{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T10:15:20Z","timestamp":1791281720315,"version":"4.1.0"},"reference-count":14,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2017,1,18]],"date-time":"2017-01-18T00:00:00Z","timestamp":1484697600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Anesthesia"],"published-print":{"date-parts":[[2017,4]]},"abstract":"<jats:title>Summary<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Sanfilippo syndrome (<jats:styled-content style=\"fixed-case\">MPS III<\/jats:styled-content>) is rare, with 97 cases in the United Kingdom between 1988 and 1998. Mucopolysaccharide infiltration of tissues in mucopolysaccharidosis (MPS) causes multi\u2010systemic pathology including difficult airways and cardiac disease. Published anesthesia case reviews of Sanfilippo syndrome have included limited numbers of patients to date.<\/jats:p><\/jats:sec><jats:sec><jats:title>Aim<\/jats:title><jats:p>To identify the perioperative management and complications of anesthesia in children with mucopolysaccharidosis Type <jats:styled-content style=\"fixed-case\">III<\/jats:styled-content> at Great Ormond Street Hospital.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A retrospective case note review of all children with <jats:styled-content style=\"fixed-case\">MPS III<\/jats:styled-content> in our institution was undertaken. All medical notes and anesthetic charts were analyzed, and conduct of anesthesia, airway management, perioperative complications, and associated comorbidities were identified.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>There were 43 patients with <jats:styled-content style=\"fixed-case\">MPS III<\/jats:styled-content>, of which 34 required anesthesia, on 86 occasions for 156 procedures between 1993 and 2015. Dental extraction was the likeliest indication for anesthesia (34%) (general surgery [30%]; ear, nose, and throat [26%]; other [10%]). Thirteen of 34 patients had cardiac pathology (valvular [<jats:italic>n<\/jats:italic> = 6], functional [<jats:italic>n<\/jats:italic> = 6], electrophysiological [<jats:italic>n<\/jats:italic> = 1]). Ten of 34 patients had evidence of clotting abnormality (mild prolonged clotting time [<jats:italic>n<\/jats:italic> = 5], low von Willebrand factor [<jats:italic>n<\/jats:italic> = 2], thrombocytopenia [<jats:italic>n<\/jats:italic> = 3]). The majority of intubations were Cormack\u2013Lehane Grade 1 (<jats:italic>n<\/jats:italic> = 47) (Grade 2 [<jats:italic>n<\/jats:italic> = 14], Grade 3 [<jats:italic>n<\/jats:italic> = 1], Grade 4 [<jats:italic>n<\/jats:italic> = 1]). In 86 anesthetics, there were 0 cases of difficulty with mask ventilation. There was 1 case of failed intubation. They were subsequently anesthetized by a different operator uneventfully at a later date. Two perioperative complications occurred: a failed intubation and bleeding during adenoidectomy.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>We demonstrate a difficult airway is unlikely when anesthetizing an <jats:styled-content style=\"fixed-case\">MPS III<\/jats:styled-content> patient although a risk does remain. A significant proportion of <jats:styled-content style=\"fixed-case\">MPS III<\/jats:styled-content> have cardiac involvement although no perioperative complications were described. With associated coagulation issues, bleeding tendency, while uncommon, can occur in this group.<\/jats:p><\/jats:sec>","DOI":"10.1111\/pan.13075","type":"journal-article","created":{"date-parts":[[2017,1,18]],"date-time":"2017-01-18T10:42:22Z","timestamp":1484736142000},"page":"363-369","source":"Crossref","is-referenced-by-count":18,"title":["Delivery of anesthesia for children with Mucopolysaccharidosis Type <scp>III<\/scp> (Sanfilippo syndrome): a review of 86 anesthetics"],"prefix":"10.1111","volume":"27","author":[{"given":"Marc A.","family":"Cohen","sequence":"first","affiliation":[{"name":"Department of Anaesthesia Great Ormond Street Hospital NHS Foundation Trust London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Grant M.","family":"Stuart","sequence":"additional","affiliation":[{"name":"Department of Anaesthesia Great Ormond Street Hospital NHS Foundation Trust London UK"},{"name":"Portex Department of Paediatric Anaesthesia UCL Great Ormond Street Institute of Child Health University College London London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2017,1,18]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-008-0838-5"},{"key":"e_1_2_9_3_1","unstructured":"MPS III \u2013 Sanfilippo. Available at:http:\/\/www.mpssociety.org.uk\/en\/diseases\/mps-diseases\/mps-iii\/. Accessed 29 February 2016."},{"key":"e_1_2_9_4_1","unstructured":"Phenotypic series \u2013 Mucopolysaccharidosis Online Mendelian Inheritance in Man. Available at:http:\/\/www.omim.org\/phenotypicSeries\/PS607014. Accessed 29 February 2016."},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/pan.12876"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(63)80279-6"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1460-9592.2012.03825.x"},{"key":"e_1_2_9_8_1","doi-asserted-by":"crossref","first-page":"198","DOI":"10.1177\/0310057X8301100303","article-title":"Anaesthetic implications of the mucopolysaccharidoses: a fifteen\u2010 year experience in a children's hospital","volume":"11","author":"Baines D","year":"1983","journal-title":"Anaesth Intens Care"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF03010548"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2044.1994.tb04360.x"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1177\/0310057X9602400408"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1460-9592.1992.tb00201.x"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.2169\/internalmedicine.49.4210"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.3174\/ajnr.A2832"},{"key":"e_1_2_9_15_1","doi-asserted-by":"crossref","first-page":"86","DOI":"10.1177\/0310057X8901700117","article-title":"A patient with Sanfilippo syndrome and pseaudocholinesterase deficiency, further complicated by post\u2010tonsillectomy haemorrhage","volume":"17","author":"Myles PS","year":"1989","journal-title":"Anaesth Intes Care"}],"container-title":["Pediatric Anesthesia"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fpan.13075","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/pan.13075","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,2]],"date-time":"2023-10-02T03:20:16Z","timestamp":1696216816000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/pan.13075"}},"subtitle":[],"editor":[{"given":"Francis","family":"Veyckemans","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"editor"}]}],"short-title":[],"issued":{"date-parts":[[2017,1,18]]},"references-count":14,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2017,4]]}},"alternative-id":["10.1111\/pan.13075"],"URL":"https:\/\/doi.org\/10.1111\/pan.13075","archive":["Portico"],"relation":{},"ISSN":["1155-5645","1460-9592"],"issn-type":[{"value":"1155-5645","type":"print"},{"value":"1460-9592","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017,1,18]]}}}