A novel surgical management of hypopharyngeal branchial anomalies.

OBJECTIVE: To review our experience treating hypopharyngeal branchial anomalies utilizing an open transcervical approach that: (1) includes recurrent laryngeal nerve (RLN) monitoring and identification if needed; (2) resection of tract if present; and (3) a superiorly based sternothyroid muscle flap for closure. METHODS: A retrospective chart review was performed to identify all patients at a tertiary level children's hospital with branchial anomalies from 2005 to 2014. The clinical presentation, evaluation, treatment and outcome were analyzed for those patients with hypopharyngeal branchial anomalies. RESULTS: Forty-seven patients who underwent excision of branchial anomalies with a known origin were identified. Thirteen patients had hypopharyngeal branchial anomalies. Six of these patients were treated by the authors of this study and are the focus of this analysis. All six underwent an open transcervical procedure with a sternothyroid muscle flap closure of a piriform sinus opening over a nine year period. Definitive surgery included a microlaryngoscopy and an open transcervical approach to close a fistula between the piriform sinus and neck with recurrent laryngeal nerve monitoring or dissection. A superiorly based sternothyroid muscle flap was used to close the sinus opening. There were no recurrences, recurrent laryngeal nerve injuries or other complications from these procedures. CONCLUSIONS: This study supports complete surgical extirpation of the fistula tract using an open cervical approach, recurrent laryngeal nerve monitoring or identification, and rotational muscle flap closure to treat patients with hypopharyngeal branchial anomalies.

Hearing preservation after middle fossa vestibular schwannoma removal: are the results durable?

OBJECTIVE: To describe 5-year hearing preservation rates following microsurgical excision of vestibular schwannoma (VS) via the middle cranial fossa (MCF) approach. STUDY DESIGN: Case series with chart review. SETTING: This study was performed at a tertiary care academic medical center. SUBJECTS AND METHODS: Fifty-seven subjects with VS underwent resection via an MCF approach between February 1998 and January 2009. Pure-tone average (PTA) and word recognition score (WRS) were obtained preoperatively, immediately postoperatively, and at 5-year follow-up. RESULTS: Preoperative serviceable hearing (American Academy of Otolaryngology-Head and Neck Surgery class A/B) was present in 49 (86%) of the 57 patients, with an average PTA of 23 dB (range, 1-50 dB) and an average WRS of 97% (range, 76%-100%). Immediate postoperative serviceable hearing was maintained in 27 (55%) patients, with an average PTA and WRS of 31 dB (5-50 dB) and 96% (70%-100%), respectively. Five-year follow-up was available for 16 of the 27 patients. Twelve (75%) of the 16 patients maintained serviceable hearing with an average PTA and WRS of 35 dB (4-49 dB) and 95% (84%-100%), respectively. Of the 16 subjects who did maintain class A or B hearing, the mean change in PTA and WRS was 5 dB and 0.4%, respectively. Of the 4 patients who did not maintain class A/B hearing, average change in PTA and WRS was 16 dB (4.5-23 dB) and 16% (0%-40%), respectively. CONCLUSIONS: For patients with VS in whom serviceable hearing is preserved following the MCF approach, the long-term hearing outcome remains durable in most patients.

BRAF V600E does not predict aggressive features of pediatric papillary thyroid carcinoma.

