Connective tissue and autoimmune disease

{}

Marfans Syndrome

Marfan’s_Calgory

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 preferably), 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

Clinical Manifestations and Anaesthetic Implications of Marfan Syndrome (MFS)

Organ system	Clinical features	Anaesthetic implications	Management
Ocular	– Ectopia lentis (lens dislocation) – Retinal detachment – Myopia – Glaucoma	Increased risk of ocular injury	Careful ocular protection and padding
Musculoskeletal	– Tall stature – Increased arm span to height ratio – Arachnodactyly – Scoliosis – Pectus deformity – Elongated face – High arched palate – Joint hypermobility – Protrusio acetabula (reduced movement in hip joint) – Pes planus – Dural ectasia (widening of the dural sac surrounding the spinal cord)	– May result in ventilatory complications – Challenging regional anaesthesia (RA) – Difficult airway management – Joint subluxation and dislocation – Can restrict spread of local anaesthetic resulting in spinal failure – Increased risk of accidental dural puncture	– Careful airway assessment and planning – Ensure careful handling and positioning – Consider MRI for identification and delineation
Cardiovascular	– Aortic root dilatation – Aortic dissection – Mitral valve prolapse – Aortic valve regurgitation – Tricuspid valve prolapse – Dilatation of the main pulmonary artery – Congestive heart failure*	– Increased risk of aortic dissection – Increased morbidity and mortality	– Thorough multi-disciplinary preoperative assessment and investigation, e.g. ECHO – Continue β-blockers – Obtund pressor response on intubation and extubation
Pulmonary	– Pulmonary hypertension – Spontaneous pneumothorax – Emphysema*		Lung-protective ventilation strategies
Other	– Recurrent hernias – Striae atrophicae

Note: Features marked with an asterisk () are critical to assess as they can significantly impact anaesthetic care.*

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 anaesthesia 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

Clinical Manifestations and Anaesthetic Implications of Ehlers-Danlos Syndrome (EDS)

Ehlers-Danlos type	Major clinical manifestations	Anaesthetic implications	Management
Classic	– Marked joint hypermobility – Skin hyperextensibility – Atrophic scarring	– Joint subluxation and dislocation	Ensure careful handling and positioning
Hypermobile	– Marked joint hypermobility – Minor skin findings
Vascular	– Thin, translucent skin – Arterial/intestinal/uterine fragility or rupture	– Caution with invasive monitoring—Increased risk of dissection – Increased bleeding risk	– Avoid adhesive tapes and dressings – Non-invasive monitoring (NIM) preferred – Use of ultrasound to establish invasive monitoring – Consider use of cell saver, tranexamic acid – Avoid intramuscular (i.m.) injections and tourniquets
Kyphoscoliotic	– Hypermobility of small joints – Extensive bruising – Tendon and muscle rupture – Characteristic facial appearance – Generalized joint hypermobility – Congenital hypotonia – Congenital and progressive scoliosis	– May result in ventilator complications – Challenging regional anaesthesia (RA)	– Consider neuromuscular monitoring
Arthrochalasia	– Scleral fragility and rupture of the ocular globe – Generalized joint hypermobility with recurrent subluxations – Congenital bilateral hip dislocation	– Increased risk of ocular injury	– Careful ocular protection and padding
Dermatosparaxis	– Severe skin fragility – Sagging, redundant skin	– Difficult intravenous (i.v.) access
Other features	– Recurrent temporomandibular joint (TMJ) dislocations, occipito-atlantoaxial instability – Increased risk of pneumothorax – Valvular abnormalities (typically in EDS types I, IV, VI) – Reduced effect of local anaesthesia – Tarlov cysts (typically present in EDS type I, II, III, VIA)	– Difficult airway management – Reduced effect of topical and RA – Increased risk of dural puncture	– Careful airway assessment and planning – Lung-protective ventilation strategies

