Perioperative care of a child with ullrich congenital muscular dystrophy

Ullrich congential muscular dystrophy [UCMD] is a severe form of congenital muscular dystrophy manifesting axial muscle contractures and distal joint hyperlaxity. Severe hypotonia and associated respiratory failure may occur early in the disease process. Given the various associated orthopedic conditions, anesthetic management may be required during surgical interventions to correct skeletal deformities or these patients may present with surgical conditions unrelated to their primary illness. We present a 4-year-old with UCMD who required operative intervention for a ruptured appendix. Anesthetic care implications included the need for a rapid airway control to limit the risks of aspiration due to the intra-abdominal process, choice of neuromuscular blocking agent for rapid sequence intubation, associated airway issues related to micrognathia and limited mouth opening, and the potential for involvement of the cardiovascular and respiratory systems. The perioperative management of patients with UCMD is discussed including the use of propofol and remifentanil for rapid sequence intubation to avoid the need for neuromuscular blocking agents

Cirugía de los miembros inferiores en la distrofia muscular progresiva: Enfermedad de Duchenne

Fueron evaluados 55 pacientes con distrofia muscular progresiva de tipo Duchenne, de los cuales 20 se intervinieron quirúrgicamente a una edad promedio de 9 años. El tiempo promedio de seguimiento postoperatorio fue de 1 año y 3 meses. Comparamos en este trabajo la persistencia de marcha entre los pacientes del grupo quirúrgico y aquellos no operados a igual tiempo de seguimiento. Se establecen los puntos primordiales de evaluación preoperatoria, el tipo de equipamiento ortésico y los cuidados postoperatorios a tener en cuenta en este tipo de pacientes

Artrodese escapular no tratamento da insuficiência escápulo-torácica da distrofia fáscio-escápulo-umeral / Scapular arthrodesis in the treatment of the scapulothoracic insufficiency of facioscapulohumeral dystrophy

A Distrofia Fáscio-Escápulo-Umeral é uma miopatia benigna que provoca grande incapacidade funcional dos membros superiores pelo comprometimento dos músculos estabilizadores da escápula. É apresentada uma técnica de artrodese da escápula que permite dispensar a imobilizaçäo prolongada do membro superior, favorecendo a reabilitaçäo precoce