Retrograde Cricopharyngeus Dysfunction: The Jefferson Experience.

OBJECTIVES: To describe our center's experience with the identification and treatment of retrograde cricopharyngeus dysfunction (R-CPD), a syndrome involving the inability to belch previously described by only one institution. Additionally, because all patients initially learned of their condition and sought treatment as a result of social media posts, we queried their source and comfort with this form of medical referral. METHODS: Retrospective chart review of patients who underwent botulism toxin injection into the cricopharyngeus muscle for treatment of R-CPD from 2019 to 2022. Demographic data, most common symptoms at presentation, and response to treatment and complications were documented. Post-treatment questionnaires were reviewed. RESULTS: A total of 85 patients were identified. Mean age at surgery was 27 years. There were 54 (63.5%) females and 31 (36.5%) males. The inability to burp (98.8%), bloating (92.9%), gurgling noises (31.8%), and excessive flatulence (21.2%) were the most common symptoms. The minimum units of botox utilized were 25, whereas the maximum was 100. The majority of patients (88.2%) had a successful response at initial follow-up visit. The most common complication was mild dysphagia (30.6%), which was transient for all patients. Most patients learned of our practice through social media, with only one patient being referred by a medical provider. CONCLUSIONS: The majority of patients in our cohort were young and female. The inability to burp and bloating were the most common presenting symptoms. Social media was the primary source of referral. Our institution favors 80-100 units for an effective response. Laryngoscope, 133:1081-1085, 2023.

Safety and efficacy of functional laryngectomy for end-stage dysphagia.

OBJECTIVES/HYPOTHESIS: To evaluate functional outcomes and complication rate after total laryngectomy (TL) for dysfunctional larynx with end-stage dysphagia. STUDY DESIGN: Retrospective chart review. METHODS: Chart review was performed on all patients who underwent TL from January 2008 to July 2016 at a single tertiary academic medical center. Patients who underwent TL for dysfunctional larynx without preoperative evidence of malignancy were included. Main outcome measures were post-TL functional swallowing and speech outcomes, and complication rate. RESULTS: The study included 19 patients from a cohort of 278 consecutive patients. All patients were previously treated with radiotherapy (RT), whereas 13/19 (68%) previously received chemoradiotherapy. The median time from RT to TL was 10.98 years (range, 0.67-23.94 years). Aspiration was evident preoperatively in 17/19 (89%) patients, with 11 experiencing recurrent aspiration pneumonia. Seventeen of 19 (89%) patients were nil per os (NPO) requiring enteral nutrition. Six of 19 (32%) patients had surgical complications, including three (16%) pharyngocutaneous fistulas. At 3-month and 1-year postoperative follow-up, there was significant improvement in mean Functional Oral Intake Scale (FOIS) score and aspiration, recurrent pneumonia, enteral nutrition, and NPO status rates (P < .05). At 1-year follow-up, no patients were NPO, and only one patient required gastrostomy tube supplementation. Mean FOIS score increased from 1.3 to 6.1 (P = .001). Eight of 13 patients (62%) were actively using a tracheoesophageal prosthesis at 1-year follow-up. CONCLUSIONS: Laryngectomy for dysfunctional larynx eliminates the morbidity of aspiration while improving diet and reducing gastrostomy tube dependence with an acceptable complication rate. LEVEL OF EVIDENCE: 4. Laryngoscope, 128:597-602, 2018.

Efficacy of large-diameter dilatation in cricopharyngeal dysfunction.