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

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

Anaesthetic Considerations in Ankylosing Spondylitis (AS) Patients

Preoperative Assessment

History & Examination:
- Focus on disease duration, current treatments, and level of disability.
- Consider the risk of fractures due to severe osteoporosis, particularly in the vertebrae and hips.
Airway Assessment:
- Challenges:
  - Fixed flexion deformities of the neck and vertebral column make airway management difficult, including laryngoscopy and intubation.
  - Temporomandibular joint (TMJ) involvement restricts mouth opening.
  - Cricoarytenoid joint involvement and hoarseness may indicate airway complications, including potential laryngeal inlet distortion.
  - Risk of C5/6 spinal fractures with minimal trauma.
  - Atlanto-axial subluxation can complicate airway management.
- Management:
  - Consider awake tracheal intubation (ATI) or fiberoptic intubation as preferred techniques.
  - Fiberoptic nasendoscopy may be useful for assessing cricoarytenoid involvement.
Cardiorespiratory Involvement:
- Cardiac:
  - Monitor for aortic regurgitation, conduction abnormalities, myocarditis, pericarditis, and pericardial effusions.
  - Consider ECG and ECHO preoperatively to assess aortic root dilation and other potential issues.
- Respiratory:
  - Limited chest expansion due to costo-vertebral involvement and fibrosing alveolitis may impair pulmonary function.
  - Assess for interstitial lung disease or bronchiectasis; consider preoperative pulmonary function tests (PFTs).
Neurological Assessment:
- Document any existing neurological deficits, including spinal cord compression and peripheral nerve lesions.
- Be cautious with head positioning, particularly in patients with vertebrobasilar insufficiency.
- Consider epilepsy and assess for signs of vertebrobasilar insufficiency preoperatively.

Intraoperative Considerations

Positioning:
- Positioning, especially prone, is challenging and requires careful planning with maximal comfort for the patient.
- Use careful padding to avoid pressure injuries, and avoid exacerbating any existing spinal deformities.
Neuraxial Anesthesia:
- Challenges:
  - Difficult due to ossification of interspinous ligaments, ankylosis, and bony bridges.
  - Epidural space obliteration and spinal fusion may make neuraxial blocks impossible or high-risk.
- Approach:
  - Consider lateral or paramedian approaches, often with ultrasound guidance, for any neuraxial anesthesia attempts.
  - Avoid epidurals due to an increased risk of epidural hematoma.
  - Caudal approaches can be considered as the sacrococcygeal membrane is typically spared.
Potential for Blood Loss:
- Patients on NSAIDs may have increased blood loss risk, especially when positioned prone.
- Manage carefully to avoid excessive intraoperative bleeding.
Pharmacological Considerations:
- Drug Therapies:
  - Continue NSAIDs preoperatively (discontinuation may cause vascular rebound and increase myocardial infarction risk).
  - Stop methotrexate (MTX) 48 hours prior to surgery.
  - Discontinue TNF-blockers for 3-5 half-lives before surgery due to increased infection risk.
- Be aware of the implications of disease-modifying antirheumatic drugs (DMARDs), NSAIDs, and biologicals on perioperative management.

Postoperative Management

Analgesia:
- Multimodal analgesia (MMA) should be used.
- Patient-controlled analgesia (PCA) may be necessary if opioids are required, but this warrants close monitoring in a high-dependency unit (HDU) due to the risk of opioid-related complications.
Mobilization & Respiratory Care:
- Early mobilization is crucial to avoid complications.
- Use incentive spirometry and breathing exercises postoperatively to optimize pulmonary function.
Positioning:
- Ensure the patient is positioned for maximum comfort during the postoperative period.

