Perioperative management of patients with amyotrophic lateral sclerosis: A narrative review.

Amyotrophic lateral sclerosis, or motor neuron disease, is an uncommon progressive neurological disorder. Professionals working in the perioperative field may encounter patients with amyotrophic lateral sclerosis only rarely. The relevant published literature on amyotrophic lateral sclerosis is broad in scope, but a contemporary review focused on the perioperative period is absent. This structured narrative review seeks to provide a summary of the contemporary management of patients and then focuses on eliciting if there are perioperative management considerations specific to amyotrophic lateral sclerosis that can be optimised. A comprehensive structured narrative literature review, including grey literature searching, indicated worsening ventilatory failure is of prime concern but that patients may present with a broad range of neurological symptoms, and that cardiovascular and cognitive dysfunction specific to amyotrophic lateral sclerosis may exist and be occult. Exacerbation of neuromuscular weakness during the perioperative period is multifaceted and requires the application of a high standard of the core principles of surgical and anaesthetic management of neuromuscular disease. Standard perioperative approaches require rigorous attention and potential exists for significant alteration. There is a potential high risk of postoperative increased morbidity from neurological decline and mortality from pulmonary complications. A meticulous approach to planning preoperative assessment, shared decision-making, intraoperative and postoperative care is required.

Thriving, not just surviving, with motor neurone disease. The outcome of the first pre-emptive 'triple-ostomy'.

The following report details the multidisciplinary treatment of a patient with motor neurone disease. The patient, who requested publication of this case, is a highly intelligent and distinguished robotic scientist. He was diagnosed with amyotrophic lateral sclerosis in 2017 and his personal approach to his condition has been to use modern technology and all treatment options to maximise his quality and duration of life. After his research, the patient decided that his life would be significantly improved by formation of an elective 'triple-ostomy', this being an end colostomy and suprapubic catheter (for continence), and a percutaneous gastrostomy (for nutrition). We report the peri-operative multidisciplinary approach taken with this case, the surgical procedures, the potential risks and the outcome. The patient is delighted with the result and aims to raise awareness that this may be a treatment option in highly selected patients.

Cochlear implants and infants: expectations and outcomes.

This study followed the communication and motor development of 3 profoundly deaf infants who underwent cochlear implantation. All infants were receiving auditory habilitation at the Sydney Cochlear Implant Centre, Australia, with the view to acquiring oral language skills and had English-speaking, hearing parents. Their progress was tracked with a battery of measurement tools before and after cochlear implantation. The results indicated noticeable growth in receptive language and motor skills after operation. Although expressive language improved, the rate of growth was much slower than changes in other areas. Although some areas of the subjects' communication skills remained delayed, the importance of tracking their progress according to their hearing age, as well as the chronological age, was evident.