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Importance: Vocal fold motion impairment (VFMI) due to neuronal injury is a known complication following thoracic aortic repair that can impair pulmonary toilet function and post-operative recovery. Objective: To demonstrate clinical outcomes of patients undergoing inpatient vocal fold medialization for VFMI after aortic surgery. Design: A 15-year retrospective chart review (2005-2019) of 259 patients with postoperative VFMI after thoracic aortic surgery registry was conducted. Data included demographics, surgery characteristics, laryngology exam, and postoperative clinical outcomes. Medialization procedures consisted of type 1 thyroplasty and injection laryngoplasty. Setting: Tertiary care hospital Participants: Two hundred and fifty-nine patients (median age 61, 71% male) with VFMI post-thoracic aortic repair met inclusion criteria; inpatient vocal fold medialization was performed for 203 (78%) patients. One hundred and twenty-six. (49%) received type 1 thyroplasty and 77 (30%) received injection laryngoplasty procedures at a median 7 days (IQR 5-8 days) from extubation. Main Outcomes: Primary study outcome measurements consisted of median LOS, median ICU LOS, complications intra- and postoperatively, and pulmonary complications (post-medialization bronchoscopies, pneumonia, tracheostomy, etc.). Results: Post-medialization bronchoscopy rates were significantly lower in the medialization (n = 11) versus the non-medialization group (n = 8) (5% vs 14%, P = .02) and significantly higher in the injection laryngoplasty group (n = 77) versus thyroplasty group (n = 126) (10% vs 2%, P = .02). Further analysis revealed no significant difference in overall LOS and pulmonary complications between the techniques. Conclusion: Inpatient thyroplasty and injection laryngoplasty are both effective vocal fold medialization techniques after extent I and II aortic repair. Thyroplasty may have a small pulmonary toilet advantage, as measured by need for post-medialization bronchoscopy, compared to injection laryngoplasty.

Objectives: To examine whether social determinants of health (SDH) factors are associated with time to diagnosis, treatment selection, and time to recurrent surgical intervention in idiopathic subglottic stenosis (iSGS) patients. Methods: Adult patients with diagnosed iSGS were recruited prospectively (2015-2017) via clinical providers as part of the North American Airway Collaborative (NoAAC) and via an online iSGS support community on Facebook. Patient-specific SDH factors included highest educational attainment (self-reported), median household income (matched from home zip code via U.S. Census data), and number of close friends (self-reported) as a measure of social support. Main outcomes of interest were time to disease diagnosis (years from symptom onset), treatment selection (endoscopic dilation [ED] vs cricotracheal resection [CTR] vs endoscopic resection with adjuvant medical therapy [ERMT]), and time to recurrent surgical intervention (number of days from initial surgical procedure) as a surrogate for disease recurrence. Results: The total 810 participants were 98.5% female, 97.2% Caucasian, and had a median age of 50 years (IQR, 43-58). The cohort had a median household income of $62 307 (IQR, $50 345-$79 773), a median of 7 close friends (IQR, 4-10), and 64.7% of patients completed college or graduate school. Education, income, and number of friends were not associated with time to diagnosis via multivariable linear regression modeling. Univariable multinominal logistic regression demonstrated an association between education and income for selecting ED versus ERMT, but no associations were noted for CTR. No associations were noted for time to recurrent surgical procedure via Kaplan Meier modeling and Cox proportional hazards regression. Conclusions: Patient education, income, and social support were not associated with time to diagnosis or time to disease recurrence. This suggests additional patient, procedure, or disease-specific factors contribute to the observed variations in iSGS surgical outcomes.

Objectives Multiple population studies have shown racial discrepancies in head and neck cancer treatment and outcomes. We sought to characterize the impact of race on clinical outcomes for patients with early glottic squamous cell carcinoma (SCC) in a tertiary institution which provides equivalent access to care. Methods We retrospectively reviewed all early glottic (T1-T2) squamous cell carcinoma at a single institution, the Michael E. DeBakey Veterans' Administration Medical Center (MEDVAMC). Data collected included demographic information, primary and adjuvant treatment modalities, time to diagnosis, time to treatment, recurrences, recurrence treatment modality, secondary malignancies, recurrence-free survival (RFS), and overall survival (OS). Results One hundred seventeen patients with a primary diagnosis of T1-T2 glottic squamous cell carcinoma were included. Black and white patients demonstrated equivalent rates of recurrence, RFS, and OS. There was no significant difference in treatment delivery by race for all recorded parameters. T1b tumors were associated with an increased risk of recurrence which did not translate into a statistically significant decrease in RFS or OS. Surgical treatment was associated with increased recurrence but similar RFS and OS compared to radiation-based treatment. Secondary malignancies were common; 12% of patients were diagnosed with a second primary lung cancer during the study period. Conclusion At our institution, race did not impact survival when access to care, treatment selection, and delivery are equivalent for early glottic SCC. Secondary lung cancer is a critical risk factor for mortality in this patient group and requires long-term surveillance and monitoring. Level of evidence 4 Laryngoscope, 130:1733-1739, 2020.

Background Vocal fold movement impairment (VFMI) secondary to neuronal injury is a known risk after aortic surgery. Total arch replacement is technically challenging, and the incidence of vocal fold movement impairment secondary to neuronal injury after this surgery is unknown. This study examined the incidence of VFMI after total arch replacement and medialization treatment outcomes. Study design Retrospective cohort study. Methods All patients who underwent total arch replacement at a tertiary care center over 11 years (2006-2017) were identified through an institutional database. End points included evidence of VFMI on flexible laryngoscopy, time to diagnosis, time to treatment, need for reintubation, and intensive care unit (ICU) and hospital length of stay. Results Of the 358 patients who underwent total arch replacement, 63 (20%) were diagnosed with VFMI during their initial inpatient stay. Fifty patients (79%) VFMIs were left-sided, nine (14%) were right-sided, and four (6%) were bilateral. Thirty-nine patients (62%) underwent inpatient vocal fold medialization: 28 (72%) by injection laryngoplasty and 11 (28%) by type 1 thyroplasty. Those with unilateral VFMI had longer ICU (8.9 days) and hospital (19.4 days) than those with no VFMI (5.7 and 16.1 days). Among patients with unilateral VFMI, those who underwent inpatient vocal fold medialization trended toward shorter ICU (6.2 vs. 14.4 days, P = .03) and hospital stays (20.1 vs. 23.3 days, P = .4) than patients who did not have a medialization procedure. Conclusion The overall incidence of VFMI after total arch replacement in our series was 20%. Both the right and left vocal folds are potentially at risk from a total arch replacement; consequently, the distribution of injury in our cohort was more heterogeneous than in other series. Level of evidence 3 Laryngoscope, 129:699-703, 2019.

