Anesthesia of a patient with congenital cataract, facial dysmorphism, and neuropathy syndrome for posterior scoliosis: A case report.
Anesthetic management
CCFDN syndrome
Case report
Endotracheal intubation
Neuromuscular scoliosis
Total intravenous anesthesia
Journal
World journal of clinical cases
ISSN: 2307-8960
Titre abrégé: World J Clin Cases
Pays: United States
ID NLM: 101618806
Informations de publication
Date de publication:
06 May 2022
06 May 2022
Historique:
received:
09
09
2021
revised:
17
12
2021
accepted:
16
03
2022
entrez:
6
6
2022
pubmed:
7
6
2022
medline:
7
6
2022
Statut:
ppublish
Résumé
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. 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. Adequate understanding of the anesthetic management of CCFDN syndrome can reduce perioperative complications and improve patient outcome after surgery.
Sections du résumé
BACKGROUND
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
METHODS
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
CONCLUSIONS
Adequate understanding of the anesthetic management of CCFDN syndrome can reduce perioperative complications and improve patient outcome after surgery.
Identifiants
pubmed: 35665120
doi: 10.12998/wjcc.v10.i13.4207
pmc: PMC9131212
doi:
Types de publication
Case Reports
Langues
eng
Pagination
4207-4213Informations de copyright
©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.
Déclaration de conflit d'intérêts
Conflict-of-interest statement: The authors declare no conflict of interest for this article.
Références
Eur Spine J. 2015 Nov;24 Suppl 7:931-6
pubmed: 26482497
Cochrane Database Syst Rev. 2014 Jun 17;(6):CD003843
pubmed: 24937564
Rom J Anaesth Intensive Care. 2015 Apr;22(1):25-33
pubmed: 28913452
Curr Osteoporos Rep. 2017 Aug;15(4):271-282
pubmed: 28620868
Orphanet J Rare Dis. 2014 Apr 01;9:46
pubmed: 24690360
J Neurosurg Pediatr. 2018 Aug;22(2):207-213
pubmed: 29749884
Curr Opin Anaesthesiol. 2017 Jun;30(3):435-440
pubmed: 28448298
Ophthalmology. 2004 Jul;111(7):1415-23
pubmed: 15234148
Neurosurg Focus. 2009 Oct;27(4):E7
pubmed: 19795956
Neurology. 2014 Oct 7;83(15):1337-44
pubmed: 25186864
Neurology. 2002 Jan 22;58(2):231-6
pubmed: 11805249
Eur J Hum Genet. 1999 Jul;7(5):560-6
pubmed: 10439962
Paediatr Anaesth. 2017 May;27(5):490-493
pubmed: 28306187
Eur J Anaesthesiol. 2017 Mar;34(3):178-180
pubmed: 28141735
Minerva Anestesiol. 2013 Apr;79(4):419-33
pubmed: 23419334
Orphanet J Rare Dis. 2006 Aug 29;1:32
pubmed: 16939648