OBJECTIVES/HYPOTHESIS: This study aimed to review the prevalence of the BRAF V600E mutation in pediatric papillary thyroid carcinoma (PTC) and any possible association with aggressive tumor behavior. STUDY DESIGN: A retrospective chart review and post hoc BRAF V600E mutational analysis of archived tumor tissue. METHODS: Patients 0 to 18 years old who underwent surgery for PTC from 1999 to 2012 were selected for a retrospective chart review to assess for aggressive disease characteristics. Microdissection was performed on archived tumor tissue, which was analyzed for the BRAF V600E mutation by pyrosequencing. RESULTS: Archived tumor specimens were available for 19/27 pediatric patients who fit the inclusion criteria. Ages ranged from 2.8 to 18 years (median, 13.7 years). Thirteen patients (68.4%) had central neck metastases, eight (42.1%) had lateral neck metastases, and five (26.3%) had pulmonary metastases. The BRAF V600E mutation was present in seven patients (36.8%). There were 11 patients with classic PTC, seven with a follicular variant of PTC, and one with an oncocytic variant. Seven (63.6%) with classical PTC were BRAF V600E positive. All histologic variants were wild type. PTC histology significantly correlated with the BRAF mutation (P = .013). The BRAF mutation was associated with a lower metastases, age at diagnosis, completeness of resection, invasion, and size of the tumor score, which trended toward significance (P = .087). Presence of lymphatic or pulmonary metastases, tumor size, overall age, lymphovascular invasion, or extrathyroidal extension were not associated with BRAF V600E. Our results are combined with existing studies for a combined incidence of 28.4%. CONCLUSIONS: BRAF V600E mutations may be more prevalent than previously thought in pediatric patients with PTC, but do not correlate with aggressive disease characteristics.

Uncommon presentations of parathyroid adenoma.

BACKGROUND: Patients with parathyroid adenomas (PAs) are commonly encountered in otolaryngology and may present with asymptomatic hypercalcemia. Retropharyngeal hematoma and recurrent laryngeal nerve (RLN) paralysis are less commonly encountered presentations and may be harbingers of a malignant process. METHODS AND RESULTS: We present 2 patients with rare presentations of parathyroid adenoma. The first is a 57-year-old man with retropharyngeal hemorrhage and progressive airway compromise. The second is a 51-year-old woman presenting with dysphonia who was found to have RLN paralysis. Both of these patients were found to have benign disease. CONCLUSIONS: Although most patients with PA present with asymptomatic hypercalcemia, this disease entity must be considered in patients with other unusual presentations including hemorrhagic neck masses and dysphonia. The importance of a broad differential diagnosis and thorough workup is emphasized.

Adverse events associated with concurrent chemoradiation therapy in patients with head and neck cancer.

OBJECTIVE: To assess toxicities, functional outcomes, and health-related quality of life associated with concurrent chemoradiation therapy (CRT) in patients with head and neck cancer. DESIGN: Prospective and retrospective outcomes study. SETTING: Tertiary care institution. PATIENTS: Participants in the longitudinal Outcomes Assessment Project whose head and neck cancer was treated with CRT between February 1, 2000, and March 1, 2007 (n = 104). INTERVENTIONS: Patients prospectively provided functional and health-related quality of life information, including data from the 1-year and most current follow-up visits. Medical records were reviewed to determine toxicity and survival rates. MAIN OUTCOME MEASURES: Well-defined acute and late toxicities; functional outcomes (diet, dentition, tracheostomies); head and neck cancer-specific, general health, and depression outcomes; and survival rates. RESULTS: Most patients had oropharyngeal or laryngeal tumors (87.5%) and advanced-stage disease (75.0%). Approximately one-half had hematologic toxicities and toxicity-related treatment delays. Approximately one-quarter had neurotoxicities and/or ototoxicites, moist desquamation, pneumonia, nausea and vomiting requiring hospitalization or intravenous fluids, dehydration or malnutrition requiring hospitalization, and mild or moderate fever. Although patients receiving the current intensity-modulated radiation therapy (IMRT) protocol using the Pinnacle(3) planning system had more toxicity-related treatment delays, they had fewer toxicities and better functional and health-related quality of life outcomes compared with those receiving conventional lateral opposing-field radiation or the initial IMRT protocol using the Best nomos PEACOCK planning system. CONCLUSIONS: Patients receiving CRT experience a substantial number of treatment-related adverse events, primarily affecting oropharyngeal and laryngeal function, with improvement noted for the current IMRT protocol. Improving dental prosthetic rehabilitation and including evaluations with speech and swallowing pathologists before and during treatment may enhance patient outcomes.