ANAESTHESIA DATABOOK APerioperative and Peripartum Manual 3RD EDITION To Chris,without whom the writing of this book would not have been possible.In affectionate memory of the late H Stuart Winsey FRCS,an anaesthetist’s surgeon,and the late Ritabrata Bose (Bosie) FRCA, who valued books. ANAESTHESIA DATABOOK A Perioperative and Peripartum Manual 3RD EDITION Rosemary Mason MB ChB DObst RCOG FRCA Consultant Anaesthetist Swansea NHS Trust Singleton Hospital Swansea SA2 8QA UK © 2001 Greenwich Medical Media Limited 137 Euston Road London NW1 2AA ISBN 1 84110067 6 First published 2001 Apart from any fair dealing for the purposes of research or private study,or criticism or review,as permitted under the UK Copyright Designs and Patents Act 1988,this publication may not be reproduced,stored,or transmitted,in any form or by any means,without the prior permission in writing of the publishers, or in the case of reprographic reproduction only in accordance with the terms of the licences issued by the appropriate Reproduction Rights Organisations outside the UK.Enquiries concerning reproduction outside the terms stated here should be sent to the publishers at the London address printed above. The right of Rosemary Mason to be identified as editor of this work has been asserted by her in accordance with the Copyright Designs and Patents Act 1988. The publisher makes no representation,express or implied,with regard to the accuracy of the information contained in this book and cannot accept any legal responsibility or liability for any errors or omissions that may be made. A catalogue record for this book is available from the British Library. Visit our website at:www.greenwich-medical.co.uk Distributed worldwide by Plymbridge Distributors Ltd Typeset by Phoenix Photosetting,Chatham,Kent Printed by Alden Press Ltd,UK Contents SECTION 1 Beckwith-Wiedmann syndrome 71 Medical disorders and Budd-Chiari syndrome 73 Buerger’s disease 74 anaesthetic problems C A Carcinoid syndrome 76 Achalasia of the oesophagus 3 Cardiac tamponade 79 Achondroplasia 5 Cardiomyopathies;dilated,hypertrophic, Acromegaly 8 restrictive 82 Addiction 12 Carnitine palmitoyl transferase deficiency 88 Addison’s disease;adrenocortical Carotid body tumour 90 insufficiency 14 Central core disease 91 v AIDS;acquired immunodeficiency C1 esterase inhibitor deficiency;acquired 93 syndrome 17 Charcot-Marie-Tooth disease (peroneal Air embolism;see section 2 525 muscular atrophy) 95 Alcoholism 23 Charge association 96 Aldosteronism,Primary 26 Cherubism 98 Alkaptonuria 26 Christmas disease (haemophilia B) 99 Alveolar proteinosis,pulmonary 27 Churg-Strauss syndrome 99 Amniotic fluid embolism 29 Coarctation of the aorta,adult 100 Amphetamine abuse 29 Cocaine abuse 102 Amyloidosis 32 Cockayne syndrome 106 Amyotrophic lateral sclerosis 36 Conn’s syndrome (primary aldosteronism) 107 Ankylosing spondylitis 36 Creutzfeldt-Jakob disease 109 Antiphospholipid syndrome (Hughes’ Cri du Chat syndrome 111 syndrome) 40 Cushing’s syndrome & Cushing’s disease 112 Aortic regurgitation 43 Cystic fibrosis 114 Aortic stenosis 46 Cystic hygroma (lymphangioma) 119 Arnold Chiari (and other Chiari) malformations 49 D Arthrogryposis multiplex congenita 51 Dandy-Walker syndrome 122 Asthma 54 Demyelinating diseases 123 Athletic heart syndrome 59 Dermatomyositis/polymyositis complex 125 Automatic implantable cardioverter Diabetes insipidus 127 defibrillators 62 Diabetes mellitus 129 Autonomic failure 66 Diffuse idiopathic skeletal hyperostosis 134 Down’s syndrome 136 B Drowning and near drowning 139 Becker muscular dystrophy 70 Duchenne muscular dystrophy 142 Anaesthesia Databook: a perioperative and peripartum manual