{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,24]],"date-time":"2026-06-24T12:00:41Z","timestamp":1782302441270,"version":"3.54.5"},"reference-count":28,"publisher":"MDPI AG","issue":"8","license":[{"start":{"date-parts":[[2022,8,21]],"date-time":"2022-08-21T00:00:00Z","timestamp":1661040000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Ministry of Science and Technology, Executive Yuan, Taiwan","award":["MOST-111-2811-B-195-001"],"award-info":[{"award-number":["MOST-111-2811-B-195-001"]}]},{"name":"Ministry of Science and Technology, Executive Yuan, Taiwan","award":["MOST-111-2811-B-195-002"],"award-info":[{"award-number":["MOST-111-2811-B-195-002"]}]},{"name":"Ministry of Science and Technology, Executive Yuan, Taiwan","award":["MOST-111-2314-B-195-017"],"award-info":[{"award-number":["MOST-111-2314-B-195-017"]}]},{"name":"Ministry of Science and Technology, Executive Yuan, Taiwan","award":["MOST-110-2314-B-195-010-MY3"],"award-info":[{"award-number":["MOST-110-2314-B-195-010-MY3"]}]},{"name":"Ministry of Science and Technology, Executive Yuan, Taiwan","award":["MOST-110-2314-B-195-014"],"award-info":[{"award-number":["MOST-110-2314-B-195-014"]}]},{"name":"Ministry of Science and Technology, Executive Yuan, Taiwan","award":["MOST-110-2314-B-195-029"],"award-info":[{"award-number":["MOST-110-2314-B-195-029"]}]},{"name":"Ministry of Science and Technology, Executive Yuan, Taiwan","award":["MOST-109-2314-B-195-024"],"award-info":[{"award-number":["MOST-109-2314-B-195-024"]}]},{"name":"Ministry of Science and Technology, Executive Yuan, Taiwan","award":["MMH-E-111-13"],"award-info":[{"award-number":["MMH-E-111-13"]}]},{"name":"Ministry of Science and Technology, Executive Yuan, Taiwan","award":["MMH-E-110-16"],"award-info":[{"award-number":["MMH-E-110-16"]}]},{"name":"Ministry of Science and Technology, Executive Yuan, Taiwan","award":["MMH-E-109-16"],"award-info":[{"award-number":["MMH-E-109-16"]}]},{"name":"Ministry of Science and Technology, Executive Yuan, Taiwan","award":["MMH-E-108-16"],"award-info":[{"award-number":["MMH-E-108-16"]}]},{"name":"MacKay Memorial Hospital","award":["MOST-111-2811-B-195-001"],"award-info":[{"award-number":["MOST-111-2811-B-195-001"]}]},{"name":"MacKay Memorial Hospital","award":["MOST-111-2811-B-195-002"],"award-info":[{"award-number":["MOST-111-2811-B-195-002"]}]},{"name":"MacKay Memorial Hospital","award":["MOST-111-2314-B-195-017"],"award-info":[{"award-number":["MOST-111-2314-B-195-017"]}]},{"name":"MacKay Memorial Hospital","award":["MOST-110-2314-B-195-010-MY3"],"award-info":[{"award-number":["MOST-110-2314-B-195-010-MY3"]}]},{"name":"MacKay Memorial Hospital","award":["MOST-110-2314-B-195-014"],"award-info":[{"award-number":["MOST-110-2314-B-195-014"]}]},{"name":"MacKay Memorial Hospital","award":["MOST-110-2314-B-195-029"],"award-info":[{"award-number":["MOST-110-2314-B-195-029"]}]},{"name":"MacKay Memorial Hospital","award":["MOST-109-2314-B-195-024"],"award-info":[{"award-number":["MOST-109-2314-B-195-024"]}]},{"name":"MacKay Memorial Hospital","award":["MMH-E-111-13"],"award-info":[{"award-number":["MMH-E-111-13"]}]},{"name":"MacKay Memorial Hospital","award":["MMH-E-110-16"],"award-info":[{"award-number":["MMH-E-110-16"]}]},{"name":"MacKay Memorial Hospital","award":["MMH-E-109-16"],"award-info":[{"award-number":["MMH-E-109-16"]}]},{"name":"MacKay Memorial Hospital","award":["MMH-E-108-16"],"award-info":[{"award-number":["MMH-E-108-16"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JPM"],"abstract":"<jats:p>Anesthesia for patients with mucopolysaccharidoses (MPS) is quite challenging due to vital systemic dysfunction following progressive accumulation of lysosomal glycosaminoglycans. Previous studies focused on perioperative difficult airway management under general anesthesia but rarely depicted the concern of choosing the size of the endotracheal tube (ETT) as well as neuraxial anesthesia. This study aimed to analyze the overall anesthetic management and related complications for a thorough anesthetic strategy. Within the study period from 2002 to 2021, each record of the anesthetic and perioperative quality assurance\/improvement system for patients with a diagnosis of MPS at MacKay Memorial Hospital was retrospectively reviewed. A total of 51 individuals with 151 anesthesia for 163 interventions were cohort studied, and there were 136 general anesthesia and 15 neuraxial anesthesia. We found that the most common interventions for MPS patients were otolaryngological surgeries (49.6%). Additionally, a