Case series: anesthetic management of patients with spinal and bulbar muscular atrophy (Kennedy’s disease)

Anesthesia and perioperative medical management of children with spinal muscular atrophy

“…The choice of airway safeguard and devices (e.g., face mask, laryngeal mask airway [LMA], endotracheal tube) is dictated by the type of surgery. However, direct laryngeal intubation may become progressively difficult with severe or advanced muscle atrophy, particularly in SMA type I, because of atrophy of masseter and other muscles of mastication as was noted unexpectedly in one patient in our series (18,23). Anesthetic technique of spontaneous breathing via face mask or LMA may be indicated for short and minimally invasive procedure such as muscle biopsy, bearing in mind the potential for laryngospasm and need for administering nondepolarizing muscles relaxant that may prolong emergence or need for prolonged ventilatory support.…”

The use of sugammadex in a patient with Kennedy′s disease under general anesthesia

“…Despite this, a Medline search of the available literature from the past 40 years revealed only one case report of the successful use of epidural anesthesia for internal urethrotomy[9] and one case series of six patients related to anesthetic management that used a search of medical records from the Mayo Clinic. [5] This is the first anesthetic report regarding the general anesthetic management of a patient with KD. This is also the first report of rocuronium and sugammadex being administered to a patient with KD.…”

“…Although a cases series of anesthetic reports on the general anesthetic management of patients with KD has been published,[5] the use of rocuronium and sugammadex have not yet been reported. Sugammadex, a newly introduced modified l-cyclodextrin, encapsulates steroidal neuromuscular blocking drugs and may therefore reverse rocuronium-induced neuromuscular block.…”

Complications After Cardiovascular Surgery in a Case of Undiagnosed Spinal-Bulbar Muscular Atrophy (Kennedy Disease)

“…No postoperative complications or exacerbation of neurologic symptoms were noted. The other study 10 reports a series of 6 patients with confirmed SBMA undergoing general anesthesia for various surgical interventions (fasciotomy, pulmonary resection, debridements, lumbar laminectomy, cervical laminectomy, knee arthroplasty, odontectomy, and appendectomy). Similarly to our patient, Niesen and colleagues 10 report major postoperative complications in 2 of their 6 patients, including dysphagia, respiratory complications requiring reintubation, tracheotomy placement, and/or prolonged mechanical ventilation.…”

“…[5][6][7] Signs and symptoms of neurodegenerative diseases can be unmasked or exacerbated after major surgery. [8][9][10] A patient with SBMA that was diagnosed incidentally after a triple coronary artery bypass procedure is presented.…”