Paediatric Laryngeal Synovial Sarcoma: Dilemmas and Decision-Making.

Primary laryngeal synovial sarcoma is a rare head and neck cancer. We describe a case of synovial sarcoma of the larynx in a previously well 9-year-old boy with a one-month history of a progressively enlarging neck lump. He was referred to our institution after incomplete surgical excision of the then undifferentiated neck mass. A partial laryngectomy including wide local excision of the residual mass was performed. An ipsilateral level I-III neck dissection was also performed concurrently. Clear re-excision margins were achieved. The neck nodes were all negative for metastatic disease. Adjuvant local radiotherapy treatment was administered to reduce the probability of local recurrence. Four years following treatment completion, the patient remains in remission with no signs of treatment-related morbidity. A review of the published literature on laryngeal synovial sarcoma was undertaken. This case represents the youngest patient to be diagnosed with the condition. Surgical excision represents the mainstay of treatment of laryngeal synovial sarcoma. At more common sites of disease, adjuvant radiotherapy has been associated with lower rates of recurrence. However, there is the potential for significant morbidity from irradiating the neck of a paediatric patient. This case report explores the challenges in treating young patients with aggressive head and neck cancers when faced with little available evidence to guide decision-making.

Systematic review of head and neck lymphedema assessment.

Head and neck lymphedema (HNL) is an increasingly recognized complication of head and neck cancer and its treatment. However, no consensus exists on the "gold-standard" assessment tool for the purposes of diagnosis, classification, or monitoring of HNL. We conducted a systematic review of the literature regarding HNL assessment to determine the optimal method/s of assessment for patients with HNL. A review of publications between January 2000 and September 2021 was undertaken on four electronic databases. Studies were excluded if no clear assessment method of HNL was documented. Sixty-seven articles were included in the study. A wide range of assessment methods for HNL have been reported in the literature. For the purposes of diagnosis and classification of physical findings, computed tomography (CT) appears the most promising tool available for both external and internal HNL. In terms of monitoring, ultrasound appears optimal for external HNL, while a clinician-reported rating scale on laryngoscopy is the gold standard for internal HNL. Patient-reported assessment must be considered alongside objective methods to classify symptom burden and monitor improvement with treatment.

Timing of Ansa Cervicalis-to-recurrent Laryngeal Nerve Reinnervation: A Systematic Review.

BACKGROUND: Ansa cervicalis-to-recurrent laryngeal nerve anastomosis (ARA) is an established technique for the treatment of recurrent laryngeal nerve (RLN) injury after head and neck surgery. However, the optimal timing of ARA remains unclear, and the evidence bases for ARA performed at each timepoint after RLN injury have not previously been clearly distinguished. We conducted a systematic review of the literature to evaluate the efficacy of ARA performed at different timepoints on postoperative voice outcomes. METHODS: A review of English-language journal articles published in the last 20 years was undertaken on three electronic databases: Ovid MEDLINE, PubMed and Embase. Studies with a focus on paediatric RLN injury, bilateral RLN injury, ansa cervicalis anatomy and non-ARA techniques alone were excluded. RESULTS: Twenty eight articles were included in the review. ARA was performed as a delayed surgery in 16/28 studies (57%), while immediate ARA was utilized in 14/28 studies (50%). On qualitative synthesis, delayed ARA was shown to be effective in improving patient-reported, subjective observer-reported and objective observer-reported voice outcomes. Likewise, a substantial body of evidence was identified demonstrating postoperative voice improvement with immediate ARA. On direct comparison of timepoints, some benefit was shown for early delayed ARA relative to late delayed operations, while no comparative data for immediate versus delayed repair were available in the literature. CONCLUSIONS: ARA at both delayed and immediate timepoints is effective in the treatment of patients with RLN injury after head and neck surgery. The timing of ARA may have some influence on its efficacy, with early delayed repair potentially associated with superior outcomes to late delayed operations, and immediate ARA offering several practical advantages relative to delayed repair. Further comparative studies are required to better characterize the optimal timing of ARA after RLN injury.

Tranexamic acid in otorhinolaryngology - A contemporary review.

