piperidines and Myotonic-Dystrophy

In general anesthesia for a patient with dystrophia myotonica (DM), respiratory depression and muscle weakness by opioid, as well as prolongation of the effect of muscle relaxant are seen postoperatively. Therefore it is desirable to choose agents with short duration of action and to dose these medicines to the minimum. We report a case of a 45-year-old woman with DM who underwent laparotomy for uterine cancer under general anesthesia combined with epidural anesthesia. Epidural catheter was placed from T 11-12, and anesthesia was inducted with propofol and remifentanil (RF). We administered rocuronium bromide (RB) 5 mg while watching TOF ratio with a muscle relaxation monitor (TOF-Watch). T1 became 0 after giving a dose of 10 mg, and intubation was performed. We maintained anesthesia by propofol and RF combined with epidural anesthesia. TOF ratio was restored to around 80% 90 minutes after RB administration, but we did not give supplemental doses because the operation went well smoothly. Recovery was smooth and fast. The respiratory depression and the muscle spasm were not noticed. RB and RE both with short duration of action, are useful in anesthesia management in DM cases.

Topics: Androstanols; Anesthesia, Epidural; Anesthesia, Intravenous; Anesthetics, Intravenous; Female; Humans; Middle Aged; Myotonic Dystrophy; Neuromuscular Nondepolarizing Agents; Piperidines; Propofol; Remifentanil; Rocuronium; Uterine Neoplasms

A 54-year-old woman, complicated with myotonic dystrophy, underwent laparoscopic cholecystectomy for cholelithiasis. The patient was given total intravenous anesthesia using propofol, remifentanil and vecuronium, combined with epidural anesthesia using ropivacaine. No complication occurred, and the patient recovered from anesthesia without delay. For the relief of postoperative pain, ropivacaine (0.2%) was given epidurally. The postoperative course was uneventful. The anesthesia with remifentanil is extremely useful for patients with myotonic dystrophy.

Topics: Anesthesia, Epidural; Anesthesia, Intravenous; Cholecystectomy, Laparoscopic; Cholelithiasis; Female; Humans; Middle Aged; Myotonic Dystrophy; Piperidines; Propofol; Remifentanil

Anesthesia for patients with Steinert's syndrome (myotonic dystrophy, MD) is a challenge for the anaesthetist. MD is a multisystemic disease and the neuromuscular symptoms can be associated with sleep apnea, endocrine disorders (diabetes, hypogonadism, hypothyroidism), cardiac, gastroenteric or cognitive disorders (mental deficiency, attention disorders). The diagnosis is facilitated when one or more of these symptoms are associated with the neuromuscular symptoms; however, the latter are not always present at the onset, which makes the diagnosis of MD a difficult and often late one. The choice of drugs and the choice of anesthesia in these patients can be very challenging for many reasons. A myotonic crisis can be triggered by several factors including hypothermia, shivering and mechanical or electrical stimulation. These patients are very sensitive to the usual anesthetics such as hypnotics and paralyzing agents (both depolarizing and nondepolarizing). The following case report describes pathophysiological considerations and a technique for anaesthesia during thoracic surgery that has been able to assure hemodynamic peroperative stability, early extubation and prolonged respiratory autonomy in a patient affected by this genetic disorder.

Topics: Aged; Anesthesia, Intravenous; Anesthetics, Intravenous; Atracurium; Humans; Male; Myotonic Dystrophy; Neuromuscular Nondepolarizing Agents; Piperidines; Propofol; Remifentanil; Respiratory Insufficiency; Thoracotomy

Topics: Brachial Plexus; Cholinesterase Inhibitors; Colles' Fracture; Contraindications; Female; Fracture Fixation, Internal; Hemodynamics; Humans; Hypnotics and Sedatives; Intraoperative Complications; Middle Aged; Myotonic Dystrophy; Nerve Block; Neuromuscular Nondepolarizing Agents; Piperidines; Remifentanil; Succinylcholine

We report two cases of intestinal pseudoobstruction caused by visceral smooth muscle involvement due to myotonic muscular dystrophy. Two patients with myotonic muscular dystrophy presented with abdominal pain, distention, constipation, and vomiting. The exclusion of mechanical obstruction by plain abdominal radiography, contrast studies, and colonoscopy led to the diagnosis of intestinal pseudoobstruction. Diagnosis was confirmed by manometric and cineradiographic findings of abnormal intestinal motility. Conservative management including laxatives and cisapride led to the resolution of the pseudoobstruction syndrome and long-term remission without relapses during a two year follow-up. In patients with known myotonic dystrophy the occurrence of intestinal pseudoobstruction should be considered in order to avoid unnecessary laparotomies.

Topics: Cathartics; Cisapride; Female; Follow-Up Studies; Humans; Intestinal Pseudo-Obstruction; Male; Manometry; Middle Aged; Muscle, Smooth; Myotonic Dystrophy; Piperidines; Radiography; Sympathomimetics