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Glaucoma, Open-Angle

Risk Open-angle glaucoma is the leading cause of blindness among African Americans and the second leading cause overall in USA. African American race, advanced age, elevated IOP, myopia, low diastolic perfusion pressures, and family Hx of open-angle glaucoma increase the risk for primary open-angle glaucoma. Incidence in US: Estimates suggest over 2.25 million Americans over age 40 have open-angle glaucoma. Perioperative Risks Vision loss secondary to…

Glaucoma, Closed-Angle

Acknowledgment The author wishes to acknowledge the contributions to the previous edition of this chapter by Dr. Kate Tobin. Risk According to the WHO, glaucoma is the second most common cause of blindness worldwide. Risk factors include hyperopia (far-sightedness), age >60, female gender, and family Hx. Perioperative Risks Postop vision loss Inducing acute ACG Worry About Causing sustained, marked elevations in IOP A chronic, narrowed angle…

Gaucher Disease

Risk General population: 1:50,000 to 1:100,000. Inheritance follows an autosomal recessive pattern. Type 1 (nonneuropathic) most common and represents 99% of cases. Population with highest prevalence is Ashkenazi Jewish. 1:18 carrier rate 3:1000 of the Ashkenazi Jewish population have type 1 disease. Types 2 (infantile) and 3 (juvenile) are exceedingly rare No specific populations are at elevated risk. Perioperative Risks Upper airway obstruction Coagulopathies GERD Insulin…

Gastroesophageal Reflux in Children

Risk Physiologic GER usually resolves by 12–15 mo of age. 10% of pyloric stenosis pts. After diaphragmatic hernia, tracheoesophageal fistula, and esophageal atresia repairs Neurologically impaired, developmentally delayed, trisomy syndromes, and hiatal hernia. Perioperative Risks Aspiration during induction of anesthesia Severe bronchospasm in pts with RAD Decreased pulm reserve secondary to chronic aspiration and pneumonitis Worry About Pulm complications from aspiration pneumonitis and RAD Anemia and…

Gastrinoma

Acknowledgment The authors would like to acknowledge Dr. Christine Piefer’s contribution to this text in the previous edition. Risk Occurs in about 0.1–3 cases per million, usually in pts aged 20–50 y; male/female predominance about equal. 65–80% of gastrinomas are sporadic and have high (40–85%) malignant potential. 20–35% of cases occur with MEN1, with low (7–12%) malignant potential. Less than 0.1% of all PUD is caused…

Galactosemia

Risk Rare autosomal recessive metabolic disorder that can present increased anesthesia risks. Inherited disease of carbohydrate metabolism affecting how the body processes the simple sugar galactose. The primary risk factor is having parents who carry the gene for galactosemia. Life-threatening complications in the newborn may occur shortly after introducing galactose into the diet. Three types of galactosemia: Type I (classic galactosemia) involves a deficiency of GALT…

Friedreich Ataxia

Risk Prevalence: 2:100,000; 80–90% have cardiac involvement. Worry About Cardiac involvement, which does not correlate with neurologic involvement. Electrophysiologic disturbances. Cardiac dysfunction and failure. Overview Progressive degeneration of posterior columns and corticospinal and posterior spinocerebellar tracts. Muscle weakness. General anesthesia can lead to postop respiratory disorders caused by thoracic kyphoscoliosis, which is associated with restrictive respiratory function. Abnormal glucose homeostasis. Most individuals have onset of symptoms…

Foreign Body Aspiration

Risk Most prevalent in children ages ≤3 y. In adults, elderly are most susceptible to FB aspiration. Risk factors include Alzheimer disease or dementia, stroke, loss of consciousness due to trauma, alcohol intoxication, or drug overdose. Foods are most commonly aspirated foreign objects. Perioperative Risks Hypoxemia due to FB obstruction Fragmentation of the FB and distal dislodgement during retrieval Severe inflammation due to presence of high…

Fat Embolism

Risk Long bone fractures and pelvic fractures: 80–100% fat embolism Less than 1–30% FES Male-female ratio: 4:1 Adult greatly increased over pediatric Multiple fractures >single fractures Pathologic fractures >traumatic fractures Total hip, total knee replacement, intramedullary nailing: 27–100% fat embolism Unknown incidence FES Unusual causes: Liposuction, fat injection, bone marrow harvest and/or transplantation, vertebroplasty, cardiopulmonary bypass, CPR, burns, pancreatitis, sickle cell disease, osteomyelitis, fatty liver, soft…

Fanconi Syndrome

Risk FS can be inherited, acquired, or caused by exogenous factors. Incidence is sporadic. Exact incidence in USA is not clear. Most diseases associated with FS are inherited in an autosomal recessive pattern. Cystinosis is the most common cause in pediatric pts. Perioperative Risks Potential for hypotension secondary to hypovolemia Renal failure, proximal renal tubular dysfunction Lyte imbalance (especially hypokalemia causing tachyarrhythmias) Worry About Polyuria, polydipsia,…

