Arun O, Oc B, Metin EN, Sert A, Yilmaz R, Oc M. Anesthetic management of a child with Cornelia de Lange Syndrome undergoing open heart surgery: A case report. World J Cardiol 2022; 14(1): 54-63 [PMID: 35126872 DOI: 10.4330/wjc.v14.i1.54]
Corresponding Author of This Article
Oguzhan Arun, MD, Professor, Department of Anesthesiology and Reanimation, Selcuk University Faculty of Medicine, Alaaddin Keykubat Kampus, Konya 42130, Turkey. oguzarun@selcuk.edu.tr
Research Domain of This Article
Anesthesiology
Article-Type of This Article
Case Report
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
World J Cardiol. Jan 26, 2022; 14(1): 54-63 Published online Jan 26, 2022. doi: 10.4330/wjc.v14.i1.54
Anesthetic management of a child with Cornelia de Lange Syndrome undergoing open heart surgery: A case report
Oguzhan Arun, Bahar Oc, Esma Nur Metin, Ahmet Sert, Resul Yilmaz, Mehmet Oc
Oguzhan Arun, Bahar Oc, Esma Nur Metin, Department of Anesthesiology and Reanimation, Selcuk University Faculty of Medicine, Konya 42130, Turkey
Ahmet Sert, Department of Pediatric Cardiology, Selcuk University Faculty of Medicine, Konya 42130, Turkey
Resul Yilmaz, Pediatric Intensive Care, Selcuk University Faculty of Medicine, Konya 42130, Turkey
Mehmet Oc, Department of Cardiovascular Surgery, Selcuk University Faculty of Medicine, Konya 42130, Turkey
Author contributions: All authors equally contributed to the preparation of the manuscript; Arun O analyzed the data and wrote the manuscript; all authors have read and approved the final manuscript.
Informed consent statement: Written informed consent was obtained from the father of the patient for publication of this report and accompanying images.
Conflict-of-interest statement: The authors certify that there is no conflict of interest with any financial organization regarding the material discussed in the manuscript.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Oguzhan Arun, MD, Professor, Department of Anesthesiology and Reanimation, Selcuk University Faculty of Medicine, Alaaddin Keykubat Kampus, Konya 42130, Turkey. oguzarun@selcuk.edu.tr
Received: April 2, 2021 Peer-review started: April 2, 2021 First decision: June 25, 2021 Revised: July 14, 2021 Accepted: December 21, 2021 Article in press: December 21, 2021 Published online: January 26, 2022
Abstract
BACKGROUND
Cornelia de Lange syndrome (CdLS) is a congenital multisystemic genetic disorder. The expected lifespan of children with this disorder has been prolonged in parallel with the advances in medicine in recent years. However, they still more frequently undergo cardiac surgery. There are some challenges for clinicians when faced with CdLS patients. We present the perioperative management of a child with CdLS undergoing open-heart surgery.
CASE SUMMARY
Severe pulmonic and subpulmonic valvular stenosis, enlargement of the right side of the heart, mild tricuspid regurgitation, atrial septal defect, and patent ductus arteriosus were diagnosed in a 14-month-old boy with manifested cyanosis, developmental delay, and malnutrition. Attempted balloon valvuloplasty was unsuccessful due to a severe stenotic pulmonary valve, therefore it was decided to perform an open surgical repair. Following a successful and uncomplicated intraoperative course, the patient was extubated on postoperative day 5, and adrenalin and dopamine infusions were gradually decreased and stopped on postoperative days 6 and 10, respectively. Moderate laryngomalacia and suboptimal vocal cord movements were diagnosed, and tracheotomy and percutaneous endoscopic gastrostomy were performed under general anesthesia in the same session at postoperative day 32. The patient was discharged on postoperative day 85 after a challenging postoperative period with additional airway and nutritional problems.
CONCLUSION
This is the first report of the perioperative anesthetic and clinical management of a CdLS patient undergoing open-heart surgery.
Core tip: Cornelia de Lange Syndrome (CdLS) is a congenital multisystemic genetic disorder with multiple congenital abnormalities. The expected lifespan of children with CdLS has been prolonged in parallel with the advances in medicine in recent years. Patients with CdLS undergo cardiac surgery more frequently. In any patients with multiple medical challenges, anesthesiologists, cardiovascular surgeons and pediatricians may face unexpectedly unusual perioperative courses with additional difficulties when undergoing congenital open-heart surgery. The case presented here demonstrates an example of a challenging perioperative management period of a child with multisystemic congenital disease undergoing multiple high-risk surgeries.
