{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,27]],"date-time":"2026-05-27T03:02:34Z","timestamp":1779850954660,"version":"3.53.1"},"reference-count":30,"publisher":"American Dental Society of Anesthesiology (ADSA)","issue":"1","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,3]]},"abstract":"<jats:p>This case report describes the dental treatment performed under general anesthesia for a boy with Sj\u00f6gren\u2013Larsson syndrome (SLS), a rare autosomal recessive genetic disorder. The main symptoms of SLS are congenital ichthyosis, limb paraplegia or tetraplegia, and intellectual disability. The main concerns regarding the administration of general anesthesia to patients with SLS include difficulty with adhesion of the electrocardiogram electrodes and intravenous (IV) catheter dressings due to ichthyosis and heat intolerance secondary to hypohidrosis. Although it was first described approximately 70 years ago, there is little in the existing literature on general anesthesia for patients with SLS. We administered deep sedation\/general anesthesia using continuous IV infusions of propofol and remifentanil along with a laryngeal mask airway in a 6-year-old boy with SLS to successfully obtain radiographic studies and extract a supernumerary tooth. No problems occurred other than difficulties with adhesives and soft tissue obstruction of the airway.<\/jats:p>","DOI":"10.2344\/24-0053","type":"journal-article","created":{"date-parts":[[2026,3,6]],"date-time":"2026-03-06T15:26:27Z","timestamp":1772810787000},"page":"44-47","source":"Crossref","is-referenced-by-count":0,"title":["General Anesthesia for a Child With Sj\u00f6gren-Larsson Syndrome"],"prefix":"10.2344","volume":"73","author":[{"given":"Yoshinao","family":"Asahi","sequence":"first","affiliation":[{"name":"Department of Dentistry, Morinomiya Hospital"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shiro","family":"Omichi","sequence":"additional","affiliation":[{"name":"Department of Dentistry, Bobath Memorial Hospital"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiroko","family":"Tsukamoto","sequence":"additional","affiliation":[{"name":"Department of Dentistry, Bobath Memorial Hospital"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yukiyo","family":"Toyohara","sequence":"additional","affiliation":[{"name":"Department of Dentistry, Morinomiya Hospital"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chiaki","family":"Kato","sequence":"additional","affiliation":[{"name":"Department of Dentistry, Morinomiya Hospital"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuka","family":"Sugimoto","sequence":"additional","affiliation":[{"name":"Department of Dentistry, Bobath Memorial Hospital"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Miho","family":"Hyodo","sequence":"additional","affiliation":[{"name":"Department of Dentistry, Morinomiya Hospital"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoshito","family":"Takasaki","sequence":"additional","affiliation":[{"name":"Department of Dentistry, Morinomiya Hospital"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1191","reference":[{"key":"i1878-7177-73-1-44-b01","series-title":"Appl Clin Genet","first-page":"13","article-title":"Sjogren-Larsson syndrome: mechanism and management","volume":"13","author":"Bindu PS.","year":"2020","unstructured":"Bindu PS. Sjogren-Larsson syndrome: mechanism and management. Appl Clin Genet. 2020;13:13\u201324."},{"key":"i1878-7177-73-1-44-b02","series-title":"Acta Derm Venereol","first-page":"68","article-title":"Sj\u00f6gren-Larsson syndrome: a study of clinical symptoms and dermatological treatment in 34 Swedish patients","volume":"89","author":"G\u00e5nemo A","year":"2009","unstructured":"G\u00e5nemo A,\nJagell S,\nVahlquist A. Sj\u00f6gren-Larsson syndrome: a study of clinical symptoms and dermatological treatment in 34 Swedish patients. Acta Derm Venereol. 2009;89:68\u201373."