{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,5,14]],"date-time":"2025-05-14T01:51:55Z","timestamp":1747187515338,"version":"3.40.5"},"reference-count":25,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2020,10,26]],"date-time":"2020-10-26T00:00:00Z","timestamp":1603670400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Anesthesia"],"published-print":{"date-parts":[[2020,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Sj\u00f6gren\u2010Larsson syndrome is a rare inherited neurocutaneous disorder characterized by congenital ichthyosis, spasticity, intellectual disability, seizures, and ophthalmologic changes. Most individuals with Sj\u00f6gren\u2010Larsson syndrome live well into adulthood and often require surgical intervention to manage their symptomatology.<\/jats:p><\/jats:sec><jats:sec><jats:title>Aims<\/jats:title><jats:p>The aim of this work was to review the clinical aspects of Sj\u00f6gren\u2010Larsson syndrome, highlight the unique anesthetic considerations associated with this disease, and provide practical recommendations about anesthetic management.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A retrospective case review from February 2013 to October 2019 was performed based on subject participation in a Sj\u00f6gren\u2010Larsson syndrome longitudinal study at the University of Nebraska Medical Center. Anesthetic and surgical records were reviewed for the following data: age, sex, relevant comorbid conditions, anesthetic induction and maintenance agents, intravenous and oral analgesics, muscle relaxants, and anesthetic\u2010related complications.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Fourteen patients with Sj\u00f6gren\u2010Larsson syndrome undergoing 48 anesthetic events were identified. A variety of anesthetic techniques was utilized. No serious adverse events were encountered. The most common clinical observations were related to the ichthyosis seen in Sj\u00f6gren\u2010Larsson syndrome, which led to difficulty in adherence of electrocardiogram leads and intravenous catheter dressings.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>We found that anesthesia can be safely administered in patients with Sj\u00f6gren\u2010Larsson syndrome. Providers should be aware of anesthetic management issues in Sj\u00f6gren\u2010Larsson syndrome including challenges placing and securing lines and monitors secondary to the ichthyosis.<\/jats:p><\/jats:sec>","DOI":"10.1111\/pan.14034","type":"journal-article","created":{"date-parts":[[2020,10,10]],"date-time":"2020-10-10T05:32:17Z","timestamp":1602307937000},"page":"1390-1395","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Sj\u00f6gren\u2010Larsson syndrome: Anesthetic considerations and practical recommendations"],"prefix":"10.1111","volume":"30","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4325-9509","authenticated-orcid":false,"given":"Marcellene H.","family":"Franzen","sequence":"first","affiliation":[{"name":"Department of Anesthesiology University of Nebraska Medical Center Omaha NE USA"},{"name":"Division of Pediatric Anesthesiology Children's Hospital and Medical Center Omaha NE 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