Anaesthetic management of a parturient with severe muscular dystrophy, lumbar lordosis and a difficult airway

Treacher-Collins syndrome (TCS) is a rare congenital disease known to be associated with a difficult airway and represents some of the most hazardous and difficult challenges that anaesthetists may encounter during their entire practice of anesthesia. Successful anaesthetic management of a case with Treacher-Collins syndrome posted for laparoscopic cholecystectomy under general anaesthesia is presented in this report. DOI: http://dx.doi.org/10.3329/jbsa.v23i2.18178 Journal of BSA, 2009; 23(2): 72-75

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Anaesthetic challenges and difficult airway in a child with Poland’s syndrome

Indian Journal of Clinical Anaesthesia ◽

10.18231/j.ijca.2021.091 ◽

2021 ◽

Vol 8 (3) ◽

pp. 479-482

Author(s):

Aikta Gupta ◽

Bhumika Kalra

Keyword(s):

Malignant Hyperthermia ◽

Difficult Airway ◽

Preoperative Evaluation ◽

Anaesthetic Techniques ◽

Anaesthetic Management ◽

Neuromuscular Blocking ◽

Poland’S Syndrome ◽

Clinical Disorder ◽

Anaesthetic Care ◽

Poland's Syndrome

: Poland’s syndrome is an uncommon clinical disorder with a typical musculoskeletal deformity of thorax and ipsilateral upper limb with a variable spectrum of associated anomalies. Although patients of this musculoskeletal disorder have several features with potential anaesthetic risks including malignant hyperthermia, previous reports about anaesthetic management of these patients are limited. : We report the successful anaesthetic management of a child of Poland’s syndrome posted for surgical correction of club foot who had an anticipated difficult airway alongwith unilateral chest wall defect with paradoxical breathing and ipsilateral syndactyly and hence, posing challenges in the insertion of endotracheal tube, ventilatory management, regional anaesthesia and prevention of any risk of development of malignant hyperthermia. Paramount in the anaesthetic care of such patients is a thorough preoperative evaluation and a cautious intraoperative anaesthetic management. Avoidance of halogenated inhalational agents and depolarising neuromuscular blocking drugs, and use of controlled ventilation and regional anaesthetic techniques are recommended. The possibility of a compromised airway should always be kept in mind while anaesthetising these patients.

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Anaesthesia and Serum Creatine Phosphokinase Levels in Patients with Duchenne's Pseudohypertrophic Muscular Dystrophy

Anaesthesia and Intensive Care ◽

10.1177/0310057x7200100209 ◽

1972 ◽

Vol 1 (2) ◽

pp. 150-153 ◽

Cited By ~ 23

Author(s):

W. C. Richards

Keyword(s):

Cardiac Arrest ◽

Muscular Dystrophy ◽

Creatine Phosphokinase ◽

Anaesthetic Management ◽

Serum Creatine Phosphokinase ◽

Wide Range ◽

Duchenne's Dystrophy

Forty-three patients with Duchenne's pseudohypertrophic muscular dystrophy receiving a total of 61 general anaesthetics were reviewed to assess the effect of the anaesthetic with particular regard to rise in temperature and its relationship, if any, to serum creatine phosphokinase (C.P.K.) levels. All popular forms of anaesthetic were administered to these patients, who had a wide range of C.P.K. levels. No patient developed hyperpyrexia or had a cardiac arrest. The features of Duchenne's dystrophy are described, the complications reviewed in the literature are outlined, and some recommendations about the anaesthetic management are made.

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Anaesthetic management of a parturient with spondylothoracic dysostosis

BMJ Case Reports ◽

10.1136/bcr-2019-232964 ◽

2020 ◽

Vol 13 (1) ◽

pp. e232964

Author(s):

Reine Zbeidy ◽

Nathalia Torres Buendia ◽

Fouad G Souki

Keyword(s):

Tracheal Intubation ◽

Lung Disease ◽

Difficult Airway ◽

Caesarean Delivery ◽

Multidisciplinary Approach ◽

Anaesthetic Management ◽

Spine Deformity ◽

Restrictive Lung Disease ◽

Rare Congenital Disorder ◽

Perioperative Planning

Spondylothoracic dysostosis is a rare congenital disorder characterised by multiple vertebral malformations, shortening of the spine and fusion of the ribs at the costovertebral junction. These abnormalities create anaesthetic challenges due to difficult airway, severe restrictive lung disease and spine deformity necessitating a multidisciplinary approach and careful perioperative planning. We present the perianaesthetic management of a parturient with spondylothoracic dysostosis who successfully underwent preterm caesarean delivery under general anaesthesia with awake videolaryngoscopy-assisted tracheal intubation.

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In Vitro Contracture Test Results and Anaesthetic Management of a Patient with Emery-Dreifuss Muscular Dystrophy for Cardiac Transplantation

Case Reports in Anesthesiology ◽

10.1155/2012/349046 ◽

2012 ◽

Vol 2012 ◽

pp. 1-4 ◽

Cited By ~ 1

Author(s):

Frank Schuster ◽

Carsten Wessig ◽

Christoph Schimmer ◽

Stephan Johannsen ◽

Marc Lazarus ◽

...

Keyword(s):

Muscular Dystrophy ◽

General Anaesthesia ◽

Cardiac Transplantation ◽

Calcium Release ◽

Pectoralis Major Muscle ◽

Anaesthetic Management ◽

Neuromuscular Disorder ◽

Test Results ◽

In Vitro Contracture Test

Emery-Dreifuss muscular dystrophy (EDMD) is a hereditary neuromuscular disorder characterized by slowly progressive muscle weakness, early contractures, and dilated cardiomyopathy. We reported an uneventful general anaesthesia using total intravenous anaesthesia (TIVA) for cardiac transplantation in a 19-year-old woman suffering from EDMD. In vitro contracture test results of two pectoralis major muscle bundles of the patient suggest that exposition to triggering agents does not induce a pathological sarcoplasmic calcium release in the lamin A/C phenotype. However, due to the lack of evidence in the literature, we would recommend TIVA for patients with EDMD if general anaesthesia is required.

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