},{"key":"i1878-7177-73-1-44-b03","series-title":"Brain","first-page":"1426","article-title":"Clinical, biochemical and molecular genetic characteristics of 19 patients with Sj\u00f6gren-Larsson syndrome","volume":"124","author":"Willemsen MA","year":"2001","unstructured":"Willemsen MA,\nIjlst L,\nSteijlen PM, et al. Clinical, biochemical and molecular genetic characteristics of 19 patients with Sj\u00f6gren-Larsson syndrome. Brain. 2001;124:1426\u20131437."},{"key":"i1878-7177-73-1-44-b04","series-title":"Ophthalmic Genet","first-page":"298","article-title":"Sj\u00f6gren-Larsson syndrome: a complex metabolic disease with a distinctive ocular phenotype","volume":"40","author":"Fouzdar-Jain S","year":"2019","unstructured":"Fouzdar-Jain S,\nSuh DW,\nRizzo WB. Sj\u00f6gren-Larsson syndrome: a complex metabolic disease with a distinctive ocular phenotype. Ophthalmic Genet. 2019;40:298\u2013308."},{"key":"i1878-7177-73-1-44-b05","series-title":"Swed Dent J","first-page":"141","article-title":"Oral conditions in Sj\u00f6gren-Larsson syndrome","volume":"7","author":"Forsberg H","year":"1983","unstructured":"Forsberg H,\nJagell S,\nReuterving CO. Oral conditions in Sj\u00f6gren-Larsson syndrome. Swed Dent J. 1983;7:141\u2013151."},{"key":"i1878-7177-73-1-44-b06","series-title":"Acta Genet","first-page":"80","article-title":"Oligophrenia combined with congenital ichthyosiform erythrodermia, spastic syndrome and macularretinal degeneration; a clinical and genetic study","volume":"6","author":"Sj\u00f6gren T.","year":"1956","unstructured":"Sj\u00f6gren T. Oligophrenia combined with congenital ichthyosiform erythrodermia, spastic syndrome and macularretinal degeneration; a clinical and genetic study. Acta Genet. 1956;6:80\u201391."},{"key":"i1878-7177-73-1-44-b07","series-title":"Acta Psychiatr Neurol Scand Suppl","first-page":"1","article-title":"Oligophrenia in combination with congenital ichthyosis and spastic disorders; a clinical and genetic study","volume":"113","author":"Sj\u00f6gren T","year":"1957","unstructured":"Sj\u00f6gren T,\nLarsson T. Oligophrenia in combination with congenital ichthyosis and spastic disorders; a clinical and genetic study. Acta Psychiatr Neurol Scand Suppl. 1957;113:1\u2013112."},{"key":"i1878-7177-73-1-44-b08","series-title":"Paediatr Anaesth","first-page":"1390","article-title":"Sj\u00f6gren-Larsson syndrome: anesthetic considerations and practical recommendations","volume":"30","author":"Franzen MH","year":"2020","unstructured":"Franzen MH,\nLeRiger MM,\nKugler JA,\nPellegrino KP,\nRizzo WB. Sj\u00f6gren-Larsson syndrome: anesthetic considerations and practical recommendations. Paediatr Anaesth. 2020;30:1390\u20131395."},{"key":"i1878-7177-73-1-44-b09","series-title":"J Clin Anesth","first-page":"1737","article-title":"Anesthetic management of a patient with Sj\u00f6gren-Larsson syndrome with laryngomalacia","volume":"18","author":"Nishida T","year":"1994","unstructured":"Nishida T,\nTakashina M,\nHagihira S,\nInagaki Y,\nYoshiya I. Anesthetic management of a patient with Sj\u00f6gren-Larsson syndrome with laryngomalacia. J Clin Anesth. 1994;18:1737\u20131738."},{"key":"i1878-7177-73-1-44-b10","series-title":"Masui","first-page":"177","article-title":"Anesthesia for an eleven year old girl with Sj\u00f6gren-Larsson syndrome","volume":"66","author":"Sakurai Y","year":"2017","unstructured":"Sakurai Y,\nUchida M. Anesthesia for an eleven year old girl with Sj\u00f6gren-Larsson syndrome. Masui. 2017;66:177\u2013179."},{"key":"i1878-7177-73-1-44-b11","series-title":"J Inherit Metab Dis","first-page":"955","article-title":"Sj\u00f6gren-Larsson syndrome in clinical practice","volume":"35","author":"Fuijkschot J","year":"2012","unstructured":"Fuijkschot J,\nTheelen T,\nSeyger MMB, et al. Sj\u00f6gren-Larsson syndrome in clinical practice. J Inherit Metab Dis. 2012;35:955\u2013962."