RESUMO
Moebius syndrome is a collection of orofacial anomalies with highly variable features affecting many different systems but characterised by bilateral facial palsy and absent eye abduction. We largely regard Moebius syndrome as a diagnosis of exclusion. Lack of awareness and knowledge means that children often fall between services, leading to treatment delays and difficulty interfacing with social care and schools, with long-term impact on physical health and psychosocial development. We developed a multidisciplinary team comprising core clinicians (lead physician, geneticist, speech and language therapist, psychologist and specialist nurse) and an expanded group to encompass the other affected systems. The interactions between our specialties lead to the development of a treatment protocol, which we present. The protocol harnesses the aspects of care of children with a range of other rare diseases at a specialised paediatric centre and synthesises them into a holistic approach for MBS and related conditions. Management is sequenced on an "ABC-style" basis, with airway, feeding, vision and speech taking priority in the early years. We define management priorities as airway stabilisation with swallow assessment, ocular surface protection and maintenance of nutritional support. Management principles for issues such as speech, reflux, drooling and sleep issues are outlined. In later years, psychological support has a prominent role geared towards monitoring and interventions for low mood, self-esteem and bullying.
RESUMO
Introduction: Transperineal (TP) biopsy has recently replaced the transrectal ultrasound (TRUS) approach as the ideal method of biopsy in the United Kingdom with growing trends to adopt. To minimise transmission of COVID-19 during the first wave of the pandemic, the British Association of Urological Surgeons Section of Oncology issued guidelines reducing general anaesthesia (GA) procedures and initiate COVID-secure 'green' site diagnostics. As a result of these guidelines and reduction in clinical diagnostics trust-wide, we ceased all TRUS diagnostics and implemented a centralised, nurse-led LA TP biopsy service. Materials and methods: A waiting list was developed for those awaiting prostate cancer diagnostics across the network. A COVID-secure 'green' site was quickly identified with TP biopsies starting soon after. Quality improvement methodology was utilised and a run chart was used to show if changes were sustainable. Results: Successful implementation and centralisation of a TP biopsy service occurred with TRUS guided biopsies ceasing across all sites on 12 May 2020. The procedures were carried out by urology advanced nurse practitioners under local anaesthesia with a select few occurring under GA. Centralising the service in a COVID-secure manner freed up dedicated theatre sessions and personal leading to increased efficiency elsewhere. The service was robust and was maintained upon lifting of COVID restrictions. Conclusions: A centralised, nurse led LA TP biopsy service in a procedural unit was implemented successfully. The service has remained resilient upon lifting of restrictions and return to business as usual. This led to improved performance across trust by freeing up valuable resources and staff to undertake more duties. The service remains highly valued trust-wide.