Dystrophica myotonica;and other Heart block 229 myotonic syndromes 147 HELLP syndrome 235 Hereditary angioneurotic oedema 238 E Hereditary haemorrhagic telangiectasia Eaton-Lambert syndrome (Lambert-Eaton (Osler-Weber-Rendu disease) 241 or myasthenic syndrome) 153 Hereditary spherocytosis 245 Ebstein’s anomaly 155 Hers’disease (Cori type VI glycogen Eclampsia and severe preeclampsia 156 storage disease) 246 Ehlers-Danlos syndrome 162 Homocystinuria 247 Eisenmenger’s syndrome 166 Hunter’s syndrome;see Hurler’s and Emery-Dreifuss muscular dystrophy 169 mucopolysaccharidoses 249 Epidermolysis bullosa (recessive dystrophic) 170 Huntington’s disease 249 Epiglottitis 172 Hurler,Hurler-Schie,Schie,and Hunter’s Epilepsy 176 syndromes 250 Hydatid disease 255 F Hypercalcaemia 256 Fabry’s disease 182 Hyponatraemia 258 Fallot’s tetralogy 183 Hypothyroidism 262 Familial dysautonomia (Riley-Day syndrome) 186 I vi Familial periodic paralyses 188 Infectious mononucleosis 267 Fat embolism syndrome 193 Insulinoma 269 Fibrodysplasia (myositis) ossificans progressiva 196 J Fraser syndrome 198 Jehovah’s Witnesses;management for Freeman-Sheldon syndrome 199 surgery 272 Friedreich’s ataxia 200 Juvenile hyaline fibromatosis 277 G K Gaucher’s disease 203 Kawasaki disease (mucocutaneous lymph Giant axonal neuropathy 204 node syndrome) 279 Gilbert’s disease (idiopathic unconjugated Kearns-Sayre syndrome 280 hyperbilirubinaemia) 205 Klippel-Feil syndrome 282 Glomus jugulare tumour 205 Klippel-Trenaunay-Weber syndrome 284 Glucagonoma 207 Glucose-6-phosphate dehydrogenase L deficiency 208 Lambert-Eaton syndrome 287 Glycogen storage diseases 209 Laryngeal papillomatosis 287 Goldenhar’s syndrome 210 Latex allergy 290 Goodpasture’s syndrome 212 Lesch-Nyan disease 291 Gorham’s syndrome 213 Ludwig’s angina 292 Guillain-Barre syndrome 214 Lysergic acid diethylamide (LSD) abuse 294 H M Haemoglobinopathies (sickle cell disease) 218 Malignant hyperthermia 296 Haemophilias A;and haemophilia B 224 Marcus-Gunn jaw winking syndrome 303 Hallervorden-Spatz disease (neuroaxonal Marfan syndrome 303 dystrophy) 228 Maroteaux-Lamy syndrome 307 Contents Mastocytoses 308 Pericardial disease 384 McArdle’s syndrome (myophosphorylase Phaeochromocytoma 384 deficiency) 311 Phenylketonuria 391 Mediastinal masses 313 Pierre-Robin syndrome and the Robin Miller’s syndrome 317 sequence 393 Mitochondrial encephalomyopathies 318 Pituitary apoplexy 397 Mitral valve disease 321 Plasma cholinesterase abnormalities 399 Morquio’s syndrome Polycythaemia vera and secondary (mucopolysaccharidosis type IV) 328 polycythaemias 402 Motor neurone disease (amyotrophic Pompe’s disease;and adult-onset acid lateral sclerosis) 330 maltase deficiency 405 Moyamoya disease 332 Porphyria 407 Mucopolysaccharidoses 335 Prader-Willi syndrome 413 Multiple endocrine neoplasia 336 Protein C deficiency 415 Multiple system atrophy (Shy-Drager Protein S deficiency 418 syndrome) 337 Pulmonary hypertension 419 Muscular dystrophies 339 Pulmonary oedema 423 Myasthenia gravis 340 Myasthenic syndrome 344 Q Myeloma,multiple 344 QT interval syndromes,prolonged 433 vii Myoglobinuria 345 Myotubular (centronuclear) myopathy 345 R Myxoedema 346 Relapsing polychondritis 439 Myxoma,cardiac 346 Rett syndrome 441 Rhabdomyolysis 442 N Rheumatoid arthritis 447 Nemaline myopathy 350 Neurofibromatosis (types 1 & 2; S von Recklinghausen’s disease) 351 Neurogenic pulmonary oedema 354 Sarcoidosis 452 Neuroleptic malignant syndrome 354 Schwartz-Jampel syndrome 454 Noonan’s syndrome 357 Scleroderma 455 Scoliosis 458 O Shy-Drager syndrome 461 Obesity 360 Sick sinus syndrome (bradycardia/ Obstructive sleep apnoea 363 tachycardia syndrome) 461 Opiate