Diagnosis and management of congenital floor of mouth masses: A systematic review.

OBJECTIVES: Determine the utility of preoperative imaging and the optimal course of management for congenital floor of mouth (FOM) cysts in infants. METHODS: A systematic review of the literature was performed conforming to PRISMA guidelines. Pubmed, Embase and Cochrane Library databases were queried to identify cases of infants with congenital floor of mouth masses. Patient demographics, presenting findings, imaging, management, complications, and outcomes were determined. RESULTS: 85 patients were evaluated. 98% of patients presented at 16 months of age or younger. The most common presenting symptom was submental mass or swelling, 31.3%. Among the patients that underwent imaging, the suspected diagnosis obtained from imaging findings was consistent with the final pathologic diagnosis 59% of the time reported and inaccurate 34% of the time. There were multiple definitive treatment modalities described in the literature review including surgical excision, 82.3%, marsupialization, 12.9%, chemical injection 2.3%, sclerotherapy 1.2%,% and radiation, 1.2%. Recurrence rate after initial definitive treatment was as follows, surgical excision, 8.8%, marsupialization, 80%, sclerotherapy, 100%, chemical injection, 50%, and radiation, 100%. CONCLUSION: Preoperative imaging studies should not be relied upon alone to determine suspected pathology and subsequent management in pediatric patients with FOM masses. It may be beneficial for these patients to undergo primary surgical excision regardless of imaging studies or suspected pathology. Needle aspiration offers limited addition to pathologic diagnosis and should only be performed in the setting of acute symptomatic management. Surgical excision should be considered as definitive treatment modality in all patients with FOM masses, regardless of the suspected diagnosis of ranula. Further multi-institutional cohort studies could be invaluable to elucidate definitive treatment guidelines in this patient population.

Endoscopic Resection of Pediatric Skull Base Tumors: An Evidence-Based Review.

Objectives To perform a systematic review examining experiences with endoscopic resection of skull base lesions in the pediatric population, with a focus on outcomes, recurrence, and surgical morbidities. Methods PubMed/MEDLINE, Cochrane Library, Embase, and Web of Science databases were evaluated. Studies were assessed for level of evidence. Bias risk was evaluated using the Cochrane Bias tool, Grades of Recommendation, Assessment, Development and Evaluation (GRADE), and Methodological Index for Non-Randomized Studies (MINORS) criteria. Patient characteristics, pathology, site of primary disease, presenting symptoms, stage, procedure specific details, and complications were evaluated. Results were reported using the Preferred Reporting Systems for Systematic Reviews and Meta-Analysis guidelines. Results Ninety-three studies met criteria for inclusion, encompassing 574 patients with skull base tumors. The GRADE and MINORS criteria determined the overall evidence to be moderate quality. The most common benign and malignant pathologies included juvenile nasopharyngeal angiofibromas ( n = 239) and chondrosarcomas ( n = 11) at 41.6 and 1.9%, respectively. Of all juvenile nasopharyngeal angiofibroma tumors, most presented at stage IIIa and IIIb (25.8 and 27.3%, respectively). Nasal obstruction (16.5%) and headache (16.0%) were common symptoms at initial presentation. Surgical approaches included endoscopic endonasal ( n = 193, 41.2%) and endoscopic extended transsphenoidal ( n = 155, 33.1%). Early (< 6 weeks) and late (>6 weeks) complications included cerebrospinal fluid leak ( n = 36, 17.3%) and endocrinopathy ( n = 43, 20.7%). Mean follow-up time was 37 months (0.5-180 months), with 86.5% showing no evidence of disease and 2.1% having died from disease at last follow-up. Conclusion Endoscopic skull base surgery has been shown to be a safe and effective method of treating a variety of pediatric skull base tumors. If appropriately employed, the minimally invasive approach can provide optimal results in the pediatric population.

Sleep and Breathing the First Night After Adenotonsillectomy in Obese Children With Obstructive Sleep Apnea.

