{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,11]],"date-time":"2026-03-11T11:51:48Z","timestamp":1773229908918,"version":"3.50.1"},"reference-count":24,"publisher":"BMJ","issue":"3","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Rep"],"accepted":{"date-parts":[[2026,2,25]]},"published-print":{"date-parts":[[2026,3]]},"abstract":"<jats:p>Neonates comprise a special population of patients undergoing surgery, and the presence of congenital defects further complicates their anaesthetic management. This report describes a neonate born with Arnold-Chiari malformation and a ruptured myelomeningocele, who was later found to also have a double-outlet right ventricle. This report outlines the careful selection of anaesthetic agents and perioperative strategies tailored to the patient\u2019s compounded neurologic and cardiac congenital defects while catering to the surgical requirements. The patient underwent repair of the MMC under total intravenous anaesthesia induced with midazolam, ketamine and atracurium and maintained with a combination of dexmedetomidine and fentanyl. The combination of dexmedetomidine and fentanyl appears to be a viable option for neonates with rigid perioperative haemodynamic goals and a need for neuroprotection. We report the safe use of dexmedetomidine for total intravenous anaesthesia pending more robust evidence in this patient population.<\/jats:p>","DOI":"10.1136\/bcr-2025-269078","type":"journal-article","created":{"date-parts":[[2026,3,10]],"date-time":"2026-03-10T14:45:10Z","timestamp":1773153910000},"page":"e269078","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":0,"title":["Anaesthetic management in a neonate with a double-outlet right ventricle undergoing repair of leaking myelomeningocele"],"prefix":"10.1136","volume":"19","author":[{"given":"Micaella Maria Lauchengco","family":"Gonzales","sequence":"first","affiliation":[{"name":"Department of Anesthesiology, University of the Philippines-Philippine General Hospital, Manila, National Capital Region, Philippines"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Geraldine Raphaela Bernardo","family":"Jose","sequence":"additional","affiliation":[{"name":"Department of Anesthesiology, University of the Philippines-Philippine General Hospital, Manila, National Capital Region, Philippines"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2026,3,10]]},"reference":[{"key":"2026031007450470000_19.3.e269078.1","doi-asserted-by":"crossref","first-page":"1026","DOI":"10.1007\/s00246-021-02576-3","article-title":"Congenital Heart Disease and Myelomeningocele in the Newborn: Prevalence and Mortality","volume":"42","author":"Wilkes","year":"2021","journal-title":"Pediatr Cardiol"},{"key":"2026031007450470000_19.3.e269078.2","doi-asserted-by":"crossref","unstructured":"Heart N , Blood Institute . Fourth report on the diagnosis, evaluation, and treatment of high blood pressure in children and adolescents. Bethesda (MD): National Heart, Lung, and Blood Institute, 2005:114\u20136.","DOI":"10.1542\/peds.114.S2.555"},{"key":"2026031007450470000_19.3.e269078.3","doi-asserted-by":"publisher","DOI":"10.1093\/ije\/dyz009"},{"key":"2026031007450470000_19.3.e269078.4","doi-asserted-by":"crossref","first-page":"838","DOI":"10.4070\/kcj.2024.0105","article-title":"Nationwide Birth Prevalence of Crucial Congenital Heart Defects From 2014 to 2018 in Korea","volume":"54","author":"Ha","year":"2024","journal-title":"Korean Circ J"},{"key":"2026031007450470000_19.3.e269078.5","doi-asserted-by":"crossref","unstructured":"Rodr\u00edguez-Blanque R , Almaz\u00e1n-Soto C , Piqueras-Sola B , et al . Chiari Syndrome: Advances in Epidemiology and Pathogenesis: A Systematic Review. J Clin Med 2023;12. doi:10.3390\/jcm12206694","DOI":"10.3390\/jcm12206694"},{"key":"2026031007450470000_19.3.e269078.6","unstructured":"StatPearls Contributors . Chiari malformation type ii. In: StatPearls. Treasure Island (FL): StatPearls Publishing, 2024."},{"key":"2026031007450470000_19.3.e269078.7","doi-asserted-by":"crossref","unstructured":"Zaganjor I , Sekkarie A , Tsang BL , et al . Describing the Prevalence of Neural Tube Defects Worldwide: A Systematic Literature Review. PLoS One 2016;11. doi:10.1371\/journal.pone.0151586","DOI":"10.1371\/journal.pone.0151586"},{"key":"2026031007450470000_19.3.e269078.8","doi-asserted-by":"crossref","unstructured":"Ntimbani J , Kelly A , Lekgwara P . Myelomeningocele - A literature review. Interdiscip Neurosurg 2020;19. doi:10.1016\/j.inat.2019.100502","DOI":"10.1016\/j.inat.2019.100502"},{"key":"2026031007450470000_19.3.e269078.9","doi-asserted-by":"crossref","first-page":"87","DOI":"10.4103\/0019-5049.198396","article-title":"Anaesthetic management in a patient of uncorrected double outlet right ventricle for emergency