addiction 368 Sipple’s syndrome (MEN 2a) 463 Opitz-Frias syndrome 370 Sjogren’s syndrome 464 Osler-Weber-Rendu syndrome 370 Solvent and volatile substance abuse 465 Osteogenesis imperfecta 370 Spinal muscular atrophy 469 Spondyloepiphyseal dysplasia 470 P Sturge-Weber syndrome 472 Pacemakers 375 Systemic lupus erythematosus 473 Paget’s disease 375 Papillomatosis 356 T Parkinson’s disease 356 Takaysu’s arteritis 477 Paroxsysmal nocturnal haemoglobinuria 380 Tetanus 480 Pemphigus vulgaris and foliaceous 382 Thalassaemia 484 Anaesthesia Databook: a perioperative and peripartum manual Thalidomide-related deformities and Anaphylactoid/anaphylactic reactions to other phocomelias 486 intravenous agents 534 Thyrotoxicosis 487 Angioneurotic oedema 542 Torsade de pointes (atypical ventricular Aortocaval fistula 543 tachycardia) 491 Arrhythmias 545 Tracheobronchomegaly 493 Treacher Collins syndrome 494 B Tuberous sclerosis 496 Bronchospasm 548 Turner syndrome 497 C V Carcinoid syndrome 549 Varicella 499 Cardiac tamponade 551 Vipoma 500 Central anticholinergic syndrome 553 Von Gierke’s disease and type 1b glycogenolysis 502 D Von Hippel-Lindau disease 504 Glycine absorption syndrome 555 Von Recklinghausen’s disease 506 Von Willebrand’s disease 506 L Latex allergy 555 W Local anaesthetic toxicity 557 viii Wegener’s granulomatosis 510 Werdnig-Hoffman disease 513 M Williams syndrome 513 Malignant hyperthermia 563 Wolff-Parkinson-White syndrome 516 Masseter muscle rigidity 566 Methaemoglobinaemia 569 Z Zenker’s diverticulum 520 P Phaeochromocytoma 572 Pituitary apoplexy 576 Pulmonary oedema 577 SECTION 2 S Emergency conditions arising Subdural block,accidental 577 during anaesthesia or in the Suxamethonium apnoea 580 immediate perioperative T period Thyrotoxic crisis or storm 581 Torsade de pointes 584 A Total spinal anaesthesia,accidental 586 Addisonian crisis 523 TURP syndrome 588 Air embolism 525 Amniotic fluid embolism 531 Appendix 594 List of abbreviations A C4 Fourth component of ACE Angiotensin-converting enzyme complement AChE Acetylcholinesterase CABG Coronary artery bypass graft ACLA Anticardiolipin antibody cAMP Cyclical adenosine ACTH Adrenocorticotrophic hormone monophosphate ADH Antidiuretic hormone CAPD Continuous ambulatory peritoneal AF Atrial fibrillation dialysis AFE Amniotic fluid embolism ChE Cholinesterase AIDS Acquired immune deficiency C1EI C1 esterase inhibitor syndrome CMV Cytomegalic virus AIP Acute intermittent porphyria CNS Central nervous system ix ALA d-Aminolaevulinic acid (synthase) CPAP Continuous positive airway AMP Adenosine monophosphate pressure APA Antiphospholipid antibody CPB Cardiopulmonary bypass APLS Antiphospholipid antibody CK Creatine kinase syndrome CPR Cardiopulmonary resuscitation APTT Activated partial thromboplastin CRF Corticotrophin-releasing factor time CSF Cerebrospinal fluid APUD Amine precursor uptake and CT Computerised tomography decarboxylation CVP Central venous pressure ARDS Adult respiratory distress syndrome CXR Chest X-ray ASD Atrial septal defect AV Atrioventricular D A-V Arteriovenous DDC 3,5-Dicarbethoxy-1,4- AVP Arginine vasopressin dihydrocolidine AZT Zidovudine (‘Retrovir’) DDAVP Desmopressin (1-desamino-8-D- arginine vasopressin) B DMD Duchenne muscular dystrophy BAL Bronchoalveolar lavage DNA Desoxyribose nucleic acid BBB Bundle branch block DVI Digital vascular imaging bd Twice per day DVT Deep venous thrombosis BG Blood glucose BMR Basal metabolic rate E BP Blood pressure EBV Epstein-Barr virus ECF Extracellular fluid C ECG Electrocardiogram C1 First component of complement ECM External cardiac massage C2 Second component of ECMO Extracorporeal membranous complement oxygenation