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Anesthesia for Muscular dystrophy

Ashwin Subramaniam, Robert Grauer, David Beilby, Ravindranath Tiruvoipati
Myotonic dystrophy (DM), though rare, can significantly complicate anesthesia due to muscular and extra-muscular involvement. When this condition is compounded by a pheochromocytoma, anesthetizing such patients becomes extra challenging. We present a case report of a 61-year-old lady with congenital DM, with the whole gamut of associated features, was diagnosed with a noradrenaline secreting paraganglionoma following investigation of refractory hypertension. We anesthetized her for an open resection of the lesion...
November 2016: Journal of Clinical Anesthesia
Özlem Özmete, Mesut Şener, Esra Çalışkan, Anış Arıboğan
Duchenne's muscular dystrophy (DMD) is a neuromuscular disease with a progressive course. It is the most common and most severe muscular dystrophic disorder for which the application of anesthesia is critical, due to muscle weakness, and cardiac and pulmonary involvement. Successful application of spinal anesthesia in a 2-year-old boy with DMD undergoing bilateral inguinal hernia repair is described in the present report. It is proposed that spinal anesthesia is an effective alternative to general anesthesia in certain pediatric patients, including those with DMD, for whom general anesthesia poses increased risk...
April 2016: Aǧrı: Ağrı (Algoloji) Derneği'nin Yayın Organıdır, the Journal of the Turkish Society of Algology
Frederic Shapiro, Umeshkumar Athiraman, David J Clendenin, Monica Hoagland, Navil F Sethna
BACKGROUND: The objectives are to review the anesthetic management and anesthetic-related adverse events in patients undergoing muscle biopsy for a broad spectrum of neuromuscular disorders (NMD). AIM: The study aims to assess the hypothesis that perceived awareness of potential anesthesia-induced hyperkalemia and MH in patients with NMD reduces the frequency of such events. METHODS: A 20-year retrospective review of 877 consecutive patients undergoing muscle biopsy to establish diagnoses of NMD has been performed...
July 2016: Paediatric Anaesthesia
Shuhei Matsui, Satoshi Tanaka, Kenkichi Kiyosawa, Toshiyuki Tanaka, Mikito Kawamata
A 39-year-old female with facioscapulohumeral muscular dystrophy (FSHD) was scheduled for thoracoscopic resection of an anterior mediastinal tumor. She had slowly progressive weakness and atrophy in the fascial and shoulder girdle muscles. General anesthesia was induced and maintained with propofol, remifentanil, and fentanyl combined with thoracic paravertebral block. Rocuronium-induced neuromuscular blockade was evaluated with acceleromyography at the corrugator supercilii, masseter, and adductor pollicis muscles...
December 2015: Masui. the Japanese Journal of Anesthesiology
Seung Uk Bang, Yee Suk Kim, Woo Jin Kwon, Sang Mook Lee, Soo Hyang Kim
General anesthesia and central neuraxial blockades in patients with severe Duchenne muscular dystrophy are associated with high risks of complications, including rhabdomyolysis, malignant hyperthermia, hemodynamic instability, and postoperative mechanical ventilation. Here, we describe peripheral nerve blocks as a safe approach to anesthesia in a patient with severe Duchenne muscular dystrophy who was scheduled to undergo surgery. A 22-year-old male patient was scheduled to undergo reduction and internal fixation of a left distal femur fracture...
April 2016: Journal of Anesthesia
Nethra H Nanjundaswamy
Calcinosis cutis is a rare clinical condition wherein calcium is deposited in the layers of the skin. Various types of calcinosis cutis have been described based on histology and location. Airway may be involved only if calcinosis cutis is associated with overlapping syndrome of connective tissue disorders and muscular dystrophy. I report a rare case of dystrophic calcinosis cutis without any connective tissue disorders with the unanticipated difficult airway. A young lady with dystrophic calcinosis cutis of the gluteal region was posted for surgical excision and flap cover procedure under general anesthesia...
September 2015: Anesthesia, Essays and Researches
R V Ranjan, T R Ramachandran, S Manikandan, Roshan John
Limb-girdle muscular dystrophy (LGMD) is an autosomal recessive disorder in which the pelvic or shoulder girdle musculature is predominantly or primarily involved. We report the management of a 27-year-old primigravida with LGMD associated with obesity posted for elective cesarean section. She was successfully managed with epidural anesthesia assisted with noninvasive positive pressure ventilation. She had an uncomplicated intra- and post-operative course.
