1/16. Anaesthetic management of a patient with myotonic dystrophy.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.- - - - - - - - - - ranking = 1keywords = breathing (Clic here for more details about this article) |
2/16. Torsion of a non-gravid leiomyomatous uterus in a patient with myotonic dystrophy complaining of acute urinary retention: anaesthetic management for total abdominal hysterectomy.Torsion of a pregnant uterus is rare, but torsion of a non-pregnant uterus is extremely rare. abdominal pain is the major symptom. Other symptoms include vaginal bleeding, urinary tract symptoms and gastro-intestinal manifestations. We present a case of a 37-year-old white nullipara who presented at the emergency room with acute urinary retention. Medical history revealed that the patient carried the disease of myotonic dystrophy, which was diagnosed two years before. physical examination revealed a tender, distended bladder, which was easily catheterized, draining 900 ml of clear urine. The abdomen was soft with no muscle guarding or rebound tenderness. A palpable large dense mass occupying the cul-de-sac was found during bimanual examination. Abdominal ultrasound examination revealed a large intramural leiomyoma approximately 10 cm in diameter, in the posterior wall of the uterus, which repelled the bladder. In neurological examination the muscular tone and reflexes were reduced in the lower extremities. Myotonic phenomenon was not found. The patient was thought to suffer from myotonic dystrophy and therefore the possibilities for pulmonary and cardiac complications or malignant hyperthermia had to be kept in mind during the anaesthetic management. The patient underwent an exploratory laparotomy and the uterus was found to have undergone a 60 degrees rotation along the corpus and the cervix uteri transition line. Total abdominal hysterectomy with bilateral salpingo-oophorectomy was perfomed. The intra- and postoperative course of the patient was uneventful. In conclusion, in this patient the uterine pathology (large leiomyoma) in combination with the disease of myotonic dystrophy seemed to be the predisposing factors for the torsion of the non-pregnant uterus. Also, the anaesthetic implications for total abdominal hysterectomy in myotonic dystrophy are discussed and the international literature is reviewed.- - - - - - - - - - ranking = 0.0021025856840016keywords = pain (Clic here for more details about this article) |
3/16. Gastric volvulus complicating myotonic dystrophy.myotonic dystrophy is an autosomal inherited disorder of both striated and smooth muscle, and is considered to be a rare cause of gastrointestinal dilatation and abnormal peristalsis. We report on a patient with myotonic dystrophy complicated by gastric volvulus. A 57-year-old female with myotonic dystrophy suddenly developed abdominal pain, nausea and vomiting. X-ray examinations revealed gastric dilatation and pyloroantral obstruction, consistent with acute gastric volvulus. The patient underwent successful emergency gastrectomy. Gastric volvulus is often an unrecognized surgical emergency, but its clinical and radiographic features are so characteristic that accurate diagnosis is possible if the condition is kept in mind. Thus, the clinician should consider the possibility of gastric volvulus when evaluating gastrointestinal complaints in patients with myotonic dystrophy.- - - - - - - - - - ranking = 0.0021025856840016keywords = pain (Clic here for more details about this article) |
4/16. scoliosis in Steinert syndrome: a case report.BACKGROUND CONTEXT: Steinert syndrome is described as an autosomal dominant condition characterized by progressive muscular wasting, myotonia, musculoskeletal manifestations and rare spinal defects. Little is reported about spinal deformity associated with this syndrome. PURPOSE: We present a patient with Steinert syndrome complicated by scoliosis. In the literature on muscular dystrophy, other than Duchenne, little mention is given to the problem of scoliosis in general and its treatment in particular. STUDY DESIGN: A case report of a patient with Steinert syndrome associated with thoracic scoliosis and hypokyphosis is presented. methods: A 17-year-old boy presented with King type II right thoracic scoliosis (T5-T11, Cobb angle of 40 degrees) and hypokyphosis--10 degrees. He was treated with posterior stabilization and instrumentation at level T3-L2 with a postoperative correction of the scoliotic curve to 20 degrees. Histopathologic examination of the muscles confirmed the diagnosis of Steinert myotonic dystrophy. RESULTS: At 30-month follow-up, the patient was clinically pain free and well balanced. Plain radiographs showed solid spine fusion with no loss of deformity correction. CONCLUSIONS: scoliosis in Steinert syndrome shares the characteristic of an arthrogrypotic neuromuscular curve and demands the extensive soft tissue release for optimal surgical correction. Intraoperative observations included profound tissue bleeding, abnormally tough soft tissues and a difficult recovery from anaesthesia.- - - - - - - - - - ranking = 0.0021025856840016keywords = pain (Clic here for more details about this article) |
5/16. myotonic dystrophy and pain management of a patient undergoing total abdominal hysterectomy in a metropolitan general hospital.patients with myotonic dystrophy presenting for surgery are exposed to the adverse effects of anaesthetics, especially if the condition has not been diagnosed. Respiratory depression due to increased sensitivity to anaesthetic and opioid drugs is of major concern in patients undergoing major surgery. Meticulous pre- and postoperative management, including pain management, is necessary to avoid morbidity and mortality.- - - - - - - - - - ranking = 0.010512928420008keywords = pain (Clic here for more details about this article) |
