Anesthetic Management of an Infant with Bilateral Radial Dysplasia and Isolated Patent Ductus Arteriosus: A Case Report and Brief Review of Literature
Bilateral radial dysplasia is a rare condition and usually associated with familial malformation syndromes and spectrum of cardiac anomalies. These patients often require corrective orthopedic surgeries in early age. Radial dysplasia associated with cardiac abnormalities is particularly seen in certain syndromes such as Holt-Oram (HOS) syndrome and TAR (thrombocytopenia, anemia, and radial aplasia) syndrome. We report a successful management of twelve months old male child with rare combination of bilateral radial dysplasia with isolated patent ductus arteriosus (PDA), for corrective orthopedic surgery. A comprehensive approach, which includes an extensive preoperative evaluation and pre-defined perioperative hemodynamic goals for PDA, plays a pivotal role in its perioperative management.
etscher DT, Baumholtz MA. Treatment of congenital upper extremity problems. Plast. Reconstr. Surg. 2007; 119(5):101e–129e.
Bossert T, Walther T, Gummert J, Hubald R, Kostelka M, Mohr FW. Cardiac malformations associated with the Holt-Oram syndrome--report on a family and review of the literature. Thorac Cardiovasc Surg. 2002; 50(5):312-4.
Hedberg VA, Lipton JM. Thrombocytopenia with absent radii. A review of 100 cases. Am J Pediatr Hematol Oncol. 1988; 10:51-64.
Baum VC, Barton DM, Gutgesell HP. Influence of congenital heart disease on mortality after noncardiac surgery in hospitalized children. Pediatrics. 2000; 105:332-5.
Ramamoorthy C, Haberkern CM, Bhananker SM, Domino KB, Posner KL, Campos JS, et al. Anesthesia-related cardiac arrest in children with heart disease: data from the Pediatric Perioperative Cardiac Arrest (POCA) registry. Anesth. Analg. 2010; 110:1376-82.
Menghraj SJ. Anaesthetic considerations in children with congenital heart disease undergoing non-cardiac surgery. Indian J Anaesth. 2012; 56:491-5.
Cox RG, Nemish U, Ewen A, Crowe M-J. Evidence-based clinical update: does premedication with oral midazolam lead to improved behavioural outcomes in children? Can J Anaesth. 2006; 53:1213-9.
Verghese S, Hannallah RS. Anaesthesia for non-cardiac surgery in children with congential heart disease. Southern African Journal of Anaesthesia and Analgesia. 2008; 14:84-6.
Fluri S, Pavlovic M, Wagner BP. Patent arterial duct, bottle-meal, and fatal myocardial ischaemia. Cardiol Young. 2010; 20:108-10.
|Issue||Vol 8 No Supplement (2022): Autumn|
|Radial dysplasia Patent ductus arteriosus Holt-Oram syndrome TAR syndrome Orthopedic|
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