Skin and musculoskeletal Flashcards
Achondroplasia
Considerations
Goals
Pregnancy Considerations
Achondroplasia
Considerations
Potential difficult airway:
Difficult bag mask ventilation, subglottic stenosis, facial anomalies, cervical spine instability (odontoid hypoplasia)
Possible atlantoaxial instability (AAI)
Pulmonary complications:
Kyphoscoliosis, obstructive & central sleep apnea, restrictive lung disease, possible cor pulmonale
Neurologic complications:
Spinal stenosis, hydrocephalus (cervical kyphoscoliosis) +/- VP shunt
Difficult neuraxial anesthesia: kyphoscoliosis, narrow epidural space, prolapsed discs, deformed vertebral bodies
Difficult IV access, regional/neuraxial, monitoring & positioning
Goals
Safe establishment of airway
Avoid neck hyperextension (brainstem compression)
Identification & optimization of cardiorespiratory complications
Goals for pulmonary hypertension if applicable
Pregnancy Considerations
Cesarean section required due to fetal:pelvic disproportion
Titrated epidural (spinal spread is unpredictable) is best, but epidural may also be difficult
Ankylosing Spondylitis
Considerations
Goals
Pregnancy Considerations
Ankylosing Spondylitis
Considerations
Potential difficult airway
Risk of ↓ c-spine mobility/fusion/instability
Atlanto-axial subluxation possible
Multisystem disease with extra-articular features:
Cardiac: aortic insufficiency, myocarditis, conduction defects, cardiomegaly, cardiomyopathy, pericardial effusion
Respiratory: restrictive lung disease from parenchymal fibrosis & chest wall musculoskeletal disease
Neuro: spondylolisthesis (cord compression), uveitis
Heme: anemia of chronic disease
Difficult/impossible regional & neuraxial anesthesia due to poor positioning, axial spine fusion, epidural space obliteration, & underlying radiculopathies/neuropathic pain
Higher risk epidural hematoma (ASRA)
Paramedian might be a better approach
Medications: NSAIDS, steroids, immunomodulators: marrow suppression, platelet dysfunction, renal impairment, need for steroid coverage, ↑ infection risk
Goals
Minimize c-spine manipulation with airway management, consider AFOI, videolaryngoscopy, lighted stylet
Management of multisystem disease features, especially cardiopulmonary
Patient positioning
Recognition of difficult neuraxial & unpredictable response to neuraxial local anesthetics
Pregnancy Considerations
Complicated due to difficult airway & difficult neuraxial technique, have multiple plans in place
For neuraxial: consider paramedian approach & ultrasound guidance
Ehlers Danlos
Background
Considerations
Pregnancy Considerations
Ehlers Danlos
Background
A type of connective tissue disorder
Characterized by skin hyperextensibility, joint hypermobility, & tissue fragility
Classified into 13 subtypes: classical, classical-like, cardiac-valvular, vascular, hypermobile, arthrochalasia, dermatosparaxis, kyphoscoliotic, Brittle Cornea syndrome, spondylodysplastic, musculocontractural, myopathic, periodontal
Vascular type associated with ↑ risk of death
Considerations
Multisystem disease:
High risk of bleeding & friable tissue → affects ETT placement, regional techniques, invasive lines
Desmopressin useful if bleeding
Cardiovascular: mitral regurgitation, proximal aortic dilatation, conduction abnormalities
Spontaneous ruptures: bowel, uterus, or major arteries
Skin laxity/fragility & joint hypermotility
↑ risk pneumothorax: keep low airway pressures
Regional anesthesia is relatively contraindicated due to hematoma risk
Pregnancy Considerations
Very difficult management, requires multidisciplinary effort
High risk of preterm labor, uterine rupture, & hemorrhage
Neuraxial relatively contraindicated, may need GA
If vascular type, recommendations are either termination of pregnancy or cesarean section before 32 weeks
Marfan’s Syndrome
Considerations
Goals
Potential Conflicts
Pregnancy Considerations
Marfan’s Syndrome
Considerations
Airway problems:
Possibly difficult: high arched palate
Potential cervical spine (C1/2) ligamentous instability
TMJ laxity & potential dislocation with laryngoscopy
Multisystem disease:
Cardiovascular dysfunction
Valvular disease (AI, MR, MVP)
Aortic arch aneurysm, aortic rupture & dissection risk
MIs secondary to medial necrosis of the coronary arterioles
Arrhythmias & conduction defects
Respiratory dysfunction:
Scoliosis, pectus carinatum/excavatum & restrictive lung disease, pulmonary hypertension, cor pulmonale
Spontaneous pneumothorax (bullous lung disease), emphysema
Ocular: lens dislocation, retinal detachement, glaucoma
Potentially difficult positioning & regional anesthesia