surgery","volume":"61","author":"Kotwani","year":"2017","journal-title":"Indian J Anaesth"},{"key":"2026031007450470000_19.3.e269078.10","first-page":"100","article-title":"Hemodynamic effects of prone positioning in neonates","volume":"35","author":"Ma","year":"2015","journal-title":"J Perinatol"},{"key":"2026031007450470000_19.3.e269078.11","doi-asserted-by":"crossref","unstructured":"Gano D , McQuillen PS . The neurological impact of congenital heart disease and prematurity. Semin Fetal Neonatal Med 2021;26. doi:10.1016\/j.siny.2021.101271","DOI":"10.1016\/j.siny.2021.101271"},{"key":"2026031007450470000_19.3.e269078.12","doi-asserted-by":"crossref","unstructured":"Xu J , Wen J , Mathena RP , et al . Early Postnatal Exposure to Midazolam Causes Lasting Histological and Neurobehavioral Deficits via Activation of the mTOR Pathway. IJMS 2024;25. doi:10.3390\/ijms25126743","DOI":"10.3390\/ijms25126743"},{"key":"2026031007450470000_19.3.e269078.13","first-page":"1065","article-title":"Effects of low doses of ketamine on intracranial pressure in patients having space-occupying lesions of the brain","volume":"31","author":"Sisodia","year":"2024","journal-title":"J Popul Ther Clin Pharmacol"},{"key":"2026031007450470000_19.3.e269078.14","doi-asserted-by":"crossref","unstructured":"Laws JC , Vance EH , Betters KA , et al . Acute effects of ketamine on intracranial pressure in children with severe traumatic brain injury. Crit Care Med 2023;51. doi:10.1097\/CCM.0000000000005801","DOI":"10.1097\/CCM.0000000000005806"},{"key":"2026031007450470000_19.3.e269078.15","doi-asserted-by":"publisher","DOI":"10.1111\/chd.12662"},{"key":"2026031007450470000_19.3.e269078.16","doi-asserted-by":"publisher","DOI":"10.1177\/1089253214528048"},{"key":"2026031007450470000_19.3.e269078.17","doi-asserted-by":"crossref","unstructured":"Liaquat Z , Xu X , Zilundu PLM , et al . The Current Role of Dexmedetomidine as Neuroprotective Agent: An Updated Review. Brain Sci 2021;11. doi:10.3390\/brainsci11070846","DOI":"10.3390\/brainsci11070846"},{"key":"2026031007450470000_19.3.e269078.18","doi-asserted-by":"publisher","DOI":"10.3389\/fphar.2022.965661"},{"key":"2026031007450470000_19.3.e269078.19","doi-asserted-by":"crossref","first-page":"495","DOI":"10.1159\/000546017","article-title":"Dexmedetomidine as a Promising Neuroprotective Sedoanalgesic in Neonatal Therapeutic Hypothermia: A Systematic Review and Meta-Analysis","volume":"122","author":"Cocchi","year":"2025","journal-title":"Neonatology"},{"key":"2026031007450470000_19.3.e269078.20","first-page":"252","article-title":"Dexmedetomidine versus standard therapy with fentanyl for sedation in mechanically ventilated premature neonates","volume":"17","author":"O\u2019Mara","year":"2012","journal-title":"J Pediatr Pharmacol Ther"},{"key":"2026031007450470000_19.3.e269078.21","doi-asserted-by":"crossref","first-page":"17","DOI":"10.4103\/0970-9185.76610","article-title":"Preliminary experience with dexmedetomidine in neonatal anesthesia","volume":"27","author":"Dilek","year":"2011","journal-title":"J Anaesthesiol Clin Pharmacol"},{"key":"2026031007450470000_19.3.e269078.22","doi-asserted-by":"crossref","DOI":"10.1186\/s13256-024-04645-6","article-title":"Dexmedetomidine as a total intravenous anesthetic in pediatric patients undergoing cleft lip and palate surgery: a case series","volume":"18","author":"Yahya","year":"2024","journal-title":"J Med Case Rep"},{"key":"2026031007450470000_19.3.e269078.23","doi-asserted-by":"crossref","first-page":"143","DOI":"10.1016\/j.pedneo.2014.06.002","article-title":"Clinical pharmacology of fentanyl in preterm infants. A review","volume":"56","author":"Pacifici","year":"2015","journal-title":"Pediatr Neonatol"},{"key":"2026031007450470000_19.3.e269078.24","doi-asserted-by":"crossref","unstructured":"Rakshasbhuvankar M , Rath C , Agrawal S , et al . Neurodevelopmental effects of opioids in preterm infants - A systematic review and meta-analysis of randomised and observational studies. Early Hum Dev 2025;210. doi:10.1016\/j.earlhumdev.2025.106357","DOI":"10.1016\/j.earlhumdev.2025.106357"}],"container-title":["BMJ Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/bcr-2025-269078","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,3,10]],"date-time":"2026-03-10T14:45:14Z","timestamp":1773153914000},"score":1,"resource":{"primary":{"URL":"https:\/\/casereports.bmj.com\/lookup\/doi\/10.1136\/bcr-2025-269078"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,3]]},"references-count":24,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2026,3,10]]},"published-print":{"date-parts":[[2026,3]]}},"alternative-id":["10.1136\/bcr-2025-269078"],"URL":"https:\/\/doi.org\/10.1136\/bcr-2025-269078","relation":{},"ISSN":["1757-790X"],"issn-type":[{"value":"1757-790X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,3]]}}}