Anesthetic Management of a Pediatric Patient with Leigh Syndrome
Background and objectives: Leigh syndrome (LS) is a rare disease caused by abnormalities of mitochondrial energy generation. The central nervous system is most frequently affected, with psychomotor underdevelopment, seizures, nystagmus, ophthalmoparesis, optic atrophy, ataxia, dystonia, or respirato...
| Main Authors: | , |
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| Format: | Article |
| Language: | English |
| Published: |
Elsevier
2013-03-01
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| Series: | Brazilian Journal of Anesthesiology |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S0104001413000420 |
| _version_ | 1811236716290244608 |
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| author | Ismail Serhat Kocamanoglu Esra Sarihasan |
| author_facet | Ismail Serhat Kocamanoglu Esra Sarihasan |
| author_sort | Ismail Serhat Kocamanoglu |
| collection | DOAJ |
| description | Background and objectives: Leigh syndrome (LS) is a rare disease caused by abnormalities of mitochondrial energy generation. The central nervous system is most frequently affected, with psychomotor underdevelopment, seizures, nystagmus, ophthalmoparesis, optic atrophy, ataxia, dystonia, or respiratory failure. Surgical and anesthetic procedures stimulate the tracheal irritability, and could exacerbate risks of aspiration, wheezing, breathing difficulties, gasping, hypoventilation, and apnea. Case Report: We present the anesthetic management for a six-year-old boy with severe form of LS, involving repair of a femur fracture. Propofol and remifentanil were infused for general anesthesia. The patient was closely monitored during anesthesia and in the intensive care unit in the early postoperative period. Conclusions: Close intraoperative monitoring of patients, including invasive arterial blood pressure monitoring and frequently measuring the levels of blood gases, glucose, and lactate, made this procedure run smoothly. Intensive care and breathing support for the patient with LS, under sedation with an analgesic combination during the early postoperative period, minimized the stress response due to pain after surgery. Keywords: Leigh Disease, Anesthesia, General, Monitoring, Physiologic, Intensive Care |
| first_indexed | 2024-04-12T12:12:48Z |
| format | Article |
| id | doaj.art-cb26d29b1c2947ee9c56c1e961666979 |
| institution | Directory Open Access Journal |
| issn | 0104-0014 |
| language | English |
| last_indexed | 2024-04-12T12:12:48Z |
| publishDate | 2013-03-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Brazilian Journal of Anesthesiology |
| spelling | doaj.art-cb26d29b1c2947ee9c56c1e9616669792022-12-22T03:33:31ZengElsevierBrazilian Journal of Anesthesiology0104-00142013-03-01632220222Anesthetic Management of a Pediatric Patient with Leigh SyndromeIsmail Serhat Kocamanoglu0Esra Sarihasan1MD; Associate Professor, Department of Anesthesia, Ondokuz Mayis School of Medicine, Samsun, Turkey; Corresponding author. Ondokuz Mayis Universitesi, Tip Fakultesi, Anestezi Anabilim Dali. Kurupelit 55139. Samsun, Turkey.MR, Department of Anesthesia, Ondokuz Mayis School of Medicine, Samsun, TurkeyBackground and objectives: Leigh syndrome (LS) is a rare disease caused by abnormalities of mitochondrial energy generation. The central nervous system is most frequently affected, with psychomotor underdevelopment, seizures, nystagmus, ophthalmoparesis, optic atrophy, ataxia, dystonia, or respiratory failure. Surgical and anesthetic procedures stimulate the tracheal irritability, and could exacerbate risks of aspiration, wheezing, breathing difficulties, gasping, hypoventilation, and apnea. Case Report: We present the anesthetic management for a six-year-old boy with severe form of LS, involving repair of a femur fracture. Propofol and remifentanil were infused for general anesthesia. The patient was closely monitored during anesthesia and in the intensive care unit in the early postoperative period. Conclusions: Close intraoperative monitoring of patients, including invasive arterial blood pressure monitoring and frequently measuring the levels of blood gases, glucose, and lactate, made this procedure run smoothly. Intensive care and breathing support for the patient with LS, under sedation with an analgesic combination during the early postoperative period, minimized the stress response due to pain after surgery. Keywords: Leigh Disease, Anesthesia, General, Monitoring, Physiologic, Intensive Carehttp://www.sciencedirect.com/science/article/pii/S0104001413000420 |
| spellingShingle | Ismail Serhat Kocamanoglu Esra Sarihasan Anesthetic Management of a Pediatric Patient with Leigh Syndrome Brazilian Journal of Anesthesiology |
| title | Anesthetic Management of a Pediatric Patient with Leigh Syndrome |
| title_full | Anesthetic Management of a Pediatric Patient with Leigh Syndrome |
| title_fullStr | Anesthetic Management of a Pediatric Patient with Leigh Syndrome |
| title_full_unstemmed | Anesthetic Management of a Pediatric Patient with Leigh Syndrome |
| title_short | Anesthetic Management of a Pediatric Patient with Leigh Syndrome |
| title_sort | anesthetic management of a pediatric patient with leigh syndrome |
| url | http://www.sciencedirect.com/science/article/pii/S0104001413000420 |
| work_keys_str_mv | AT ismailserhatkocamanoglu anestheticmanagementofapediatricpatientwithleighsyndrome AT esrasarihasan anestheticmanagementofapediatricpatientwithleighsyndrome |