Faculty Bibliography

IMPORTANCE: Children with poor muscle tone may demonstrate upper airway obstruction due to several mechanisms including obstructive sleep apnea, laryngopharyngeal reflux, and laryngomalacia. Though hypotonia has been shown to compromise the pediatric airway, and some authors suggest that neurologic deficits can compromise the success of laryngotracheal reconstruction (LTR), to our knowledge no studies have evaluated the effect of neurologic diagnoses or hypotonia on outcomes in LTR. OBJECTIVE: To determine whether hypotonic children with subglottic stenosis have lower rates of successful decannulation after LTR compared with children without neurologic deficit. DESIGN, SETTING, AND PARTICIPANTS: A retrospective medical chart review was conducted for 27 children aged 0 to 6 years, who underwent LTR for subglottic stenosis between December 2007 and December 2012 at a tertiary care children's hospital. Children were classified based on documented neurologic findings. Group 1 comprised those children without neurologic impairment (n = 16). Group 2 included those children with a documented neurocognitive or neuromuscular diagnosis but without evidence of hypotonia (n = 7). Group 3 comprised hypotonic children (n = 4). INTERVENTIONS Laryngotracheal reconstruction. MAIN OUTCOMES AND MEASURES: The number of procedures performed after LTR to optimize the airway and whether the child was successfully decannulated. RESULTS: All 16 of the neurologically intact patients (100%) were decannulated. Among children with a neurologic deficit, 5 of 7 (71%) were ultimately decannulated. No hypotonic children 0 of 4 were decannulated. The difference in rates of decannulation between unaffected and normotonic children with a neurologic deficit was not statistically significant (P = .08). However, the difference in outcomes between hypotonic children and neurologically intact patients was statistically significant (P < .001). CONCLUSIONS AND RELEVANCE: Findings from this study suggest that hypotonic children may experience poorer rates of post-LTR decannulation compared with children without neurologic deficit. Dynamic upper airway obstruction may be unappreciated in hypotonic children. Future research may be directed at the appropriate evaluation and treatment of children with poor muscle tone and subglottic stenosis.

OBJECTIVES/HYPOTHESIS: The aims of the study were to identify trends in surgical management of laryngotracheal stenosis (LTS) based on lesion location, as well as to recognize factors associated with recurrence and repeat surgical intervention. STUDY DESIGN: The study is a retrospective review of all adult patients cared for at a tertiary care laryngology practice with a diagnosis of laryngotracheal stenosis between October 2001 and July 2010, following Institutional Review Board approval. METHODS: This study collectively measured demographics, comorbidities, etiologies, sites of stenoses, treatment modalities, and recurrences. RESULTS: Incisions made with the carbon dioxide (CO2 ) laser were the most common modality of treatment; patients with multilevel tracheal stenosis were most likely to have undergone at least one operation in which the CO2 laser was used to make incisions (78.7%). Balloon dilation was most commonly employed in patients with multilevel tracheal stenosis (66.0%). Lowest rates of decannulation were identified in patients with a pure tracheal stenosis (23.1%). Patients presenting with multilevel tracheal stenosis underwent the most procedures (6.7). Supraglottic, glottic, and multilevel laryngeal stenosis recurred at the lowest rates. Patients suffering from diabetes mellitus recurred in an average period of 3.9 months, a shorter time frame than those without diabetes, who recurred every 10.5 months. CONCLUSIONS: Laser incision and/or balloon dilation are most effective in treatment of pure glottic, subglottic, and tracheal stenosis. Multilevel tracheal stenosis warrants closer follow-up, and is more likely to require multiple procedures. Worsening stenosis despite endoscopic management warrants an open procedure. Diabetes is associated with an earlier recurrence of stenosis. LEVEL OF EVIDENCE: Level 2b (Individual retrospective cohort study). Laryngoscope, 2013.

Castleman disease is most commonly found in the mediastinum, while the head and neck is the second most common location. The disease exists in a unicentric and multicentric variety and is usually successfully treated with surgical resection alone. Early identification is important for treatment planning. Castleman disease has been reported to mimic other disease processes, however there has been only one report of the disease mimicking a nerve sheath tumor in the parapharyngeal space. Here we report the second case of Castleman disease mimicking a schwannoma in the parapharyngeal space.

PURPOSE: To evaluate the impact of radiation therapy (RT) on the long-term outcomes and pattern of failure for T1-2 glottic cancer. MATERIALS AND METHODS: This is a single-institution, retrospective study. From January (1997 to 2010), 253 patients with early glottic cancer underwent RT by 2-dimensional or 3-dimensional RT with Co or linear accelerator. Appropriate daily bolus was applied with linear accelerator-based RT to avoid under dosage of the anterior commissure. RESULTS: A total of 253 patients with T1-T2 glottic cancer were treated with definitive RT. The median age was 65 and males made up 87% of the population. T1 was 77% of the population. The median dose and fraction size were 63 and 2.25 Gy, respectively. After a median follow-up of 83 months, the locoregional control (LRC) for the whole cohort was 98%. Specifically, LRCs for T1 (195 patients) and T2 (58 patients) were 99.5% and 91%, respectively. Kaplan-Meier curve shows the 5-year cause-specific survival to be 100%. CONCLUSIONS: Single-modality RT provides an excellent and effective treatment for T1-T2 glottic cancer with remarkable functional preservation and 5-year LRC of 98% with negligible long-term toxicity.

