[{"data":1,"prerenderedAt":-1},["ShallowReactive",2],{"doc-seo-639066-105":3,"detail-sidebar-cat-0-en-105":81,"doc-detail-639066-en":130},{"code":4,"msg":5,"data":6},0,"ok",{"site_id":7,"language":8,"slug":9,"title":10,"keywords":11,"description":12,"schema_data":13,"social_meta":74,"head_meta":76,"extra_data":78,"updated_unix":80},105,"en","anesthetic-management-of-leigh-syndrome-case-report","Anesthetic Management of Leigh Syndrome - Case Report","","Leigh syndrome is an extremely rare infant and childhood neurodegenerative disorder marked by subacute necrotizing encephalomyelopathy and progressive clinical decline, often presenting with developmental delay, seizures, dysarthria, ataxia, and myopathy. A pediatric patient required general anesthesia for percutaneous endoscopic gastrostomy, highlighting the need for meticulous evaluation of respiratory, cardiac, and renal function during the perioperative period. Long-acting opioids and neuromuscular agents may worsen postoperative respiratory complications, and preoperative dextrose-containing fluids are recommended to mitigate perioperative lactic acidosis risk.",{"@graph":14,"@context":73},[15,34,56],{"@type":16,"itemListElement":17},"BreadcrumbList",[18,23,27,31],{"item":19,"name":20,"@type":21,"position":22},"https://docshare.wps.com","Home","ListItem",1,{"item":24,"name":25,"@type":21,"position":26},"https://docshare.wps.com/document/","Document",2,{"item":28,"name":29,"@type":21,"position":30},"https://docshare.wps.com/document/healthcare/","Healthcare",3,{"item":32,"name":10,"@type":21,"position":33},"https://docshare.wps.com/document/anesthetic-management-of-leigh-syndrome-case-report/639066/",4,{"url":32,"name":10,"@type":35,"image":36,"author":41,"headline":10,"publisher":44,"fileFormat":47,"inLanguage":8,"description":12,"dateModified":48,"datePublished":49,"encodingFormat":47,"isAccessibleForFree":50,"interactionStatistic":51},"DigitalDocument",{"url":37,"@type":38,"width":39,"height":40},"https://docshare.wps.com/thumbnails/anesthetic-management-of-leigh-syndrome-case-report/639066.png","ImageObject",300,407,{"name":42,"@type":43},"Genevieve","Person",{"url":19,"name":45,"@type":46},"DocShare","Organization","application/pdf","2026-11-12","2026-10-22",true,{"@type":52,"interactionType":53,"userInteractionCount":55},"InteractionCounter",{"@type":54},"ViewAction",5,{"@type":57,"mainEntity":58},"FAQPage",[59,65,69],{"name":60,"@type":61,"acceptedAnswer":62},"What key problems define Leigh syndrome relevant to anesthesia?","Question",{"text":63,"@type":64},"Leigh syndrome involves progressive neurodegeneration with subacute necrotizing encephalomyelopathy and commonly shows developmental delay, seizures, dysarthria, ataxia, and myopathy. It also requires attention to mitochondrial energy defects that can lead to biochemical issues perioperatively.","Answer",{"name":66,"@type":61,"acceptedAnswer":67},"Why is careful evaluation of organ functions important before anesthesia induction?",{"text":68,"@type":64},"Respiratory, cardiac, and renal functions should be carefully assessed in the perioperative period. Increased airway reactivity and existing hypotonia/spasticity can increase the risk of airway and neuromuscular complications.",{"name":70,"@type":61,"acceptedAnswer":71},"How can perioperative management reduce respiratory complications and metabolic risk?",{"text":72,"@type":64},"The case discusses that long-acting opioids and neuromuscular agents can increase postoperative respiratory complications related to neuromuscular myopathy. Because lactic acidosis can develop perioperatively, preoperative fluids with dextrose are