Rare Co-existing Disease Flashcards
Amyloidosis
Background
Considerations
Goals & Conflicts
Amyloidosis
Background
Refers to the extracellular tissue deposition of fibrils
Primary amyloidosis: is a plasma cell disorder marked by the accumulation of immunoglobulin light chains.
Secondary amyloidosis: is observed in association with several other conditions, including multiple myeloma, rheumatoid arthritis, & a prolonged antigenic challenge, such as may be produced by chronic infection
Considerations
Difficult airway: macroglossia, laryngo-tracheo-bronchial tree involvement, stridor
Aspiration risk
Multisystem infiltrative disease:
Cardiovascular:
Restrictive cardiomyopathy
Diastolic dysfunction
Complete heart block
Risk of sudden death
Coronary heart disease
Respiratory: interstitial lung disease
Renal: nephrotic syndrome & renal failure
GI: dysphagia, risk of aspiration secondary to autonomic/peripheral neuropathy
Nervous system: autonomic neuropathy, mixed sensory/motor neuropathy
Heme: bleeding risk → factor X deficiency, ↓ coagulation factors, platelet dysfunction
Potential co-existing secondary causes: e.g. multiple myeloma & rheumatoid arthritis
Medications: steroids/chemotherapy
Goals & Conflicts
RSI for possible gastroparesis, aspiration risk vs. potential difficult airway
Prior to neuraxial/regional technique:
Document pre-existing neurodeficits
Confirm coagulation profile & clinical assessment of coagulopathy
Mindful of restrictive cardiomyopathy, hemodynamic instability due to autonomic neuropathy
Glycogen Storage Disorders
Background
Considerations
Glycogen Storage Disorders
Background
Inherited disorders caused by abnormalities of enzymes that regulate glycogen synthesis & breakdown
3 common features with all these disorders:
Acidosis secondary to fat & protein metabolism
Risk of hypoglycemia secondary to the failure to metabolize glycogen to glucose
Cardiac & hepatic dysfunction secondary to destruction of normal tissue by accumulated glycogen
Considerations
Careful monitoring of glucose & give D5W during fasting periods
Careful evaluation of cardiac & hepatic function
Careful review of previous anesthetics & obtain endocrinology consultation before surgery
Monitor glucose & pH intra-op
Avoid lactate containing solutions (they cannot convert lactate to glycogen)
Hereditary Angioedema (C1 Esterase Deficiency)
Background
Considerations
Goals
Management (Prophylaxis, Treatment)
Hereditary Angioedema (C1 Esterase Deficiency)
Background
A hereditary disorder that results in angioedema without urticaria
Due to autosomal dominant deficiency or dysfunction of C1 esterase inhibitor → release of vasoactive mediators → vascular permeability & edema formation
Presents as recurrent bouts of angioedema involving the extremities, face, oropharynx, larynx, GI, and/or GU tract lasting 24-72 hours
Considerations
Potential acute life threatening airway edema & obstruction that is not treated by conventional methods such as steroids, epinephrine & antihistamines
Need to avoid triggers: oral/dental surgery, laryngoscopy, trauma, infection, stress/light anesthesia
Need for prophylaxis prior to surgical procedures & availability of C1 esterase inhibitor to treat acute attacks
Need for postop monitoring (onset may be delayed 60 mins to 36 hours after trigger)
Goals
Pre-op prophylaxis (see management)
Avoid/minimize triggers
Gentle airway manipulation
Regional anesthesia if feasible
Ensure ready availability of treatment modalities for acute attack including difficult airway kit/surgical airway
Management
Prophylaxis
C1INHRP (C1 esterase inhibitor replacement protein)
Androgens (if C1INHRP not available) e.g. danazol (2.5 to 10 mg/kg per day to a maximum of 600 mg/day), beginning five days before and extending for five days after the procedure.
TXA (not as good as C1NHRP or Androgens)
Treatment of Acute Event
C1 inhibitor concentrate (25 units/kg)
FFP (2-4 units) to replace the deficient enzyme
NOT HELPFUL: androgens, epinephrine, antihistamines, antifibrinolytics
Hereditary Hemorrhagic Telangiectasias (HHT) (Osler-Weber-Rendu Disease)
Considerations
Goals & Conflicts
Pregnancy Considerations
Hereditary Hemorrhagic Telangiectasias (HHT) (Osler-Weber-Rendu Disease)
Considerations
Risk of excessive bleeding from variety of surfaces/organs:
Nose: epistaxis (nasal intubation contraindicated)
Upper airway hemorrhage (potential for difficult airway)
Spinal AVM: risk of paralysis from epidural hematoma (image before neuraxial technique!)