Rule out dural ectasia
Goals
Minimize ↑ in aortic wall tension through avoidance of sustained ↑ in systolic BP
Establish airway with minimal c-spine movement
Maintain hemodynamic goals of associated valvular lesions
Lung protective ventilation considering restrictive lung disease & potential bullae
Careful positioning (lax joints & potential peripheral nerve injury)
Post-op pain (neuraxial or regional preferrably), post-op disposition
These patients are for elective aortic repair when ≥ 5cm
Potential conflicts
Coexisting aortic root dilation (need to reduce cardiac output) vs MR/AI/LV dysfunction
Pregnancy Considerations
If ∅ symptoms & aorta diameter < 4cm → no special considerations & vaginal delivery ok
If aortic root dilatation/AI → multidisciplinary management with cardiology/cardiac surgery/obstetrics
Some authorities recommend cesarean section for aortic diameter > 4.5cm, labor if > 4 & < 4.5cm
Issues:
Airway might be even more difficult
Neuraxial very good option for vaginal delivery & cesarean section
Aortic dilatation with risk dissection/rupture
Monthly echocardiography during pregnancy
Big focus is to reduce shear forces on aorta
Consider very early epidural
Need invasive monitoring
Drug therapy to prevent tachycardia & elevated BP (keep systolic < 120mmHg) = labetalol good agent
Avoid ergotamine due to hypertension risk
Dural ectasia:
NOT an absolute contraindication to epidural placement but higher risk for failed block & dural puncture & PDPH
Widening of the dural sac, asymptomatic or may present with low back pain, headache, or proximal leg pain, weakness, or numbness
Consider CT/MRI
Osteogenesis Imperfecta
Background
Considerations
Conflicts
Pregnancy Considerations
Osteogenesis Imperfecta
Background
Rare, autosomal dominant, inherited disease of connective tissue that affects bones, the sclera, & the inner ear, bones are extremely brittle
Considerations
Difficult airway: ↓ C-spine mobility, fragile C-spine with fracture risk, mandibular fractures, large head, short neck, brittle teeth
Kyphoscoliosis & pectus excavatum
Possible restrictive lung disease, pulmonary hypertension, & RV dysfunction
Cardiac involvement (aortic regurgitation & mitral regurgitation)
↑ risk of fractures:
BP cuffs may be hazardous
Careful positioning & padding essential
Succinylcholine fasciculations may cause fractures
Bleeding tendency secondary to ↓ platelet function possible
Hypermetabolic: prone to hyperthermia but not malignant hyperthermia (MH) risk
Conflicts
Full stomach/RSI vs. difficult airway
Regional vs. bleeding tendency
Monitoring/positioning vs. brittle bones
Pregnancy Considerations
↑ obstetrical risks:
High incidence cephalo-pelvic-disproportion → mandatory cesarean section
Higher incidence intra-partum & post partum hemorrhage
↑ risk uterine rupture/pelvic fracture
Even more difficult airway
Succinylcholine can cause bone fractures: give defasciculating NdMR or use rocuronium
Potential contraindication to regional due to platelet dysfunction: if patient history reassuring along with platelet count/INR/PTT, go ahead with regional
Rheumatoid Arthritis
Considerations
Goals
Pregnancy Considerations
Approach to atlanto-axial instability
Rheumatoid Arthritis
Considerations
Potential for difficult airway & unstable c-spine:
TMJ involvement, atlanto-axial instability (AAI)*, cricoarytenoid arthritis
Multisystem disease:
Respiratory: interstitial fibrosis, pulmonary hypertension, pleural effusions
Cardiovascular: pericarditis, AI, pericardial effusions, conduction system defects, LV dysfunction, accelerated CAD
CNS: peripheral neuropathy resulting from nerve compression, carpal tunnel syndrome, & tarsal tunnel syndrome are common, chronic pain
Renal: chronic renal failure possible (drugs, amyloidosis, vasculitis)
Heme: chronic anemia, thrombocytopenia, neutropenia (Felty’s syndrome)
Medication side effects: corticosteroids, NSAIDs, immunosuppressives (cyclosporine, cyclophosphamide, methotrexate), stress dose steroids if needed
Technical difficulties with lines & patient positioning, fragile skin
Goals
Safe establishment of airway & preservation of c-spine integrity
Careful positioning & documentation of pre-existing neurologic symptoms
Rule out systemic disease & manage any existing abnormalities, especially cardiopulmonary
Pregnancy Considerations
Obstetric management: vaginal delivery is preferred, cesarean section is reserved for obstetrical indications
Anesthesia:
Regional definitely ok if platelets within normal limits
Document pre-existing injuries
If GA: very cautious airway management!!