OBJECTIVES/HYPOTHESIS: To investigate patient outcomes with large-diameter bougienage in isolated cricopharyngeal dysfunction and understand how esophageal dilatation can be used as an effective diagnostic and therapeutic modality in treating dysphagia. STUDY DESIGN: Retrospective review. METHODS: A retrospective chart review was performed on 46 patients meeting the criteria for cricopharyngeal dysphagia from 2004 to 2008 presenting in the outpatient setting. Patients were treated with 60 French esophageal dilators. Outcomes were analyzed as a function of symptomatology, manometry, duration of benefit, and safety. RESULTS: Over the period reviewed, 59 dilatations were performed on 46 patients with cricopharyngeal dysfunction. Eight patients were dilated more than once. Four patients were lost to follow-up. The average starting Functional Outcome Swallowing Score (FOSS) was 2.07. Of the patients reviewed, 64.29% experienced an improvement in their FOSS with a median duration of 741 days. There were five minor complications and no major complications. CONCLUSIONS: In the largest series of esophageal dilatation for cricopharyngeal dysfunction in the literature, we found large-bore bougienage to have significant utility due to its efficacy, ease of use, and safety when compared to other modalities such as botulinum injection, balloon dilatation, and cricopharyngeal myotomy.

Detection of Merkel cell carcinoma polyomavirus in mucosal Merkel cell carcinoma.

A 61-year-old woman presented with solitary lymphadenopathy suspicious for lymphoma. An excisional biopsy of a right inguinal lymph node demonstrated metastatic Merkel cell carcinoma (MCC). No skin lesions were detected, but a primary nasopharyngeal mass was identified. A microscopic examination of the nasopharyngeal tumor and the lymph node metastasis showed a monotonous population of small- to intermediate-sized, round blue cells with vesicular nuclei, finely granular and dusty chromatin, and multiple nucleoli. Immunohistochemistry showed perinuclear dot-like staining for cytokeratins AE1/AE3 and CK20. Microscopic appearance and immunohistochemical stains were consistent with MCC. MCC was recently shown to harbor a novel polyomavirus, Merkel cell polyomavirus (MCPyV), in the majority of cases. In this case, MCPyV was detected in the primary tumor and metastasis using polymerase chain reaction and CM2B4 immunohistochemical stain. This is the first report to demonstrate the presence of MCPyV and CM2B4 in a mucosal MCC and its metastasis.

Tracheal stenosis after placement of percutaneous dilational tracheotomy.

OBJECTIVES: Percutaneous dilational tracheotomy procedures have been used successfully as a bedside alternative to open surgical tracheotomy. At our institution, we have seen patients with tracheal injuries following this procedure. In this paper, we review those cases to demonstrate that tracheal stenosis is a potential long-term complication of percutaneous dilational tracheotomy. STUDY DESIGN: Case series. METHODS: Patients were evaluated with computed tomography and operative endoscopy. Inpatient and outpatient records were reviewed retrospectively. RESULTS: Nine patients were referred to our practice for management of tracheal stenosis after percutaneous dilational tracheotomy between 2003 and 2006. Presence of anterior tracheal ring compression and destruction or lateral wall collapse was noted in each case. Endoscopy revealed stenosis secondary to anterior tracheal wall injury in all cases. In eight of nine cases, operative intervention was needed to correct the stenotic segment. CONCLUSIONS: It has been demonstrated in the literature that with 20 years of experience, the percutaneous dilational tracheotomy procedure is more affordable, faster to perform, and a generally safe procedure when performed under appropriate conditions. Most case series of percutaneous dilational tracheotomy reveal an equal or lower risk of short-term complications than open tracheotomy. This series demonstrates that tracheal stenosis is a potential long-term complication. Longitudinal follow-up of patients undergoing percutaneous dilational tracheotomy is indicated.

Identification and monitoring of the recurrent laryngeal nerve during thyroidectomy.

Careful dissection of the recurrent laryngeal nerve (RLN) represents perhaps the most critical component of thyroidectomy. It long has been established that routine identification of the nerve reduces the risk of iatrogenic injury. In recent years, much attention has been paid to the role that functional monitoring plays in identification and preservation of the RLN. This article explores methods for detecting and identifying the RLN. It then examines the evolution of functional RLN monitoring, its potential advantages and disadvantages, statistical validity, and its role in the current medicolegal climate.

Cervical discitis and epidural abscess after tonsillectomy.