secured airway played a marked preference for the most general anesthesia (87.1%). The incidence of difficult intubation was 12.5%. In view of ETT size, a smaller than estimated size was used in MPS type II, III, IV, and VI patients and also in patients who received intubation with multiple attempts. However, a larger than estimated size of ETT was adopted whilst choosing cuffed ones. For neuraxial anesthesia, two failed spinal anesthesia procedures were converted to general anesthesia and 73 percent of the patients received perioperative sedation. In conclusion, through the individualized anesthetic strategy and build-up of an experienced team for airway management, high-quality anesthesia can be ensured in each patient.<\/jats:p>","DOI":"10.3390\/jpm12081343","type":"journal-article","created":{"date-parts":[[2022,8,21]],"date-time":"2022-08-21T23:51:01Z","timestamp":1661125861000},"page":"1343","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["The Anesthetic Strategy for Patients with Mucopolysaccharidoses: A Retrospective Cohort Study"],"prefix":"10.3390","volume":"12","author":[{"given":"Hsuan-Chih","family":"Lao","sequence":"first","affiliation":[{"name":"Department of Anesthesiology, MacKay Memorial Hospital, Taipei 10449, Taiwan"},{"name":"Institute of Brain Science, College of Medicine, National Yang Ming Chiao Tung University, Taipei 11203, Taiwan"},{"name":"Department of Medicine, MacKay Medical College, New Taipei City 25245, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8629-5449","authenticated-orcid":false,"given":"Ying-Chun","family":"Lin","sequence":"additional","affiliation":[{"name":"Department of Anesthesiology, MacKay Memorial Hospital, Taipei 10449, Taiwan"},{"name":"Department of Medicine, MacKay Medical College, New Taipei City 25245, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Muh-Lii","family":"Liang","sequence":"additional","affiliation":[{"name":"Department of Medicine, MacKay Medical College, New Taipei City 25245, Taiwan"},{"name":"Department of Neurosurgery, Mackay Memorial Hospital, Taipei City 104217, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ying-Wei","family":"Yang","sequence":"additional","affiliation":[{"name":"Department of Anesthesiology, MacKay Memorial Hospital, Taipei 10449, Taiwan"},{"name":"Department of Medicine, MacKay Medical College, New Taipei City 25245, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ya-Hsien","family":"Huang","sequence":"additional","affiliation":[{"name":"Department of Anesthesiology, MacKay Memorial Hospital, Taipei 10449, Taiwan"},{"name":"Department of Medicine, MacKay Medical College, New Taipei City 25245, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ying-Lun","family":"Chan","sequence":"additional","affiliation":[{"name":"Department of Anesthesiology, MacKay Memorial Hospital, Taipei 10449, Taiwan"},{"name":"Department of Medicine, MacKay Medical College, New Taipei City 25245, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yung-Wei","family":"Hsu","sequence":"additional","affiliation":[{"name":"Department of Anesthesiology, MacKay Memorial Hospital, Taipei 10449, Taiwan"},{"name":"Department of Medicine, MacKay Medical College, New Taipei City 25245, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shuan-Pei","family":"Lin","sequence":"additional","affiliation":[{"name":"Department of Medicine, MacKay Medical College, New Taipei City 25245, Taiwan"},{"name":"Department of Pediatrics, MacKay Memorial Hospital, Taipei 10449, Taiwan"},{"name":"Department of Medical Research, MacKay Memorial Hospital, Taipei 10449, Taiwan"},{"name":"The Rare Disease Center, MacKay Memorial Hospital, Taipei 10449, Taiwan"},{"name":"Department of Infant and Child Care, National Taipei University of Nursing and Health Sciences, Taipei 11219, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9776-2092","authenticated-orcid":false,"given":"Chih-Kuang","family":"Chuang","sequence":"additional","affiliation":[{"name":"Department of Medical Research, MacKay Memorial Hospital, Taipei 10449, Taiwan"},{"name":"College of Medicine, Fu-Jen Catholic University, Taipei 24205, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2384-7856","authenticated-orcid":false,"given":"Jen-Kun","family":"Cheng","sequence":"additional","affiliation":[{"name":"Department of Anesthesiology, MacKay Memorial Hospital, Taipei 10449, Taiwan"},{"name":"Department of Medicine, MacKay Medical College, New Taipei City 