Objectives Veterans with laryngeal and oropharyngeal cancer remain an understudied patient population despite a high incidence of disease and decreased survival compared to the general population. Our objective was to evaluate treatment patterns for laryngeal and oropharyngeal cancer in patients treated at one of the Veterans Health Administration's busiest cancer centers in order to generate some basic benchmarks for treatment delivery in the veteran population. Methods We reviewed 338 patients treated at the Michael E. DeBakey Veterans Affairs Medical Center between 2000 and 2012. Results Oropharyngeal site and advanced age were associated with worse overall and disease-free survival. Treatment periods (mean) were as follows: 1) referral-diagnosis, 26 days; 2) diagnosis-surgery, 29 days; and 3) diagnosis-radiation, 58 days. Adjuvant radiation was initiated within 6 weeks of surgery in 42% of patients and 68% of patients had a total treatment package time ≤100 days. Time from diagnosis to treatment initiation, surgery to adjuvant radiation interval and total treatment package time did not impact survival. Conclusions This study establishes basic benchmarks for laryngeal and oropharyngeal cancer treatment delivery in veterans. Additional efforts are warranted to improve consistency and provide treatment in line with NCCN recommendations and literature consensus. Level of Evidence 2b.

Radiation therapy is a mainstream strategy in the treatment of several cancer types that are surgically unresectable. Unfortunately, cancer patients often suffer from unintended consequences of radiotherapy, including the development of skin inflammation (dermatitis), which may progress to fibrosis. These morbid complications often require interruption of radiotherapy and threaten the relapse of underlying cancer. Current treatment options for radiation dermatitis are suboptimal and compel the need to develop safer, more effective therapies. In this study, we assessed the biophysical properties of topically-formulated esomeprazole (here referred to as dermaprazole) and performed proof-of-concept studies to evaluate its efficacy in vitro and in vivo. We found that dermaprazole induced nuclear translocation of erythroid 2-related factor 2 (Nrf2) and significantly upregulated heme oxygenase 1 (HO1) gene and protein expression in a 3D human skin model. Our animal study demonstrated that dermaprazole improved macroscopic appearance of the irradiated skin and accelerated healing of the wounds. Histopathology data corroborated the photographic evidence and confirmed that both prophylactically and therapeutically administered dermaprazole conferred potent anti-inflammatory and antifibrotic effects. Gene expression data showed that dermaprazole downregulated several pro-oxidant, pro-inflammatory and profibrotic genes. In conclusion, topical formulation of the FDA-approved drug esomeprazole is highly effective in attenuating dermal inflammation and fibrosis.

Objective:Fibrovascular polyps of the hypopharynx and esophagus are rare, with few case reports in the literature. In this article, we present our institutional experience with a focus on airway and surgical management.Study Design:Case series.Setting:Tertiary academic institution.Methods:A retrospective review was conducted of 4 patients that presented to a tertiary medical center with fibrovascular polyps between 1990 and 2012. Patient demographics, clinical presentation, diagnostic studies, and surgical approaches were reviewed. A review of the published literature was also performed.Results:The average age at presentation was 72 years (range, 59-85 years). Among the 4 patients, 2 presented with airway compromise requiring tracheotomy. All patients had removal of the polyp shortly after presentation; 2 underwent transcervical approaches with lateral pharyngotomy/esophagotomy, and the other 2 had endoscopic removal. The polyps arose from the hypopharynx in 3 patients and upper esophagus in 1. Three patients had complete resolution of their symptoms and remained disease free. One patient had recurrence of the polyp 2 years later and is currently being observed.Conclusion:Fibrovascular polyps are rare tumors of the hypopharynx/esophagus that present in older adults. Although benign, they can cause life-threatening airway compromise that may necessitate tracheotomy. We present 4 cases of fibrovascular polyps and discuss our evolving surgical management, including endoscopic removal.

IntroductionIdiopathic subglottic stenosis (iSGS) is an unexplained progressive obstruction of the upper airway that occurs almost exclusively in adult, Caucasian women. The disease is characterised by mucosal inflammation and localised fibrosis resulting in life-threatening blockage of the upper airway. Because of high recurrence rates, patients with iSGS will frequently require multiple procedures following their initial diagnosis. Both the disease and its therapies profoundly affect patients’ ability to breathe, communicate and swallow. A variety of treatments have been advanced to manage this condition. However, comparative data on effectiveness and side effects of the unique approaches have never been systematically evaluated. This study will create an international, multi-institutional prospective cohort of patients with iSGS. It will compare three surgical approaches to determine how well the most commonly used treatments in iSGS ‘work’ and what quality of life (QOL) trade-offs are associated with each approach.Methods and analysisA prospective pragmatic trial comparing the ‘Standard of Care’ for iSGS at multiple international institutions. Patients with a diagnosis of iSGS without clinical or laboratory evidence of vasculitis or a history of endotracheal intubation 2 years prior to symptom onset will be included in the study. Prospective evaluation of disease recurrence requiring operative intervention, validated patient-reported outcome (PRO) measures as well as patient-generated health data (mobile peak flow recordings and daily steps taken) will be longitudinally tracked for 36 months. The primary endpoint is treatment effectiveness defined as time to recurrent operative procedure. Secondary endpoints relate to treatment side effects and include PRO measures in voice, swallowing, breathing and global QOL as well as patient-generated health data.Ethics and disseminationThis protocol was approved by the local IRB Committee of the Vanderbilt University Medical Center in July 2015. The findings of the trial will be disseminated through peer-reviewed journals, national and international conference presentations and directly to patient with iSGS via social media-based support groups.Trial registration numberNCT02481817.