STUDY OBJECTIVES: There are few studies measuring postoperative respiratory complications in obese children with obstructive sleep apnea (OSA) undergoing adenotonsillectomy (AT). These complications are further compounded by perioperative medications. Our objective was to study obese children with OSA for their respiratory characteristics and sleep architecture on the night of AT. METHODS: This was a prospective study at a tertiary pediatric hospital between January 2009-February 2012. Twenty obese children between 8-17 years of age with OSA and adenotonsillar hypertrophy were recruited. Patients underwent baseline polysomnography (PSG) and AT with or without additional debulking procedures, followed by a second PSG on the night of surgery. Demographic and clinical variables, surgical details, perioperative anesthetics and analgesics, and PSG respiratory and sleep architecture parameters were recorded. Statistical tests included Pearson correlation coefficient for correlation between continuous variables and chi-square and Wilcoxon rank-sum tests for differences between groups. RESULTS: Baseline PSG showed OSA with mean obstructive apnea-hypopnea index (oAHI) 27.1 ± 22.9, SpO2 nadir 80.1 ± 7.9%, and sleep fragmentation-arousal index 25.5 ± 22.0. Postoperatively, 85% of patients had abnormal sleep studies similar to baseline, with postoperative oAHI 27.0 ± 34.3 (P = .204), SpO2 nadir, 82.0 ± 8.7% (P = .462), and arousal index, 24.3 ± 24.0 (P = .295). Sleep architecture was abnormal after surgery, showing a significant decrease in REM sleep (P = .003), and a corresponding increase in N2 (P = .017). CONCLUSIONS: Obese children undergoing AT for OSA are at increased risk for residual OSA on the night of surgery. Special considerations should be taken for postoperative monitoring and treatment of these children. COMMENTARY: A commentary on this article appears in this issue on page 775.

Otolaryngology Concerns for Illicit and Prescription Drug Use.

Concern for illicit and restricted drug use in otolaryngology is similar to other surgical specialties with a few notable exceptions. Many illicit drugs are consumed transnasally. Repeated nasal exposure to stimulants or narcotics can cause local tissue destruction that can present as chronic rhinosinusitis or nasoseptal perforation. Further, the Food and Drug Administration has taken a stance against codeine for pediatric patients undergoing adenotonsillectomy. They have identified an increased risk of death postoperatively with these medications. Because codeine has been the most commonly prescribed narcotic, this has shifted the standard practice.

Randomized Controlled Trial Assessing the Feasibility of Shortened Fasts in Intubated ICU Patients Undergoing Tracheotomy.

OBJECTIVE: American Society of Anesthesiology guidelines recommend preoperative fasts of 6 hours after light snacks and 8 hours after large meals. These guidelines were designed for healthy patients undergoing elective procedures but are often applied to intubated intensive care unit (ICU) patients. ICU patients undergoing routine procedures may be subjected to unnecessary prolonged fasts. This study tests whether shorter fasts allow for better nutrition delivery and patient outcomes without increasing the risk. STUDY DESIGN: Randomized blinded controlled trial. SETTING: Tertiary academic medical center. SUBJECTS: ICU patients undergoing bedside tracheotomy. METHODS: Intubated ICU patients who were receiving enteral feeding and for whom bedside tracheotomy was indicated were enrolled prospectively and randomly allocated to 2 parallel preoperative fasting regimens: a 6-hour fast (control) and a 45-minute fast (intervention). Patients were assessed for aspiration, caloric delivery, metabolic markers, and infectious and noninfectious complications. RESULTS: Twenty-four patients were enrolled and randomized. There were no complications related to the procedure. There were no cases of intraoperative aspiration identified. There was a single postoperative pneumonia in the control group. Median (interquartile range) length of fast and caloric delivery were significantly different between the control group and the shortened fast group: 22 hours (18, 34) vs 14 hours (5, 25; P < .001) and 429 kcal (57, 1125) vs 1050 kcal (825, 1410; P = .01), respectively. CONCLUSIONS: Shortening preoperative fasts in intubated ICU patients allowed for better caloric delivery in the preoperative period.

