Pompe disease (PD) is a type II glycogen storage disease, characterised by abnormal glycogen deposition, mainly in heart and skeletal muscles, leading to progressive loss of muscle function. The infantile variety is associated with severe hypertrophic cardiomyopathy and generally do not reach adulthood. The juvenile variety presents with progressive muscle weakness and respiratory failure. Anaesthetic management concerns in the patient reported here were mainly due to respiratory failure, myopathy and sensitivity to muscle relaxants and significant haemodynamic changes perioperatively. We successfully managed a 13-year-old girl with juvenile PD on respiratory support scheduled for thoracolumbar kyphoscoliosis corrective surgery. Ketamine and dexmedetomidine were used for induction of anaesthesia and maintenance. Muscle relaxants were diligently avoided in this case.

Background and Aims:Twin pregnancy with monochorionic placenta may be associated with arteriovenous vascular anastomosis of the placental vessels resulting in twin-to-twin transfusion syndrome (TTTS) and twin reversed arterial perfusion syndrome (TRAP). Foetoscopic LASER ablation (FLA) is the treatment of choice in reducing foetal mortality related to this.Methods:A retrospective review of medical records of 41 FLA procedures for TTTS and TRAP were analysed for anaesthetic management. Thirty-four patients received subarachnoid block, three combined spinal-epidural block, three general anaesthesia and one local anaesthesia with sedation. Nitroglycerine 5 mg patch was used for tocolysis 1 h before the procedure and continued for 24–48 h postoperatively.Results:Bupivacaine was used in 34 patients, and ropivacaine in three patients. Mean dose of bupivacaine 0.5% was 2.43 ± 0.32 ml and ropivacaine 0.75% was 2.85 ± 0.19 ml. The mean duration of surgery was 117.07 ± 28 min. Mild hypotension occurred in all patients under spinal anaesthesia and was treated with vasopressors. The foetal outcome among all 41 patients were 13 delivered live twins, 15 had a single live baby with intrauterine death of other twin baby. In 12 patients, both babies were intrauterine death. One patient was lost for follow-up.Conclusion:Foetoscopic procedures can be done under central neuraxial block, however occasionally general anaesthesia may be required.

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