Tranexamic acid (TXA) is an anti-fibrinolytic agent which has been proven beneficial in multiple surgical specialties where significant bleeding can occur. Whilst it has been widely available for over 40 years its use within Otorhinolaryngology is still limited. Operations in Otorhinolaryngology are particularly varied with some such as tonsillectomy having the potential for significant life threatening bleeding. Other operations are performed within small confined surgical fields and even small amounts of bleeding can significantly detriment surgical field and increase technical difficulty and operative time. This review evaluated the current literature on the benefits of tranexamic acid within the field of Otorhinolaryngology and Head and Neck Surgery. Overall TXA was demonstrated to be a safe drug with no major adverse effects including thromboembolic events reported in any study. It has been shown to be of particular benefit in rhinology by improving surgical field, reducing operative time and reducing postoperative swelling and ecchymosis. The benefit in tonsillectomy is less clear and further studies are required to evaluate its potential use in the reduction of post tonsillectomy haemorrhage rates.

Laryngeal Synovial Sarcoma: A Systematic Review of the Last 40 Years of Reported Cases.

Primary laryngeal synovial sarcoma is an extremely rare tumor predominantly affecting young adults. There are currently no well-defined guidelines to direct investigation and management, and treatment is largely based on what is known for synovial sarcoma of the upper and lower limbs. This PROSPERO-registered study aims to review the diagnostic methods, treatment regimens, and survival outcomes for patients with synovial sarcoma of the larynx. A systematic search of databases Medline, Embase, SCOPUS, and Web of Science was undertaken in December 2017. The literature search identified 1031 potentially relevant studies, and after the deletion of duplicates and excluded papers, 98 full-text articles were screened. A total of 39 cases were reviewed from 32 studies in the data extraction. The average age at the time of laryngeal synovial sarcoma diagnosis was 32 years (range, 11-79 years). In all cases (n = 39), patients underwent wide surgical excision, with 20 patients requiring a partial or total laryngectomy. A total of 18 patients received adjuvant and 3 received neoadjuvant radiotherapy. Chemotherapy was used in 10 cases, with ifosfamide the most frequently used agent. There was considerable variability in the order and combinations of the abovementioned treatments. No clinicopathologic factors or treatment regimens were associated with improved overall survival or lower rate of recurrence. There is a paucity of literature and heterogeneity in clinical approaches to this highly aggressive sarcoma. Reporting of cases must be standardized and formal guidelines must be established to guide clinical management.

Acinic cell carcinoma of the salivary gland in the adult and paediatric population: a survival analysis.

BACKGROUND: Acinic cell carcinoma (AcCC) is a rare malignant neoplasm of the salivary glands and generally considered to be a low-grade tumour. Surgical treatment is often curative, but a more aggressive high-grade variant has been associated with poorer survival and propensity for distant metastasis. No standard treatment guidelines exist and the approach to treatment is varied in the published series. The aim of this study is to present the experience of three major hospitals in Sydney, Australia, in treating AcCC of the salivary gland, with a focus on clinico-pathological features of disease and their associations with survival outcomes. METHODS: Adult and paediatric cases of AcCC of the salivary gland during the time period 1979-2018 were retrospectively included. Demographic, clinico-pathological, treatment and survival outcome data were extracted. Survival analysis was undertaken to assess the effect of clinical and pathological variables on overall and disease-free survival. RESULTS: Thirty-two cases were reviewed (29 adult and three paediatric). Thirty tumours (93.8%) were parotid gland primary tumours. Mean overall and disease-free survival was 17.0 ± 0.7 and 16.0 ± 0.9 years, respectively. Features associated with poorer survival were cT staging >1, presence of preoperative clinical facial nerve deficit and local recurrence. Positive margins were associated with recurrence. CONCLUSION: These data suggest that disease-free and overall survival in AcCC of the salivary gland is excellent with surgery as the first-line treatment. Poor survival outcomes are uncommon and may be associated with locally advanced disease in the presence of other well-established high-risk features.

Meta-analysis on the rate of pharyngocutaneous fistula in early oral feeding in laryngectomy patients.

