Neurosurgical Treatment for Hydrocephalus, Subdural Hematomas, and Arachnoid Cysts in Glutaric Aciduria Type 1

Jf, Martínez-Lage

doi:10.1055/s-2007-973806

Cited by 13 publications

(3 citation statements)

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“…In 19 out of 20 cases cerebral imaging showed brain abnormalities specific for GA1. MRI and CT of the brain in these cases show widening of insular cisterns (Sylvian fissure/open opercula) in 93 % of patients in the literature and 90 % in this review [ 35 , 53 , 54 ]. These specific findings should therefore always lead to further investigation of GA1 [ 35 ].…”

Section: Discussionmentioning

confidence: 79%

Subdural hematomas: glutaric aciduria type 1 or abusive head trauma? A systematic review

Vester

Bilo

Karst

et al. 2015

Forensic Sci Med Pathol

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PurposeGlutaric aciduria type 1 (GA1) is a rare metabolic disorder of glutaryl-CoA-dehydrogenase enzyme deficiency. Children with GA1 are reported to be predisposed to subdural hematoma (SDH) development due to stretching of cortical veins secondary to cerebral atrophy and expansion of CSF spaces. Therefore, GA1 testing is part of the routine work-up in abusive head trauma (AHT). This systematic review addresses the coexistence of GA1 and SDH and the validity of GA1 in the differential diagnosis of AHT.MethodsA systematic literature review, with language restriction, of papers published before 1 Jan 2015, was performed using Pubmed, PsychINFO, and Embase. Inclusion criteria were reported SDHs, hygromas or effusions in GA1 patients up to 18 years of age. Of 1599 publications, 20 publications were included for analysis.ResultsIn total 20 cases, 14 boys and 6 girls, were included. In eight cases (40 %) a child abuse work-up was performed, which was negative in all cases. Clinical history revealed the presence of trauma in eight cases (40 %). In only one case neuroradiology revealed no abnormalities related to GA1 according to the authors, although on evaluation we could not exclude AHT.ConclusionFrom this systematic review we conclude that SDHs in 19/20 children with GA1 are accompanied by other brain abnormalities specific for GA1. One case with doubtful circumstances was the exception to this rule.

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Section: Discussionmentioning

confidence: 79%

Subdural hematomas: glutaric aciduria type 1 or abusive head trauma? A systematic review

Vester

Bilo

Karst

et al. 2015

Forensic Sci Med Pathol

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“…In these instances, performing a surgical procedure might cause a further worsening of the patients’ clinical condition. Accordingly, it seems crucial to note that patients with GA‐1 found to have hydrocephalus, subdural collections or arachnoid cysts by neuroimaging should be considered for surgery only if it is strictly essential (12). Surgical treatment should be only undertaken if the patients show clinical evidence of raised intracranial pressure or if they have abnormal recordings of intracranial pressure.…”

Section: Discussionmentioning

confidence: 99%

“…Surgical treatment should be only undertaken if the patients show clinical evidence of raised intracranial pressure or if they have abnormal recordings of intracranial pressure. The risks of encephalopathic crises and metabolic decompensation these patients may present during anesthesia, and the surgical and postsurgical periods should not be ignored (12).…”

Section: Discussionmentioning

confidence: 99%

Anesthetic management in two siblings with glutaric aciduria type 1

Hérnández-Palazón

Sánchez-Ródenas

Martínez‐Lage

et al. 2005

Pediatric Anesthesia

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Glutaric aciduria type 1 (GA-1) is an inborn error of metabolism that results from a deficiency of glutaryl-CoA dehydrogenase. This disorder mainly manifests in early childhood and most patients with this condition develop a dystonic-dyskinetic syndrome. We report the anesthetic management of two sisters with GA-1, aged 30 and 17 months respectively at the time of surgery, who presented with macrocephaly and psychomotor delay. The children required CSF shunting procedures for hydrocephalus and subdural fluid collections, which were performed under total intravenous anesthesia with propofol and remifentanil.

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Glutaric Aciduria Type 1

2005

Magnetic Resonance of Myelination and Myelin Disorders

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Neurosurgical Treatment for Hydrocephalus, Subdural Hematomas, and Arachnoid Cysts in Glutaric Aciduria Type 1

Cited by 13 publications

References 0 publications

Subdural hematomas: glutaric aciduria type 1 or abusive head trauma? A systematic review

Subdural hematomas: glutaric aciduria type 1 or abusive head trauma? A systematic review

Anesthetic management in two siblings with glutaric aciduria type 1

Glutaric Aciduria Type 1

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