UNLABELLED: Approximately 6 weeks after an uncomplicated tonsillectomy for chronic tonsillitis, a 37-year-old woman presented to our emergency department with complaints of odynophagia and cervical pain persistent since surgery. Computed tomographic and magnetic resonance imaging revealed cervical spinal osteomyelitis with epidural abscess at C2 to 3. The patient underwent treatment with intravenous antibiotics, operative debridement, and cervical spinal stabilization. She recovered with no neurologic deficit. CONCLUSIONS: Significant infectious complications of tonsillectomy are uncommon, and cervical spinal osteomyelitis and epidural abscess are exceptionally rare occurrences. In the presence of prolonged pain and dysphagia, imaging can be considered to evaluate for such sequelae.

A third branchial pouch cyst presenting as a lateral neck mass in an adult.

Anomalies of the branchial apparatus occur with some frequency in the adult and pediatric populations. Branchial anomalies are most often derivatives of the first or second pouch. Branchial anomalies involving the third pouch may present as cysts, sinuses, fistulas, and ectopic glands. They are relatively rare, and they respond well to surgical removal. We report the case of a 53-year-old woman who was referred to us for evaluation of a persistent left upper neck mass. The patient had no history of a cervical mass as a child or young adult. The mass was excised uneventfully, and the final pathology revealed a normocellular parathyroid gland and thymic tissue.

Treatment of chronic posterior laryngitis with esomeprazole.

OBJECTIVE: To evaluate the efficacy of acid-suppressive therapy with the proton pump inhibitor esomeprazole on the signs and symptoms of chronic posterior laryngitis (CPL) in patients with suspected reflux laryngitis. STUDY DESIGN: Prospective, multicenter, randomized, parallel-group trial that compared twice-daily esomeprazole 40 mg with placebo for 16 weeks. METHODS: Eligible patients had a history of one or more CPL symptoms (throat clearing, cough, globus, sore throat, or hoarseness) and laryngoscopic signs indicating reflux laryngitis based on CPL index (CPLI) scores measured during a screening laryngoscopy. Patients were randomized to treatment if their 7-day screening diary-card recordings showed a cumulative primary symptom score of 9 or higher and they had 3 or more days with moderately severe symptoms based on a 7-point scale. Efficacy was assessed by changes in symptoms as recorded by patients and investigators and by changes in CPLI scores based on laryngoscopic examinations. RESULTS: The patients' primary CPL symptom at final visit (primary efficacy end point) was resolved in 14.7% (14/95) and 16.0% (8/50) of patients in the esomeprazole and placebo groups, respectively (P=.799). Esomeprazole and placebo were not significantly different for change from baseline to the final visit in mean total CPLI (-1.66+/-2.13 vs. -2.0+/-2.55, respectively; P=.446) or any other secondary efficacy end points based on patient diary card or investigator assessments. CONCLUSION: This study provides no evidence of a therapeutic benefit of treatment with esomeprazole 40 mg twice daily for 16 weeks compared with placebo for signs and symptoms associated with CPL.

Increased incidence of carcinoma of the tongue in patients with systemic sclerosis.

OBJECTIVE: To describe the incidence of carcinoma of the tongue in a large cohort of patients with systemic sclerosis (SSc). METHODS: In total, 769 patients with SSc were prospectively followed over 16 years for the development of cancer. Patients with a diagnosis of carcinoma of the tongue were identified to determine the incidence of this cancer in SSc. The results were compared to the incidence of tongue cancer in the SEER cancer registries. RESULTS: A total of 3775 patient-years of followup of 769 patients with SSc (392 diffuse cutaneous, 377 limited cutaneous) prospectively evaluated for the occurrence of cancer disclosed 9 patients who were diagnosed with oral cavity and pharyngeal carcinomas. Six of these patients had squamous cell carcinoma of the tongue. One of these had both pharyngeal and tongue squamous cell carcinomas within a 4-year period, and another had 3 separate squamous cell carcinomas of the tongue. The standardized incidence ratio of squamous cell carcinoma of the tongue observed in this cohort of patients with SSc was 25-fold higher than that expected in an age adjusted population from the SEER cancer registries. All patients with SSc identified within this cohort with oral cavity carcinomas had the diffuse subset of the disease. CONCLUSION: There is a highly significant increase in the incidence of squamous cell carcinoma of the tongue in patients with SSc. A remarkable observation was that all patients within this cohort who developed oral cancer had the diffuse subset of SSc. This suggests a relationship between the etiology or pathogenesis of the diffuse form of SSc and development of squamous cell carcinoma of the tongue in this group of patients.