Vocal fold movement impairment (VFMI) secondary to neuronal injury is a known risk after aortic surgery. Total arch replacement is technically challenging, and the incidence of vocal fold movement impairment secondary to neuronal injury after this surgery is unknown. This study examined the incidence of VFMI after total arch replacement and medialization treatment outcomes.Retrospective cohort study.All patients who underwent total arch replacement at a tertiary care center over 11 years (2006-2017) were identified through an institutional database. End points included evidence of VFMI on flexible laryngoscopy, time to diagnosis, time to treatment, need for reintubation, and intensive care unit (ICU) and hospital length of stay.Of the 358 patients who underwent total arch replacement, 63 (20%) were diagnosed with VFMI during their initial inpatient stay. Fifty patients (79%) VFMIs were left-sided, nine (14%) were right-sided, and four (6%) were bilateral. Thirty-nine patients (62%) underwent inpatient vocal fold medialization: 28 (72%) by injection laryngoplasty and 11 (28%) by type 1 thyroplasty. Those with unilateral VFMI had longer ICU (8.9 days) and hospital (19.4 days) than those with no VFMI (5.7 and 16.1 days). Among patients with unilateral VFMI, those who underwent inpatient vocal fold medialization trended toward shorter ICU (6.2 vs. 14.4 days, P = .03) and hospital stays (20.1 vs. 23.3 days, P = .4) than patients who did not have a medialization procedure.The overall incidence of VFMI after total arch replacement in our series was 20%. Both the right and left vocal folds are potentially at risk from a total arch replacement; consequently, the distribution of injury in our cohort was more heterogeneous than in other series.3 Laryngoscope, 129:699-703, 2019.

Tracheobronchopathia osteochondroplastica (TBOC) is a rare disease of the trachea and bronchi characterized by submucosal nodules of osseous and cartilaginous tissue. In this series, we present three cases highlighting the varied clinical presentations of this rare disease process, which ranged from a rough voice to a chronic cough to lobar pneumonia. The disorder may mimic other lesions. We review the clinical presentations, pathophysiology, lab tests, imaging, diagnosis, and management of TBOC patients. Laryngoscope, 126:2006-2009, 2016.

Objectives/Hypothesis Idiopathic subglottic stenosis (iSGS) is a rare and potentially life-threatening disease marked by recurrent and progressive airway obstruction frequently requiring repeated surgery to stabilize the airway. Unknown etiology and low disease prevalence have limited the ability to characterize the natural history of iSGS and resulted in variability in surgical management. It is uncertain how this variation relates to clinical outcomes. Study Design Medical record abstraction. Methods Utilizing an international, multi-institutional collaborative, we collected retrospective data on patient characteristics, treatment, and clinical outcomes. We investigated variation between and within open and endoscopic treatment approaches and assessed therapeutic outcomes; specifically, disease recurrence and need for tracheostomy at last follow-up. Results Strikingly, 479 iSGS patients across 10 participating centers were nearly exclusively female (98%, 95% confidence interval [CI], 96.1–99.6), Caucasian (95%, 95% CI, 92.2–98.8), and otherwise healthy (mean age-adjusted Charlson Comorbidity Index 1.5; 95% CI, 1.44–1.69). The patients presented at a mean age of 50 years (95% CI, 48.8–51.1). A total of 80.2% were managed endoscopically, whereas 19.8% underwent open reconstruction. Endoscopic surgery had a significantly higher rate of disease recurrence than the open approach (chi2 = 4.09, P = 0.043). Tracheostomy was avoided in 97% of patients irrespective of surgical approach (95% CI, 94.5–99.8). Interestingly, there were outliers in rates of disease recurrence between centers using similar treatment approaches. Conclusion Idiopathic subglottic stenosis patients are surprisingly homogeneous. The heterogeneity of treatment approaches and the observed outliers in disease recurrence rates between centers raises the potential for improved clinical outcomes through a detailed understanding of the processes of care. Level of Evidence 4. Laryngoscope, 2015

Importance Surgical treatment comparisons in rare diseases are difficult secondary to the geographic distribution of patients. Fortunately, emerging technologies offer promise to reduce these barriers for research. Objective To prospectively compare the outcomes of the 3 most common surgical approaches for idiopathic subglottic stenosis (iSGS), a rare airway disease. Design, Setting, and Participants In this international, prospective, 3-year multicenter cohort study, 810 patients with untreated, newly diagnosed, or previously treated iSGS were enrolled after undergoing a surgical procedure (endoscopic dilation [ED], endoscopic resection with adjuvant medical therapy [ERMT], or cricotracheal resection [CTR]). Patients were recruited from clinician practices in the North American Airway Collaborative and an online iSGS community on Facebook. Main Outcomes and Measures The primary end point was days from initial surgical procedure to recurrent surgical procedure. Secondary end points included quality of life using the Clinical COPD (chronic obstructive pulmonary disease) Questionnaire (CCQ), Voice Handicap Index-10 (VHI-10), Eating Assessment Test-10 (EAT-10), the 12-Item Short-Form Version 2 (SF-12v2), and postoperative complications. Results Of 810 patients in this cohort, 798 (98.5%) were female and 787 (97.2%) were white, with a median age of 50 years (interquartile range, 43-58 years). Index surgical procedures were ED (n = 603; 74.4%), ERMT (n = 121; 14.9%), and CTR (n = 86; 10.6%). Overall, 185 patients (22.8%) had a recurrent surgical procedure during the 3-year study, but recurrence differed by modality (CTR, 1 patient [1.2%]; ERMT, 15 [12.4%]; and ED, 169 [28.0%]). Weighted, propensity score–matched, Cox proportional hazards regression models showed ED was inferior to ERMT (hazard ratio [HR], 3.16; 95% CI, 1.8-5.5). Among successfully treated patients without recurrence, those treated with CTR had the best CCQ (0.75 points) and SF-12v2 (54 points) scores and worst VHI-10 score (13 points) 360 days after enrollment as well as the greatest perioperative risk. Conclusions and Relevance In this cohort study of 810 patients with iSGS, endoscopic dilation, the most popular surgical approach for iSGS, was associated with a higher recurrence rate compared with other procedures. Cricotracheal resection offered the most durable results but showed the greatest perioperative risk and the worst long-term voice outcomes. Endoscopic resection with medical therapy was associated with better disease control compared with ED and had minimal association with vocal function. These results may be used to inform individual patient treatment decision-making.

