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先天性多发性关节挛缩症成年患者腹股沟疝手术的麻醉管理:一例报告

Anesthetic Management of Inguinal Hernia Surgery in an Adult Patient With Arthrogryposis Multiplex Congenita: A Case Report.

作者信息

Asimakopoulos Thalis, Prodromakis Panagiotis, Kouroukli Irene C

机构信息

Department of Anesthesiology and Pain Medicine, Hippocratio General Hospital of Athens, Athens, GRC.

出版信息

Cureus. 2025 Mar 9;17(3):e80300. doi: 10.7759/cureus.80300. eCollection 2025 Mar.

Abstract

Arthrogryposis multiplex congenita (AMC) poses major anesthetic challenges due to severe joint contractures, airway restrictions, and a debated risk of malignant hyperthermia. Despite these complexities, anesthetic reports in adult AMC patients remain extremely limited, leaving perioperative management largely unstandardized. We present the anesthetic approach for a 35-year-old male patient with AMC undergoing open inguinal hernia repair. Given the high risk of airway compromise and joint immobility, we opted for ultrasound-guided ilioinguinal and iliohypogastric nerve blocks with dexmedetomidine sedation, avoiding endotracheal intubation and volatile anesthetics. This case highlights regional anesthesia as a critical alternative to general anesthesia, providing valuable insights into safe, tailored anesthetic strategies for this rare and underreported population.

摘要

先天性多发性关节挛缩症(AMC)因严重的关节挛缩、气道受限以及恶性高热风险存在争议等因素,给麻醉带来了重大挑战。尽管存在这些复杂情况,但关于成年AMC患者的麻醉报告仍然极为有限,使得围手术期管理在很大程度上未标准化。我们介绍了一名35岁患有AMC的男性患者接受开放性腹股沟疝修补术的麻醉方法。鉴于气道受损和关节活动受限的高风险,我们选择了超声引导下的髂腹股沟和髂腹下神经阻滞并给予右美托咪定镇静,避免气管插管和挥发性麻醉剂。该病例突出了区域麻醉作为全身麻醉的关键替代方法,为这一罕见且报道不足的人群提供了关于安全、个性化麻醉策略的宝贵见解。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8583/11977437/19bd6d979cbb/cureus-0017-00000080300-i01.jpg

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本文引用的文献

1
Difficult airway management in children and young adults with arthrogryposis.
World J Otorhinolaryngol Head Neck Surg. 2018 Jul 13;4(2):122-125. doi: 10.1016/j.wjorl.2018.04.003. eCollection 2018 Jun.
2
Anaesthesiology for Children With Arthrogryposis.
J Pediatr Orthop. 2017 Jul/Aug;37 Suppl 1:S27-S28. doi: 10.1097/BPO.0000000000000998.
3
Arthrogryposis multiplex congenita: classification, diagnosis, perioperative care, and anesthesia.
Front Med. 2017 Mar;11(1):48-52. doi: 10.1007/s11684-017-0500-4. Epub 2017 Mar 2.
4
Epidural anesthesia for labor pain and cesarean section in a parturient with arthrogryposis multiplex congenita.
J Anaesthesiol Clin Pharmacol. 2016 Jul-Sep;32(3):410-1. doi: 10.4103/0970-9185.188828.
5
Truncating Mutations of MAGEL2, a Gene within the Prader-Willi Locus, Are Responsible for Severe Arthrogryposis.
Am J Hum Genet. 2015 Oct 1;97(4):616-20. doi: 10.1016/j.ajhg.2015.08.010. Epub 2015 Sep 10.
6
Anesthesia in patients with arthrogryposis multiplex congenita: a report of 10 patients.
Korean J Anesthesiol. 2014 Dec;67(Suppl):S89-90. doi: 10.4097/kjae.2014.67.S.S89.
8
Multiple congenital contractures: birth prevalence, etiology, and outcome.
J Pediatr. 2002 Jan;140(1):61-7. doi: 10.1067/mpd.2002.121148.
9
Lethal congenital arthrogryposis presents with increased nuchal translucency at 10-14 weeks of gestation.
Ultrasound Obstet Gynecol. 1997 May;9(5):310-3. doi: 10.1046/j.1469-0705.1997.09050310.x.

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