Pulmonary hemorrhage requiring lung isolation
Cerebral AVM: risk of intracranial hemorrhage
GI bleeding
Hepatic AVM: portal HTN, high output heart failure
Paradoxical emboli from intrapulmonary shunts (AVM): de-air lines!
Chronic anemia with potential for a difficult cross-match
Coagulopathy: low grade DIC, reduced platelet function
Goals & Conflicts
May present for laser treatment of epistaxis, embolization of pulmonary AVMs, craniotomy for AVM resection, treatment of GI bleeding
Thorough preoperative workup if suspected AVMs:
Pulmonary AVM: chronic hypoxemia, risk of pulmonary hemorrhage
Renal & liver function
Spinal MRI to rule out spinal AVM prior to neuraxial anesthesia
Rule out preoperative anemia, group & screen
Antibiotic prophylaxis if AVMs
Intraoperative care:
De-airing of all lines
Avoidance of airway manipulation if telangiectasias are present
Avoid nasal intubation if history of epistaxis
Modify anesthesia if high-output cardiac failure
Pregnancy Considerations
High risk for worsening of AVMs during pregnancy & peripartum period
Pulmonary hemorrhage, intracerebral & spinal hemorrhage, GI bleeding
Shunt-induced high output cardiac failure
Systemic embolism
Spinal MRI prior to epidural & spinal anesthesia
Avoid HTN during anesthetic management
HIV and AIDS
Considerations (Multisystem disease, medications)
Pregnancy Considerations
HIV and AIDS
Considerations
Need for universal precautions
Multisystem disease:
CNS: Dementia, peripheral neuropathies, myopathys, encelphalopathy, masses with ↑ICP
Airway: upper a/w obstruction with Kaposi sarcoma, possible difficult intubation
Resp: Pneumonia, pHTN of HIV, opportunistic resp. infxns
CVS: Autonomic neuropathy, accelerated CAD for HART, HIV related DCM
Renal: End stage nephropathy GN
Heme: Anemia, thrombocytopenia, lymphopenia
Immunocompromised:
Opportunistic infections: Meningitis, encephalitis, pneumonia/TB, esophagitis, enteritis
Malignancy: Lymphoma, Karposi Sarcoma
Failure to thrive, Cachexia, and Deconditioning
Medications (Antiretrovirals)
Bone marrow suppression
Renal failure
Peripheral neuropathy
p450 activation
Lactic acidosis
Insulin resistance
Dyslipidemia
Chronic pain and co-existing substance abuse
Pregnancy Considerations
↓ perinatal transmission with AZT
Elective C/S may reduce risk of transmission. All should be offered this but labor also ok esp. if Viral load <1000 copies/ml
Regional OK (document peripheral neuropathy, coagulation, strict sterile technique with gown!)
PDPH/blood patch NOT contraindicated
Huntington’s Disease
Background
Considerations
Huntington’s Disease
Background
Autosomal dominant inherited disease characterized by progressive neurodegeneration
Clinical features: choreiform movements, depression, & dementia
Onset is typically between 35-40 years of age, but can be as late as 80 years. The disease continues to progress for several years with death occuring 17-20 years after diagnosis & is usually from malnutrition or aspiration pneumonitis
Considerations
Bulbar dysfunction with risk of aspiration
Dementia, potentially uncooperative, end of life care, advanced directives, DNR status
Anesthetic medications:
All IV anesthetics OK, caution with slower recovery
NDMRs are ok, but may require ↓ dose
Succinylchonline is ok, but duration may be longer due to ↓ plasma cholinesterase activity
Neuraxial/regional is ok, document pre-existing deficits
Preoperative sedation using butyrophenones such as droperidol or haloperidol may be helpful in controlling choreiform movements
Neurofibromatosis
Background
Considerations (Airway, Respiratory, Cardiovascular, CNS, Medications)
Optimization/Consults
Pregnancy Considerations
Neurofibromatosis
Background
Cafe au lait spots are the most characteristic finding
2 types: NF-1 (more common) & NF-2
Neurofibromas nearly always involve the skin, but they can also occur in the deeper peripheral nerves & nerve roots & in or on viscera or blood vessels innervated by the autonomic nervous system
Considerations
Potential difficult airway:
Airway obstruction/distortion from laryngeal, pharyngeal, tongue, cervical tumors
Macrocephaly & macroglossia
Tumors can be very vascular
Respiratory:
Restrictive lung disease (kyphoscoliosis ~25%)
Potential for pulmonary fibrosis from parenchymal tumours
Pulmonary hypertension/RV failure
