Anesthetic considerations for scoliosis surgery in a patient with recessive severe/lethal form of osteogenesis imperfecta.

Osteogenesis imperfecta is a heterogeneous genetic disorder characterized by bone fragility, with disease ranging from mild fractures to death in utero. We describe a child with autosomal recessive osteogenesis imperfecta type VIII (severe or lethal phenotype), who successfully underwent posterior spinal fusion, was extubated on postoperative day 1 and discharged home 25 days later. Recently identified recessive forms of osteogenesis imperfecta are associated with severe/lethal phenotype. Special consideration is needed in scoliosis surgery, with challenges arising from prone positioning, neurophysiology monitoring, and blood loss.

Using quality improvement methods to reduce clear fluid fasting times in children on a preoperative ward.

OBJECTIVES: We applied quality improvement (QI) methodology to identify the different aspects of why children fasted for prolonged periods in our institution. Our aim was for 75% of all children to be fasted for clear fluid for less than 4 hours. BACKGROUND: Prolonged fasting in children can increase thirst and irritability and have adverse effects on haemodynamic stability on induction. By reducing this, children may be less irritable, more comfortable and more physiologically stable, improving the preoperative experience for both children and carers. METHODS: We conducted a QI project from January 2014 until August 2016 at a large tertiary pediatric teaching hospital. Baseline data and the magnitude of the problem were obtained from pilot studies. This allowed us to build a key driver diagram, a process map and conduct a failure mode and effects analysis. Using a framework of Plan-Do-Study-Act cycles our key interventions primarily focused on reducing confusion over procedure start times, giving parents accurate information, empowering staff and reducing variation by allowing children to drink on arrival (up to one hour) before surgery. RESULTS: Prior to this project, using the 6,4,2 fasting rule for solids, breast milk, and clear fluids, respectively, 19% of children were fasted for fluid for less than 4 hours, mean fluid fasting time was 6.3 hours (SD 4.48). At the conclusion 72% of patients received a drink within 4 hours, mean fluid fasting reduced to 3.1 hours (SD 2.33). The secondary measures of aspiration (4.14:10 000) and cancellations have not increased since starting this project. CONCLUSIONS: By using established QI methodology we reduced the mean fluid fasting time for day admissions at our hospital to 3.1 hours and increased the proportion of children fasting for less than 4 hours from 19% to 72%.

Delivery of anesthesia for children with Mucopolysaccharidosis Type III (Sanfilippo syndrome): a review of 86 anesthetics.

BACKGROUND: Sanfilippo syndrome (MPS III) is rare, with 97 cases in the United Kingdom between 1988 and 1998. Mucopolysaccharide infiltration of tissues in mucopolysaccharidosis (MPS) causes multi-systemic pathology including difficult airways and cardiac disease. Published anesthesia case reviews of Sanfilippo syndrome have included limited numbers of patients to date. AIM: To identify the perioperative management and complications of anesthesia in children with mucopolysaccharidosis Type III at Great Ormond Street Hospital. METHODS: A retrospective case note review of all children with MPS III in our institution was undertaken. All medical notes and anesthetic charts were analyzed, and conduct of anesthesia, airway management, perioperative complications, and associated comorbidities were identified. RESULTS: There were 43 patients with MPS III, of which 34 required anesthesia, on 86 occasions for 156 procedures between 1993 and 2015. Dental extraction was the likeliest indication for anesthesia (34%) (general surgery [30%]; ear, nose, and throat [26%]; other [10%]). Thirteen of 34 patients had cardiac pathology (valvular [n = 6], functional [n = 6], electrophysiological [n = 1]). Ten of 34 patients had evidence of clotting abnormality (mild prolonged clotting time [n = 5], low von Willebrand factor [n = 2], thrombocytopenia [n = 3]). The majority of intubations were Cormack-Lehane Grade 1 (n = 47) (Grade 2 [n = 14], Grade 3 [n = 1], Grade 4 [n = 1]). In 86 anesthetics, there were 0 cases of difficulty with mask ventilation. There was 1 case of failed intubation. They were subsequently anesthetized by a different operator uneventfully at a later date. Two perioperative complications occurred: a failed intubation and bleeding during adenoidectomy. CONCLUSION: We demonstrate a difficult airway is unlikely when anesthetizing an MPS III patient although a risk does remain. A significant proportion of MPS III have cardiac involvement although no perioperative complications were described. With associated coagulation issues, bleeding tendency, while uncommon, can occur in this group.

The anaesthetic management of conjoined twins.

The management of anaesthesia for conjoined twins poses unique anatomical, physiological and logistic challenges. Although many possible configurations of union exist and each is unique, we describe the principles of anaesthesia for conjoined twins drawing on our institutional experience of managing 26 sets for a variety of procedures including separation.