Journal of Japanese Dental Society of Anesthesiology
Online ISSN : 2433-4480
Short Communication
Anesthetic Management of a Pediatric Patient with Cardio-facio-cutaneous Syndrome
Yoshiki SHIONOYAMaki YAMAMOTOKatsuhisa SUNADAKiminari NAKAMURA
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JOURNAL FREE ACCESS

2019 Volume 47 Issue 2 Pages 44-46

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Abstract

  Cardio-facio-cutaneous (CFC) syndrome is a RASopathy, a family of syndromes that includes Noonan and Costello syndromes and presents with overlapping phenotypic similarities. The major features of CFC syndrome include a characteristic craniofacial dysmorphology, congenital heart disease, dermatologic abnormalities, seizures, and intellectual disability. Musculoskeletal findings, such as muscle weakness and decreased muscle mass, are also observed in all RASopathies but are particularly prominent in CFC syndrome. We performed dental treatment under general anesthesia in an 8-year-boy with CFC syndrome. The boy was 120 cm tall, weighed 22 kg, and had a history of epileptic seizures and micrognathia. No abnormalities were noted in a cardiac evaluation including echocardiogram and electrocardiogram. However, respiratory muscle weakness leading to profound postoperative respiratory depression following the administration of general anesthetic, sedative, and opioid drugs was a major concern because of the presence of CFC syndrome. Anesthesia was induced by the inhalation of sevoflurane, N2O, and oxygen. Although mask ventilation and intubation were difficult to perform, success was eventually achieved using a nasal airway and the McGRATHTM MAC laryngoscope (McGRATH). Tracheal intubation was performed using sevoflurane and remifentanil without the use of a muscle relaxant. Anesthesia was maintained with desflurane and continuous intravenous remifentanil. Respiratory depression and epileptic seizures did not occur postoperatively. In patients with CFC syndrome who have muscle weakness, a rapid offset of desflurane may be effective to prevent respiratory depression after general anesthesia. The present case suggested that a preoperative cardiac evaluation, airway management, and the selection of an anesthetic agent to prevent postoperative respiratory depression are important for performing anesthesia safely in patients with CFC syndrome.

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© 2019 The Japanese Dental Society of Anesthesiology
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