Hydrocephalus in mucopolysaccharidosis Type VI successfully treated with endoscopic third ventriculostomy

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dc.contributor.authorSilva Neto, Ângelo Raimundo da
dc.contributor.authorHolanda, Gervina Brady Moreira
dc.contributor.authorFarias, Maria Cláudia Saldanha
dc.contributor.authorCosta, Gladstone Santos da
dc.contributor.authorPereira, Hougelle Simplício Gomes
dc.contributor.authorIDhttps://orcid.org/0000-0002-3819-8951pt_BR
dc.date.accessioned2023-05-12T19:42:16Z
dc.date.available2023-05-12T19:42:16Z
dc.date.issued2013
dc.description.resumoMucopolysaccharidosis (MPS) Type VI, or Maroteaux-Lamy syndrome, is characterized by a deficiency of the enzyme arylsulfatase B (ASB). In patients with this disorder, craniocervical compression, carpal tunnel syndrome, and communicating hydrocephalus are common. Traditionally, hydrocephalus occurring in patients with MPS VI has been treated with shunt placements. Considering obstruction of the outlets from the fourth ventricle at the craniocervical transition, the authors decided to treat a female patient with MPS VI via endoscopic third ventriculostomy. She was 12 years old and had refractory headaches. This seems to be the first reported instance of the neuroendoscopic treatment of hydrocephalus in a patient with MPS VI. The pathophysiology is briefly discussed.pt_BR
dc.identifier.citationSILVA NETO, Ângelo Raimundo da; HOLANDA, Gervina Brady Moreira; FARIAS, Maria Cláudia Saldanha; COSTA, Gladstone Santos da; PEREIRA, Hougelle Simplício Gomes. Hydrocephalus in mucopolysaccharidosis Type VI successfully treated with endoscopic third ventriculostomy. Journal Of Neurosurgery: Pediatrics, [S.L.], v. 11, n. 3, p. 327-330, mar. 2013. Journal of Neurosurgery Publishing Group (JNSPG). http://dx.doi.org/10.3171/2012.11.peds11419.pt_BR
dc.identifier.doi/10.3171/2012.11.PEDS11419
dc.identifier.urihttps://repositorio.ufrn.br/handle/123456789/52354
dc.languagept_BRpt_BR
dc.publisherJournal of Neurosurgery: Pediatricspt_BR
dc.subjectmaroteaux-Lamy syndromept_BR
dc.subjecthydrocephaluspt_BR
dc.subjectneuroendoscopypt_BR
dc.titleHydrocephalus in mucopolysaccharidosis Type VI successfully treated with endoscopic third ventriculostomypt_BR
dc.typearticlept_BR

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