J Dent Anesth Pain Med.  2022 Apr;22(2):155-159. 10.17245/jdapm.2022.22.2.155.

A Cohen syndrome patient whose muscle-relaxant effect may have been prolonged during general anesthesia: a case report

• Ishikawa E
• Shibuya M
• Kimura Y
• Kamekura N
• Fujisawa T

• KMID: 2527614
• DOI: http://doi.org/10.17245/jdapm.2022.22.2.155

Abstract

Cohen syndrome is a rare genetic disorder associated with mutations in the VPS13B gene. Individuals with this disorder present with diverse clinical manifestations, including muscle hypotonia, intellectual disabilities, and typical facial characteristics, such as prominent upper central incisors and micrognathia. General anesthesia was administered to a 23-year-old man with Cohen syndrome. Although we observed prominent upper central incisors, an overjet of 10 mm, micrognathia, and thyromental distance of 4 cm, hypotonia was not observed in the patient. Intubation was rendered difficult when performing a direct laryngoscopy. However, smooth intubation was achieved using a video laryngoscope. The patient’s train of four (TOF) count remained zero close to 60 min after rocuronium administration, suggesting that the drug’s muscle-relaxant effect may have been prolonged. A TOF ratio of 0.79 was confirmed 130 min after rocuronium administration, and a TOF ratio of 1.0 was confirmed after administration of 150 mg of sugammadex. The patient’s respiration remained stable after extubation, and no recurarization of muscle relaxation was observed. As demonstrated in this case report, it is important to closely monitor recovery from muscle relaxation and prepare multiple techniques for airway management in general anesthesia management of patients with Cohen syndrome.

Keyword

Anesthesia; General; Cohen Syndrome; Muscle Hypotonia; Rocuronium