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 Consideration

↑ 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

Anaesthetic Considerations for Patients with Rheumatoid Arthritis (RA)

1. Airway Management

Challenges:
- Limited oral opening due to temporomandibular joint (TMJ) involvement.
- Arthritis of the cervical spine with atlantoaxial instability increases the risk of cervical spine injury during airway manipulation.
- Fixed flexion deformities, cricoarytenoid joint involvement, and risk of C5/6 spinal fractures may complicate intubation.
- Airway management may be difficult, requiring techniques like fiberoptic intubation.
Considerations:
- Fiberoptic intubation may be the preferred technique, especially in patients with cervical spine involvement.
- Assess for signs of hoarseness, which may indicate cricoarytenoid arthritis.

2. Respiratory Considerations

Pulmonary Involvement:
- Diffuse interstitial fibrosis, fibrosing alveolitis, and pleural disease with intrapulmonary nodules are common, leading to restrictive lung disease and limited chest expansion.
- Reduced chest wall compliance and potential pleural effusions may impair ventilation.
- Patients with severe respiratory involvement may require postoperative ventilation.
Preoperative Assessment:
- Pulmonary function tests (PFTs) may be required to assess baseline respiratory function.
- Post-extubation edema is a risk due to cricoarytenitis.

3. Cardiovascular Considerations

Involvement:
- RA can cause pericardial effusions, pericarditis, myocarditis, amyloidosis, and coronary artery disease.
- Aortic regurgitation, pulmonary hypertension, restrictive pericarditis, and left ventricular failure are significant risks.
- Increased risk of cardiovascular events, especially in patients with seropositive disease (Rheumatoid factor, anti-cyclic citrullinated peptide antibodies), heart failure, or rheumatoid cachexia.
Preoperative Management:
- Conduct ECG and echocardiogram (ECHO) for cardiac assessment.
- Consider increased red-cell transfusion requirements due to pre-existing anaemia.

4. Haematological and Drug Considerations

Anaemia:
- Normocytic normochromic anaemia and iron deficiency anaemia are common, especially with chronic disease and non-steroidal anti-inflammatory drug (NSAID) use.
- Increased risk of gastrointestinal and renal complications due to NSAIDs.
Immunosuppression and Steroid Use:
- Long-term steroid use (e.g., glucocorticoids) necessitates supplementation during surgery to avoid adrenal insufficiency.
- Disease-modifying antirheumatic drugs (DMARDs) and biological agents increase the risk of infection, particularly during prosthesis insertion.
Drug Management:
- Glucocorticoid supplementation may be necessary.
- Be cautious of NSAID-related gastrointestinal and renal side effects, requiring careful monitoring.
- Consider the potential for reduced response to regional anaesthesia due to chronic inflammation or joint deformities.

5. Neurological and Ocular Considerations

Peripheral and Central Nervous System:
- Cervical spine involvement with atlantoaxial subluxation may lead to spinal cord compression, requiring careful preoperative imaging and head positioning.
- Peripheral neuropathy and autonomic dysfunction are associated with RA.
- Vertebrobasilar insufficiency can complicate positioning and neurological assessments.
Ocular Involvement:
- Dry eyes and keratoconjunctivitis sicca (Sjogren’s syndrome) are common, requiring eye protection during anaesthesia.

6. Neuraxial Anaesthesia

Challenges:
- Difficulty performing neuraxial blocks due to ossification of the interspinous ligaments, vertebral ankylosis, and spinal fusion.
- Increased risk of higher-than-expected spinal block levels.
- Epidural space obliteration and a higher risk of epidural haematoma complicate neuraxial techniques.
Approach:
- Paramedian approach is preferred for neuraxial techniques, but regional anaesthesia may not be possible in all cases.
- Extreme care must be taken to avoid epidural haematoma.

7. Positioning and Fragile Skin

Positioning Difficulties:
- Deformities and fixation of joints make patient positioning challenging, particularly prone positioning.
- Joint deformities and pain may limit mobility, requiring careful handling and padding.
Skin Fragility:
- Very fragile skin is prone to injury, necessitating extreme care during positioning and handling.