BACKGROUND: Laryngectomy remains a common operation in head and neck units. The operation holds significant risk of post-operative morbidity including swallowing dysfunction. The most significant post-operative concern is the formation of a pharyngocutaneous fistula [PCF], the reported incidence of which is between 3% and 65%. The purpose of this systematic review and meta-analysis was to assess the safety of initiating early oral feeding following laryngectomy and the risk of PCF formation. METHODS: A literature search was conducted through online databases: MEDLINE, EMBASE and PubMed. Eligible studies were included which contained cohorts of patients who had undergone laryngectomy, with early oral feeding commencing within seven days compared to late oral feeding. The primary outcome assessed was the incidence of PCF. Studies were excluded if cohorts had not included laryngectomy or if no comment was made on PCF formation. Meta-analysis was used to examine associations between oral feeding and PCF formation using Fixed Effect models. RESULTS: Twelve studies and 1883 patients were included after systematic review. Six studies were non-interventional whereas the remaining were randomized clinical trials. Exposure included those with early oral feeding (before seven days) or late feeding oral feeding (after seven days) and the outcome assessed was the risk of PCF formation. Results from observational studies showed a higher risk of PCF formation for early feeders compared to late feeders [RR = 1.56, 95% CI: 1.15, 2.11]. Higher risk was also observed for RCT but was not significant [RR = 1.40, 95% CI: 0.85, 2.30]. Overall, there was a 50% greater risk of PCF formation for early oral feeding compared to late oral feeding [RR = 1.51, 95% CI: 1.17, 1.96]. CONCLUSION: While early oral feeding can reduce post-laryngectomy patients' hospital stay and improve psychological wellbeing, there is a significant relative risk of PCF development within this group. However, this must be taken in context of the significant heterogeneity that exists within the literature.

Systematic review of laryngocele and pyolaryngocele management in the age of robotic surgery.

OBJECTIVE: A laryngocele is a space that develops as a result of pathological dilatation of the laryngeal saccule. However, the reported management of laryngoceles varies. We conducted a systematic review of the literature regarding the surgical management of laryngoceles and pyolaryngoceles, to understand the evolving nature of treatment for this rare condition. METHODS: We searched for publications in the PubMed, Cochrane Library, JBI Library of Systematic Reviews, and Ovid databases using the terms "laryngocele", "pyolaryngocele", and "laryngopyocele", and reviewed the identified articles. RESULTS: After removal of repeated studies and filtering for relevance and studies written in English, a total of 227 studies were included in this review. No meta-analyses or randomized controlled trials have been published. The identified studies have been summarized in 14 reviews conducted since 1946. The meta-analysis determined that endoscopy was the preferred approach for internal laryngoceles, while combined laryngoceles benefited from both internal and external surgical approaches. CONCLUSIONS: Laryngocele management has progressed since its initial description, from open surgery to an endoscopic approach, and more recently to a robotic-assisted surgical approach. The uptake of robotic surgery as a possible treatment modality over the last decade shows much promise for the treatment of these conditions.

Operative team checklist for aerosol generating procedures to minimise exposure of healthcare workers to SARS-CoV-2.

OBJECTIVES: In many countries around the world, the COVID-19 pandemic has resulted in health services being diverted to manage patients with the condition. There are situations however that still require the undertaking of aerosol generating procedures (AGP) with potentially high exposure of healthcare workers to SARS-CoV-2 transmission through droplet, contact and possibly airborne routes. The objective of this paper is to explore a structured way for the operative team to approach AGP to reduce aerosolisation of secretions, decrease open airway time and minimise staff exposure. METHODS: The authors (otolaryngologists, anaesthetists and nursing staff) created a unified operative team checklist based on collation of national and international specialty society statements, local state government recommendations, hospital policies and literature review. Simulation was undertaken and the checklist was refined after performing AGP on patients with unknown (presumed positive) COVID-19 status. RESULTS: An 8 step operative team checklist is provided describing details for the immediate pre-operative, intra-operative and post-operative journey of the patient to encourage healthcare workers to reflect upon and modify usual practice during AGP to mitigate exposure to SARS-CoV-2. The example of paediatric laryngo-bronchoscopy for diagnostic purposes or retrieval of an inhaled airway foreign body is used to illustrate the steps however the checklist structure is modifiable for other AGP and adaptable for local needs. CONCLUSIONS: At a time of overwhelming and changing information and recommendations, an operative team checklist may provide some structure to healthcare workers undertaking AGP to reduce anxiety, maintain focus, prompt consideration of alternatives and potentially reduce risk.