January 2015: Anesthesia, Essays and Researches
Hirohito Ishii, Kunihide Nakamura, Hiroyuki Nagahama, Masakazu Matsuyama, Jouji Endo, Masanori Nishimura
Duchenne muscular dystrophy (DMD) is an X-linked disease, but female carriers infrequently have some symptoms, who are called manifesting carriers. Here we report a case of a manifesting carrier of DMD with skeletal muscle weakness and cardiac abnormalities such as deterioration of cardiac function and left ventricular dilatation, who successfully underwent cardiac surgery. A 79-year-old female with acute heart failure for severe mitral regurgitation was admitted to our hospital. Surgical replacement of the mitral valve was performed under general anesthesia with intravenous anesthetics and non-depolarizing muscle relaxant...
February 2015: Kyobu Geka. the Japanese Journal of Thoracic Surgery
Satoshi Yamaguchi, Giichiro Ohno, Jiro Kitamura
A 70-year-old man with Becker muscular dystrophy (BMD) underwent laparoscopic rectopexy under general anesthesia. For anesthetic induction, we administered total 0.6 mg · kg-1 of rocuronium with titration. Eight minutes later, train-of-four (TOF) count reached to 0 and the patient was intubated smoothly. One hundred and five minutes later, TOF ratio recovered to 100% and we administered rocuronium 10 mg additionally. Surgery was finished without any problems 95 minutes after thereafter. TOF ratio was 45% and we administered sugammadex 3 mg · kg-1, reversing neuromuscular blockade to TOF ratio 100% within 1...
October 2014: Masui. the Japanese Journal of Anesthesiology
Emmanuelle Salort-Campana, Karine Nguyen, Rafaelle Bernard, Elisabeth Jouve, Guilhem Solé, Aleksandra Nadaj-Pakleza, Julien Niederhauser, Estelle Charles, Elisabeth Ollagnon, Françoise Bouhour, Sabrina Sacconi, Andoni Echaniz-Laguna, Claude Desnuelle, Christine Tranchant, Christophe Vial, Frederique Magdinier, Marc Bartoli, Marie-Christine Arne-Bes, Xavier Ferrer, Thierry Kuntzer, Nicolas Levy, Jean Pouget, Shahram Attarian
BACKGROUND: Facioscapulohumeral muscular dystrophy type 1(FSHD1) is an autosomal dominant disorder associated with the contraction of D4Z4 less than 11 repeat units (RUs) on chromosome 4q35. Penetrance in the range of the largest alleles is poorly known. Our objective was to study the penetrance of FSHD1 in patients carrying alleles ranging between 6 to10 RUs and to evaluate the influence of sex, age, and several environmental factors on clinical expression of the disease. METHODS: A cross-sectional multicenter study was conducted in six French and one Swiss neuromuscular centers...
2015: Orphanet Journal of Rare Diseases
V M Mikhaĭlov, G I Mamaeva
We have studied the influence of NADP+ on routine ECG in 6 months old C57BL/6 and mdx mice. The animals were anestheized by ether before ECG recording. ECG registration was made with the speed of 100 mm per sec. The first ECG recording was made before intraperitoneal NADP+ injection in a dose of 13 or 80 mg/kg. The second ECG recording was made in 10 min after NADP+ injection. Then anesthesia was cut off. The mice were occasionally anestheized 45-60 min later and the third ECG was recorded in 1 h after injection of NADP+...
2013: Tsitologiia
Keitetsu So, Kei Shinoda, Emiko Watanabe, Toru Mashiko, Atsushi Mizota
PURPOSE: Retinal vascular abnormalities are rare in patients with Duchenne muscular dystrophy. We present a patient with Duchenne muscular dystrophy who developed severe proliferative retinopathy for which vitrectomy was successfully performed in one eye. METHOD: Case presentation. A 23-year-old Japanese man with Duchenne muscular dystrophy complicated by cardiac and respiratory insufficiency had reduced vision in both eyes. His best-corrected visual acuity was 0...
2012: Retinal Cases & Brief Reports
Hiromi Matsuda, Masayasu Arai, Hirotsugu Okamoto
We described the anesthetic management of a 17-year-old male patient with Fukuyama congenital muscle dystrophy (FCMD) who underwent surgical repair for scoliosis under total intravenous anesthesia. The patient had severe constructive lung disease (%VC 18.6%). Left ventricular wall motion was reduced (left ventricular ejection fraction 40%). Propofol and remifentanil were continuously infused to maintain anesthesia, but we did not use any muscle relaxant throughout the course. We used arterial pressure-based cardiac output and stroke volume variation as a guide for circulatory management...