Familial Periodic Paralysis

Risk Rare; hyperPP approximately 1:200,000 and hypoPP approximately 1:100,000 HyperPP with childhood onset; hypoPP with teenage onset Perioperative Risks In hyperPP, succinylcholine may provoke severe myotonia, provide no relaxation, and cause hyperkalemia (resulting postop muscle weakness over days and rhythm disturbances). HypoPP associated with supraventricular or conduction defect-type cardiac arrhythmias; weakness may be enhanced by β-adrenergic blocking drugs, and postop resp muscle weakness may occur. Hypermetabolic…

Familial Dysautonomia (Riley-Day Syndrome)

Risk Autosomal recessive transmission Complete penetrance, marked variability in expression Predominantly affects Ashkenazi Jewish population (incidence 1:10,000–20,000; carrier frequency 1:27-32) Perioperative Risks Intraop: Primarily cardiovascular with hemodynamic variability Postop: Primarily cyclic vomiting and pulmonary complications Worry About Paroxysmal dysautonomic crisis triggered by physiologic or psychologic stress characterized by intractable vomiting, Htn, tachycardia, diaphoresis, erythematous macular rash Resp status compromised by dysfunctional swallowing, leading to repeated aspiration…

Factor V Leiden Mutation

Risk Most common hereditary thrombophilia Autosomal dominant inheritance pattern Heterozygous form in 5% of white population in USA (up to 15% in Europe), 2% of Hispanic Americans, 1% in both African and Native Americans Homozygosity in white population 1:5000 May account for 85–95% of pts with APC resistance Relative risk of venous thrombosis sevenfold in heterozygous and 80-fold in homozygotes Perioperative Risks VTE: DVT most likely;…

Fabry Disease

Risk Genetic disease with reported annual incidence of approximately 1:100,000, but rarity may lead to underestimation of true prevalence Panethnic Perioperative Risks Autonomic instability, particularly with neuraxial anesthesia Cardiovascular instability from conduction abnormalities and structural heart disease Periop stroke in those with cerebrovascular disease Worry About Poor respiratory function, particularly in smokers Renal impairment Sudden swings in blood pressure Pain control in pts with chronic pain…

Epiglottitis

Risk Prevalent in children 1–7 y; sometimes called supraglottitis, it does occur in adults (decreasing incidence in children >3 y related to vaccines against Haemophilus influenzae type B, but still found, particularly if pt is not immunized). Adult incidence remains constant with organisms group A Streptococcus pneumoniae , Staphylococcus aureus , and Klebsiella pneumoniae. Perioperative Risks Acute deterioration of airway patency resulting in complete obstruction worse…

Epidermolysis Bullosa

Risk 1:17,000, 50% dystrophic form Racial distribution equal Perioperative Risks Difficult IV access, airway, intraop positioning, reflux, steroid dependence, intraop hemorrhage, sepsis, iatrogenic corneal abrasion, blister formation, and airway obstruction Worry About Problems similar to those found in pts with severe skin burns; severely compromised pts Difficult intubation (23%) secondary to microstomia Establishing monitoring and IV access Dehydration and malnutrition Anemia, hypoalbuminemia, electrolyte imbalance, and thrombocytosis…

Endocarditis

Risk Incidence: 3–10:100,000 population. Rheumatic heart disease is a key risk factor in lower-middle income countries. Valvular and cyanotic heart disease, DM, cancer, and IV drug use are risk factors in higher income countries. Perioperative Risks Septic embolization to other organs (CNS, renal, and lung) is seen in 25–50% of pts. Worry About Acute heart failure (valvular regurgitation or obstruction), stroke, and metastatic infection (i.e., epidural…

Endocardial Cushion Defect (Atrioventricular Canal)

Risk 4% of all congenital heart disease and 0.3–0.4:1000 live births 40–50% of AV canal defects are associated with trisomy 21 Perioperative Risks Paradoxical air embolism Shunt reversal (from left to right to right to left) because of vasodilating volatile and IV anesthetics (reduced systemic vascular resistance) Endocarditis; prophylactic antibiotics for pts with a complete repair or a jet lesion Arrhythmias after AV canal repair Reactive…

Encephalopathy, Postanoxic

Risk After successful prehospital cardiac resuscitation: 59–65% of pts remain comatose. 0–5% of successful resuscitations result in chronic vegetative state. Perioperative Risks Worsening of neurologic status; blindness most common residuum. Postpone surgery in all but emergency situations. Do what is necessary to treat precipitating cause and to decrease sequelae (e.g., treat elevated ICP). Worry About Repeat of events that initially caused encephalopathy (e.g., arrhythmias leading to…

Encephalopathy, Metabolic

Risk 3.4– 11% of medical ICU admissions 12–33% of multiple-organ dysfunction pts Perioperative Risks With predisposing conditions (e.g., hepatic insufficiency), risk of developing or exacerbating metabolic encephalopathy Increasing severity of preexisting encephalopathy Worry About Worsening hepatic insufficiency causing hepatic encephalopathy Diabetics becoming hypoglycemic or with DKA/hyperosmolar coma Postop hyponatremia Deteriorating renal insufficiency leading to uremic encephalopathy Preexisting encephalopathy may be exacerbated by anesthetics (e.g., benzodiazepines) in…