6/16. Neonatal myotonic dystrophy associated with prenatal ventriculomegaly. A case report.myotonic dystrophy is an autosomal dominant neuromuscular disease in which the maternal manifestations may be mild or subclinical. However, the congenital form, transmitted only from affected mothers, is reported to have a neonatal mortality rate as high as 50%. Ultrasonographic features of pregnancies with affected fetuses include hydramnios and decreased fetal movement, breathing motion and swallowing. Several reports have also noted an occasional infant with ventriculomegaly or hydrocephalus. It appears that prenatal ventriculomegaly may also be an occasional ultrasonographic finding heralding neonatal myotonic dystrophy.- - - - - - - - - - ranking = 1keywords = breathing (Clic here for more details about this article) |
7/16. Aspiration pneumonia and coma--an unusual presentation of dystrophic myotonia.A 30-year-old female patient presented in a comatose state with clinical and radiographic signs of aspiration pneumonia 16 hours following elective surgery. Subsequent clinical assessment and investigations revealed the characteristic facies, proximal muscle weakness, lenticular opacities, pulmonary function defects, arterial desaturation and abnormal breathing during rapid eye movement (REM) sleep often associated with myotonia dystrophica. Although these characteristic features were evident on clinical examination postoperatively they were not noted in the preoperative assessment. The aspiration pneumonia and coma were unusual presenting features of this disease. Unsuspected myotonia dystrophica should be considered in the differential diagnosis of unexplained respiratory depression, aspiration or comatose state following surgery. Recognition of the disorder during the preoperative assessment is the key to avoiding complications during the perioperative management of such patients.- - - - - - - - - - ranking = 1keywords = breathing (Clic here for more details about this article) |
8/16. Dysphagia and dystrophia myotonica. A case report.A 26-year-old Indian man who presented with a long history of vomiting, upper abdominal pain and dysphagia is described. The dysphagia had been largely overlooked and investigation delayed. The diagnosis of dystrophia myotonica (DM) was apparent on clinical examination and his symptoms responded well to phenytoin therapy. The cause of his symptoms is discussed and the importance of recognizing dysphagia and other gastro-intestinal manifestations of DM is emphasized.- - - - - - - - - - ranking = 0.0021025856840016keywords = pain (Clic here for more details about this article) |
9/16. Absence or impaired response of fetal breathing to intravenous glucose is associated with pulmonary hypoplasia in congenital myotonic dystrophy.Two fetuses of a patient affected with myotonic dystrophy were studied ultrasonically from 28 to 34 weeks' gestation. After a 1-hour observation period, an intravenous injection of 25 gm of 50% glucose solution was given to the mother. Fetal breathing movements were 0% during the control period and increased to only 10% at 90 minutes after the injection of glucose; the episode lasted approximately 30 minutes. The infants, who were delivered at 33 and 35 weeks, had generalized hypotonia, normal arterial cord blood gases, and died shortly after birth from pulmonary insufficiency, in spite of maximum ventilatory support. Postmortem pulmonary hypoplasia was confirmed by a lung weight/body weight ratio of less than 0.019. We postulate that fetal breathing activity and its response to the injection of glucose may be a potential clinical test by which normal fetuses can be differentiated from fetuses affected by neuromuscular disorders, including myotonic dystrophy.- - - - - - - - - - ranking = 6keywords = breathing (Clic here for more details about this article) |
10/16. Anaesthesia for caesarean section in a patient with myotonic dystrophy receiving warfarin therapy.A 31-yr-old parturient with myotonic dystrophy and asthma presented for elective Caesarean section. The patient was receiving warfarin having had two previous episodes of thromboembolism. Anticoagulation was subsequently provided by heparin in the weeks prior to delivery. The combination of the patient's medical conditions and the continuing need for anticoagulation presented a considerable anaesthetic problem in planning anaesthesia and analgesia for both elective and emergency delivery. heparin was discontinued on the day prior to surgery and restarted immediately after surgery. During surgery flowtron anti-embolitic boots were used. warfarin therapy was recommenced on the seventh postoperative day. Anaesthesia for Caesarean section was provided using a combined spinal epidural technique using a separate needle, separate interspace method. Postoperative pain was relieved by using a continuous epidural infusion, transcutaneous nerve stimulation and diclofenac. No new neurological problems arose despite the use of epidural analgesia in the presence of heparin anticoagulation. This method of providing anaesthesia and postoperative analgesia without the use of opioids in an anticoagulated, asthmatic, myotonic parturient has not been described elsewhere.- - - - - - - - - - ranking = 0.0021025856840016keywords = pain (Clic here for more details about this article) |
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