25245, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9619-0990","authenticated-orcid":false,"given":"Hsiang-Yu","family":"Lin","sequence":"additional","affiliation":[{"name":"Department of Medicine, MacKay Medical College, New Taipei City 25245, Taiwan"},{"name":"Department of Pediatrics, MacKay Memorial Hospital, Taipei 10449, Taiwan"},{"name":"Department of Medical Research, MacKay Memorial Hospital, Taipei 10449, Taiwan"},{"name":"The Rare Disease Center, MacKay Memorial Hospital, Taipei 10449, Taiwan"},{"name":"MacKay Junior College of Medicine, Nursing and Management, Taipei 11260, Taiwan"},{"name":"Department of Medical Research, China Medical University Hospital, China Medical University, Taichung 40402, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,8,21]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","unstructured":"Fecarotta, S., Tarallo, A., Damiano, C., Minopoli, N., and Parenti, G. (2020). Pathogenesis of Mucopolysaccharidoses, an Update. Int. J. Mol. Sci., 21.","DOI":"10.3390\/ijms21072515"},{"key":"ref_2","doi-asserted-by":"crossref","unstructured":"Chuang, C.K., Lee, C.L., Tu, R.Y., Lo, Y.T., Sisca, F., Chang, Y.H., Liu, M.Y., Liu, H.Y., Chen, H.J., and Kao, S.M. (2021). Nationwide Newborn Screening Program for Mucopolysaccharidoses in Taiwan and an Update of the \u201cGold Standard\u201d Criteria Required to Make a Confirmatory Diagnosis. Diagnostics, 11.","DOI":"10.3390\/diagnostics11091583"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"314","DOI":"10.1186\/s13023-020-01598-z","article-title":"Survival and diagnostic age of 175 Taiwanese patients with mucopolysaccharidoses (1985\u20132019)","volume":"15","author":"Lin","year":"2020","journal-title":"Orphanet J. Rare Dis."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"1","DOI":"10.17305\/bjbms.2017.2201","article-title":"Anesthesia for patients with mucopolysaccharidoses: Comprehensive review of the literature with emphasis on airway management","volume":"18","author":"Clark","year":"2018","journal-title":"Bosn. J. Basic Med. Sci."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"3373","DOI":"10.7150\/ijms.61827","article-title":"Otorhinolaryngological management in Taiwanese patients with mucopolysaccharidoses","volume":"18","author":"Lee","year":"2021","journal-title":"Int. J. Med. Sci."},{"key":"ref_6","first-page":"306","article-title":"Clinical experience in anesthetic management for children with mucopolysaccharidoses: Report of ten cases","volume":"42","author":"Chan","year":"2001","journal-title":"Acta Paediatr. Taiwanica"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"55","DOI":"10.1002\/ped4.12116","article-title":"Successful spinal anesthesia in a patient with mucopolysaccharidosis type I under femoral fracture reduction and external fixation","volume":"3","author":"Sun","year":"2019","journal-title":"Pediatr. Investig."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"99","DOI":"10.1016\/j.jclinane.2015.08.025","article-title":"Successful use of spinal anesthesia for inguinal hernia repair in a child with Hunter syndrome with difficult airway","volume":"30","author":"Kumar","year":"2016","journal-title":"J. Clin. Anesth."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"1270","DOI":"10.1111\/j.1460-9592.2011.03671.x","article-title":"Caudal anesthesia in Hurler\u2019s syndrome","volume":"21","author":"Yalcin","year":"2011","journal-title":"Pediatr. Anesth."},{"key":"ref_10","first-page":"641","article-title":"Anaestethic problems in Sanfilippo syndrome: A rare case of adult patient","volume":"69","author":"Ingrosso","year":"2003","journal-title":"Minerva Anestesiol."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"242","DOI":"10.1016\/S0952-8180(99)00007-0","article-title":"Anesthetic care for the child with Morquio syndrome: General versus regional anesthesia","volume":"11","author":"Tobias","year":"1999","journal-title":"J. Clin. Anesth."},{"key":"ref_12","first-page":"333","article-title":"Continuous subarachnoid analgesia in two adolescents with severe scoliosis and impaired pulmonary function","volume":"16","author":"Sethna","year":"1991","journal-title":"Reg. Anesth."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"484","DOI":"10.1111\/j.1399-6576.1986.tb02459.x","article-title":"Anaesthetic problems in Hurler-Scheie syndrome. Report of two cases","volume":"30","author":"Pedersen","year":"1986","journal-title":"Acta Anaesthesiol. Scand."},{"key":"ref_14","first-page":"846","article-title":"[Anesthesia for three patients with Hunter syndrome]","volume":"60","author":"Kodani","year":"2011","journal-title":"Masui."