8. Postoperative Management

Pain Management:
- Patients often experience significant pain and require multimodal analgesia.
- Consider patient-controlled analgesia (PCA) or regional techniques where feasible.
Ventilatory Support:
- Patients with significant respiratory involvement or myopathy may require postoperative ventilatory support.
Neurological Monitoring:
- Postoperative neurological damage, especially in those with cervical spine instability, should be closely monitored.

This comprehensive summary outlines the critical anaesthetic considerations for managing patients with rheumatoid arthritis, addressing preoperative, intraoperative, and postoperative care to mitigate risks associated with airway, cardiovascular, respiratory, neurological, and musculoskeletal involvement.

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

Musculoskeletal Changes

• Cricoarytenoid joint involvement → dyspnoea, stridor, hoarseness ± severe upper airway obstruction
• Laryngeal mass (also 2° to Cricoarytenoid RA) → significant destruction of surrounding structures
• Laryngeal amyloidosis and rheumatoid nodules → laryngeal obstruction
• TMJ involvement → limitated mouth opening ⇒ direct laryngoscopy impossible!
• Atlanto-axial subluxation

Cervical Spine Instability

Anterior (more common) – C1 moves forward on C2 due to transverse ligament destruction ⇒ spinal cord compression. ∴ Avoid neck flexion (e.g. Pillow). Advisable to keep the upper c-spine supported whilst the head is not moved anteriorly, e.g. using a doughnut head ring. Direct laryngoscopy should be tolerated.
Posterior – C1 moves backward on C2 due to odontoid peg destruction. ∴ Avoid neck extension → avoid direct laryngoscopy. Keep c-spine flexed. Consider ATI.

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

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 Considerations

Raynaud’s phenomenon: Peripheral vasoconstriction → Avoid hypothermia
Dermal thickening, calcifications, contractures: Peripheral IV difficulties
Skin tightening, microstomia, decreased neck flexibility: Difficult airway management (consider AFOI – awake fiberoptic intubation)
Telangiectasias: Oral or nasal bleeding
Esophageal dilatation, decreased LES tone: Aspiration risk
Intestinal malabsorption: Decreased vitamin K-dependent clotting factors
Restrictive pulmonary disease: Increased positive airway pressure, increased oxygen concentration, potential extubation delays
Myocardial fibrosis, pulmonary hypertension: Ventricular hypertrophy, diastolic dysfunction, conduction defects, coronary vasospasm → Invasive monitoring may be necessary
Renal disease: Hypertension, decreased renal clearance

NB: Use regional anesthesia if possible, provided coagulation status allows.

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

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)

Introduction

Considerations

Preoperative Management for Systemic Lupus Erythematosus (SLE)

History

Review the disease activity index, organ damage, and drug history, particularly focusing on steroids, immunosuppressants, antimalarials, NSAIDs, and anticoagulants.

Examination

Comprehensive cardiovascular, respiratory, and neurological assessments, including testing for atlantoaxial subluxation, joint and skin manifestations, and systemic complications.

Airway

Generally no issues, but assess for:
- Mouth ulcers
- Subglottic stenosis
- Cervical spine (C-spine) and cricoarytenoid arthritis
- Recurrent laryngeal nerve palsies

Cardiovascular (CVS)

Increased risk of pericarditis (effusions), myocarditis (conduction defects, heart failure), and endocarditis (aortic regurgitation, mitral regurgitation).
Higher prevalence of coronary artery disease (CAD) and Raynaud’s phenomenon.

Respiratory

Higher incidence of infections, pulmonary embolism (PE), pleuritis, pleural effusions, and fibrotic lung disease.

Neurological

Risk of cranial and peripheral nerve lesions (due to arteritis and ischemia).
Potential for depression, psychosis, seizures, and increased risk of stroke with antiphospholipid antibodies.

Renal

Lupus nephritis and progression to chronic renal failure.

Hepatic

Possible liver dysfunction.

