Incidence of Airway Abnormalities in Children With Craniosynostosis.

INTRODUCTION: Children with syndromic craniosynostosis are known to have a high propensity for associated airway abnormalities. However, this has not been investigated using a large-scale national database. METHODS: For this retrospective cohort study, the 2016 Healthcare Cost and Utilization Project Kid's Inpatient Database was queried for craniosynostosis patients. Data on demographics, airway diagnoses, and comorbidities were analyzed. RESULTS: Four thousand nine hundred fourteen children with craniosynostosis with a mean age of 1.7±3.6 years were identified. Of these, 51% were female and 136 children had an associated syndrome. Choanal atresia was present in 31% of patients with an associated syndrome versus 2.5% without. Syndromic patients are 4.59 times more likely (95% CI 2.65-7.94) to have airway anomalies than nonsyndromic patients. After age and sex adjustment, craniosynostosis patients have higher likelihoods of presenting with other anomalies, with syndromic having higher incidences: 5.23 times (95% CI 2.63-10.39) more likely to have laryngomalacia, 18.30 times (95% CI 3.27-102.36) more likely to have tracheal stenosis, and 4.58 times (95% CI 1.36- 15.43) more likely to have tracheomalacia. Incidence of tracheostomy was 5.84 times (95% CI 3.77-9.04) higher in syndromic patients with craniosynostosis. Tracheostomy rates were 28.4% and 4.6% in craniosynostosis patients with and without associated syndrome, respectively. CONCLUSION: Syndromic craniosynostosis patients had significantly higher incidences of choanal atresia and other airway anomalies. Given a high incidence of airway anomalies, syndromic craniosynostosis patients likely warrant routine airway evaluation. Providers should also be vigilant about airway evaluation in patients with nonsyndromic craniosynostosis when aerodigestive symptoms arise.

Association of meningitis and clival canal defect: case illustration, management, and systematic review of the literature.

OBJECTIVE: Improper embryological development of the clivus, a bony structure that comprises part of the skull base, can lead to a clival canal defect. Previously thought to be a benign condition, clival canals have been reported to be associated with meningitis and meningoceles. In this review, the authors sought to present an unpublished case of a patient with a clival canal defect associated with meningitis and to evaluate all other reported cases. METHODS: In October 2020, a search of PubMed, Web of Science, and Scopus was conducted to identify all cases of clival canals reported from January 1, 1980, through October 31, 2020. RESULTS: Including the case presented herein, 13 cases of clival canals, 11 in children (84.6%) and 2 in adults (15.4%), have been identified. Of the pediatric patients, 5 (45.5%) had an associated meningocele, and 8 (72.7%) had meningitis. Nine of the 13 patients (69.2%) had defects that were treated surgically, 5 (38.5%) by a transnasal approach and 4 (30.8%) by a transoral approach. Two patients (15.4%) were treated with drainage and antibiotics, 1 patient (7.7%) was treated solely with antibiotics, and 1 patient (7.7%) was not treated. In the literature review, 8 reports of clival canals were found to be associated with meningitis, further contributing to the notion that the clival canal may be an overlooked source of recurrent infection. In several of these cases, surgical repair of the lesion was curative, thus preventing continued episodes of meningitis. CONCLUSIONS: When a patient has recurrent meningitis with no clear cause, taking a closer look at clival anatomy is recommended. In addition, if a clival canal defect has been identified, surgical repair should be considered a safe and effective primary treatment option.

Congenital and Neonatally Acquired Sensorineural Hearing Loss.

Hearing loss is one of the most common congenital diagnoses, recognized in large part by a robust newborn hearing screening program. Ensuring appropriate follow-up of failed newborn hearing screening is crucial to avoid delay in initiation of treatment for hearing loss. The most common etiology for congenital hearing loss is genetic, but some cases can be acquired. Understanding of the etiology aids in counseling for the family and in direction of treatment. Early diagnosis and treatment results in dramatically improved speech and developmental outcomes for affected children. Treatment including amplification, speech therapy, adaptations in the classroom, and family support leads to gains in academic performance, parental satisfaction, and quality of life. Early cochlear implantation has been shown to be beneficial in obtaining speech and language skills in patients with severe to profound sensorineural hearing loss and should be considered in the appropriate patient population. [Pediatr Ann. 2021;50(7):e292-e296.].