Objectives/Hypothesis Laryngotracheal stenosis (LTS) is largely considered a structural entity, defined on anatomic terms (i.e., percent stenosis, distance from vocal folds, overall length). This has significant implications for identifying at-risk populations, devising systems-based preventive strategies, and promoting patient-centered treatment. The present study was undertaken to test the hypothesis that LTS is heterogeneous with regard to etiology, natural history, and clinical outcome. Study Design Retrospective cohort study of consecutive adult tracheal stenosis patients from 1998 to 2013. Methods Subjects diagnosed with laryngotracheal stenosis (ICD-9: 478.74, 519.19) between January 1, 1998, and January 1, 2013, were identified. Patient characteristics (age, gender, race, follow-up duration) and comorbidities were extracted. Records were reviewed for etiology of stenosis, treatment approach, and surgical dates. Stenosis morphology was derived from intraoperative measurements. The presence of tracheostomy at last follow-up was recorded. Results One hundred and fifty patients met inclusion criteria. A total of 54.7% had an iatrogenic etiology, followed by idiopathic (18.5%), autoimmune (18.5%), and traumatic (8%). Tracheostomy dependence differed based on etiology (P

Chondroblastoma-like chondroma (CLC) of soft tissue is a rare benign neoplasm that usually involves the soft tissues of the hand. This report describes the first case of CLC of soft tissue arising in the base of the skull. A 33-year-old man was seen with a slow growing mass in the right parotid region of his face. The noncontrast computed tomographic scans showed an 8.5-cm mass with calcifications involving the right masticator space and extending through the bone into the middle cranial fossa. The radiologic differential diagnosis included osteosarcoma, leiomyosarcoma, chondrosarcoma, and giant cell tumor. During surgery, the large lateral skull base tumor appeared to involve the middle and infratemporal fossae and eroded the surrounding bone. Although the tumor was removed piecemeal, total excision was performed. On microscopic examination, the tumor displayed lobules of mature hyaline cartilage with numerous chondroblasts, coarse calcifications including chicken wire calcifications, and scattered osteoclasts. No atypia, mitoses, necrosis, or osteoid formation was seen. The tumor was diagnosed as chondroma with chondroblastoma features of the soft tissue. His postoperative clinical course was uneventful; however, after 7 months, he had a local recurrence identified on follow-up magnetic resonance imaging. He underwent repeat surgical excision of the tumor, which showed similar histology as the previous excision. This large skull based tumor eroding the bone, which clinically and radiologically mimicked a malignant process, was an unusual presentation of a benign cartilaginous neoplasm. Pathologists should be aware that CLC may occur in the base of the skull and this lesion should be differentiated from the other benign or malignant tumors arising in this area. These lesions have a potential for local recurrence; hence, a close follow-up is recommended.

Objective: To describe this institution’s experience with and the long-term outcomes of early type 1 thyroplasty for unilateral vocal fold paralysis (UVFP) following surgery on the aortic arch. Study Design: Retrospective chart review with telephone questionnaire. Setting: Academic tertiary care center. Subjects and Methods: Three hundred forty-eight patients with UVFP following surgery on the aortic arch since 1999 were identified; 40 were available for follow-up. The number of revision procedures following initial thyroplasty was ascertained, and the Voice Handicap Index (VHI) was administered by telephone. The hypothesis that early thyroplasty produced voice outcomes and revision rates comparable to injection laryngoplasty was established prior to the initiation of data collection. Results: Six out of the 40 patients (15%) required revision thyroplasty following their initial procedure. Mean VHI of all patients was 36.0 (SD, 27.2). Mean VHI was significantly different in the 18 to 39 age group (13.1) when compared to the 40 to 59 (51.8) and 60+ (37.7) age groups ( P = .013). Mean follow-up since initial thyroplasty was 46.5 months (SD, 42.2). Conclusions: In the setting of aortic arch surgery with injury to the recurrent laryngeal nerve, early thyroplasty produces voice outcomes comparable to those achieved in the literature with repeated injection and delayed thyroplasty and can be considered in select populations.

Idiopathic subglottic stenosis (iSGS) is a rare and potentially life-threatening disease marked by recurrent and progressive airway obstruction frequently requiring repeated surgery to stabilize the airway. Unknown etiology and low disease prevalence have limited the ability to characterize the natural history of iSGS and resulted in variability in surgical management. It is uncertain how this variation relates to clinical outcomes.Medical record abstraction.Utilizing an international, multi-institutional collaborative, we collected retrospective data on patient characteristics, treatment, and clinical outcomes. We investigated variation between and within open and endoscopic treatment approaches and assessed therapeutic outcomes; specifically, disease recurrence and need for tracheostomy at last follow-up.Strikingly, 479 iSGS patients across 10 participating centers were nearly exclusively female (98%, 95% confidence interval [CI], 96.1-99.6), Caucasian (95%, 95% CI, 92.2-98.8), and otherwise healthy (mean age-adjusted Charlson Comorbidity Index 1.5; 95% CI, 1.44-1.69). The patients presented at a mean age of 50 years (95% CI, 48.8-51.1). A total of 80.2% were managed endoscopically, whereas 19.8% underwent open reconstruction. Endoscopic surgery had a significantly higher rate of disease recurrence than the open approach (chi(2) = 4.09, P = 0.043). Tracheostomy was avoided in 97% of patients irrespective of surgical approach (95% CI, 94.5-99.8). Interestingly, there were outliers in rates of disease recurrence between centers using similar treatment approaches.Idiopathic subglottic stenosis patients are surprisingly homogeneous. The heterogeneity of treatment approaches and the observed outliers in disease recurrence rates between centers raises the potential for improved clinical outcomes through a detailed understanding of the processes of care.4. Laryngoscope, 126:1390-1396, 2016.