*Approach to AAI
Indications for X-ray are controversial
Indications suggested in literature:
Severe disease requiring steroids, methotrexate, & immunosuppressants
Obvious symptoms
Disease >10 years
On X-ray distance from the anterior arch of the atlas to the odontoid process > 3 mm confirms the presence of atlantoaxial subluxation
This abnormality is important, because the displaced odontoid process can compress the cervical spinal cord or medulla or occlude the vertebral arteries. When atlantoaxial subluxation is present, care must be taken to minimize movement of the head & neck during direct laryngoscopy to avoid further displacement of the odontoid process & damage to the spinal cord. It is helpful to evaluate preoperatively whether there is interference with vertebral artery blood flow during flexion, extension, or rotation of the head & cervical spine. This can be accomplished by having the awake patient demonstrate head movement or positioning that can be tolerated without discomfort or other symptoms.
Scleroderma
Considerations
Anaestethic goals/issues
Conflicts
Treatment of Limb Raynaud’s Crisis due to Artrial line
Pregnancy Considerations
Scleroderma
Considerations
Potential difficult airway (microstomia, ↓ neck mobility, bleeding nasal/oral telangiectasia)
Aspiration risk (esophageal dysmotility, hypotonic lower esophageal sphincter)
Multi-system disease:
Cardiovascular: hypertension, coronary disease, myocardial fibrosis & LV failure, arrhythmia
Resp: restrictive lung disease, pulmonary fibrosis, pulmonary hypertension, cor pulmonale
Renal failure & “renal crisis”
Skin: raynaud’s, vasoconstriction, sensitive to cold:
Radial arterial line may be contraindicated
Consequences of dermal thickening, contractures:
Difficult vascular access/positioning/monitoring
Nerve entrapment/pain syndromes
Medications: immunosuppressant, vasodilators (ACE inhibitors), pain medications
Anesthetic Goals/Issues
Secure airway safely & avoid aspiration:
Potential difficulty with bag mask ventilation, laryngoscopy & surgical technique
Consider regional over GA
Recognize several potential precipitants for hemodynamic instability:
Volume depletion, hypertension
Myocardial dysfunction, arrythmia, pulmonary hypertension, cor pulmonale
Avoid precipitants of:
Vasoconstriction episodes: e.g., hypothermia, sympathetic stimulation
Pulmonary hypertension
Address pulmonary disease
Potential for hypoxemia (fibrosis), acute lung injury/barotrauma (restrictive lung disease)
Post-op ventilation
Conflicts
Aspiration vs. cardiac disease & hemodynamic instability
Aspiration vs. difficult airway
? difficult regional vs. risk of post-op ventilation
TEE vs compromised esophagus
Treatment of Limb Raynaud’s Crisis Due to Arterial Line
Emergency situation
Warm limb
Consult vascular surgery
Consider sympathetic block (i.e., stellate ganglion)
Pregnancy Considerations
Essentially as above
Very careful airway management & aspiration prevention
Possible prolonged neural blockade reported in literature so very carefully titrated epidural warranted
Stevens-Johnson Syndrome
Considerations
Treatment
Stevens-Johnson Syndrome
Considerations
Severe life threatening drug reaction with high morbidity/mortality
Possible difficult airway (edema & ulcerations)
Respiratory:
Hypoxia
Hypersecretions
Pulmonary edema & ARDS
Blebs & pneumothorax
Tracheobronchitis
Cardiovascular:
Septic shock common cause of death
Hypovolemia, third spacing