June 2014: Masui. the Japanese Journal of Anesthesiology
Thomas Hachenberg, Christine Schneemilch
PURPOSE OF REVIEW: Patients with diseases affecting the central nervous system present a wide range of clinical manifestations increasing the perioperative risk. The following review focused on recommendations for anaesthesiological management in patients with both neurologic and psychiatric diseases. RECENT FINDINGS: The heterogeneity of disorders affecting the central nervous system and the variability of comorbidities make definition of standards for anaesthesiological management difficult...
August 2014: Current Opinion in Anaesthesiology
Emine Arzu Kose, Bulent Bakar, Gokay Ates, Didem Aliefendioglu, Alpaslan Apan
BACKGROUND AND OBJECTIVES: Walker-Warburg Syndrome is a rare, autosomal recessive congenital muscular dystrophy manifested by central nervous system, eye malformations and possible multisystem involvement. The diagnosis is established by the presence of four criteria: congenital muscular dystrophy, type II lissencephaly, cerebellar malformation, and retinal malformation. Most of the syndromic children die in the first three years of life because of respiratory failure, pneumonia, seizures, hyperthermia and ventricular fibrillation...
March 2014: Brazilian Journal of Anesthesiology
Ahmed Abdelgawwad Wefki Abdelgawwad Shousha, Maria Sanfilippo, Antonio Sabba, Paolo Pinchera
Duchenne's muscular dystrophy (DMD) is the most common and severe form of myopathy. Patients with DMD are more sensitive to sedative, anesthetic, and neuromuscular blocking agents which may result in intraoperative and early postoperative cardiovascular and respiratory complications, as well as prolonged recovery from anesthesia. In this case report, we describe a 25-year-old male patient admitted for cholecystectomy under general anesthesia. We induced our anesthesia by oxygen, propofol, fentanyl, and rocuronium bromide...
2014: Case Reports in Anesthesiology
Hiromi Kako, Marco Corridore, John Kean, Jerry R Mendell, Kevin M Flanigan, Joseph D Tobias
BACKGROUND: Duchenne muscular dystrophy (DMD) possesses many potential challenges for anesthetic care. Invasive and noninvasive procedures with corresponding sedation or general anesthesia are frequent and necessary for affected patients. There remains a need for a better agent or agents for procedural sedation in patients with comorbid diseases. This study prospectively evaluated a combination of ketamine with two different doses of dexmedetomidine for sedation during muscle biopsy in patients with DMD...
August 2014: Paediatric Anaesthesia
G Remmers, D W Hayden, M A Jaeger, J M Ervasti, S J Valberg
There are few reports of naturally occurring muscular dystrophy in domestic animals. Herein, we describe a case of muscular dystrophy in a 4-year-old neutered male American domestic shorthair cat that died unexpectedly following anesthesia for an elective surgical procedure. Macroscopic muscular hypertrophy and histologic evidence of myofiber size variation, mineralization, myofiber degeneration, and necrosis were compatible with a diagnosis of muscular dystrophy. Extensive endomysial fibrosis was noted histologically in the diaphragm...
January 2015: Veterinary Pathology
C Vandepitte, Ph Gautier, P Bellen, H Murata, E A Salviz, A Hadzic
Duchenne muscular dystrophy is a progressive neuromuscular disease. Mortality is typically related to combined respiratory failure and dilated cardiomyopathy. Surgery under general anesthesia or deep sedation presents increased risks for pulmonary complications or ventilator dependency postoperatively. We describe the utility of ultrasound guided intercostal nerve blocks for surgery on the chest wall in a patient with Duchenne muscular dystrophy and severe respiratory compromise.
2013: Acta Anaesthesiologica Belgica
Palak Turakhia, Brian Barrick, Jeffey Berman
This article describes the preoperative preparation of patients with neuromuscular disorders. These entities are a relatively rare and diverse group of diseases that can affect various organ systems in addition to the central nervous system. The anesthetic implications for the various comorbidities are varied and can be profound. These patients should be optimized before surgery, with the involvement of a multidisciplinary team of specialists.
November 2013: Medical Clinics of North America
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