The study goal was to assess the use of digital laryngeal videostroboscopy (LVS) in the detection of objective improvement of the larynx after 6 weeks of proton pump inhibitor (PPI) therapy and to evaluate a clinical grading scale for findings of laryngopharyngeal reflux disease (LPRD).We conducted a prospective analysis of digital LVS examinations from a tertiary referral center for laryngology by 3 independent blinded examiners.The mean score on the Laryngopharyngeal Reflux Disease Index for experimental patients was significantly higher than that for control patients (9.50 versus 2.92, P0.001), and posttreatment scores were significantly lower than pretreatment scores (7.35 versus 9.50, P0.001). Useful items on the grading scale for assessing the presence of LPRD and improvement in LPRD included supraglottic edema and erythema, glottic edema and erythema, and subglottic edema and erythema. Nonuseful items included the presence of pachydermia, granulomas, nodules, leukoplakia, and polyps.Objective improvement of signs of LPRD can be detected after 6 weeks of PPI therapy using digital LVS. The Laryngopharyngeal Reflux Disease Index is a useful valid clinical tool for following treatment response to PPI therapy.

Study background: A two patient case series describing the surgical management of upper cervical sarcoma. Due to the density of critical neurovascular structures in the upper cervical spine, these rare sarcomas require primary surgical treatment that preempts local recurrence. Recurrence secondary to tumor spillage is problematic due to scar tissue formation and radiation effect creating surgically inaccessible tissue planes. En bloc resection of sarcomas during an index procedure provides the best chance at cure and prevention of local recurrence. Meticulous planning, familiarity with anatomy and surgical technique is critical for the success of these operations. Methods: Two patients: a 30-year-old and 36-year-old female, were referred to our institution with malignant spine tumors involving C1. The first was found to have a left sided synovial sarcoma anterolateral to C1 and C2. The second presented with metastatic alveolar soft tissue sarcoma at C1. Both patients underwent multi-stage en bloc surgical removal of their tumors. Results: Successful en bloc tumor excision and instrumented stabilization of the cervical spine without neurovascular complication was performed. Tumor margins were negative and x-rays demonstrated adequate spinal alignment. At six month followup, MRI evaluation demonstrated no local recurrence in either patient. Conclusions: En bloc resection is a highly effective, but technically demanding method of treating upper cervical sarcomas. In conjunction with adjuvant radiotherapy, en bloc surgery has the lowest risk of local recurrence and highest quality of life outcomes. Due to the proximity of critical neurovascular structures in the upper cervical spine, meticulous planning, staging and technique is required. A multidisciplinary surgical team should be assembled that includes a head and neck, skull-base, neuro-endovascular and spine surgeon. With appropriate planning, understanding of anatomy and surgical technique, en bloc resections of upper cervical sarcomas can be successfully performed.

The treatment of head and neck cancer has evolved from conventional fields encompassing large volumes of normal tissue to focused treatment aimed at conforming the dose around the target while avoiding normal tissue. Intensity modulated radiation therapy has changed the way radiation oncologists think about head and neck cancer. Using the concepts of conformal treatment and avoidance, the therapeutic ratio can be improved and technology exploited to the patients' advantage. This is particularly evident with head and neck irradiation, where a common side effect is xerostomia. By decreasing xerostomia through conformal avoidance of the parotid glands, we can improve patient satisfaction and quality of life. In this study, xerostomia is assessed through a subjective salivary gland function questionnaire. This article examines the use of intensity modulated radiation therapy in the treatment of head and neck cancer to decrease xerostomia. The purpose of this article is to evaluate the significance of parotid gland dosimetry in relation to subjective salivary gland function. Semin Oncol 29 (suppl 19):71-79. Copyright 2002, Elsevier Science (USA). All rights reserved.

Routine computed tomography (CT) for vocal fold movement impairment (VFMI) without an apparent cause is common. However, given increased cancer risk associated with ionizing radiation exposure, our purpose is to evaluate the utility of routine scans for these patients.Retrospective case series.Houston, Texas.A 5-year review of patients with VFMI diagnosed at an academic institution was conducted. For patients without an apparent cause (eg, recent head, neck, or cardiothoracic surgery or known malignancy), CT head/neck and chest was performed to evaluate the recurrent laryngeal nerve course. Data included demographics, symptoms, radiography, and interventions. Statistical analyses were performed via χ(2) analysis.Of 406 patients with VFMI, 47 (11%) patients had no apparent cause clinically. Routine CT revealed abnormalities in 10 (21%) patients, of which only 3 (6%) could account for VFMI: benign thyroid adenoma (1), papillary thyroid cancer (1), and an esophageal mass (1). The most common lesion detected involved the thyroid. Demographic data and symptom type were not significantly associated with detection of a VFMI-attributable lesion on CT. Overall, routine CT did not identify a focal etiology in 94% patients with VFMI without an apparent cause.Routine pan-CT evaluation failed to reveal an etiology in 94% of patients with VFMI without an apparent cause. Patients may be subjected to health risks associated with radiation exposure without significant diagnostic benefit. Further studies should consider more judicious use of CT in the context of risk factors and safer imaging modalities as the initial diagnostic step.

We studied the association between the severity of pretreatment CT scan findings and the improvement in symptoms of chronic sinusitis after treatment.We analyzed data from an ongoing prospective, nonrandomized clinical outcomes study of patients treated for chronic sinusitis at a tertiary-care academic medical center (n = 57, mean age 46.8 years). Disease-specific symptom severity was measured with a validated health status instrument, and CT stage was quantified with the Harvard CT staging system.In a multivariate analysis, symptom severity based on CT findings emerged as a strong predictor of both improvement in symptom score outcome (beta = 0.47, P = 0.01) and absolute symptom level after treatment (beta = -0.58, P = 0.001).Severity as assessed by a pretreatment CT scan is a strong predictor of outcome. Patients with higher symptom severity based on CT scans showed significantly larger improvement and lower absolute levels of symptom severity after treatment. This study links CT scan findings and subjective patient-based outcomes (symptom scores) using a validated outcomes instrument.