Patient selection in congenital pyriform aperture stenosis repair - 14 year experience and systematic review of literature.

PURPOSE: Congenital nasal pyriform aperture stenosis (CNPAS) is a rare cause of respiratory distress in neonates that may necessitate early surgical intervention. Restenosis and granulation are postoperative concerns that may prompt a return to the operating room. Reoperation places children at increased risk of perioperative complications and prolonged hospital stays. We are presenting a review of our institutional experience of 16 patients treated for CNPAS over a 14 year period and a systematic review with pooled data analysis to determine the effect of craniofacial and neurologic anomalies on surgical success. METHODS: Retrospective chart review of all cases of CNPAS treated at our tertiary children's hospital between 1999 and 2013. Systematic review of English language literature was conducted adhering to the PRISMA statement to determine the effect of neurologic anomalies and craniofacial dysmorphism (CFD) on surgical failure for CNPAS treatment. Univariate and exact multiple logistic regression were used for analysis of an individual patient data analysis. RESULTS: 10 patients had surgery and 6 were treated medically. Average pyriform apertures were 5.71±1.72mm for the surgical group and 4.83±1.26mm for the medical group (p=0.38). 31% had neurological impairments. 31% had craniofacial dysmorphisms (CFD). 2 patients developed restenosis and 1 required tracheotomy. Both of these patients had other CFDs. Literature review captured 63 surgical patients and 9 failures in 6 series of CNPAS. 4.6% of patients without CFD and 36.8% of patients with CFD required surgical revision (p=0.023, OR13.8). CONCLUSION: When repairing CNPAS, co-morbidities must be considered. Impaired respiration, central neurologic deficits and extensive craniofacial anomalies may require additional surgeries or an alternative approach.

Transoral resection of stylopharyngeus calcification: a unique manifestation of a stylohyoid complex syndrome.

BACKGROUND: Eagle syndrome is often considered in the work-up of odynophagia and neck pain. Classically, this is manifested by ossification or calcification of the stylohyoid ligament or styloid process. There are no reported cases of stylopharyngeal calcification leading to these symptoms. CASE: We describe a patient with a suspected submucosal pharyngeal foreign body who was found to have a calcified stylopharyngeus muscle and tendon during surgery. The patient experienced full resolution of symptoms after transoral robotic resection. This diagnosis was initially missed because the radiology was inconsistent with Eagle syndrome. CONCLUSION: This is the first report of isolated stylopharyngeal calcification, and this unique manifestation of a stylohyoid complex syndrome should be considered in patients with symptoms of Eagle syndrome without styloid elongation.

Complicated pediatric subglottic granular cell tumor with extensive intraluminal and extraluminal invasion.

Subglottic granular cell tumors (GCT) are rare, potentially life threatening benign tumors. Complete resection is necessary, yet care must be taken to preserve laryngeal function. We present the first description of a pediatric subglottic GCT with extensive invasion beyond the confines of the subglottis to include the vocal folds and central neck. Urgent endoscopic debulking avoided tracheotomy and facilitated extubation. Later, complete resection required hemithyroidectomy, laryngofissure and partial cricotracheal resection. We conclude that endoscopic debulking is an appropriate initial treatment. Transmural extension should be suspected in tumors larger than 1cm and warn of the need for tracheal resection.

Intraoperative inflation of laryngocele with LMA.

