Perioperative anesthetic management of reductive glossoplasty in a patient with Beckwith-Wiedemann syndrome. / Manejo anestésico perioperatorio de glosoplastia reductora en paciente con síndrome de Beckwith-Wiedemann.

INTRODUCTION: Postoperative management of patients with the congenital growth disorder Beckwith-Wiedemann syndrome (BWS) can be complicated. The main clinical manifestations of the syndrome are macroglossia - which may hamper airway management -, prematurity, hemihypertrophy, omphalocele, embryonal tumours and episodes of neonatal hypoglycaemia. OBJECTIVE: Our main objective is to describe the perioperative management and potential anaesthetic complications in paediatric patients with BWS undergoing glossectomy. METHODS: Case report and literature review. RESULTS: We describe the case of an 11-month-old patient diagnosed with BWS who underwent reduction glossoplasty. We performed a comprehensive preoperative evaluation, taking into account potential anaesthetic complications derived from both macroglossia and prematurity, and the risk of hypoglycaemia. The procedure was performed under general anaesthesia. Intubation - performed according to difficult airway management algorithms - was uneventful and the patient was successfully extubated in the operating room. The patient remained stable during the postoperative period, with good respiratory dynamics, SatO2>96% and good glycaemic control. Oral intake was started 4hours after surgery, and she was discharged to the ward at 24hours. CONCLUSION: BWS patients require a multimodal approach that includes detailed preoperative planning and knowledge of potential airway-related and systemic complications.

Impact of empirical treatment with antifungal agents on survival of patients with candidemia.

OBJECTIVE: The objective of this study was to evaluate the impact of echinocandins and fluconazole) on mortality 7 and 30 days after candidemia onset and overall in-hospital mortality), in patients with candidemia at a Spanish tertiary hospital. METHODS: A retrospective study was conducted that enrolled all non-neutropenic adult patients diagnosed with candidemia at Hospital Clínico Universitario de Valladolid between 2007 and 2016. A total of 179 patients were evaluated, they were divided into two sub-groups: surviving patients (n = 92) and non-surviving patients (n = 87). RESULTS: The 7-day mortality was 25,1% (45), 30-day mortality was 46,9% (84), and overall in-hospital mortality was 48,6% (87). 40.8% of patients received no antifungal treatment (43.8% of surviving patients and 37.8% of non-surviving patients; p=0.15). A total of 106 (59.2%) patients were treated, of which 90 patients (50.3%) received empiric treatment. 19.6% and 47.8% of surviving patients were treated with echinocandins and fluconazole, respectively. By contrast, of non-surviving patients, 31.0% were treated with echinocandins and 47.1% received fluconazole. Survival for the first 7 days was significantly higher in treated with antifungal agents (log-rank = 0.029), however, there were not significant differences in 30-day survival. Factors linked to a significant increase in overall in-hospital mortality were age (OR 1.040), septic shock (OR 2.694) and need for mechanical ventilation > 48 h (OR 2.812). CONCLUSIONS: Patients who received antifungal treatment, regardless of whether they received fluconazole or echinocandins, had a significantly lower mortality rate after 7 days than untreated patients, although no significant differences in 30-day mortality were seen.

Anaesthetic management of a paediatric patient with congenital fibre type disproportion myopathy. / Tratamiento anestésico en una paciente pediátrica con miopatía congénita por desproporción del tipo de fibras.

Congenital fibre type disproportion (CFTD) is a rare type of myopathy that is characterised by muscle weakness and hypotonia during childhood. Clinical features include motor delay, feeding difficulties, limb weakness, joint contractures, and scoliosis. A report is presented of the anaesthetic management of a 3-year-old girl with CFTD myopathy associated with a mutation of the TPM3 gene, scheduled for adenotonsillectomy because of obstructive sleep apnoea hypopnoea syndrome (OSAHS). The main concerns were the possible susceptibility to malignant hyperthermia, the risk of anaesthesia-induced rhabdomyolysis, a greater sensitivity to non-depolarising muscle relaxants, and the presence of OSAHS. Total intravenous anaesthesia with propofol and the use of rocuronium/sugammadex appear to be safe options. Given the high risk of respiratory compromise and other complications, patients should be closely monitored in the post-operative period.

Descriptive study of ultrasound images of the upper airway obtained after insertion of laryngeal mask. / Imágenes ultrasonográficas de vía aérea superior tras la inserción de mascarilla laríngea: estudio descriptivo y utilidad clínica.

OBJECTIVE: To evaluate clinical usefulness of ultrasound images of the upper airway in order to check correct laryngeal mask placement. MATERIAL AND METHODS: A prospective observational study was conducted on patients scheduled for abdominal surgery under general anaesthesia, in whom the patency of the upper airway was ensured using an Ambu®AuraGainTM laryngeal mask. An ultrasound scan was performed of the upper-airway in the cranio-caudal direction and with longitudinal scans in the anterior midline and parasagittal axis, in three moments: before, after inserting and after removing the mask. All recorded images were evaluated in a second time by a radiologist-expert in upper airway ultrasound. Subsequently, the ultrasound data were related to the clinical difficulty of the insertion and presence of air leaks. RESULTS: Data was collected from 30 patients (20 females and 10 males) being operated on for abdominal hysterectomy (15), eventroplasty (6), uterine myomectomy (3), and umbilical (4) and inguinal herniorrhaphy (2). The blind insertion of the masks did not present difficulties in 24 (80%) patients. Air leakage was detected in 8 (26.7%) patients, which was moderate in 7 cases and severe in one of them. The ultrasound findings confirmed good mask placement in 22 (73.3%) patients. Anatomical airway changes after laryngeal mask extraction were only observed in 3 (12%) patients, all of them minor. There was a statistically significant association (P<.05) between difficulty in inserting the device and the level of air leakage. CONCLUSIONS: Upper airway ultrasound is a useful diagnostic method to evaluate laryngeal mask placement. Laryngeal oedema was not observed after removal of the device.