},{"key":"i1878-7177-73-1-44-b12","series-title":"Dev Med Child Neurol","first-page":"1019","article-title":"Oral health status of children and adolescents with intellectual disabilities: a systematic review and meta-analysis","volume":"59","author":"Zhou N","year":"2017","unstructured":"Zhou N,\nWong HM,\nWen YF,\nMcgrath C. Oral health status of children and adolescents with intellectual disabilities: a systematic review and meta-analysis. Dev Med Child Neurol. 2017;59:1019\u20131026."},{"key":"i1878-7177-73-1-44-b13","series-title":"Spec Care Dentist","first-page":"262","article-title":"Intellectual disability and impact on oral health: a paired study","volume":"33","author":"Oliveira JS","year":"2013","unstructured":"Oliveira JS,\nPrado J\u00fanior RR,\nde Sousa Lima KR,\nde Oliveira Amaral H,\nMoita Neto JM,\nMendes RF. Intellectual disability and impact on oral health: a paired study. Spec Care Dentist. 2013;33:262\u2013268."},{"key":"i1878-7177-73-1-44-b14","series-title":"J Clin Med","first-page":"5343","article-title":"Teeth damage during general anesthesia","volume":"12","author":"Neto JM","year":"2023","unstructured":"Neto JM,\nTeles AR,\nBarbosa J,\nSantos O. Teeth damage during general anesthesia. J Clin Med. 2023;12:5343."},{"key":"i1878-7177-73-1-44-b15","series-title":"J Thorac Dis","first-page":"822","article-title":"The pathological oral cavity as preventable source of postoperative pneumonia in thoracic surgery: a prospective observational study","volume":"14","author":"Ploenes T","year":"2022","unstructured":"Ploenes T,\nPollok A,\nJ\u00f6ckel KH, et al. The pathological oral cavity as preventable source of postoperative pneumonia in thoracic surgery: a prospective observational study. J Thorac Dis. 2022;14:822\u2013831."},{"key":"i1878-7177-73-1-44-b01","series-title":"Appl Clin Genet","first-page":"13","article-title":"Sjogren-Larsson syndrome: mechanism and management","volume":"13","author":"Bindu PS.","year":"2020","unstructured":"Bindu PS. Sjogren-Larsson syndrome: mechanism and management. Appl Clin Genet. 2020;13:13\u201324."},{"key":"i1878-7177-73-1-44-b02","series-title":"Acta Derm Venereol","first-page":"68","article-title":"Sj\u00f6gren-Larsson syndrome: a study of clinical symptoms and dermatological treatment in 34 Swedish patients","volume":"89","author":"G\u00e5nemo A","year":"2009","unstructured":"G\u00e5nemo A,\nJagell S,\nVahlquist A. Sj\u00f6gren-Larsson syndrome: a study of clinical symptoms and dermatological treatment in 34 Swedish patients. Acta Derm Venereol. 2009;89:68\u201373."},{"key":"i1878-7177-73-1-44-b03","series-title":"Brain","first-page":"1426","article-title":"Clinical, biochemical and molecular genetic characteristics of 19 patients with Sj\u00f6gren-Larsson syndrome","volume":"124","author":"Willemsen MA","year":"2001","unstructured":"Willemsen MA,\nIjlst L,\nSteijlen PM, et al. Clinical, biochemical and molecular genetic characteristics of 19 patients with Sj\u00f6gren-Larsson syndrome. Brain. 2001;124:1426\u20131437."},{"key":"i1878-7177-73-1-44-b04","series-title":"Ophthalmic Genet","first-page":"298","article-title":"Sj\u00f6gren-Larsson syndrome: a complex metabolic disease with a distinctive ocular phenotype","volume":"40","author":"Fouzdar-Jain S","year":"2019","unstructured":"Fouzdar-Jain S,\nSuh DW,\nRizzo WB. Sj\u00f6gren-Larsson syndrome: a complex metabolic disease with a distinctive ocular phenotype. Ophthalmic Genet. 2019;40:298\u2013308."},{"key":"i1878-7177-73-1-44-b05","series-title":"Swed Dent J","first-page":"141","article-title":"Oral conditions in Sj\u00f6gren-Larsson syndrome","volume":"7","author":"Forsberg H","year":"1983","unstructured":"Forsberg H,\nJagell S,\nReuterving CO. Oral conditions in Sj\u00f6gren-Larsson syndrome. Swed Dent J. 1983;7:141\u2013151."},{"key":"i1878-7177-73-1-44-b06","series-title":"Acta Genet","first-page":"80","article-title":"Oligophrenia combined with congenital ichthyosiform erythrodermia, spastic syndrome and macularretinal degeneration; a clinical and genetic study","volume":"6","author":"Sj\u00f6gren T.","year":"1956","unstructured":"Sj\u00f6gren T. Oligophrenia combined with congenital ichthyosiform erythrodermia, spastic syndrome and macularretinal degeneration; a clinical and genetic study. Acta Genet. 