BACKGROUND: A 270-degree partially tubed pectoralis major myocutaneous flap (PMMF) is an excellent option for total circumferential pharyngoesophageal defects in patients who are not candidates for more complex reconstructions. METHODS: Patients undergoing circumferential pharyngoesophageal reconstruction with partially tubed PMMF were reviewed. End points were stricture, fistula, resumption of oral intake, perioperative death, and recurrence. RESULTS: Eleven patients underwent 270-degree PMMF for reconstruction: 6 (55%) were men and 5 (45%) were women (mean, 62 years; range, 42-78 years). Three patients (27%) developed fistulas and 2 (18%) developed stenosis. Ten patients (91%) were able to resume adequate nutrition via oral intake. There were no perioperative deaths. CONCLUSIONS: Patients with severe comorbidities, metastatic disease, a lack of donor vessels, or a potentially hostile abdomen may not be ideal candidates for free tissue transfer. For these patients, partially tubed PMMF using the prevertebral fascia provides a reliable alternative for reconstruction with excellent functional results.

BACKGROUND: The BRAF V600E (BRAF+) mutation activates the mitogen-activated protein kinase (MAPK/ERK) pathway and may confer an aggressive phenotype in papillary thyroid cancer (PTC). Clinically, the behavior of BRAF+ PTC, however, varies from an indolent to an aggressive course. SPRY2 is a negative feedback regulator of the MAPK/ERK pathway. We hypothesize that the level of SPRY2 expression contributes to MAPK/ERK pathway output and accounts for BRAF+ and clinical heterogeneity. METHODS: A tissue microarray with BRAF-positive PTCs (BRAF+ PTCs) was constructed and analyzed for SPRY2 expression and MAPK/ERK output. Data were studied in the context of clinicopathologic factors to develop a risk stratification system predictive of tumor biology. SPRY2 function was studied by silencing SPRY2 in BRAF+ PTC cells. These cells were treated with MAPK/ERK pathway inhibitors and assessed for growth effects. RESULTS: BRAF+ PTCs with an intact MAPK/ERK feedback pathway do not exhibit lymph node metastases. BRAF+ PTCs with dysregulated feedback pathways have nodal metastasis. When SPRY2 is silenced, the BRAF+ PTC cells are significantly more sensitive to MAPK/ERK inhibition. CONCLUSION: PTC behavior likely is dependent on both the driver of the MAPK/ERK pathway and its regulatory feedback. When the feedback pathway is intact, the tumor phenotype seems to be less aggressive. This observation has direct and important clinical implications and may alter our treatment strategies.

OBJECTIVES/HYPOTHESIS: In pediatric patients with congenital malformations of the inner ear, anomalies within the anatomy may facilitate unintentional insertion of the cochlear implant electrode into the internal auditory canal. Revision procedures for removal and replacement of cochlear implant electrodes following internal auditory canal insertion are fraught with potential danger, including the theoretical risk of injury to vasculature within the internal auditory canal, repeat insertion within the internal auditory canal, and cerebrospinal fluid leak. The objective of this presentation is to describe a technique for revision cochlear implantation following internal auditory canal insertion to minimize the potential associated risks. STUDY DESIGN: Case series. METHODS: A retrospective chart review was performed on all patients at a tertiary care facility who underwent revision cochlear implantation for internal auditory canal insertion between January 1999 and July 2011. RESULTS: A total of four patients referred from outside institutions have undergone revision cochlear implantation for internal auditory canal insertion. The records from these patients were reviewed. Electrodes were safely removed in all cases without injury to the anterior inferior cerebellar artery or its branches (i.e., labyrinthine artery). Complete insertion was accomplished on reimplantation. Neural response telemetry was performed in all cases, and responses were noted. Fluoroscopy was utilized to visualize electrode progression during insertion. A detailed description of the operative technique is provided. CONCLUSIONS: This case series describes a technique for revision cochlear implantation that appears to be safe and effective in preventing potential associated complications. Laryngoscope, 2013.

BACKGROUND: Typically, thyroid follicular neoplasm/suspicious for follicular neoplasm (FN/SFN) cases show moderate to marked cellularity and scant or absent colloid. Recently, cases have been noted with microfollicular cellularity in the background of moderate to abundant amount of colloid. The purpose of this study was to compare these "colloid-rich" FN/SFN cases to the typical FN/SFN cases. METHODS: Thyroid cytology specimens with the features of FN/SFN were searched in cytopathology files from September 2008 to June 2012. Cases with absent or minimal colloid were designated "typical colloid-poor" FN/SFN and cases with moderate to abundant colloid were designated "colloid-rich" FN/SFN. From these cases, those with surgical pathology resection follow-up were identified. Cytologic, surgical pathology resection, and molecular features (BRAF, RAS, RET/PTC, and PAX8-PPARgamma) were investigated for the typical colloid-poor FN/SFN cases and were compared with those of the colloid-rich FN/SFN cases. RESULTS: Of 431 FN/SFN cases with surgical pathology resection follow-up, 360 (83.5%) cases showed features of typical colloid-poor FN/SFN and 71 (16.5%) cases showed features of colloid-rich FN/SFN. Papillary carcinoma was the most common malignant outcome for the 2 groups. Although the proportion of malignant outcome was similar for the 2 groups, the "colloid-rich" FN/SFN cases showed a greater proportion of nodular hyperplasia among the cases with benign outcome. In addition, the "colloid-rich" FN/SFN cases demonstrated a greater proportion of cases with a mutation. CONCLUSIONS: Approximately one-sixth of cases of FN/SFN show "colloid-rich" features. Comparison to the typical colloid-poor FN/SFN demonstrated similar risk for malignancy but contrasting resection outcome and molecular characteristics.