and 17% had abnormal vocal quality. This suggests that voice and vocal fold abnormalities are important to consider in this population. Distinguishing between speech and voice abnormalities in patients with 22q11.2 deletion can help ensure appropriate intervention.

Assisted Reproduction is Not Associated with Increased Risk of Congenital Head and Neck Defects

This abstract was presented at the American Academy of Otolaryngology-Head and Neck Surgery Annual Meeting, Orlando, FL, September 2014 with the abstract published (Neumann C, Thompson D, and Sidman J; Assisted reproduction is not associated with increased risk of head and neck defects; Otolaryngology-Head and Neck Surgery; Vol 151, Issue 1, supplement, 2014). Objectives - Compare the rate of head and neck anomalies between children conceived via artificial reproductive technology (ART) versus those conceived via natural methods. - Determine the risk of congenital head and neck abnormalities associated with ART. Study design A retrospective chart review cross-sectional study from 2004-2014 of all patients admitted to the neonatal intensive care unit (NICU) at a tertiary pediatric hospital. Results A total of 14,857 charts were examined; 2,288 patients were conceived via ART, while 12,569 patients were conceived via natural methods. There were 8,022 males and 6,637 females. There were 40 patients born with defects via ART, while there were 681 patients born with defects via natural conception. The total occurrence of congenital malformations was higher for patients conceived naturally versus those conceived with artificial reproduction (5.41% vs. 1.74%). The odds ratio was 0.31 with a 95% CI of 0.23 to 0.43 and a P-value of < 0.0001; the relative risk of having any one of the head and neck defects with ART was 1.04 with a 95% CI of 1.03 to 1.05 and a P-value < 0.0001. Conclusion There appears to be no increased risk of congenital head and neck defects in children conceived via ART versus those conceived naturally.