1956;6:80\u201391."},{"key":"i1878-7177-73-1-44-b07","series-title":"Acta Psychiatr Neurol Scand Suppl","first-page":"1","article-title":"Oligophrenia in combination with congenital ichthyosis and spastic disorders; a clinical and genetic study","volume":"113","author":"Sj\u00f6gren T","year":"1957","unstructured":"Sj\u00f6gren T,\nLarsson T. Oligophrenia in combination with congenital ichthyosis and spastic disorders; a clinical and genetic study. Acta Psychiatr Neurol Scand Suppl. 1957;113:1\u2013112."},{"key":"i1878-7177-73-1-44-b08","series-title":"Paediatr Anaesth","first-page":"1390","article-title":"Sj\u00f6gren-Larsson syndrome: anesthetic considerations and practical recommendations","volume":"30","author":"Franzen MH","year":"2020","unstructured":"Franzen MH,\nLeRiger MM,\nKugler JA,\nPellegrino KP,\nRizzo WB. Sj\u00f6gren-Larsson syndrome: anesthetic considerations and practical recommendations. Paediatr Anaesth. 2020;30:1390\u20131395."},{"key":"i1878-7177-73-1-44-b09","series-title":"J Clin Anesth","first-page":"1737","article-title":"Anesthetic management of a patient with Sj\u00f6gren-Larsson syndrome with laryngomalacia","volume":"18","author":"Nishida T","year":"1994","unstructured":"Nishida T,\nTakashina M,\nHagihira S,\nInagaki Y,\nYoshiya I. Anesthetic management of a patient with Sj\u00f6gren-Larsson syndrome with laryngomalacia. J Clin Anesth. 1994;18:1737\u20131738."},{"key":"i1878-7177-73-1-44-b10","series-title":"Masui","first-page":"177","article-title":"Anesthesia for an eleven year old girl with Sj\u00f6gren-Larsson syndrome","volume":"66","author":"Sakurai Y","year":"2017","unstructured":"Sakurai Y,\nUchida M. Anesthesia for an eleven year old girl with Sj\u00f6gren-Larsson syndrome. Masui. 2017;66:177\u2013179."},{"key":"i1878-7177-73-1-44-b11","series-title":"J Inherit Metab Dis","first-page":"955","article-title":"Sj\u00f6gren-Larsson syndrome in clinical practice","volume":"35","author":"Fuijkschot J","year":"2012","unstructured":"Fuijkschot J,\nTheelen T,\nSeyger MMB, et al. Sj\u00f6gren-Larsson syndrome in clinical practice. J Inherit Metab Dis. 2012;35:955\u2013962."},{"key":"i1878-7177-73-1-44-b12","series-title":"Dev Med Child Neurol","first-page":"1019","article-title":"Oral health status of children and adolescents with intellectual disabilities: a systematic review and meta-analysis","volume":"59","author":"Zhou N","year":"2017","unstructured":"Zhou N,\nWong HM,\nWen YF,\nMcgrath C. Oral health status of children and adolescents with intellectual disabilities: a systematic review and meta-analysis. Dev Med Child Neurol. 2017;59:1019\u20131026."},{"key":"i1878-7177-73-1-44-b13","series-title":"Spec Care Dentist","first-page":"262","article-title":"Intellectual disability and impact on oral health: a paired study","volume":"33","author":"Oliveira JS","year":"2013","unstructured":"Oliveira JS,\nPrado J\u00fanior RR,\nde Sousa Lima KR,\nde Oliveira Amaral H,\nMoita Neto JM,\nMendes RF. Intellectual disability and impact on oral health: a paired study. Spec Care Dentist. 2013;33:262\u2013268."},{"key":"i1878-7177-73-1-44-b14","series-title":"J Clin Med","first-page":"5343","article-title":"Teeth damage during general anesthesia","volume":"12","author":"Neto JM","year":"2023","unstructured":"Neto JM,\nTeles AR,\nBarbosa J,\nSantos O. Teeth damage during general anesthesia. J Clin Med. 2023;12:5343."},{"key":"i1878-7177-73-1-44-b15","series-title":"J Thorac Dis","first-page":"822","article-title":"The pathological oral cavity as preventable source of postoperative pneumonia in thoracic surgery: a prospective observational study","volume":"14","author":"Ploenes T","year":"2022","unstructured":"Ploenes T,\nPollok A,\nJ\u00f6ckel KH, et al. The pathological oral cavity as preventable source of postoperative pneumonia in thoracic surgery: a prospective observational study. 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