Combined General and Regional Anesthesia for a Patient With Duchenne Muscle Dystrophy With an Implanted Left Ventricular Assisted Device Undergoing Orthopedic Surgery.

Duchenne muscular dystrophy (DMD) is an X-linked inherited dystrophinopathy, with an incidence of 1 in 3,600 - 5,000 male live-born infants. The leading cause of death is often cardiomyopathy-related heart failure. Given the progressive nature of the disorder with involvement of skeletal muscle, respiratory and cardiac function, perioperative care remains challenging with an increased incidence of perioperative morbidity and mortality. Perioperative care can be challenging due to life-threatening perioperative adverse events related to associated end-organ effects, as well as sensitivity to various anesthetic agents, rhabdomyolysis, hyperkalemia, hyperthermia, and cardiac arrest. We present a 22-year-old DMD patient with left ventricular assisted device (LVAD), who presented for repair of both left distal femur and tibial diaphysis fractures. Anesthetic care included the unique combination of total intravenous anesthesia with dexmedetomidine and remimazolam combined with regional anesthesia including a supra-inguinal fascia iliaca block, saphenous nerve block, and popliteal nerve block. The basics of dystrophinopathies are presented, perioperative concerns discussed, and previous reports of the use of regional anesthesia as an adjunct to general anesthesia in adult and pediatric patients with DMD are reviewed.

Evaluation of postoperative analgesia in pediatric patients after hip surgery: lumbar plexus versus caudal epidural analgesia.

Background:There continues to be focus on the value of regional and neuraxial anesthetic techniques when combined with general anesthesia to improve postoperative analgesia. The reported advantages include decreased postoperative opioid requirements, decreased medication-related adverse effects, decreased hospital length of stay, and increased patient satisfaction. Orthopedic procedures of the hip may be amenable to such techniques as there is significant postoperative pain with the requirement for hospital admission and the administration of parenteral opioids. Given the surgical site, various regional anesthetic techniques may be used to provide analgesia including caudal epidural anesthesia (CEA) or lumbar plexus blockade (LPB). Purpose: The objective of this study was to assess the effectiveness of LPB versus CEA as an analgesic thechnique for patients undergoing elective hip surgery from the opioid consumption and pain scores perspective. Patients and methods : The current study retrospectively reviews our experience with CEA and LPB for postoperative analgesia after hip surgery in the pediatric population. Regional anesthesia technique was reviewed as well as opioid requirements and pain scores. Results: The study cohort included 61 patients, 29 who received an LPB and 32 who received CEA. No difference was noted in the demographics between the two groups. Intraoperative opioid use was 0.7 (IQR: 0.5, 1.1) mg/kg of oral morphine equivalents (MEs) in the LPB group compared to 0.6 (IQR: 0.5, 0.9) in the CEA group (p=0.479). Postoperative opioid use over the first 48 hrs was 4 (IQR: 1, 6) mg/kg of oral ME in the LPB group, compared to 2 (interquartile range [IQR]: 1, 3) in the CEA group (p=0.103). Over the first 24 hrs after surgery, the median pain score in the LPB group was 5 (IQR: 1-6), compared to 3 (IQR: 0, 5) in the CEA group (p=0.014).Conclusion: These retrospective data suggest a modest postoperative benefit of CEA when compared to LPB following hip surgery in the pediatric population. Postoperative pain scores were lower in patients receiving CEA; however, no difference in the intraoperative or postoperative opioid requirements was noted between the two groups.

Caudal epidural block instead of general anesthesia in an adult with Duchenne muscular dystrophy.

Duchenne muscular dystrophy (DMD), first described in 1834, is an X-linked dystrophinopathy, leading to early onset skeletal muscle weakness. Life expectancy is reduced to early adulthood as a result of involvement of voluntary skeletal muscles with respiratory failure, orthopedic deformities, and associated cardiomyopathy. Given its multisystem involvement, surgical intervention may be required to address the sequelae of the disease process. We present a 36-year-old adult with DMD, who required anesthetic care during surgical debridement of an ischial pressure sore. Given his significant respiratory muscle involvement, ultrasound-guided caudal epidural anesthesia was used instead of general during the surgical procedure. The technique and its applications are discussed, with particular emphasis on the feasibility and safety of using regional anesthetic techniques in patients with DMD.

Evaluating interest in narrative therapy for decision making about pectus excavatum treatment.

OBJECTIVES: The decision to proceed with surgical treatment for pectus excavatum (PE) is rarely clear-cut. Patients interested in treatment are referred for evaluation by numerous different specialists, but psychosocial counselling is currently not included in this process. Our objective was to assess whether PE patients would be interested in formal assistance with the decision-making process surrounding PE surgery using narrative therapy principles. METHODS: Ninety-seven untreated PE patients at 5 different institutions in 4 countries completed a questionnaire consisting of 13 questions, with 3 questions specifically evaluating interest in narrative therapy. RESULTS: Eighty-two percent of participants were interested in narrative therapy to assist with the decision-making process surrounding PE surgery. Individuals most interested in narrative therapy tend to be more interested in correction (P < 0.05) to improve the way they feel about their body (P < 0.05). CONCLUSIONS: The majority of PE patients are interested in narrative therapy to aid the decision-making process about treatment. We propose that narrative therapy should be offered to PE patients during the treatment evaluation process to address the psychosocial difficulties associated with PE and the surgical decision-making process overall. Future studies should assess the effectiveness of this interdisciplinary model.

A rare form of trifascicular block with intermittent complete atrioventricular block in a patient with Chagas disease.

Trifascicular block, which consists of impaired conduction in the three main fascicles of the ventricular conduction system, may progress to high-grade or complete atrioventricular block. Exceptionally, it is possible to register in the same patient paroxysmal alternating atrioventricular block and bilateral bundle branch block. This is the electrocardiogram of a male, 60 year-old patient coming from an endemic area, with positive serology for Chagas disease, with the exclusively dromotropic form (there are no signs of cardiac muscle involvement), manifest by repetitive pre-syncope and syncope episodes.