Mediastinal neurofibroma: may present with cardiopulmonary complaints
Cardiovascular:
Hypertension
Usually essential, secondary: RAS»_space; pheochromocytoma (<1%)
Dysrhythmias, idiopathic cardiomyopathy
RV outflow tract tumour
CNS:
↑ ICP (5-10% have intracranial tumors, hydrocephalus) +/- contraindication to neuraxial
Seizure disorder
Cognitive deficits, learning disabilities
Peripheral neuropathy
Unpredictable response to NMB drugs:
Variable responses (sensitivity or resistance to succinylcholine, sensitivity to NDMR)
Difficult regional & epidural/spinal, may be contraindicated if spinal neurofibroma
Possible endocrine problems: pheochromocytoma, hypoglycemia, pituitary tumours, hyperparathyroidism, medullary thyroid carcinoma
Optimization/Consults
Neurology: optimize ICP, antiepileptics
Cardiology/endocrine: optimize antihypertensives (phenoxybenzamine) if pheochromocytoma
Consider MRI (r/o ↑ICP) if planning regional technique
Respirology: if restrictive lung disease
Conflicts:
↑ICP vs neuraxial
Spinal neuromas vs neuraxial
↑ ICP vs difficult airway
Pregnancy Considerations
Higher maternal complications
If pelvic/abdominal neurofibromas → cesarean section necessary (dystocia, obstructed labor or respiratory embarrassment)
Higher fetal complications: preterm labor, IUGR, abortion
GA: very cautious of difficult airway & underlying hypertension
Neuraxial: must have imaging (MRI/CT) of spine before epidural/spinal
Periodic Paralysis
Background (Hyperkalemic periodic paralysis, Hypokalemic periodic paralysis)
Considerations
Periodic Paralysis
Background
Due to hereditary skeletal muscle ion channelopathies
Hyperkalemic periodic paralysis:
Episodes of myotonia & muscle weakness that may last for several hours
Weakness can occur during rest after strenuous exercise, infusion of potassium, metabolic acidosis, or hypothermia
The hyperkalemia is transient & occurs only at the time of weakness
Acute attacks can be fatal because of cardiac dysrhythmias or respiratory failure
A chronic myopathy frequently develops in older patients
Treatment consists of a low potassium diet & the administration of thiazide diuretics
Hypokalemic periodic paralysis:
Paralysis can be produced by a ↓ in serum potassium levels caused by strenuous exercise, infusion of glucose and insulin, or ingestion of carbohydrates & sodium rich food
Paralysis usually affects the limbs and trunk, but spares the diaphragm. Chronic muscle weakness occurs in most patients as they age
Considerations
Risk of periodic paralysis attacks, with potential for respiratory failure & cardiac dysrhythmias
Avoid triggers of attacks:
Hyperkalemic Periodic Paralysis (attacks when K >5.5)
K infusion
Metabolic acidosis
Hypothermia
Hypokalemic Periodic Paralysis (attacks when K <3.0):
Glucose/insulin infusion & other K lowering shift drugs
↓ K
Hypothermia
Carbohydrate load
Sensitivity to NDMRs: use ↓ dose, continuous monitoring with twitch monitor
Avoid succinylcholine (due to ↑ K) in hyperkalemic periodic paralysis (ok with hypokalemic periodic paralysis)
Frequent electrolyte monitoring esp. K
Sarcoidosis
Background
Considerations
Goals
Sarcoidosis
Background
Sarcoidosis is a multisystem granulomatous disorder of unknown etiology that is characterized by presence of noncaseating granulomas in involved organs. It initially presents with one or more of the following abnormalities:
Bilateral hilar adenopathy
Pulmonary reticular opacities
Skin, joint, and/or eye lesions
Considerations
Potential difficult airway (laryngeal/tracheal granulomatous disease. nasal mucosal sarcoidosis)
Multisystem granulamotous disease:
Pulmonary
A/W Tree: Endobroncial sarcoid, laryngeal sarcoidosis/stenosis
Pulmonary fibrosis, pHTN and cor-pulmonale, ↓ DLCO
CVS:
Conduction defects, CHF, restrictive CM, MR from pap muscle involvement, systolic/diastolic function
CNS: central DI (granulomas), dementia, encephalopathy, seizures, polyneuropathy
GI: Hepatomegaly & liver dysfunciton
Metabolic derrangement: Hypercalcemia
Medications: maybe on steroid Rx → stress dose
Goals
Anticipate potential difficult airway
Manage potential cardiomyopathy/AICD
Appropriate ventilatory strategy for restrictive lung disease and pulmonary fibrosis
Avoid exacerbation of cor pulmonale and pulmonary hypertension
Consider stress dose steroids
Anticipate complications: prolonged NMB, hypercalcemia, arrhythmias, etc