Purpose: To report the initial experience in the definitive treatment of head and neck carcinomas using SMART (Simultaneous Modulated Accelerated Radiation Therapy) boost technique. Radiation was delivered via IMRT (Intensity Modulated Radiotherapy). The following parameters were evaluated: acute toxicity, initial tumor response, clinical feasibility, dosimetry and cost. Methods and Materials: Between January 1996 and December 1997, 20 patients with primary head and neck carcinomas were treated with SMART boost technique. The treatment fields encompassed two simultaneous targets. The primary target included palpable and visible disease sites. The secondary target included regions at risk for microscopic disease. Daily fractions of 2.4 Gy and 2 Gy were prescribed and delivered to the primary and secondary targets to a total dose of 60 Gy and 50 Gy, respectively. Lower neck nodes were treated with a single conventional anterior portal. This fractionation schedule was completed in 5 weeks with 5 daily fractions weekly. Toxicity was evaluated by RTOG acute toxicity grading criteria, evidence of infection at immobilization screw sites, subjective salivary function, weight loss, and the need for treatment split. Mean follow-up was 15.2 months. Initial tumor response was assessed by clinical and radiographical examinations. Clinical feasibility was evaluated by the criteria: time to treat patient, immobilization, and treatment planning and QA time. In dosimetry, we evaluated the mean doses of both targets and normal tissues and percent targets’ volume below goal. To evaluate cost, Medicare allowable charge for SMART boost was compared to those of conventional fractionated and accelerated radiotherapy. Results: Acute toxicity: None of the patients had a screw site infection and all patients healed well after completion of radiotherapy. Sixteen of 20 patients (80%) completed the treatment within 40 days without any split. Sixteen patients (80%) had RTOG Grade 3 mucositis while 10 patients (50%) had Grade 3 pharyngitis. Three of 20 patients (15%) had weight loss greater than 10% of their pretreatment weight. Ten patients (50%) required intravenous fluids, tube feeding or both. Nine patients (45%) reported moderate xerostomia with significant relief reported within 6 months. Initial tumor response: 19 patients (95%) had complete response (CR) while one had partial response (PR). The patient with PR had stable disease on imaging at 12 months follow-up. Two patients were found to have lung metastases at 2 months and 5 months follow-up. To date, there have been two local recurrences in the complete responders. Both patients had nasopharyngeal primary; one was retreated with radioactive Cesium-137 implant and the other died from the disease. Clinical feasibility: The average treatment time for a three-arc treatment was 17.5 minutes and 2.5 minutes for each additional arc. Eleven patients (55%) had four-arc treatment while six patients (30%) had five-arc treatment and three patients (15%) had three-arc treatment. Immobilization was reproducible within less than 2 mm. The treatment planning, QA and documentation prior to treatment averaged 2 days. Dosimetry: The mean doses to the primary and secondary targets were 64.4 Gy and 54.4 Gy, respectively; 8.9% of the primary target volume and 11.6% of the secondary target volume were below prescribed dose goal. The mean dose delivered to the mandible was 30 Gy, spinal cord 17 Gy, ipsilateral parotid 23 Gy, and contralateral parotid 21 Gy. Cost: Total Medicare allowable charge for SMART boost was $7000 compared to $8600 (conventional ) and $9400 (accelerated fractionation). Conclusions: SMART boost technique is an accelerated radiotherapy scheme that can be delivered with acceptable toxicity. It allows parotid sparing as evidenced both clinically and by dosimetry. Initial tumor response has been encouraging. It is clinically feasible and cost saving. A larger population of patients and a long-term follow-up are warranted to evaluate ultimate tumor control and late toxicity.

Two parotid mucoepidermoid carcinomas with predominant oncocytic features were initially assessed on frozen section. Because of extensive oncocytic change, it was inferred that the lesions were most likely benign. Permanent sections revealed low-grade mucoepidermoid carcinoma with prominent oncocytic change (in more than 75% of the neoplasms) in both cases. Review of 48 additional consecutive cases of mucoepidermoid carcinoma of the salivary glands revealed prominent oncocytic change (accounting for 60% of the neoplasm) in one high-grade lesion. Phosphotungstic acid-hematoxylin stains revealed strong granular cytoplasmic staining in the oncocytic elements; immunohistochemical stains for antimitochondrial antibodies also showed intense immunoreactivity in these cells. Oncocytic change is not typically a prominent feature of mucoepidermoid carcinoma of the salivary glands, and to our knowledge, only three such cases have been reported previously. Because most salivary gland lesions with oncocytic change are benign, it is important to distinguish mucoepidermoid carcinoma from other entities that may show prominent oncocytic change. We report three additional examples of this rare lesion, two low-grade tumors and one high-grade tumor, and review our experience with oncocytic change in mucoepidermoid carcinoma of the salivary glands.

Intensity-modulated beam radiotherapy (IMRT) delivers a highly conformal, three-dimensional (3-D) distribution of radiation doses that is not possible with conventional methods. When administered to patients with head and neck tumors, IMRT allows for the treatment of multiple targets with different doses, while simultaneously minimizing radiation to uninvolved critical structures such as the parotid glands, optic chiasm, and mandible. With 3-D computerized dose optimization, IMRT is a vast improvement over the customary trial-and-error method of treatment planning. We retrospectively reviewed the charts of the first 28 head and neck patients at our institution who were treated with IMRT. All had head and neck neoplasms, including squamous cell carcinoma, adenoid cystic carcinoma, paraganglioma, and angiofibroma. Total radiation doses ranged from 1,400 to 7,100 cGy, and daily doses ranged from 150 to 400 cGy/day. A quality assurance system ensured that computer-generated dosimetry matched film dosimetry in all cases. For midline tumors, this system allowed us to decrease the dose to the parotid glands to less than 3,000 cGy. The incidence of acute toxicity was drastically lower than that seen with conventional radiotherapy delivery to similar sites. This is the first report of the application of IMRT strictly to head and neck neoplasms. We discuss the indications, technique, and initial results of this promising new technology. We also introduce the concept of the Simultaneous Modulated Accelerated Radiation Therapy boost technique, which has several advantages over other altered fractionation schemes.