Association of Velopharyngeal Insufficiency With Quality of Life and Patient-Reported Outcomes After Speech Surgery.

IMPORTANCE: Patients with cleft palate and other causes of velopharyngeal insufficiency (VPI) suffer adverse effects on social interactions and communication. Measurement of these patient-reported outcomes is needed to help guide surgical and nonsurgical care. OBJECTIVES: To further validate the VPI Effects on Life Outcomes (VELO) instrument, measure the change in quality of life (QOL) after speech surgery, and test the association of change in speech with change in QOL. DESIGN, SETTING, AND PARTICIPANTS: Prospective descriptive cohort including children and young adults undergoing speech surgery for VPI in a tertiary academic center. Participants completed the validated VELO instrument before and after surgical treatment. MAIN OUTCOMES AND MEASURES: The main outcome measures were preoperative and postoperative VELO scores and the perceptual speech assessment of speech intelligibility. The VELO scores are divided into subscale domains. Changes in VELO after surgery were analyzed using linear regression models. VELO scores were analyzed as a function of speech intelligibility adjusting for age and cleft type. The correlation between speech intelligibility rating and VELO scores was estimated using the polyserial correlation. RESULTS: Twenty-nine patients (13 males and 16 females) were included. Mean (SD) age was 7.9 (4.1) years (range, 4-20 years). Pharyngeal flap was used in 14 (48%) cases, Furlow palatoplasty in 12 (41%), and sphincter pharyngoplasty in 1 (3%). The mean (SD) preoperative speech intelligibility rating was 1.71 (1.08), which decreased postoperatively to 0.79 (0.93) in 24 patients who completed protocol (P < .01). The VELO scores improved after surgery (P<.001) as did most subscale scores. Caregiver impact did not change after surgery (P = .36). Speech Intelligibility was correlated with preoperative and postoperative total VELO score (P < .01) and to preoperative subscale domains (situational difficulty [VELO-SiD, P = .005] and perception by others [VELO-PO, P = .05]) and postoperative subscale domains (VELO-SiD [P = .03], VELO-PO [P = .003]). Neither the VELO total nor subscale score change after surgery was correlated with change in speech intelligibility. CONCLUSIONS AND RELEVANCE: Speech surgery improves VPI-specific quality of life. We confirmed validation in a population of untreated patients with VPI and included pharyngeal flap surgery, which had not previously been included in validation studies. The VELO instrument provides patient-specific outcomes, which allows a broader understanding of the social, emotional, and physical effects of VPI. LEVEL OF EVIDENCE: 2.

Septorhinoplasty in the Pediatric Patient.

Pediatric septorhinoplasty has been an area of controversy because early surgical intervention can prevent normal growth. There are certain conditions where early correction of the nose is indicated, such as in cleft lip nasal deformities, severe traumatic deformities, and congenital nasal lesions. Animal and clinical studies have been helpful in elucidating certain areas of the nose that are potential growth zones that should be left undisturbed when performing nasal surgeries on pediatric patients. We discuss the timing, indications, and surgical technique in pediatric septorhinoplasty.

Cleft Lip Repair, Nasoalveolar Molding, and Primary Cleft Rhinoplasty.

Cleft lip and palate are the fourth most common congenital birth defect. Management requires multidisciplinary care owing to the complexity of these clefts on midface growth, dentition, Eustachian tube function, and lip and nasal cosmesis. Repair requires planning, but can be performed systematically to reduce variability of outcomes. The use of primary rhinoplasty at the time of cleft lip repair can improve nose symmetry and reduce nasal deformity. Use of nasoalveolar molding ranging from lip taping to the use of preoperative infant orthopedics has played an important role in improving functional and cosmetic results of cleft lip repair.

Pediatric laryngotracheal separation following a go-cart injury.

Less than one percent of trauma admission cases are categorized as pediatric neck trauma [13]. Nevertheless, due to an increasingly mobile society, there has been an increasing frequency of pediatric neck trauma with motor vehicle accidents being the most common mechanism of injury [8]. We present a case of laryngotracheal separation from a blunt, clothesline injury to the neck in a pediatric patient. We also review the literature and discuss the benefit of balloon airway dilation and its assistance in the management of laryngeal trauma and its resultant effects.

Primary surgery for advanced-stage laryngeal cancer: A stage and subsite-specific survival analysis.