OBJECTIVES: Laryngoceles are pathologic air filled dilations of the laryngeal ventricle. They are most often benign and incidental findings. Resection may be necessary in the setting of infection, airway obstruction, dysphagia, and phonatory disturbances. External laryngoceles are almost universally treated with open resection via a lateral or midline cervical approach. Care must be taken to resect the laryngocele in its entirety to avoid recurrence. In cases of recurrent infection, normal surgical planes are often fibrosed and obscured increasing the risk of neurovascular sacrifice and functional losses. METHODS: We are reporting a case of recurrent infections in a large, palpable external laryngocele. During resection the patient was ventilated using an endotracheal tube (ETT). Additionally, a laryngeal mask airway (LMA) was inserted posterior to the ETT, resting in the hypopharynx and attached to a Jackson Rees circuit. Air was passed through the LMA to inflate the laryngocele and better define its borders. The LMA was also used to identify the root of the laryngocele in the paraglottic space and ensure its airtight closure. RESULTS: The LMA assisted our dissection and helped progress the surgery safely in a fibrosed surgical field. We have not seen this method described previously. The patient continues to be free of recurrence 2 years after surgery. CONCLUSION: While in most cases, with careful surgical technique, even a fibrotic and scarred laryngocele can be excised in its entirety without neurovascular sacrifice. In some cases where this may be difficult with a traditional approach, we offer the intra-operative trumpet maneuver as a viable method of better delineating the borders of a laryngocele.

Pectus excavatum in children with laryngomalacia.

BACKGROUND: Laryngomalacia is the most common congenital laryngeal anomaly and is associated with several disorders including gastric reflux, sleep apnea, hypotonia and failure to thrive. Pectus excavatum (PE) is the most common chest wall deformity affecting 1-300/1000 individuals. Though many authors presume a relationship between PE and laryngomalacia, there is no published data to establish this association. GOAL: To test the hypothesis that patients referred to our pediatric otolaryngology clinic for evaluation of laryngomalacia exhibit higher rates of PE than the general population. METHODS: Retrospective review of prospectively enrolled children who presented with laryngomalacia (January 2008-June 2012) to a tertiary care, hospital based, pediatric otolaryngology practice. Each chart was examined for a concurrent diagnosis of pectus deformity. RESULTS: Of the 137 laryngomalacia patients, 9 (6.6%) had documented PE. This represents a significantly increased rate of PE when compared to children without laryngomalacia (p = 0.001). Four of the 9 children with PE underwent supraglottoplasty for laryngomalacia, a significantly greater proportion than the 9/128 of the children with isolated laryngomalacia who underwent supraglottoplasty (p = 0.004). CONCLUSIONS: This study suggests an association between laryngomalacia and PE. Pediatric otolaryngologists should be cognizant of this relationship, though further studies are needed to elucidate the nature of this association.

Adenotonsillectomy in obese children with obstructive sleep apnea syndrome: magnetic resonance imaging findings and considerations.

OBJECTIVE: The reasons why adenotonsillectomy (AT) is less effective treating obese children with obstructive sleep apnea syndrome (OSAS) are not understood. Thus, the aim of the study was to evaluate how anatomical factors contributing to airway obstruction are affected by AT in these children. METHODS: Twenty-seven obese children with OSAS (age 13.0 ± 2.3 y, body mass index Z-score 2.5 ± 0.3) underwent polysomnography and magnetic resonance imaging of the head during wakefulness before and after AT. Volumetric analysis of the upper airway and surrounding tissues was performed using commercial software (AMIRA®). RESULTS: Patients were followed for 6.1 ± 3.6 mo after AT. AT improved mean obstructive apnea-hypopnea index (AHI) from 23.7 ± 21.4 to 5.6 ± 8.7 (P < 0.001). Resolution of OSAS was noted in 44% (12 of 27), but only in 22% (4 of 18) of those with severe OSAS (AHI > 10). AT increased the volume of the nasopharynx and oropharynx (2.9 ± 1.3 versus 4.4 ± 0.9 cm(3), P < 0.001, and 3.2 ± 1.2 versus 4.3 ± 2.0 cm(3), P < 0.01, respectively), reduced tonsils (11.3 ± 4.3 versus 1.3 ± 1.4 cm(3), P < 0.001), but had no effect on the adenoid, lingual tonsil, or retropharyngeal nodes. A small significant increase in the volume of the soft palate and tongue was also noted (7.3 ± 2.5 versus 8.0 ± 1.9 cm(3), P = 0.02, and 88.2 ± 18.3 versus 89.3 ± 24.4 cm(3), P = 0.005, respectively). CONCLUSIONS: This is the first report to quantify volumetric changes in the upper airway in obese children with OSAS after adenotonsillectomy showing significant residual adenoid tissue and an increase in the volume of the tongue and soft palate. These findings could explain the low success rate of AT reported in obese children with OSAS and are important considerations for clinicians treating these children.