Objectives:Fibrovascular polyps of the hypopharynx and esophagus are rare, and the majority of the literature is comprised of case reports. The goals of this study are: 1) Present one of the largest series of patients from a single institution that presented with fibrovascular polyps; 2) Describe their presentation focusing on airway management and clinical demographics; 3) Discuss our surgical management and demonstrate how it has evolved from open to endoscopic approaches.Methods:A retrospective review was conducted with four patients that presented to a tertiary medical center with fibrovascular polyps between 1990 and 2012. Patient demographics, clinical presentations, surgical approaches, and diagnostic studies were analyzed.Results:The average age at presentation was 72 years (range 59 to 85 years). Among the four patients, two presented with airway compromise requiring tracheotomy. All patients had removal of the polyp shortly after presentation; two underwent transcervical approaches with lateral ...

Purpose: Spontaneous enophthalmos and hypoglobus, in the absence of other symptoms and unrelated to trauma or surgery, may be alarming to both physician and patient. The authors describe the clinicopathologic features of a benign syndrome ("silent sinus syndrome") with this constellation of features and discuss the possible pathophysiology. Methods: A multicenter retrospective search for similar clinical cases was performed. All clinical records, computed tomographs, and pathology reports for each case were reviewed at one center. A literature search for similar cases also was conducted. Results: Nineteen cases of a new syndrome are presented. This syndrome affects individuals at approximately the fourth decade of life (average age, 36 years; range, 29–46 years); is characterized by bone resorption and remodeling of the orbital floor due to otherwise asymptomatic maxillary sinus disease; is associated with ipsilateral maxillary sinus hypoplasia; and is not fully explained by any previously described, classic cystic lesion of the maxillary antrum. Conclusion: Enophthalmos and hypoglobus unassociated with prior trauma, surgery, or other symptoms may represent "silent sinus syndrome," which is ipsilateral maxillary sinus hypoplasia and orbital floor resorption.

Objectives: We review our experience with endoscopic management of Zenker's diverticulum. We sought to analyze and determine risk factors for success or failure of endoscopic diverticulum treatment. Methods: We performed a retrospective review of 72 consecutive patients who underwent attempted endoscopic management of a Zenker's diverticulum between January 2000 and April 2006. The procedures were performed by either of 2 otolaryngologists. There were 50 men and 22 women ranging in age from 44 to 93 years. A total of 85 procedures were performed. The medical records were reviewed for preoperative diverticulum size (small, 1 to 2 cm; moderate, 2.1 to 3.0 cm; and large, more than 3.0 cm), intraoperative diverticulum characteristics, patient anatomic limitations that prevented adequate endoscopic visualization, surgical complications, and management of recurrences. Results: Of our 72 patients, 61 (85%) were able to undergo endoscopic cricopharyngeal myotomy with diverticulum elimination. Of the 61 endoscopic procedures, 47 (77%) resulted in complete symptom resolution. The most common risk factors for recurrence were diverticulum size (more than 3 cm) and amount of redundant mucosa. Of the 14 patients with a recurrence, 10 (71%) underwent a repeat procedure. Six of the 14 (43%) had a successful excision via a cervical approach, and 4 of the 14 (29%) underwent a repeat endoscopic myotomy. There was 1 major complication (esophageal tear), and there were 3 minor complications (mucosal abrasions). Conclusions: Most patients with a Zenker's diverticulum are good candidates for endoscopic management. In our series, 84% of those who underwent endoscopic treatment ultimately achieved relief of symptoms. The patient morbidity is minimal. A large diverticulum with redundant mucosa is a risk factor for recurrence after endoscopic treatment.

In the past five years, 477 patients with various focal dystonias and hemifacial spasm received 3,806 injections of botulinum A toxin for relief of involuntary spasms. A definite improvement with a global rating greater than or equal to 2 on a 0-4 scale, was obtained in all 13 patients with spasmodic dysphonia, 94% of 70 patients with blepharospasm, 92% of 13 patients with hemifacial spasm, 90% of 195 patients with cervical dystonia, 77% of 22 patients with hand dystonia, 73% of 45 patients with oromandibular dystonia, and in 90% of 21 patients with other focal dystonia who had adequate follow up. While the average duration of maximum improvement lasted about 11 weeks after an injection (range seven weeks in patients with hand dystonia to 15 weeks in patients with hemifacial spasm), some patients benefited for over a year. Only 16% of the 941 treatment visits with follow up were not successful. Except for transient focal weakness, there were very few complications or systemic effects attributed to the injections. This study supports the conclusion that botulinum toxin injections are a safe and effective therapy for patients with focal dystonia and hemifacial spasm.

Metastatic squamous cell carcinoma involving an intraparotid lymph node, although uncommon, remains of practical consideration in the differential diagnosis of a parotid mass. This is of particular importance when the primary skin lesion is not readily identifiable, or has been inadequately managed in the past. A representative case is presented, and a review of the literature follows with special emphasis on the anatomy of the parotid lymphatics and the implications for appropriate treatment.

We describe a 13-year-old boy with Tourette's syndrome (TS) manifested chiefly by severe coprolalia, obsessive compulsive disorder, and attention deficit hyperactivity disorder. He was treated with unilateral injections of botulinum toxin to the vocal cord and experienced marked improvement in coprolalia as well as marked reduction in the premonitory urges associated with the vocal tics and coprolalia. As a result of the improvement, the patient was able to attend school and church and was able to socialize. Botulinum toxin may interfere with a sensory reflex arc required to perpetuate tics and coprolalia in TS, possibly by reducing local build-up of tension or muscle contraction in affected areas.

