{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,13]],"date-time":"2026-08-13T19:35:23Z","timestamp":1786649723478,"version":"build-2736575974"},"reference-count":7,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"3","license":[{"start":{"date-parts":[[2025,6,16]],"date-time":"2025-06-16T00:00:00Z","timestamp":1750032000000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-sa\/4.0"}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2025,7]]},"abstract":"<jats:p>This report discusses the anesthetic management of a 7-year-old child with aromatic L-amino acid decarboxylase (AADC) deficiency, a rare neurometabolic disorder. The patient underwent ventilation tube insertion and adenotonsillectomy. Similar to other adenotonsillectomy procedures, this surgery carries a higher risk of postoperative nausea and vomiting, necessitating a careful management strategy. We opted for dexamethasone as the primary antiemetic agent and limited opioid use to a single dose of fentanyl, while also incorporating dexmedetomidine for enhanced pain management alongside ketorolac and paracetamol. This case highlights the need for specialized anesthesia protocols for AADC deficiency patients to enhance safety and outcomes, particularly addressing the challenges of nausea and vomiting.<\/jats:p>","DOI":"10.4103\/sja.sja_627_24","type":"journal-article","created":{"date-parts":[[2025,6,16]],"date-time":"2025-06-16T01:00:23Z","timestamp":1750035623000},"page":"425-427","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":0,"title":["Anesthetic management of a child with aromatic L-amino acid decarboxylase deficiency: A case report"],"prefix":"10.4103","volume":"19","author":[{"given":"Rayan","family":"Muawad","sequence":"first","affiliation":[{"name":"Pediatric Anesthesia Department, King Abdullah Specialist Children Hospital, Ministry of National Guard Health Affairs (MNGHA), Riyadh, Saudi Arabia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Abdullah","family":"AlDhuwaihy","sequence":"additional","affiliation":[{"name":"College of Medicine, King Saud University, Riyadh, Saudi Arabia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Abdulrahman","family":"AlGhamdi","sequence":"additional","affiliation":[{"name":"College of Medicine, King Saud University, Riyadh, Saudi Arabia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ahmed","family":"Abdurazaq","sequence":"additional","affiliation":[{"name":"Pediatric Anesthesia Department, King Abdullah Specialist Children Hospital, Ministry of National Guard Health Affairs (MNGHA), Riyadh, Saudi Arabia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2025,6,16]]},"reference":[{"key":"R1-20260423","doi-asserted-by":"crossref","first-page":"38","DOI":"10.1016\/j.pediatrneurol.2019.11.022","article-title":"Prevalence of aromatic l-amino acid decarboxylase deficiency in at-risk populations","volume":"106","author":"Hyland","year":"2020","journal-title":"Pediatr Neurol"},{"key":"R2-20260423","doi-asserted-by":"crossref","first-page":"301","DOI":"10.1007\/BF01799380","article-title":"Aromatic amino acid decarboxylase deficiency in twins","volume":"13","author":"Hyland","year":"1990","journal-title":"J Inherit Metab Dis"},{"key":"R3-20260423","doi-asserted-by":"crossref","first-page":"1121","DOI":"10.1002\/jimd.12247","article-title":"AADC deficiency from infancy to adulthood: Symptoms and developmental outcome in an international cohort of 63 patients","volume":"43","author":"Pearson","year":"2020","journal-title":"J Inherit Metab Dis"},{"key":"R4-20260423","doi-asserted-by":"crossref","first-page":"1016239","DOI":"10.3389\/fped.2022.1016239","article-title":"Case report: Aromatic L-amino acid decarboxylase deficiency in three patient cases from the Kingdom of Saudi Arabia","volume":"10","author":"Abukhaled","year":"2023","journal-title":"Front Pediatr"},{"key":"R5-20260423","doi-asserted-by":"crossref","first-page":"82","DOI":"10.1111\/j.1460-9592.2005.01605.x","article-title":"Anesthesia management in a young child with aromatic l\u2010amino acid decarboxylase deficiency","volume":"16","author":"Vutskits","year":"2006","journal-title":"Pediatr Anesth"},{"key":"R6-20260423","doi-asserted-by":"crossref","first-page":"e01275","DOI":"10.1213\/XAA.0000000000001275","article-title":"Anesthesia management for cesarean delivery in a woman with aromatic l-amino acid decarboxylase deficiency: A case report","volume":"14","author":"Viviani","year":"2020","journal-title":"A A Pract"},{"key":"R7-20260423","doi-asserted-by":"crossref","first-page":"e12152","DOI":"10.1002\/anr3.12152","article-title":"Anaesthesia management of a child with aromatic L\u2010amino acid decarboxylase deficiency","volume":"10","author":"McCarthy","year":"2022","journal-title":"Anaesth Rep"}],"container-title":["Saudi Journal of Anaesthesia"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ovid.com\/10.4103\/sja.sja_627_24","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,13]],"date-time":"2026-08-13T18:57:37Z","timestamp":1786647457000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ovid.com\/10.4103\/sja.sja_627_24"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,6,16]]},"references-count":7,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2025]]}},"URL":"https:\/\/doi.org\/10.4103\/sja.sja_627_24","relation":{},"ISSN":["1658-354X","0975-3125"],"issn-type":[{"value":"1658-354X","type":"print"},{"value":"0975-3125","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,6,16]]},"assertion":[{"value":"2024-10-04","name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-10-21","name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}}]}}