Anesthesia of a patient with congenital cataract, facial dysmorphism, and neuropathy syndrome for posterior scoliosis: A case report

Kód výsledku v IS VaVaI

<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F65269705%3A_____%2F22%3A00076076" target="_blank" >RIV/65269705:_____/22:00076076 - isvavai.cz</a>

Nalezeny alternativní kódy

RIV/00216224:14110/22:00126081

Výsledek na webu

<a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9131212/pdf/WJCC-10-4207.pdf" target="_blank" >https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9131212/pdf/WJCC-10-4207.pdf</a>

DOI - Digital Object Identifier

<a href="http://dx.doi.org/10.12998/wjcc.v10.i13.4207" target="_blank" >10.12998/wjcc.v10.i13.4207</a>

Jazyk výsledku

angličtina

Název v původním jazyce

Anesthesia of a patient with congenital cataract, facial dysmorphism, and neuropathy syndrome for posterior scoliosis: A case report

Popis výsledku v původním jazyce

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 &quot;can not intubate, can not oxygenate &quot; 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 &amp; DEG;. 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.

Název v anglickém jazyce

Anesthesia of a patient with congenital cataract, facial dysmorphism, and neuropathy syndrome for posterior scoliosis: A case report

Popis výsledku anglicky

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 &quot;can not intubate, can not oxygenate &quot; 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 &amp; DEG;. 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.

Druh

J_imp - Článek v periodiku v databázi Web of Science

CEP obor

—

OECD FORD obor

30218 - General and internal medicine

Projekt

—

Návaznosti

I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Rok uplatnění

2022

Kód důvěrnosti údajů

S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Název periodika

World Journal of Clinical Cases

ISSN

2307-8960

e-ISSN

2307-8960

Svazek periodika

10

Číslo periodika v rámci svazku

13

Stát vydavatele periodika

US - Spojené státy americké

Počet stran výsledku

7

Strana od-do

4207-4213

Kód UT WoS článku

000806174700024

EID výsledku v databázi Scopus

2-s2.0-85129944377