Recurrent respiratory papillomatosis (RRP) is a histologically benign disease of the larynx, trachea, and bronchi. Here we report on the histologic and molecular characteristics of 7 cases of malignant transformation of RRP to squamous cell carcinoma (SCCA). The clinical histories of 7 patients with RRP who developed SCCA were carefully reviewed. Sequential biopsies were available from 5 of the 7 cases of spontaneous transformation of RRP to SCCA and were reviewed. In addition, p53 protein overexpression and human papillomavirus (HPV) typing for all cases was examined. The average age of patients with juvenile-onset RRP was 3 years, and that of patients with adult-onset RRP was 31 years. The average age of onset of transformation to SCCA was 28 years. All patients had laryngeal involvement with RRP, and 3 of the 7 patients had tracheal extension of disease. Five patients were tracheotomy-dependent. Four of the 7 patients developed SCCA of the lung, while 3 patients developed laryngeal SCCA. There was no consistent histologic progression from squamous papilloma to papilloma with dysplasia, and all but 1 of the SCCAs were well differentiated. The overexpression of p53 protein was variable in each of the 5 patients. We detected HPV types 6/11 in papillomas from 3 patients, and HPV types 6/11, 16/18, and 31/33/51 in a papilloma of a fourth patient. No HPV DNA was detected in papillomas of 2 patients. We found HPV 6/11 in 4 of the carcinomas. We conclude that the spontaneous transformation of RRP to SCCA is not characterized by a histologic progression through dysplasia over time. Transformation can result in the loss of HPV expression. It does not appear that p53 is a molecular marker for monitoring the transformation process. Thus, these cancers may be very difficult to diagnose histologically and clinically early in the course of the transformation of the disease.

These case series are presented to describe the application and advantages of intensity modulated radiotherapy (IMRT) for the treatment of extensive and/or recurrent juvenile angiofibroma. Two patients were diagnosed with recurrence at 11 and 13 months postoperatively, and one was surgically unresectable. The affected areas included the base of skull, cavernous sinus, pterygopalatine fossa, infratemporal fossa, posterior orbit and nasopharynx. Highly conformal IMRT was delivered with limited radiation doses to the optic nerves, optic chiasm, brainstem, brain, spinal cord, lens, retina, mandible, and parotid. The total dose delivered to the tumor varied from 3400 to 4500 cGy. The tumor shrunk radiographically in all three cases and there was no endoscopic evidence of disease in two cases at 15 months and 40 months. There was no acute toxicity. Late toxicity was limited to one episode of epistaxis and persistent rhinitis in one patient. In conclusion, IMRT provides several advantages over conventional radiotherapy in the treatment of recurrent juvenile angiofibroma.

Practice guidelines (PG) (or clinical pathways) are increasingly important tools for standardizing health care delivery, improving efficiency, monitoring quality, and controlling costs. Health services researchers divide the delivery of health care into three stages: structure, process, and outcome. PGs are a technique to standardize the process of health care delivery, which may result in improved clinical outcomes or may maintain clinical outcomes while increasing process efficiency and decreasing costs. We describe the development and implementation of a PG for endoscopic sinus surgery at an academic center, and report preliminary results on the effects of the PG on the health care process. The PG was developed using a multidisciplinary combination of consensus-building and evidence-based techniques. Initially, participation in the PG was voluntary and at the attending physician's discretion. One year after implementation of the PG, 41 patients had been enrolled by members of the medical school's full-time faculty. Process and short-term outcome variables on those patients were compared to a random sample of 50 patients treated by the same physicians, but not using the PG. There was no evidence of selection bias into the PG based on demographics, severity of sinusitis, or the presence of comorbid factors. There were no differences in time spent in the operating room, postanesthesia care unit, or day surgery observation unit, between patients using the PG and not using the PG. However, patients not using the PG had a significantly higher rate of unplanned admission. Patients using the PG had significantly lower median hospital costs and charges than did patients not using the PG. In addition, median hospital costs and charges decreased steadily for all patients (not just those using the PG), simultaneous with the development and implementation of the PG. There were no differences in short-term clinical outcomes between PG and non-PG patients. In summary, the development and implementation of a PG for endoscopic sinus surgery resulted in lower hospital costs and charges while maintaining acceptable short-term clinical outcomes. PGs have important implications for improving the efficiency of the health care process.

Neuroendocrine neoplasms of the larynx are a rare group of tumors that include carcinoid tumor, atypical carcinoid tumor, and small cell carcinoma. These neoplasms pose interesting diagnostic, prognostic, and therapeutic dilemmas, and they are, as a whole, aggressive tumors with a tendency for local and distant spread. The authors of this study examined six new cases of laryngeal neuroendocrine neoplasms. One case manifested itself as a primary atypical carcinoid tumor and caused a "carcinoid syndrome." The remaining five cases were small cell carcinomas of the larynx. Histologic, immunocytochemical, DNA flow cytometric, and p53 studies were performed on all cases. The expression of neuron-specific enolase and chromogranin were the most useful markers in this group of tumors. Overexpression of p53 protein was present in the majority of cases, including the atypical carcinoid tumor. The implications of these studies for diagnosis, classification, and treatment are discussed.

Giant fibrovascular polyps of the esophagus and hypopharynx are benign tumors of the upper digestive tract Although a rare cause of asphyxiation, laryngeal impaction by a regurgitated polyp of the esophagus may be the initial symptom that brings the patient to request medical attention. Two new cases of giant fibrovascular polyps with dramatic and potentially life-threatening presentations illustrate the unpredictable behavior of these unusual tumors. Both patients presented to the emergency center with a history of coughing and eructation followed by temporary airway obstruction that was relieved by clenching a regurgitated fleshy mass between the teeth. Diagnostic and therapeutic intervention requires aggressive airway management, radiographic and endoscopic evaluation, and definitive surgical treatment. Tracheotomy was required in one patient, and successful endotracheal intubation provided satisfactory airway control in the second. Esophagoscopy revealed the origin of both tumors to be near the cricopharyngeal muscle. Complete surgical excision was curative in both cases.

MTC is a neoplasm derived from the thyroid C-cell. There are two clinical types of MTC, a sporadic form which accounts for the majority of tumors and a less common hereditary variety. Four variants of hereditary MEN 2 have been identified: 1) Multiple endocrine neoplasia type 2A (MEN 2A), the most common form, has three components; medullary thyroid carcinoma, pheochromocytoma, and hyperparathyroidism. 2) MEN 2B, is characterized by MTC, pheochromocytoma, and mucosal neuromas. 3) Familial MTC may occur without other components of MEN 2A. 4) MEN 2 in association with a pruritic skin lesion located over the upper back (cutaneous lichen amyloidosis) is the rarest variant (1). Recent efforts in the field have focused on defining the disease genes for hereditary MTC, with the expectation that the sporadic form of MTC will probably share some or all of the molecular defects. A broad outline of the molecular events implicated in the development of MTC has evolved, but none of the disease genes have been identified or cloned (2).