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.

Outcomes of immediate facial nerve reanimation with nerve transfer for facial nerve neoplasm-induced paralysis: a retrospective review.

BACKGROUND: The purpose of this study was to evaluate the outcomes of our polyneural, zone-based reanimation approach for patients with neoplasm-induced facial paralysis. METHODS: A retrospective review of consecutive patients who underwent facial reanimation surgery using multiple donor nerve transfers was undertaken. In each case, the selection of donor nerves was based on the availability of donor nerve and the viability of the motor endplate on the affected side. Sources of the neural inputs utilized included the remnant facial nerve stump, masseteric nerve, partial hypoglossal nerve, and branches of the contralateral facial nerve. Clinical outcomes were scored by expert raters. Ratings were undertaken using the modified House-Brackmann, eFACE and MEEI FACEgram scoring systems. RESULTS: Between 2017 and 2020, 12 patients were included in the study (mean age 60 years; range 26-81 years). Eight patients (67%) achieved a grade III outcome on the modified House-Brackmann grading scale. Mean eFACE static and dynamic scores were 76 and 57 respectively, reflecting a high degree of symmetry at rest and moderate restoration of dynamic movement. Mean time to movement was 5.4 months (SD 1.9). Objective FACE-gram measurements confirmed restoration of midface movement with an average improvement in smile excursion and mouth angle excursion of 3.19 mm (SD 3.18) and 4.81° (SD 2.90) respectively. CONCLUSION: Facial reanimation using multiple nerve transfers is effective in achieving improvements in facial function and symmetry.

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.

Accessibility of Primary, Specialist, and Allied Health Services for Aboriginal People Living in Rural and Remote Communities: Protocol for a Mixed-Methods Study.

BACKGROUND: Primary, specialist, and allied health services can assist in providing equitable access in rural and remote areas, where higher proportions of Aboriginal and Torres Strait Islander people (Aboriginal Australians) reside, to overcome the high rates of chronic diseases experienced by this population group. Little is currently known about the location and frequency of services and the extent to which providers believe delivery is occurring in a sustained and coordinated manner. OBJECTIVE: The objective of this study will be to determine the availability, accessibility, and level of coordination of a range of community-based health care services to Aboriginal people and identify potential barriers in accessing health care services from the perspectives of the health service providers. METHODS: This mixed-methods study will take place in 3 deidentified communities in New South Wales selected for their high population of Aboriginal people and geographical representation of location type (coastal, rural, and border). The study is designed and will be conducted in collaboration with the communities, Aboriginal Community Controlled Health Services (ACCHSs), and other local health services. Data collection will involve face-to-face and telephone interviews with participants who are health and community professionals and stakeholders. Participants will be recruited through snowball sampling and will answer structured, quantitative questions about the availability and accessibility of primary health care, specialist medical and allied health services and qualitative questions about accessing services. Quantitative data analysis will determine the frequency and accessibility of specific services across each community. Thematic and content analysis will identify issues relating to availability, accessibility, and coordination arising from the qualitative data. We will then combine the quantitative and qualitative data using a health ecosystems approach. RESULTS: We identified 28 stakeholder participants across the ACCHSs for recruitment through snowball sampling (coastal, n=4; rural, n=12; and border, n=12) for data collection. The project was funded in 2017, and enrolment was completed in 2017. Data analysis is currently under way, and the first results are expected to be submitted for publication in 2019. CONCLUSIONS: The study will give an indication of the scope and level of coordination of primary, specialist, and allied health services in rural communities with high Aboriginal populations from the perspectives of service providers from those communities. Identification of factors affecting the availability, accessibility, and coordination of services can assist ways of developing and implementing culturally sensitive service delivery. These findings could inform recommendations for the provision of health services for Aboriginal people in rural and remote settings. The study will also contribute to the broader literature of rural and remote health service provision. INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): DERR1-10.2196/11471.

Return to Sports and Physical Activities After Open Reduction and Internal Fixation of Lisfranc Injuries in Recreational Athletes.

BACKGROUND: The purpose of this study was to assess participation in sport and physical activity following open reduction and internal fixation of a Lisfranc injury in a cohort of recreational athletes. METHODS: This study identified all adult patients aged 55 years or younger who presented with a Lisfranc injury and underwent open reduction and internal fixation (ORIF) using a Lisfranc screw combined with bridge plating technique. Sports and physical activity participation was assessed with a new sports-specific, patient-administered questionnaire. Clinical outcomes were assessed with the Foot and Ankle Outcome Score (FAOS). Thirty-three patients qualified for the study (21 men/12 women). Mean age and follow-up were 31.2 (range, 18-55) years and 2.9 (range, 1.5-5.4) years, respectively. RESULTS: Postoperatively, 31 patients (94%) were able to return to some form of sport. Twenty-two patients (66%) returned to playing sport at or above their preinjury level. Of the 11 patients who played less sport, 6 had ongoing pain, and the remaining 5 were asymptomatic but were participating less frequently because of other lifestyle reasons. In addition, of the 33 patients, 11 (33%) had some degree of ongoing pain that might limit their ability to return to sports and physical activities. There was strong correlation between overall FAOS and the Sports Questionnaire. CONCLUSION: Most patients who sustained a Lisfranc injury could return to sport and physical activity after ORIF. Patients should be counseled preoperatively that about 1 in 3 might experience continued pain at the injury site Level of Evidence: Level IV, retrospective case series.