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Lian X, Jing Y, Luo T, Guo Y, Lin Y. Pharmacological interventions for the management of anesthesia and sedation in patients with Duchenne muscular dystrophy: a systematic review and meta-analysis. Front Med (Lausanne) 2025; 12:1497538. [PMID: 39931560 PMCID: PMC11808030 DOI: 10.3389/fmed.2025.1497538] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/19/2024] [Accepted: 01/06/2025] [Indexed: 02/13/2025] Open
Abstract
Background Patients with duchenne muscular dystrophy (DMD) have an increased risk of complications when they undergo sedation or general anesthesia. However, due to improvements in cardiopulmonary therapies during anesthetic care, patients with DMD are experiencing an unprecedented duration of survival. We performed a systematic analysis to assess the benefits and risks of pharmacological interventions for the management of anesthesia and sedation in DMD patients. Methods We included any type of study reporting any drug intervention to manage anesthesia and sedation in participants previously diagnosed with DMD. Our primary outcomes were the onset time, recovery time, and neurodevelopmental disabilities. Seven electronic databases and three clinical trial registry platforms were searched. Data from the eligible studies were combined to calculate pooled risk ratios or standardized mean differences, and some included studies are presented in a narrative synthesis. Results Forty studies with 196 DMD participants were included in the analysis. Compared with those of the control group, the sensitivity of patients with DMD to neuromuscular blocking agents (NMBAs) may have resulted in a prolonged onset time [MD = -0.96, 95% CI (0.71, 2.60), I 2 = 33%, P < 0.0001] and recovery time [MD = 2.22, 95% CI (1.14, 3.30), I 2 = 76%, P < 0.0001] from anesthesia. The neuromuscular blocking effects showed a significant age dependence in DMD patients, and the safe use of 2 mg/kg sugammadex to antagonize deep neuromuscular blockade and rapid recovery has been reported. Furthermore, DMD patients are at risk of developing malignant hyperpyrexia with general/inhaled anesthesia, and dantrolene is often used for effective rescue. In addition, general anesthesia and central neuraxial blockade in patients with severe DMD are unsafe because respiratory depression and myocardial complications may occur after the administration of volatile anesthetics and depolarizing muscle relaxants (succinylcholine) during the induction of anesthesia. Conclusions Patients with DMD are more sensitive to NMBAs with delayed onset times and prolonged recovery times. Precautions for DMD patients should include quantitative neuromuscular monitoring, electrocardiographic monitoring and rapid airway protection throughout anesthesia. Compared with general anesthesia, regional anesthesia may be a relatively safe option.
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Affiliation(s)
- Xianghong Lian
- Department of Pharmacy, West China Second University Hospital, Sichuan University, Chengdu, China
- Evidence-Based Pharmacy Center, West China Second University Hospital, Sichuan University, Chengdu, China
- Key Laboratory of Birth Defects and Related Diseases of Women and Children, Sichuan University, Ministry of Education, Chengdu, China
| | - Yang Jing
- Department of Pharmacy, West China Second University Hospital, Sichuan University, Chengdu, China
- Evidence-Based Pharmacy Center, West China Second University Hospital, Sichuan University, Chengdu, China
- Key Laboratory of Birth Defects and Related Diseases of Women and Children, Sichuan University, Ministry of Education, Chengdu, China
| | - Ting Luo
- Department of Pharmacy, West China Second University Hospital, Sichuan University, Chengdu, China
- Evidence-Based Pharmacy Center, West China Second University Hospital, Sichuan University, Chengdu, China
- Key Laboratory of Birth Defects and Related Diseases of Women and Children, Sichuan University, Ministry of Education, Chengdu, China
| | - Yixin Guo
- Department of Pharmacy, West China Second University Hospital, Sichuan University, Chengdu, China
- Evidence-Based Pharmacy Center, West China Second University Hospital, Sichuan University, Chengdu, China
- Key Laboratory of Birth Defects and Related Diseases of Women and Children, Sichuan University, Ministry of Education, Chengdu, China
| | - Yunzhu Lin
- Department of Pharmacy, West China Second University Hospital, Sichuan University, Chengdu, China
- Evidence-Based Pharmacy Center, West China Second University Hospital, Sichuan University, Chengdu, China
- Key Laboratory of Birth Defects and Related Diseases of Women and Children, Sichuan University, Ministry of Education, Chengdu, China
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Peng L, Wei W. Anesthesia management in a pediatric patient with Becker muscular dystrophy undergoing laparoscopic surgery: A case report. World J Clin Cases 2021; 9:8852-8857. [PMID: 34734066 PMCID: PMC8546828 DOI: 10.12998/wjcc.v9.i29.8852] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/18/2021] [Revised: 06/22/2021] [Accepted: 08/09/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Patients with Becker muscular dystrophy (BMD) have a high risk of developing hyperkalemia, rhabdomyolysis, and malignant hyperthermia when exposed to volatile anesthetics and depolarizing muscle relaxants. Patients with BMD are also prone to respiratory depression after general anesthesia. Thus, it is extremely challenging for anesthesiologists to manage anesthesia in BMD patients, particularly in pediatric BMD patients. Here, we present successful anesthesia management using transversus abdominis plane block (TAPB) combined with total intravenous anesthesia (TIVA) in a pediatric BMD patient undergoing laparoscopic inguinal hernia repair.