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"95","DOI":"10.1046\/j.1460-9592.2000.00464.x","article-title":"Failed epidural anaesthesia in a patient with Hurler\u2019s disease","volume":"10","author":"Vas","year":"2000","journal-title":"Pediatr. Anesth."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"45","DOI":"10.1007\/s12630-014-0247-1","article-title":"Paraplegia after epidural-general anesthesia in a Morquio patient with moderate thoracic spinal stenosis","volume":"62","author":"Drummond","year":"2015","journal-title":"Can. J. Anesth."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"1105","DOI":"10.1111\/j.1365-2044.1984.tb08932.x","article-title":"Difficult tracheal intubation in obstetrics","volume":"39","author":"Cormack","year":"1984","journal-title":"Anaesthesia"},{"key":"ref_18","first-page":"672","article-title":"Pediatric formulas for the anesthesiologist","volume":"94","author":"Cole","year":"1957","journal-title":"AMA J. Dis. Child."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"181","DOI":"10.1111\/pan.13787","article-title":"Airway management and perioperative adverse events in children with mucopolysaccharidoses and mucolipidoses: A retrospective cohort study","volume":"30","author":"Dohrmann","year":"2020","journal-title":"Pediatr. Anesth."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"981","DOI":"10.1007\/s12630-017-0906-0","article-title":"Airway management changes in patients with mucopolysaccharidoses: The role of video laryngoscopy","volume":"64","author":"Clark","year":"2017","journal-title":"Can. J. Anesth."},{"key":"ref_21","first-page":"477","article-title":"Securing the airway in children with the Morquio-Brailsford syndrome","volume":"42","author":"Tzanova","year":"1993","journal-title":"Anaesthesist"},{"key":"ref_22","first-page":"284","article-title":"[Estimation of size of the uncuffed endotracheal tube for pediatric cardiac anesthesia\u2014Cole\u2019s formula cannot estimate the appropriate tube size]","volume":"50","author":"Shiroyama","year":"2001","journal-title":"Masui"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"363","DOI":"10.1111\/pan.13075","article-title":"Delivery of anesthesia for children with mucopolysaccharidosis type III (Sanfilippo syndrome): A review of 86 anesthetics","volume":"27","author":"Cohen","year":"2017","journal-title":"Pediatr. Anesth."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"1241","DOI":"10.1111\/pan.13267","article-title":"Role of ultrasound measuring position and ventilation pressure in determining correct tube size in children","volume":"27","author":"Schramm","year":"2017","journal-title":"Pediatr. Anesth."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"819","DOI":"10.1097\/ALN.0b013e3181ef6757","article-title":"Prediction of pediatric endotracheal tube size by ultrasonography","volume":"113","author":"Shibasaki","year":"2010","journal-title":"Anesthesiology"},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"333","DOI":"10.4097\/kja.21114","article-title":"Prediction of endotracheal tube size using a printed three-dimensional airway model in pediatric patients with congenital heart disease: A prospective, single-center, single-group study","volume":"74","author":"Park","year":"2021","journal-title":"Korean J. Anesthesiol."},{"key":"ref_27","first-page":"CD011954","article-title":"Cuffed versus uncuffed endotracheal tubes for general anaesthesia in children aged eight years and under","volume":"11","author":"Andrade","year":"2017","journal-title":"Cochrane Database Syst. Rev."},{"key":"ref_28","unstructured":"Figueir\u00eado, B.B., Reinaux, C., Fuzari, H., Sarmento, A., Fernandes, J., and Andrade, A.D.d. (2022). Chest wall volumes, diaphragmatic mobility, and functional capacity in patients with mucopolysaccharidoses. Disabil. Rehabil., 1\u201310."}],"container-title":["Journal of Personalized Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2075-4426\/12\/8\/1343\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T00:13:08Z","timestamp":1760141588000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2075-4426\/12\/8\/1343"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,8,21]]},"references-count":28,"journal-issue":{"issue":"8","published-online":{"date-parts":[[2022,8]]}},"alternative-id":["jpm12081343"],"URL":"https:\/\/doi.org\/10.3390\/jpm12081343","relation":{},"ISSN":["2075-4426"],"issn-type":[{"value":"2075-4426","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,8,21]]}}}