A Case of Focal Dermal Hypoplasia (Goltze Syndrome) Masquerading as Lingual Tonsillar Hypertrophy.

Focal dermal hypoplasia is a rare condition affecting organ systems of mesodermal origin. We present a rare case of this condition presenting with apparent palatine tonsillar regrowth and outline our management. Airway management should always be a consideration in this rare condition.

Radiofrequency coblation of congenital nasopharyngeal teratoma: a novel technique.

Introduction. Congenital nasopharyngeal teratomas are rare tumours that pose difficulties in diagnosis and surgical management. We report the first use of radiofrequency coblation in the management of such tumours. Case Report. A premature baby with a perinatal diagnosis of a large, obstructing nasooropharyngeal mass was referred to the ENT service for further investigations and management. The initial biopsy was suggestive of a neuroblastoma, but the tumour demonstrated rapid growth despite appropriate chemotherapy. In a novel use of radiofrequency coblation, the nasooropharyngeal mass was completely excised, with the final histopathology revealing a congenital nasopharyngeal teratoma. Conclusion. We report the first use of radiofrequency coblation to excise a congenital nasopharyngeal teratoma and discuss its advantages.

Near-complete supraglottic transection of the larynx after a motorbike accident.

Severe laryngeal trauma is rare in the civilian environment and requires appropriate and timely surgical intervention. We report a case from Sydney, Australia, which was managed with open reduction and internal fixation of the larynx with resorbable plates. The use of resorbable plates for operative fixation of the larynx has rarely been reported in literature but may be a viable alternative.

Giant de novo pleomorphic adenoma arising from the parapharyngeal space.

Introduction. De novo pleomorphic adenomas in the parapharyngeal space are rare and cause difficulties in its surgical management. We report the largest de novo pleomorphic adenoma arising from the parapharyngeal space and discuss its surgical management. Presentation of Case. A 34-year-old male presented with a giant de novo pleomorphic adenoma arising from the parapharyngeal space, which was initially misdiagnosed as an impacted wisdom tooth. Measuring 8.4 × 6.5 × 3.9 cm in size and weighing 87.3 g, this is the largest primary salivary gland tumour arising de novo from the parapharyngeal space reported in the literature, presenting challenges in its surgical management. Discussion. Parapharyngeal space tumours cause nonspecific symptoms and may be difficult to diagnose, which can allow the tumours to become very large and cause obstructive and compressive symptoms in an anatomically difficult area. A combined trans-cervical and trans-oral approach can be used to safely perform an en bloc resection. Conclusion. We report the diagnosis and surgical management of the largest pleomorphic adenoma arising de novo from the parapharyngeal space reported in the literature.

Down-regulation of KAI1/CD82 protein expression in oral cancer correlates with reduced disease free survival and overall patient survival.

Oral Squamous Cell Carcinoma (OSCC) is a common malignancy. Treatment failure is mainly due to loco-regional disease recurrence. KAI1 is a newly discovered metastasis suppressor gene. Fifty-seven patients with primary OSCC underwent surgery alone or surgery and adjuvant radiotherapy. Immunohistochemical evaluation of KAI1/CD82 and p53 proteins was carried out on specimen obtained at surgery. Within neoplastic fields, KAI1/CD82 expression was downregulated and negative in 42/57 (73.7%) cases. p53 expression was positive in 26/57 (45.6%) cases. No correlation was noted between KAI1/CD82 and p53 expression or clinicopathological parameters. Univariate and multivariate Cox proportional hazard models showed a correlation between KAI1/CD82 expression with disease free survival (P = 0.01, P = 0.009) and overall survival (P = 0.04, P = 0.053) respectively.