Total Intravenous Anesthesia (TIVA) in an Infant with Werdnig-Hoffmann Disease. Case Report

Resende, Marco Antonio Cardoso de; Silva, Elizabeth Vaz da; Nascimento, Osvaldo J. M.; Gemal, Alberto Esteves; Quintanilha, Giseli; Vasconcelos, Eliana Maria

doi:10.1016/s0034-7094(10)70022-2

Cited by 5 publications

(5 citation statements)

References 21 publications

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“…22 Total intravenous anesthesia with propofol for open gastrostomy in a patient with Werdnig-Hoffmann disease was safe. 23 Several other patients with nonmitochondrial disorder neuromuscular disorder received propofol without major adverse reactions.…”

Section: Resultsmentioning

confidence: 99%

Propofol Is Mitochondrion-Toxic and May Unmask a Mitochondrial Disorder

Finsterer

Frank

2016

J Child Neurol

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There are indications that preexisting mitochondrial disorders or beta-oxidation defects predispose for propofol infusion syndrome. This review aimed at investigating if propofol infusion syndrome occurs exclusively in patients with mitochondrial disorder and if propofol can unmask a mitochondrial disorder. Propofol infusion syndrome has been reported in genetically confirmed mitochondrial disorder patients. In addition, muscle biopsy of patients with propofol infusion syndrome revealed complex IV or complex II deficiency. In animal studies propofol disrupted the electron flow along the respiratory chain and decreased complex I, complex II, and complex III of the respiratory chain. In addition, propofol disrupted the permeability transition pore and reduced the mitochondrial membrane potential. In conclusion, propofol is mitochondrion-toxic and mitochondrial disorder patients should not receive propofol in high dosages over a prolonged period of time. Short-term application of propofol should be safe even in mitochondrial disorder patients. Not only does propofol infusion syndrome occur in mitochondrial disorder patients, but mitochondrial disorder patients are likely at higher risk to develop propofol infusion syndrome. Patients who develop propofol infusion syndrome should be screened for mitochondrial disorder. Propofol infusion syndrome is preventable if risk factors are thoroughly assessed, and if long-term propofol is avoided in patients at risk for propofol infusion syndrome.

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Section: Resultsmentioning

confidence: 99%

Propofol Is Mitochondrion-Toxic and May Unmask a Mitochondrial Disorder

Finsterer

Frank

2016

J Child Neurol

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“…The different opinions exist in the literature about the effectiveness of ETV in children under 1 year age [7-10]. There seems to be growing evidence that the success of ETV depends mainly on the etiology of the hydrocephalus and not on the age of the patient alone [2, 10-15]. The largest study of ETV success in young children was conducted in Uganda and involved 153 children younger than 1 year [16].…”

Section: Discussionmentioning

confidence: 99%

“…Its use in children younger than 1 year of age still remains controversial. The different opinions exist in the literature about the effectiveness of ETV in children under 1 year age [7][8][9][10].…”

Section: Discussionmentioning

confidence: 99%

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Endoscopic Third Ventriculostomy in Infants Less than One Year of Age: A Short Series of 14 Cases

et al. 2021

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Background: Endoscopic third ventriculostomy (ETV) is currently considered as an alternative to cerebrospinal fluid (CSF) shunt systems in the treatment of obstructive hydrocephalus. This procedure allows the CSF to drain in the basal cisterns and reabsorbed by arachnoid granulations, and avoiding implantation of exogenous material. Aims and Objectives: The purpose of this study was to assess the success rate of ETV in infants less than 1 year of age with congenital noncommunicating hydrocephalus. Material and Methods: This study was a 2-year prospective study from August 2017 to July 2019. ETVs were performed in 14 patients younger than 1 year with diagnosis of noncommunicating hydrocephalous. A failure was defined as the need for shunt implantation after ETV. Phase-contrast MRI of the brain was done after 6 months to see patency of ETV fenestration and CSF flow through ventriculostomy. Results: ETV was tried in 18 patients and successfully performed in 14 patients. Out of the 14 patients, shunt implantation after ETV was performed in 3 patients (failed ETV). In the successful cases, etiology was idiopathic aqueductal stenosis in 8, shunt complications in 2, and 1 case was a follow-up case of occipital encephalocele; the mean age was 7.7 months (range 3–12). In the 3 failed cases, etiology was aqueductal stenosis, mean age was 7.6 months (range 3–11). In all ETVs, failed patients MPVP shunting was done. Follow-up of nonshunted patients was done from 6 to 24 months (mean 15 months). There was no mortality or permanent morbidity noted following ETV. Conclusion: ETV is a good surgical procedure for less than 1-year-old children.

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“…The main issue related to ETV is whether if it is a safer and better treatment for pediatric patients with hydrocephalus as compared to VPS/VAS. [4][5][6] Although different opinions exist in the literature about the effectiveness of ETV in children under 1-year age, [7][8][9][10] the question whether infants and very young children have a higher risk of treatment failure after ETV than older children is still being debated. There seems to be growing evidence that the success of ETV depends mainly on the etiology of the hydrocephalus and not on the age of the patient alone.…”

Section: Discussionmentioning

confidence: 99%

Endoscopic third ventriculostomy in noncommunicating hydrocephalus: Report on a short series of 53 children

et al. 2019

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Introduction:Endoscopoic third ventriculostomy (ETV) is currently considered the best alternative to cerebrospinal fluid (CSF) shunt systems in the treatment of obstructive hydrocephalus. The aim of ETV is to communicate the third ventricle with the interpendicular cistern and create CSF flow which bypasses an obstruction to the circulation of the CSF.Aims and Objectives:The purpose of this study was to elucidate the indications, efficacy, safety and outcome Of ETV pediatric patients of noncommunicating hydrocephalus.Material and Methods:This study is a 3 year prospective study from June 2012 to May 2015. Records were kept for age, gender, etilogical factors, symptoms, signs, previous use of shunt or external ventricular device, imaging findings, and surgical complications (intraoperative and postoperative). Only those patients with age between 6months and 18 years with symptoms of intracranial hypertension and radiographic evidence of noncommunicating hydrocephalus were included in the study.Results:A total of 53 patients were studied, out of these 29 were boys and 24 were girls. The mean age of the patients was 6.6 years. Overall a total of 50 successful ETVs were done in 53 patients. The success rate is estimated to be 94%. There was no mortality. The average postoperative hospital stay was 4 days. The followup ranged from 6 to 16 months (mean, 12 months).Conclusion:ETV in children is a safe, simple and effective treatment and a logical alternative to shunting procedure for patients of noncommunicating hydrocephalus.

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Total Intravenous Anesthesia (TIVA) in an Infant with Werdnig-Hoffmann Disease. Case Report

Abstract: [Rev Bras Anestesiol 2010;60(5): 563-564] ©Elsevier Editora Ltda.

Cited by 5 publications

References 21 publications

Propofol Is Mitochondrion-Toxic and May Unmask a Mitochondrial Disorder

Propofol Is Mitochondrion-Toxic and May Unmask a Mitochondrial Disorder

Endoscopic Third Ventriculostomy in Infants Less than One Year of Age: A Short Series of 14 Cases

Endoscopic third ventriculostomy in noncommunicating hydrocephalus: Report on a short series of 53 children

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