Rayan Muawad, Abdullah AlDhuwaihy, Abdulrahman AlGhamdi, Ahmed Abdurazaq
{"title":"芳香l -氨基酸脱羧酶缺乏症患儿的麻醉处理:1例报告。","authors":"Rayan Muawad, Abdullah AlDhuwaihy, Abdulrahman AlGhamdi, Ahmed Abdurazaq","doi":"10.4103/sja.sja_627_24","DOIUrl":null,"url":null,"abstract":"<p><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.</p>","PeriodicalId":21533,"journal":{"name":"Saudi Journal of Anaesthesia","volume":"19 3","pages":"425-427"},"PeriodicalIF":1.3000,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12240520/pdf/","citationCount":"0","resultStr":"{\"title\":\"Anesthetic management of a child with aromatic L-amino acid decarboxylase deficiency: A case report.\",\"authors\":\"Rayan Muawad, Abdullah AlDhuwaihy, Abdulrahman AlGhamdi, Ahmed Abdurazaq\",\"doi\":\"10.4103/sja.sja_627_24\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><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.</p>\",\"PeriodicalId\":21533,\"journal\":{\"name\":\"Saudi Journal of Anaesthesia\",\"volume\":\"19 3\",\"pages\":\"425-427\"},\"PeriodicalIF\":1.3000,\"publicationDate\":\"2025-07-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12240520/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Saudi Journal of Anaesthesia\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.4103/sja.sja_627_24\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2025/6/16 0:00:00\",\"PubModel\":\"Epub\",\"JCR\":\"Q3\",\"JCRName\":\"ANESTHESIOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Saudi Journal of Anaesthesia","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.4103/sja.sja_627_24","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2025/6/16 0:00:00","PubModel":"Epub","JCR":"Q3","JCRName":"ANESTHESIOLOGY","Score":null,"Total":0}
Anesthetic management of a child with aromatic L-amino acid decarboxylase deficiency: A case report.
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.