Thyroplasty revisions: frequency and predictive factors.

UNLABELLED: Thyroplasty is the most commonly performed type of laryngeal framework surgery, and the surgical indications are gradually being expanded. Although many reports have described thyroplasty results and rates of success, no study has attempted to determine predictors of the need for revisions or other secondary surgical procedures. METHODS: Retrospective review of 118 primary thyroplasty procedures performed on 96 patients. Secondary surgical procedures were divided into planned second-stage procedures (all fat implantation due to scarring), touchup procedures (primarily fat or collagen injections to close localized glottic gaps), and thyroplasty revisions (for implant extrusion or slippage). Statistical analysis was performed via the chi-squared technique with p < 0.05 considered significant. RESULTS: 96 patients underwent thyroplasty, 58 for vocal fold paresis or paralysis and 38 for other indications. Thirty-two (33%) patients underwent secondary surgical procedures, including 4 planned second stage procedures, and 6 patients with progression of their underlying disease. Thirty-two secondary procedures were performed in the other 22 patients. Professional singers were found to be more likely to require touchup or planned second-stage procedures (p = 0.029). Patients with abnormal preoperative noise-toharmonic ratios were more likely to undergo secondary surgeries (p = 0.039). Maximum phonation time was not associated with need for secondary surgery. Implant material did not influence revision rates. CONCLUSIONS: Professional singers and those patients with severe voice disorders (as measured by more abnormal noise-to-harmonic ratios) are more likely to undergo secondary surgical procedures. The choice of implant material does not affect need for secondary surgical procedures.

Prolonged ulcerative laryngitis.

Acute and prolonged laryngitis commonly are diagnosed and treated by primary care physicians. These ailments come to the attention of the otolaryngologist when symptoms are prolonged despite treatment. Since the beginning of 1991, the senior authors (RTS, JRS) have recognized a clinical syndrome defined by prolonged hoarseness, prolonged laryngeal inflammation, and vocal fold ulceration. Patients are frequently young, nonsmokers, and nondrinkers who have had an upper respiratory infection prior to, or associated with, the onset of laryngeal symptomatology. Patients may be affected for up to 1 year despite aggressive medical therapy. This review of 14 patients highlights the features of this previously undescribed entity.

Vocal tremor reduction with deep brain stimulation: a preliminary report.

Vocal tremor has been a challenging problem for patients and their physicians. In some cases, it has been possible to lesson the symptom's tremor through medications and/or voice therapy. However, in most cases no good treatment has been available. Chronic stimulation of the thalamus has been successful in controlling tremors of the upper limb and other portions of the body. Our preliminary experience suggests that it may also be helpful in controlling vocal tremor.

Chondrosarcoma of the skull base.

OBJECTIVES: Sarcomas of the skull base are challenging, potentially lethal tumors. Prognosis is considered poor. The present report reviews treatment options and presents a case of treatment with en bloc resection of the temporal bone and adjacent skull base. STUDY DESIGN: Single case report and literature review. RESULTS: Extensive skull base resection for chondrosarcoma can be performed successfully and may be curative. CONCLUSION: There is a role for en bloc resection of large areas of the skull base for treatment of chondrosarcoma. It appears that treatment combining surgery and radiation therapy is most likely to be effective.

Contraleteral deafness following ear surgery

Rare cases of contralateral hearing loss following surgery have been reported, usually associated with acoustic neuroma resection. We present one case of contralateral sensorineural hearing loss following middle ear reconstruction for congenital malformation, and one case following resection of a glomus jugulare tumor. Review of the literature confirms the rarity of contralateral hearing loss following ear surgery, and an autoimmune mechanisms seems most likely. Aggressive evaluation and treatment are indicated