Renal: electrolyte abnormalities, renal failure
Significant pain requiring multimodal analgesia
Treatment
Transfer to ICU/burn unit
Institute supportive care:
Fluid & electrolyte management (fluids: use parkland formula in acute phase)
Analgesia
Nutritional support
Temperature management
Antibiotics for superinfections
Adjunctive therapies (all controversial, no good evidence):
Steroids if indicated
IVIG
Cyclosporine
Plasmapheresis
Anti-TNF monoclonal antibodies
Systemic Lupus Erythematosus (SLE)
Considerations
Pregnancy Considerations
Systemic Lupus Erythematosus (SLE)
Considerations
Potential difficult airway but usually NOT an issue:
C-spine arthritis
Cricoarytenoid arthritis
Recurrent laryngeal nerve palsy
Multisystem complications of SLE:
CNS: central & peripheral sensorimotor & autonomic neuropathies, seizure, stroke, mood, confusion, organic disease, transverse myelitis
Cardiovascular: pancarditis with myocarditis (conduction disease, CHF), pericarditis (effusions/tamponade), non-infectious endocarditis (aortic & mitral regurgitations), accelerated coronary disease, hypertension, pulmonary hypertension
Pulmonary: restrictive lung disease (ILD, pleuritis, effusions), pulmonary hypertension/RV failure, infection, pulmonary hemorrhage
Renal: lupus nephritis, CRF
Hematology: antiphospholipid-Ab syndrome common, risk of thromboembolism, anemia, thrombocytopenia, leukopenia factor deficiency (VIII, IX, XII) with implications for regional anesthesia
Liver dysfunction can occur
MSK: vasculitis, arthritis, joint immobility, migratory polyarthritis
Medications:
Steroids (Cushings/hyperglycemia/adrenal suppression/need for stress dose)
Immunosuppresants/antimalarials
NSAIDs
Anticoagulants/ASA
Potential for acute exacerbation with surgery, stress, infection & pregnancy
Obstetric & cardiac anesthesia, especially in those with aPL antibodies or APS, requires multidisciplinary management at a specialist center
Pregnancy Considerations
↑ risk of preterm labour & intrauterine fetal death
Multidisciplinary management: rheumatology, obstetrics, anesthesia
Potential coagulopathy may contraindicate use of neuraxial → ensure early hematology consult
↑ PTT can have 2 causes:
Presence of lupus anticoagulant: just a lab abN so clinically ok for neuraxial, these patients can actually be hyper-coagulable
Autoantibodies against specific coagulation factors (e.g., VIII, IX, XII): risk of true coagulopathy → neuraxial contraindicated
Document any pre-existing central or peripheral sensorimotor & autonomic neuropathies prior to regional technniques
Potential for neonatal lupus & congenital heart block
Wegener’s Granulomatosis
Considerations
Goals & Conflicts
Wegener’s Granulomatosis
Considerations
Potential difficult airway: laryngeal stenosis, subglottic stenosis, tracheal stenosis, friable bleeding tissue
Multisystem granulomatous disease:
CNS: cerebral aneurysms, peripheral neuropathy
Cardiovascular: cardiac valve destruction, conduction defects, myocardial ischemia
Pulmonary: sinusitis, pulmonary fibrosis, pulmonary hypertension, pneumonia, hemoptysis, & bronchial destruction
Renal: hematuria, renal failure
Possible contraindication to arterial line due to peripheral arteritis
Medications including immunosupressants
Goals & Conflicts
Careful airway management
Document neurologic deficits prior to regional techniques
May present with massive hemoptysis & require emergency lung isolation
