Anaesthetic management of a patient with myotonic dystrophy

Abstract

A 13-year-old boy with myotonic dystrophy underwent insertion of a percutaneous gastrostomy feeding tube under general anaesthesia. We used a laryngeal mask airway and a spontaneously breathing technique with propofol total intravenous anaesthesia. Postoperative vomiting and aspiration, 12 h after the procedure, subsequently required intubation and ventilation. We discuss the anaesthetic management of this case and review the features of the disease to be considered when contemplating anaesthesia in such patients.

The reproductive outcome of female patients with myotonic dystrophy type 1 (DM1) undergoing PGD is not affected by the size of the expanded CTG repeat tract

“…The results of this study suggest that regardless of the size of the expanded CTG repeat PGD treatment is justified in DM1 patients, as far as reproductive function is concerned and not taking into account the physical status of the patient that may well contraindicate reproductive treatment. The symptomatic condition of DM1 patients and the associated risk of developing cardiac and respiratory insufficiency are largely related to the genetic status, resulting in congenital and childhood types being at a much higher risk of severe morbidity and mortality [21,22]. Our centre adheres to a policy of pre-treatment assessment of the cardiological and neurological status of all female DM1 patients, in order to anticipate any periconceptional and obstetrical risk, and advize against pregnancy if and when necessary.…”

Total intravenous anesthesia in a 10-month-old patient with congenital myotonic dystrophy undergoing endoscopic third ventriculostomy -A case report-

“…Patients with myotonic dystrophy are known to vary in terms of sensitivity to neuromuscular blockers [9,15]. TOF monitoring is thus especially helpful in enabling judicious administration of these drugs.…”

“…It should be noted that once mechanical ventilation is initiated in such patients, the probability that postoperative ventilatory assistance will be required is very high [15]. A period of at least 24 hours of observation in a facility capable of rapid intubation and ventilation has been recommended [15].…”

Preimplantation genetic diagnosis for myotonic dystrophy type 1: upon request to child

“…General anaesthesia should be avoided because of the risk of respiratory insufficiency and the need for aspiration because of incompetence of the lower oesophageal sphincter. 34,35 After a careful cardiac work up leading to the use of adjusted anaesthetic drugs no complications occurred in the five patients who underwent oocyte retrieval under general anaesthesia. An emergency C/S procedure under general anaesthesia in a woman affected by DM1 lead to one major complication.…”