Leigh syndrome: Anesthetic management in complicated endoscopic procedures

Gozal, David; Goldin, Eran; Shafran-Tikva, Sigal; Tal, Dalia; Wengrower, Dov

doi:10.1111/j.1460-9592.2005.01678.x

Cited by 13 publications

(10 citation statements)

References 29 publications

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“…During radiological examinations, focal, bilateral, symmetric, spongiform lesions, particularly those localized to the brainstem and thalamus, can be detected (1). Clinical findings of Leigh syndrome can be observed in different clinical pictures according to the enzyme deficiency.…”

Section: Discussionmentioning

confidence: 99%

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Leigh Sendromlu Hastada Anestezi Yönetimi

Tütüncü¹,

Kendigelen²,

Kaya³

2017

Bezmialem Science

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Leigh 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.

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Section: Discussionmentioning

confidence: 99%

“…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).…”

Section: Introductionmentioning

confidence: 99%

Leigh Sendromlu Hastada Anestezi Yönetimi

Tütüncü¹,

Kendigelen²,

Kaya³

2017

Bezmialem Science

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“…There are many reports of "propofol infusion syndrome" in children, indicating that this syndrome is not common with usual doses, but does occur when propofol is administered by infusion for more than 48 hours, and at a dose of more than 4.5 mg.kg -1 .h -1 . Most patients on short-term infusions show no clinical signs of mitochondrial disturbance 8 . Since succinylcholine has the risk of exaggerated rhabdomyolysis and hyperkalemia, it is not preferred in patients with myopathy.…”

Section: Discussionmentioning

confidence: 99%

“…Preoperative laboratory evaluation revealed; hemoglobin 12.4 g.dL -1 (13-17), hematocrit 37.9% (40-50), a white cell count of 8,800.µL -1 (4,300-10,300), and a platelet count of 265,000.µL -1 (156,000-363,000). Glucose was 89 mg.dL -1 (70-110) and his serum lactate was 15 mg.dL -1 (4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20). He had been fasting for six hours before surgery, in order to prevent aspiration.…”

Section: Case Reportmentioning

confidence: 99%

Abordagem anestésica em paciente pediátrico com síndrome de Leigh

Kocamanoğlu¹,

Sarihasan²

2013

Rev. Bras. Anestesiol.

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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 diffi culties, 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.

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“…Acquired Leigh's disease as a result of spontaneous mutation also happens [3]. Reports of mitochondrial myopathies with cardiac involvement remain sparse [4].…”

Section: Introductionmentioning

confidence: 99%

A Case of Mitochondrial Myopathy with Cardiac Involvement: Management in the Same Day Surgery Setting

Pisklakov¹

2012

J Anesthe Clinic Res

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Leigh syndrome: Anesthetic management in complicated endoscopic procedures

Cited by 13 publications

References 29 publications

Leigh Sendromlu Hastada Anestezi Yönetimi

Leigh Sendromlu Hastada Anestezi Yönetimi

Abordagem anestésica em paciente pediátrico com síndrome de Leigh

A Case of Mitochondrial Myopathy with Cardiac Involvement: Management in the Same Day Surgery Setting

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