Hematological

Risk of clotting disorders or hypercoagulable states.
Thrombocytopenia and prolonged aPTT common.
Note: Antiphospholipid syndrome present in one-third of patients, leading to a hypercoagulable state despite prolonged aPTT.

Investigations

Full Blood Count (FBC): Assess for anemia, thrombocytopenia, and leucopenia.
Serum Electrolytes, Creatinine, Urea: Renal function assessment for lupus nephritis.
Liver Function Tests: Screen for drug-induced or autoimmune hepatotoxicity.
Coagulation Studies: Investigate for prolonged aPTT and lupus anticoagulant.
Anti-dsDNA and Complement Levels: Reflect lupus activity based on baseline comparisons.
Urinalysis: Check for proteinuria, red cells, and cellular casts as indicators of renal involvement.
Electrocardiogram (ECG): Assess for silent ischemia, myocarditis, pericarditis, and conduction abnormalities.
Chest Radiograph: Look for pleural effusion, interstitial pneumonitis, pericardial effusion, or subglottic stenosis.

Intraoperative Management for SLE

Airway

Generally not problematic, but assess for:
- C-spine and cricoarytenoid arthritis
- Recurrent laryngeal nerve palsy
- Minimize airway manipulation to reduce the risk of inflammation and post-extubation airway edema.
- Be aware of potential vocal cord paralysis, subglottic stenosis, or laryngeal edema making intubation challenging.
- Laryngeal Mask Airway (LMA) use can help reduce airway trauma in susceptible patients.

Cardiac

5-lead electrocardiography and intra-arterial blood pressure monitoring to observe for:
- Pericarditis, endocarditis, valvular vegetations
- Conduction defects
- Accelerated coronary artery disease

Temperature

Maintain normothermia to prevent Raynaud’s phenomenon, as hypothermia can induce vasospasm in these patients.

Respiratory

Monitor for:
- Pleuritis, pleural effusion
- Interstitial lung disease

Renal

Maintain renal protective strategies:
- Monitor for lupus nephritis
- Ensure adequate urine output and avoid hypotension.
- Use nephrotoxic drugs with caution.

Hematological

Anemia and thrombocytopenia are common.
Assess platelets and APTT preoperatively, especially before administering spinal or neuraxial anesthesia. If APTT is raised, consult with hematology regarding the safety of neuraxial anesthesia.
Antithrombotic prophylaxis is crucial in patients with antiphospholipid syndrome or previous thromboembolic events. Consult with a hematologist for therapeutic anticoagulation management.

Immunosuppression

Higher risk of infection due to immunosuppressive therapy, necessitating strict asepsis.
Consider steroid supplementation in patients on long-term corticosteroids to prevent adrenal suppression.

Muscle Relaxants

Exercise caution with muscle relaxants, particularly with medications like azathioprine and cyclophosphamide, which may interact.

Regional Anesthesia

Document any neuropathies prior to neuraxial or regional anesthesia.
Review coagulation status before proceeding with regional anesthesia, ensuring safe levels of platelets and APTT.

Patient Positioning

Ensure careful positioning due to the risk of peripheral neuropathies and osteoporosis.

Eye Protection

Protect against corneal abrasions, especially in patients with Sjogren’s syndrome.

Postoperative Management for SLE

Pain Management
- Early involvement of a pain specialist to minimize systemic side effects.
Antithrombotic Prophylaxis
- Continuation of mechanical and pharmacological prophylaxis depending on surgical factors.