recommended.","https://schema.org",{"og:url":32,"og:type":75,"og:title":10,"og:site_name":45,"og:description":12},"article",{"robots":77,"canonical":32},"index,follow",{"doc_id":79,"site_id":7},639066,1793142217,{"code":4,"msg":82,"data":83},"success",[84,88,92,96,100,105,109,114,119,122,126],{"id":22,"doc_module":4,"doc_module_name":25,"category_name":85,"show_sort_weight":86,"slug":87},"Story & Novel",90,"story-novel",{"id":26,"doc_module":4,"doc_module_name":25,"category_name":89,"show_sort_weight":90,"slug":91},"Literature",80,"literature",{"id":33,"doc_module":4,"doc_module_name":25,"category_name":93,"show_sort_weight":94,"slug":95},"Exam",70,"exam",{"id":55,"doc_module":4,"doc_module_name":25,"category_name":97,"show_sort_weight":98,"slug":99},"Comic",60,"comic",{"id":101,"doc_module":4,"doc_module_name":25,"category_name":102,"show_sort_weight":103,"slug":104},6,"Technology",50,"technology",{"id":106,"doc_module":4,"doc_module_name":25,"category_name":29,"show_sort_weight":107,"slug":108},7,40,"healthcare",{"id":110,"doc_module":4,"doc_module_name":25,"category_name":111,"show_sort_weight":112,"slug":113},8,"Research & Report",30,"research-report",{"id":115,"doc_module":4,"doc_module_name":25,"category_name":116,"show_sort_weight":117,"slug":118},9,"Religion & Spirituality",20,"religion-spirituality",{"id":117,"doc_module":4,"doc_module_name":25,"category_name":120,"show_sort_weight":117,"slug":121},"World Cup","world-cup",{"id":123,"doc_module":4,"doc_module_name":25,"category_name":124,"show_sort_weight":123,"slug":125},10,"Lifestyle","lifestyle",{"id":127,"doc_module":4,"doc_module_name":25,"category_name":128,"show_sort_weight":55,"slug":129},19,"General","general",{"code":4,"msg":82,"data":131},{"doc_id":79,"user_id":132,"nickname":42,"user_avatar":133,"doc_module":4,"category_id":106,"category_name":29,"doc_title":10,"doc_description":12,"doc_content":134,"file_id":135,"file_url":136,"file_type":137,"file_size":138,"view_count":55,"is_deleted":4,"is_public":22,"is_downloadable":22,"audit_status":22,"page_count":26,"language":139,"language_code":8,"site_id":7,"html_lang":8,"table_of_contents":140,"faqs":141,"seo_title":142,"seo_description":12,"update_tm":143,"read_time":55,"preview_page_count":4},1374391974585,"https://ap-avatar.wpscdn.com/davatar_276721f389ce27ea32af1340a28f341c","Case Report Bezmialem Science 2017; 5: 84-5  \nDOI: 10.14235/bs.2016.855  \nAnesthetic Management of Leigh Syndrome  \nAyşe ÇiğdemTÜTÜNCÜ, Pınar KENDİGELEN, Güner KAYA  \nDepartment of Anesthesiology and Reanimation, İstanbul University Cerrahpaşa School of Medicine, İstanbul, Turkey  \nABSTRACT  \nLeigh syndrome is an extremely rare disorder in infants and children. It is characterized by a progressive neurodegenerative course with subacute necrotizing encephalomyelopathy, and it presents with developmental delay, seizures, dysarthria, ataxia, and myopathy. We present a patient with Leigh syndrome who required general anesthesia for percutaneous endoscopic gastrostomy. The respiratory, cardiac, and renal system functions should be very carefully evaluated in the peroperative period. The use of long-acting opioids and neuromuscular agents increases postoperative respiratory complications related to neuromuscular myopathy. Considering that lactic acidosis can develop in the perioperative period, it is recommended to give pre-perioperative fluids with dextrose.  \nKeywords: Child, anesthesia, Leigh syndrome  \nIntroduction  \nLeigh syndrome is a rare infant and childhood disease. In this disease, there are mitochondrial and nuclear genomic mutations. The disease progresses with neurodegeneration and subacute necrotizing encephalomyelopathy and clinically manifests as growth retardation, convulsion, dysarthria, ataxia, and myopathy. Patients with this disease may require general anesthesia induction for endoscopic interventions, dental problems, and the like (1, 2) .  \nIn this case, anesthesia induction in a child with Leigh syndrome in whom surgery was planned due to percutaneous endoscopic gastrostomy is reported.  \nCase Report  \nMotor and growth retardation was found in a girl who was born mature with a weight of 3600 g, in whom neuromotor development was normal until 1 year of age, and who faced difficulties in eating and walking later. Changes secondary to pyruvate deficiency were observed on performing radiological examinations: mitochondrial complex IV deficiency was found in the laboratory and high levels of lactate and pyruvate were found in the CSF and urine. Surgery was planned due to percutaneous endoscopic gastrostomy for the patient who was 6 years and who weighed 10 kg and who was followed up with a diagnosis of Leigh syndrome.  \nIn the physical examination, it was observed that the patient was conscious but could only provide limited cooperation; she had abdominal type of respiration and hyperventilation, and her heart sounds were normal. The patient was taken to an operating room without sedation before the surgery. Following standard anesthesia monitoring, anesthesia induction was performed with 0.1 mg/kg of midazolam and 2.5 mg/kg of ketamine; it was then observed that her heart rate decreased from 140-160 beats/min to 65 beats/min. Her heart rate increased after atropine administration. The patient was intubated without using neuromuscular blockers, and anesthesia maintenance was provided with 0.1-0.5 mcg/kg/ min ofremifentanil and 2% sevoflurane. The mean arterial pressure was 35-40 mmHg and heart rate was 135-145 beats/  \n84  \nAddress for Correspondence: Pınar KENDİGELEN; Department of Anesthesiology and Reanimation, İstanbul University  \nCerrahpaşa School of Medicine, İstanbul, Turkey E-mail: [pinarken@gmail.com](pinarken@gmail.com)  \n©[Copyright 2017 by Bezmialem Vakif University-Available online at www.bezmialemscience.org](Copyright 2017 by Bezmialem Vakif University-Available online at www.bezmialemscience.org)  \nReceived Accepted  \n: 19.01.2016  \n: 14.03.2016  \nTütüncü et al. Anesthesia for Leigh Syndrome  \nmin when 2% dextrose-isolate mixture was infused during the surgery. The surgery was completed in 45 min without any complications. The patient was extubated after providing adequate spontaneous respiration and consciousness and was sent to a ward after being observed in the post-anesthesia care unit for 2 h","cbCaidETVIFNJKZp","https://ap.wps.com/l/cbCaidETVIFNJKZp","pdf",95879,"English","# Introduction\n# Case Report\n## Preoperative assessment and anesthesia induction\n## Intraoperative management and recovery\n# Discussion","[{\"question\":\"What key problems define Leigh syndrome relevant to anesthesia?\",\"answer\":\"Leigh syndrome involves progressive neurodegeneration with subacute necrotizing encephalomyelopathy and commonly shows developmental delay, seizures, dysarthria, ataxia, and myopathy. It also requires attention to mitochondrial energy defects that can lead to biochemical issues perioperatively.\"},{\"question\":\"Why is careful evaluation of organ functions important before anesthesia induction?\",\"answer\":\"Respiratory, cardiac, and renal functions should be carefully assessed in the perioperative period. Increased airway reactivity and existing hypotonia/spasticity can increase the risk of airway and neuromuscular complications.\"},{\"question\":\"How can perioperative management reduce respiratory complications and metabolic risk?\",\"answer\":\"The case discusses that long-acting opioids and neuromuscular agents can increase postoperative respiratory complications related to neuromuscular myopathy. Because lactic acidosis can develop perioperatively, preoperative fluids with dextrose are recommended.\"}]","Anesthetic Management of Leigh Syndrome - Case Report | PDF",1792701491]