Anesthetic management in children with Hurler′s syndrome undergoing emergency ventriculoperitoneal shunt surgery
Mucopolysaccharidoses (MPSs) are a group of lysosomal storage disorder characterized by progressive multiorgan accumulation of glycosaminoglycans. Patients with MPS I (Hurler′s syndrome) present as one of the most difficult airway problems to be managed by anesthesiologists. Hydrocephalus with incre...
| Main Authors: | , , , , |
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| Format: | Article |
| Language: | English |
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Wolters Kluwer Medknow Publications
2012-01-01
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| Series: | Saudi Journal of Anaesthesia |
| Subjects: | |
| Online Access: | http://www.saudija.org/article.asp?issn=1658-354X;year=2012;volume=6;issue=2;spage=178;epage=180;aulast=Gupta |
| _version_ | 1811306083558359040 |
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| author | Nidhi Gupta Girija Prasad Rath Renu Bala Bapura Kiran Reddy Arvind Chaturvedi |
| author_facet | Nidhi Gupta Girija Prasad Rath Renu Bala Bapura Kiran Reddy Arvind Chaturvedi |
| author_sort | Nidhi Gupta |
| collection | DOAJ |
| description | Mucopolysaccharidoses (MPSs) are a group of lysosomal storage disorder characterized by progressive multiorgan accumulation of glycosaminoglycans. Patients with MPS I (Hurler′s syndrome) present as one of the most difficult airway problems to be managed by anesthesiologists. Hydrocephalus with increased intracranial pressure is a frequent complication seen in these patients. The rarity of the syndrome accounts for the small number of case reports and anecdotal information on the intracranial manifestations of this syndrome. We describe the successful anesthetic management of 2 children with Hurler′s syndrome who underwent ventriculoperitoneal shunt surgery for acute hydrocephalus. |
| first_indexed | 2024-04-13T08:38:03Z |
| format | Article |
| id | doaj.art-a1a1f5084a2b442a9ce09389c8679de8 |
| institution | Directory Open Access Journal |
| issn | 1658-354X |
| language | English |
| last_indexed | 2024-04-13T08:38:03Z |
| publishDate | 2012-01-01 |
| publisher | Wolters Kluwer Medknow Publications |
| record_format | Article |
| series | Saudi Journal of Anaesthesia |
| spelling | doaj.art-a1a1f5084a2b442a9ce09389c8679de82022-12-22T02:54:02ZengWolters Kluwer Medknow PublicationsSaudi Journal of Anaesthesia1658-354X2012-01-016217818010.4103/1658-354X.97036Anesthetic management in children with Hurler′s syndrome undergoing emergency ventriculoperitoneal shunt surgeryNidhi GuptaGirija Prasad RathRenu BalaBapura Kiran ReddyArvind ChaturvediMucopolysaccharidoses (MPSs) are a group of lysosomal storage disorder characterized by progressive multiorgan accumulation of glycosaminoglycans. Patients with MPS I (Hurler′s syndrome) present as one of the most difficult airway problems to be managed by anesthesiologists. Hydrocephalus with increased intracranial pressure is a frequent complication seen in these patients. The rarity of the syndrome accounts for the small number of case reports and anecdotal information on the intracranial manifestations of this syndrome. We describe the successful anesthetic management of 2 children with Hurler′s syndrome who underwent ventriculoperitoneal shunt surgery for acute hydrocephalus.http://www.saudija.org/article.asp?issn=1658-354X;year=2012;volume=6;issue=2;spage=178;epage=180;aulast=GuptaAirwayanesthetic managementHurler′s syndromehydrocephalusmucopolysaccharidosis |
| spellingShingle | Nidhi Gupta Girija Prasad Rath Renu Bala Bapura Kiran Reddy Arvind Chaturvedi Anesthetic management in children with Hurler′s syndrome undergoing emergency ventriculoperitoneal shunt surgery Saudi Journal of Anaesthesia Airway anesthetic management Hurler′s syndrome hydrocephalus mucopolysaccharidosis |
| title | Anesthetic management in children with Hurler′s syndrome undergoing emergency ventriculoperitoneal shunt surgery |
| title_full | Anesthetic management in children with Hurler′s syndrome undergoing emergency ventriculoperitoneal shunt surgery |
| title_fullStr | Anesthetic management in children with Hurler′s syndrome undergoing emergency ventriculoperitoneal shunt surgery |
| title_full_unstemmed | Anesthetic management in children with Hurler′s syndrome undergoing emergency ventriculoperitoneal shunt surgery |
| title_short | Anesthetic management in children with Hurler′s syndrome undergoing emergency ventriculoperitoneal shunt surgery |
| title_sort | anesthetic management in children with hurler s syndrome undergoing emergency ventriculoperitoneal shunt surgery |
| topic | Airway anesthetic management Hurler′s syndrome hydrocephalus mucopolysaccharidosis |
| url | http://www.saudija.org/article.asp?issn=1658-354X;year=2012;volume=6;issue=2;spage=178;epage=180;aulast=Gupta |
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