RT Book, Section A1 Sankholkar, Kriti A2 Atchabahian, Arthur A2 Gupta, Ruchir SR Print(0) ID 57260792 T1 Chapter 38. Coexisting Diseases with Anesthetic Implications T2 The Anesthesia Guide YR 2013 FD 2013 PB The McGraw-Hill Companies PP New York, NY SN 978-0-07-176049-2 LK accessanesthesiology.mhmedical.com/content.aspx?aid=57260792 RD 2024/03/29 AB Table Graphic Jump Location|Download (.pdf)|PrintDiseaseDisease characteristicsAnesthetic considerationsAchondroplasiaAutosomal dominantMost common cause of dwarfismOdontoid hypoplasia, atlantoaxial instability, bulging discs, and severe cervical kyphosisCentral sleep apnea and OSA may be a featurePulmonary HTNCor pulmonaleHydrocephalusProportionately smaller airwaysPotential airway difficulties, cervical spine instability, and the potential for spinal cord trauma with neck extension; obtain cervical spine x-rays. Consider FOB intubationConsider PFTsETT size should be weight basedRelative contraindication to neuraxial blocksAlport syndrome (hereditary nephritis)Ocular abnormalities and hearing lossDisease culminates in systemic HTN and renal failureIntraglomerular pressure may be lowered by ACEISame anesthetic considerations as in renal failure and HTNAnkylosing spondylitisChronic, progressive, inflammatory disease spine articulations and adjacent soft tissuesOccurs more commonly in menSpine exam reveals skeletal muscle spasm, loss of lordosis, and decreased mobilityWeight loss, fatigue, low-grade fever, conjunctivitis, and uveitis are systemic signsPulmonary involvement may reveal apical cavitary lesions with fibrosis and pleural thickening with ↓ pulmonary compliance and vital capacity secondary to arthritis; restrictive syndrome (30–45% of patients after 15 years)Associated psoriasisTMJ involvement with limitation of mouth openingCardiovascular: AI (up to 10% after 30 years), dysrhythmias, conduction abnormalitiesRenal insufficiency: rareMay need awake FOB intubation if cervical spine is involved (major kyphosis)Restrictive lung disease may result in higher airway pressuresSpinal deformity and instability: careful positioning and paddingNeuraxial anesthesia is acceptable but may be difficultNeurologic monitoring should be considered for corrective spine surgeryArthrogryposisOne type, autosomal recessive, is a myopathyOther type: polymalformative syndromeMultiple extremity contractures due to periarticular fibrosis with muscular atrophyPossible TMJ and spine involvement (cervical spine ⇒ intubation issues; thoracic spine ⇒ restrictive syndromePlan for difficult intubationCareful positioningSensitive to sedatives/anestheticsMyopathy: avoid succinylcholine; monitor NMBPossible MHMonitor postoperatively for possible respiratory failureBartter syndromeAutosomal recessive renal diseaseHypokalemic, hypochloremic metabolic alkalosisNormal blood pressure, no edemaElevated plasma renin and aldosteroneTreatment aimed at prostaglandin synthetase inhibition with indomethacinPatients may be treated with indomethacin, β-blockers, and spironolactoneMonitoring of urine output (compensate for polyuria), arterial blood pressure, and CVP is indicatedPatients are resistant to effects of exogenous angiotensin and norepinephrineWatch for hypokalemia and hypotoniaBullous dermatitis (Lyell, Stevens–Johnson, pemphigus, bullous pemphigoid, etc.)Separation of epidermisSkin and mucous membrane bullous lesions may lacerateMost severe form is Stevens–Johnson syndromeMay be associated with viral infection, streptococci, cancer, autoimmunity, collagen vascular diseases or drug-inducedTachycardia, high fever, and tachypnea may be featuresTreatment may involve long-term corticosteroidsAssociated diseases include porphyria, amyloidosis, multiple myeloma, DM, and hypercoagulable statesMalnutrition, anemia, electrolyte derangements, and hypoalbuminemia are commonUpper airway and trachea may have lesions → airway management should be approached with cautionRegional anesthesia may be given if skin is intactPulmonary blebs may be present → ↑ risk of PTXAvoid NO if blebs are suspectedSteroid supplementation may be necessary intraoperativelyDehydration and hypokalemia may be present due to skin lossesAdequately protect skin at sites of taping, tourniquet, BP cuff, face maskSuture in all linesDermatomyositisInflammatory myopathy and skin changesProximal muscle weaknessDysphagia, pulmonary aspiration, and pneumonia from pharyngeal and respiratory muscle weaknessHeart block, myocarditis, and left ventricular dysfunction may also be presentPulmonary aspiration is a riskNormal response to both SCh and NDMRs has been shownDistal tubular acidosisAutosomal dominantInability to excrete H+ (exchanged for K), with hyperchloremic hypokalemic acidosisRenal Ca loss with ...