Pharmacotherapy in Systemic Lupus Erythematosus (SLE) and Anesthetic Implications

Drug	Indication	Anesthetic Implications
Anti-malarials (hydroxychloroquine)	Cutaneous SLE, Pleuritis, Pericarditis, Arthritis	Retinotoxicity, Neuromyotoxicity, Cardiotoxicity
Corticosteroids (prednisone, methylprednisolone)	Cutaneous SLE, Nephritis, Arthritis	Hyperglycemia, Hypercholesterolemia, Hypertension, Osteoporosis
Aspirin/NSAIDs	Pleuritis, Arthritis, Mesenteric vasculitis	Peptic ulceration, Platelet inhibition, Renal impairment
Cyclophosphamide	Nephritis, NPSLE	Hemorrhagic cystitis, Myelosuppression, Cardiotoxicity
Azathioprine	Arthritis	Hepatotoxicity, Myelosuppression
Methotrexate	Arthritis, Cutaneous SLE	Hepatic fibrosis, Pulmonary infiltrates, GI upset
Mycophenolate mofetil	Nephritis, Hemolytic anemia, Thrombocytopenia	Pseudocholinesterase inhibition, Pancytopenia

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

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

Anaesthetic Management

Systemic Pathological Features and Anaesthetic Implications

1. Nervous System

Pathological Features:
- Short and stenosed carotid arteries
- Cerebrovascular accidents
Anaesthetic Implications:
- Maintain cerebral perfusion pressure (CPP)
- Neutral head position
- Hemodynamic monitoring
- Maintain mean arterial pressure (MAP)
- Monitor for perioperative myocardial infarction (MI)
- Prevent stress response

2. Cardiovascular System

Pathological Features:
- Multiple arterial occlusion
- Pulseless disease
- Hypertensive heart disease
- Coronary artery, endocardial, and vascular disease
Anaesthetic Implications:
- Maintain MAP
- Hemodynamic monitoring
- Prevent perioperative MI
- Prevent stress response

3. Pulmonary System

Pathological Features:
- Pulmonary artery hypertension
- Pulmonary artery narrowing
Anaesthetic Implications:
- Avoid factors increasing pulmonary vascular resistance

4. Musculoskeletal System

Pathological Features:
- Ankylosing spondylitis
- Rheumatoid arthritis
Anaesthetic Implications:
- Difficult airway and careful head and neck positioning
- Limited joint mobility

5. Renal System

Pathological Features:
- Renal artery stenosis
- Renal hypertension
Anaesthetic Implications:
- Maintain MAP
- Consider renal protection strategies

6. Endocrine System

Pathological Features:
- Adrenal suppression due to chronic steroid therapy
Anaesthetic Implications:
- Perioperative steroid replacement

Monitoring: TKA^ – difficult A-line due to impalpable pulses → use US; monitor UL + LL due to differences
Neck: Prone to ischaemic events from carotid stenosis → Avoid hyperextension, tight neck tube ties; maintain CPP
BP: TKA – Avoid BP spikes → can cause aortic dissection / cerebral haemorrhage → premed to obtund intubation response, but maintain MAP
Regional: TKA – Aids BP control, permits neurological assessement. GPA* – Avoids manipulating airway (upper airway granulomas, tracheal stenoses, lung nodules)
Treatment: Continue steroid supplementation, standard DVT prophylaxis

Links

Endocrine and Metabolic

References:

1. Chetcuti, S., Jones, R., & Varley, J. (2016). Heritable connective tissue diseases, vasculitides, and the anaesthetist. BJA Education, 16(9), 316-322. https://doi.org/10.1093/bjaed/mkw016
2. The Calgary Guide to Understanding Disease. (2024). Retrieved June 5, 2024, from https://calgaryguide.ucalgary.ca/
3. Anesthesia Considerations. (2024). Retrieved June 5, 2024, from https://www.anesthesiaconsiderations.com/
4. ICU One Pager. (2024). Retrieved June 5, 2024, from https://onepagericu.com/

Summaries
Considerations_CTD

Please log in to view your notes.

The Art of Persuasion Building Bridges Through Negotiation

December 18, 2024 No Comments

Welcome to Your Anaesthesia Resource Hub! Welcome to Your Anaesthesia Resource Hub! Explore comprehensive perioperative medicine resources developed by anaesthetists for evidence-based practitioners. Fundamental (Free)

The Resilient Mind Unlocking Mental Strength and Clarity

December 18, 2024 No Comments