RESUMO
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.
RESUMO
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.
RESUMO
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.
