{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,3]],"date-time":"2026-04-03T09:53:57Z","timestamp":1775210037570,"version":"3.50.1"},"reference-count":41,"publisher":"MDPI AG","issue":"5","license":[{"start":{"date-parts":[[2024,2,28]],"date-time":"2024-02-28T00:00:00Z","timestamp":1709078400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Background: Mucopolysaccharidoses (MPSs) are rare congenital lysosomal storage disorders due to a deficiency of enzymes metabolising glycosaminoglycans, leading to their accumulation in tissues. This multisystem disease often requires surgical intervention, including valvular cardiac surgery. Adult MPSs have complex airways making anaesthesia risky. Methods: We report novel three-dimensional (3D) modelling airway assessments and multidisciplinary peri-operative airway management. Results: Five MPS adults underwent cardiac surgery at the national MPS cardiac centre (type I = 4, type II = 1; ages 20, 24, 33, 35, 37 years; two males, three females). All had complex airway abnormalities. Assessments involved examination, nasendoscopy, imaging, functional studies, 3D reconstruction, virtual endoscopy, virtual reality and simulation using computerised, physical modelling. Awake oral fibre-optic intubation was achieved via airway conduit. Staged extubation was performed on the first post-operative day under laryngo-tracheoscopic guidance. The post-operative period involved chest physiotherapy and occupational therapy. All patients had safe intubation, ventilation and extubation. Four had good cardiac surgical outcomes, one (MPS type I; age 35 years) was inoperable due to endocarditis. None had post-operative airway complications. Conclusions: Expertise from cardiovascular-heart team, multidisciplinary airway management, use of novel techniques is vital. Traditional airway assessments are insufficient, so ENT input, radiology and computerised methods to assess and simulate the airway in 3D by collaboration with clinical engineering is essential.<\/jats:p>","DOI":"10.3390\/jcm13051366","type":"journal-article","created":{"date-parts":[[2024,2,28]],"date-time":"2024-02-28T03:37:57Z","timestamp":1709091477000},"page":"1366","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Airway and Anaesthetic Management of Adult Patients with Mucopolysaccharidoses Undergoing Cardiac Surgery"],"prefix":"10.3390","volume":"13","author":[{"given":"David","family":"Mayhew","sequence":"first","affiliation":[{"name":"Department of Anaesthesia, Liverpool Heart and Chest Hospital, Liverpool L14 3PE, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kenneth","family":"Palmer","sequence":"additional","affiliation":[{"name":"Department of Anaesthesia, Liverpool Heart and Chest Hospital, Liverpool L14 3PE, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ian","family":"Wilson","sequence":"additional","affiliation":[{"name":"Department of Cardiac Surgery, Liverpool Heart and Chest Hospital, Liverpool L14 3PE, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stuart","family":"Watson","sequence":"additional","affiliation":[{"name":"Medical Physics Department, Salford Care Organisation, Northern Care Alliance NHS Foundation Trust, Manchester M6 8HD, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0148-6332","authenticated-orcid":false,"given":"Karolina M.","family":"Stepien","sequence":"additional","affiliation":[{"name":"Adult Inherited Metabolic Department, Salford Care Organisation, Northern Care Alliance NHS Foundation Trust, Manchester M6 8HD, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Petra","family":"Jenkins","sequence":"additional","affiliation":[{"name":"Adult Congenital Heart Disease Centre, Liverpool Heart and Chest Hospital, Liverpool L14 3PE, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9495-7764","authenticated-orcid":false,"given":"Chaitanya","family":"Gadepalli","sequence":"additional","affiliation":[{"name":"Ear Nose and Throat Department, Salford Care Organisation, Northern Care Alliance NHS Foundation Trust, Manchester M6 8HD, UK"},{"name":"School of Medical Education, The University of Manchester, Manchester M14 4PX, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2024,2,28]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","unstructured":"Mehta, A.B., and Winchester, B. 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