Hudec J, Kosinova M, Prokopova T, Filipovic M, Repko M, Stourac P. Anesthesia of a patient with congenital cataract, facial dysmorphism, and neuropathy syndrome for posterior scoliosis: A case report . World J Clin Cases 2022; 10(13): 4207-4213 [PMID: 35665120 DOI: 10.12998/wjcc.v10.i13.4207]
Corresponding Author of This Article
Martina Kosinova, PhD, Associate Professor, Department of Pediatric Anesthesiology and Intensive Care Medicine, University Hospital Brno, Medical Faculty of Masaryk University, Jihlavska 20, Brno 62500, Czech Republic. mata.kosinova@gmail.com
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 Clin Cases. May 6, 2022; 10(13): 4207-4213 Published online May 6, 2022. doi: 10.12998/wjcc.v10.i13.4207
Anesthesia of a patient with congenital cataract, facial dysmorphism, and neuropathy syndrome for posterior scoliosis: A case report
Jan Hudec, Martina Kosinova, Tereza Prokopova, Milan Filipovic, Martin Repko, Petr Stourac
Jan Hudec, Tereza Prokopova, Department of Anesthesiology and Intensive Care Medicine, University Hospital Brno, Medical Faculty of Masaryk University, Brno 62500, Czech Republic
Jan Hudec, Martina Kosinova, Tereza Prokopova, Petr Stourac, Department of Simulation Medicine, Medical Faculty of Masaryk University, Brno 62500, Czech Republic
Martina Kosinova, Petr Stourac, Department of Pediatric Anesthesiology and Intensive Care Medicine, University Hospital Brno, Medical Faculty of Masaryk University, Brno 62500, Czech Republic
Milan Filipovic, Martin Repko, Department of Orthopedic Surgery, University Hospital Brno, Medical Faculty of Masaryk University, Brno 62500, Czech Republic
Author contributions: Hudec J performed anesthesia on the patient; Hudec J, Kosinova M, Prokopova T, and Stourac P had a significant contribution in writing the manuscript; Repko M and Filipovic F operated on the patient, and analyzed and interpreted the patient data regarding motor evoked potentials; all authors read and approved the final manuscript.
Informed consent statement: The legal representative provided informed consent for this publication.
Conflict-of-interest statement: The authors declare no conflict of interest for this article.
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: Martina Kosinova, PhD, Associate Professor, Department of Pediatric Anesthesiology and Intensive Care Medicine, University Hospital Brno, Medical Faculty of Masaryk University, Jihlavska 20, Brno 62500, Czech Republic. mata.kosinova@gmail.com
Received: September 9, 2021 Peer-review started: September 9, 2021 First decision: December 9, 2021 Revised: December 17, 2021 Accepted: March 16, 2022 Article in press: March 16, 2022 Published online: May 6, 2022 Processing time: 232 Days and 7.5 Hours
Abstract
BACKGROUND
Congenital cataract, facial dysmorphism, and neuropathy (CCFDN) syndrome is an extremely rare multiorgan disorder. Characteristics include congenital cataracts, facial deformation, extremity deformities, and demyelinating neuropathy. CCFDN syndrome is associated with increased risk during anesthesia including rhabdomyolysis or epileptic seizures. There is a lack of published information about difficult airways in these patients. Difficult airways during intubation represent one of the most dreaded anesthesia complications: A "can not intubate, can not oxygenate" scenario. Presented herein is the first described successful endotracheal intubation of a CCFDN syndrome patient.
CASE SUMMARY
We report the anesthetic management of a 13-year-old girl with CCFDN syndrome scheduled for posterior neuromuscular scoliosis correction surgery. The patient suffered from extensive progressive neuromuscular scoliosis with a Cobb angle of 83°. Her limitations included neuropathy and a scoliotic curve. This condition negatively impacted her quality of life. This case reflects the potential anesthetic complications for posterior scoliosis correction and CCFDN syndrome. The challenge for our anesthetic team was the limited amount of data about anesthetic management of this condition. In total, one case report without any data about endotracheal intubation of patients with this condition was available. Endotracheal intubation in our case was uncomplicated. Another focus of our case was the prevention of possible complications associated with this syndrome, including rhabdomyolysis and seizures. Rhabdomyolysis can be triggered by some types of anesthetic agents like suxamethonium or volatile anesthetics, especially in patients with certain types of myopathies.
CONCLUSION
Adequate understanding of the anesthetic management of CCFDN syndrome can reduce perioperative complications and improve patient outcome after surgery.
Core Tip: We report on a rare case of anesthetic management of a patient with congenital cataract, facial dysmorphism, and neuropathy (CCFDN) syndrome for posterior neuromuscular scoliosis correction. Additionally, this case report is unique as it for the first time presents successful endotracheal intubation in a patient with CCFDN syndrome associated with facial dysmorphism.