BACKGROUND: Treatment recommendations for advanced-stage laryngeal squamous cell carcinoma (SCC) have evolved significantly over the last 2 decades. METHODS: We retrospectively analyzed patients in the Surveillance, Epidemiology, and End Results (SEER) database with advanced-stage laryngeal SCC treated between 2004 and 2012. RESULTS: A total of 6797 patients were identified in the SEER database who met inclusion criteria, with 2051 patients undergoing primary surgery and 4746 patients undergoing primary radiotherapy (RT) or chemoradiotherapy (CRT). Disease-specific survival (DSS) and overall survival (OS) were significantly better for patients treated with primary surgery when compared using Kaplan-Meier curves and a Cox multivariate regression. When survival analysis was repeated for patients stratified by T classification, N classification, and subsite, OS and DSS benefits from primary surgery were observed for patients with T3 and T4a tumors, N0 neck disease, or supraglottic primaries. CONCLUSION: Patients with advanced-stage laryngeal SCC with T3 and T4a tumors, N0 neck disease, or supraglottic primaries have the greatest chance of survival when treated with primary surgery. © 2016 Wiley Periodicals, Inc. Head Neck 38: 1380-1386, 2016.

One-way valve bottle contamination rates in the immediate post-functional endoscopic sinus surgery period.

BACKGROUND: Sinonasal saline irrigation has become an accepted practice in the immediate postoperative management of functional endoscopic sinus surgery (FESS) patients. Recent studies have found that valveless delivery systems of sinonasal irrigation are colonized with bacteria. An alternative delivery system uses a one-way valve to reduce saline backflow and may limit bottle contamination. Our sole objective was to determine whether this system in post-FESS patients eliminates microbial bottle contamination. METHODS: Eight patients undergoing FESS were given one-way valve irrigation bottles to use immediately after surgery. Bottles were collected after 1 week of use and another set of bottles after an additional week. Endoscopic-directed cultures of the middle meatus were performed at the time of surgery. Returned used bottles were swabbed for bacteria and the valve system of the bottle was analyzed under scanning electron microscopy (SEM) for the presence of bacteria. RESULTS: All sinus swabs collected at the time of surgery grew bacteria with Staphylococcus sp. present in all samples. After the 1st week of use, 5/8 bottles grew bacterial cultures and showed bacterial presence on the valves by SEM. After the 2nd week, 4/5 bottles had positive culture results and also showed bacterial presence on the valves by SEM. CONCLUSION: Despite commercial claims that the use of valves and limit of backflow into the bottle will eliminate contamination, our study showed that one-way valve delivery systems become contaminated with bacteria after 1 week of use. We also showed that the bottle valves themselves harbor bacteria after 1 week of use.

Peripheral inflammation induces tumor necrosis factor dependent AMPA receptor trafficking and Akt phosphorylation in spinal cord in addition to pain behavior.

In the present study, intraplantar carrageenan induced increased mechanical allodynia, phosphorylation of PKB/Akt and GluR1 ser 845 (PKA site) as well as GluR1, but not GluR2 movement into neuronal membranes. This change in membrane GluR1/GluR2 ratio is indicative of Ca(2+) permeable AMPA receptor insertion. Pain behavior was reduced and biochemical changes blocked by spinal pretreatment, but not post-treatment, with a tumor necrosis factor (TNF) antagonist, Etanercept (100microg). Pain behavior was also reduced by spinal inhibition of phosphatidylinositol 3-kinase (PI-3K) (wortmannin; 1 and 5microg) and LY294002; 50 and 100microg) and Akt (Akt inhibitor IV; 3microg). Phosphorylated Akt was found exclusively in neurons in grey matter and in oligodendrocytes in white matter. Interestingly, this increase was seen first in superficial dorsal horn and alpha-motor neurons (peak 45min) and later (peak 2h post-injection) in deep dorsal horn neurons. Akt and GluR1 phosphorylation, AMPA receptor trafficking and mechanical allodynia were all TNF dependent. Whether phosphorylation of Akt and of GluR1 are in series or in parallel or upstream of pain behavior remains to be determined. Certainly, TNF-mediated GluR1 trafficking appears to play a major role in inflammatory pain and TNF-mediated effects such as these could represent a path by which glia contribute to neuronal sensitization (spinal LTP) and pathological pain.