CASE SUMMARY A 2-year-old boy, weighing 15 kg, with BMD, was scheduled for laparoscopic inguinal hernia repair. TIVA was used for induction, and continuous infusions of short-acting intravenous anesthetics combined with TAPB were performed for anesthesia maintenance. Moreover, TAPB provided good postoperative analgesia. The patient underwent uneventful surgery and anesthesia, and over the 17 mo follow-up period showed no anesthesia-induced complications.
CONCLUSION TAPB combined with TIVA, using short-acting intravenous anesthetic agents, can provide safe and effective anesthesia management in pediatric BMD patients undergoing short-term abdominal surgery.
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Affiliation(s)
- Ling Peng
- Department of Anaesthesiology, West China Hospital of Sichuan University, Chengdu 610041, Sichuan Province, China
| | - Wei Wei
- Department of Anaesthesiology, West China Hospital of Sichuan University, Chengdu 610041, Sichuan Province, China
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Zhou SY, Wang D, Liu C, Zhang S, Shan BL, Ma HC. Laparoscopic gynecological surgery in an adult woman with Becker muscular dystrophy performed with sevoflurane with cisatracurium anesthesia: A case report. Medicine (Baltimore) 2020; 99:e19733. [PMID: 32311965 PMCID: PMC7220258 DOI: 10.1097/md.0000000000019733] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/28/2022] Open
Abstract
RATIONALE Becker muscular dystrophy (BMD) and Duchenne muscular dystrophy (DMD) are progressive neuromuscular disorders caused by mutations in the dystrophin gene. The management of anesthesia in patients with BMD is complicated because they are highly sensitive to the conventional anesthetics such as volatile anesthetics and muscle relaxants. It is reported that anesthesia in patients with DMD is associated with several complications. However, a few case reports have been published on adult patients with BMD undergoing surgery with general anesthesia. Reports indicate that children with BMD may experience some serious complications with flurane-inhaled anesthesia. However, no study has yet shown that the use of flurane-induced anesthesia in adults with DMD carries high risks. PATIENT CONCERNS We describe a 56-year-old woman with BMD who was scheduled for laparoscopic hysterectomy and bilateral adnexectomy under general anesthesia due to a mass in the uterus. The patient was diagnosed with BMD 20 years back and reported that during this period, she was able to walk slowly with help during her daily life. Additionally, she also had a history of hypertension since 4 years and type 2 diabetes mellitus since 2 years. DIAGNOSIS The patient was postmenopausal and presented with abnormal uterine bleeding and elevated CA125. Abdominal ultrasonography revealed diffuse enlargement of the uterus and hypoechoic internal echoes. These findings were suggestive of diffuse adenomyosis with multiple uterine leiomyomas, which would have adverse effects later in her life. Therefore, the patient required surgery to address the symptoms and further confirm the diagnosis. The final diagnosis was confirmed by histopathological analysis. INTERVENTIONS The patient was scheduled for laparoscopic hysterectomy and bilateral adnexectomy. Anesthesia was induced and maintained by a combination of intravenous and inhalation anesthetic agents, particularly cisatracurium besilate and inhaled. sevoflurane. OUTCOMES The duration of anesthesia and postoperative period were uneventful. At the end of the operation, the patient had normal vital signs and was fully conscious. The patient was followed up for 8 months and no complications were noted during this period. LESSONS The combination of sevoflurane and cisatracurium besilate is a safe and effective method for the anesthetic management of adult patients with BMD scheduled for laparoscopic gynecological surgery. On the other hand, it is important to be aware of even rare complications of procedures, so that necessary precautions can be undertaken. Further investigations are necessary to determine the safe dosage of volatile anesthetics specifically for this clinical scenario so that anesthesiologists can use this combination method more accurately and precisely.
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Affiliation(s)
| | | | | | - Shi Zhang
- Department of Orthopaedic Surgery, The First Hospital of Jilin University, Changchun, Jilin, China
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