Radiofrequency ablation of pediatric vallecular cysts.

OBJECTIVE: Pediatric congenital vallecular cysts are an infrequent cause of infantile airway obstruction that can cause difficulty breathing or feeding, and lead to failure to thrive or death. There have been many different techniques proposed for effective treatment. We present three cases of congenital vallecular cyst, all excised utilizing a bipolar radiofrequency plasma ablation (RFA) device. The bipolar RFA device provides excellent hemostasis without the risk of airway fire. We offer a review of literature and a discussion of the potential advantages of this modality in treating neonatal/infantile vallecular cysts. METHODS: Retrospective case series of three infants all between 2 and 3 months old who presented with failure to thrive due to partially obstructive vallecular cysts. The cysts were removed surgically utilizing a bipolar RFA device. Patients were followed by complications and signs of recurrence for periods ranging from 9 to 20months. RESULTS: All cysts were excised completely in a single operation. All children are feeding well and without stridor or evidence of recurrence between 9 and 20 months postoperatively. CONCLUSIONS: RFA appears to be an effective and safe modality for removing vallecular cysts. We were able to demonstrate complete removal of lesions in our patients. This provides an alternative to other ablative modalities in treating cystic lesions of the vallecula and larynx.

Lysine residues Lys-19 and Lys-49 of beta-catenin regulate its levels and function in T cell factor transcriptional activation and neoplastic transformation.

Wnt signaling regulates cell fate determination, proliferation, and survival, among other processes. Certain Wnt ligands stabilize the beta-catenin protein, leading to the ability of beta-catenin to activate T cell factor-regulated genes. In the absence of Wnts, beta-catenin is phosphorylated at defined serine and threonine residues in its amino (N) terminus. The phosphorylated beta-catenin is recognized by a beta-transducin repeat-containing protein (betaTrCP) and associated ubiquitin ligase components. The serine/threonine residues and betaTrCP-binding site in the N-terminal region of beta-catenin constitute a key regulatory motif targeted by somatic mutations in human cancers, resulting in constitutive stabilization of the mutant beta-catenin proteins. Structural studies have implicated beta-catenin lysine 19 as the major target for betaTrCP-dependent ubiquitination, but Lys-19 mutations in cancer have not been reported. We studied the consequences of single amino acid substitutions of the only 2 lysine residues in the N-terminal 130 amino acids of beta-catenin. Mutation of Lys-19 minimally affected beta-catenin levels and functional activity, and mutation of Lys-49 led to reduced beta-catenin levels and function. In contrast, beta-catenin proteins with substitutions at both Lys-19 and Lys-49 positions were present at elevated levels and had the ability to potently activate T cell factor-dependent transcription and promote neoplastic transformation. We furthermore demonstrate that the K19/K49 double mutant forms of beta-catenin are stabilized as a result of reduced betaTrCP-dependent ubiquitination. Our findings suggest that Lys-19 is a primary in vivo site of betaTrCP-dependent ubiquitination and Lys-49 may be a secondary or cryptic site. Moreover, our results inform understanding of why single amino acid substitutions at lysine 19 or 49 have not been reported in human cancer.