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Mediastinal Masses

Acknowledgment I wish to thank Dr. Frank Gencorelli for his work on this chapter in the earlier edition of this book. Risk Usually a congenital lesion, occurring at 1:5000; no gender bias Benign or malignant; cysts or aneurysms that arise from the lung, pleura, or another structure of anterior mediastinum; middle mediastinum: LN enlargement and vascular masses, posterior mediastinum: neurogenic tumors and esophageal abnormalities. In children,…

Mastocytosis

Risk Rare clinical condition with an estimated incidence at 1:150,000. Perioperative Risks Not an allergic disease. Not a risk factor of IgE-mediated drug allergy. Mast cell degranulation induced by various nonspecific triggers with subsequent histamine and other mediators (e.g., tryptase, leukotrienes, prostaglandins) release may result in periop clinical features involving the skin and the cardiovascular system. Bronchospasm usually does not occur. Periop course is usually uneventful…

Marfan Syndrome

Risk Prevalence is estimated at 1:5000 people. Inherited as autosomal dominant trait, though 25% of cases are sporadic. The 2010 Ghent Nosology for Marfan Syndrome guides Dx, heavily prioritizing aortic root dilation, ectopia lentis, and family history. Perioperative Risks Aortic rupture and dissection, mitral valve prolapse, mitral or aortic valve regurgitation, arrhythmias, pneumothorax, restrictive lung disease, and chest wall and spine deformity Worry About Ascending aortic…

Malnutrition

Risk Rate approximately 5% in general population and 10–20% in surgical pts; increases to 40% or more in severely ill hospital admissions. Risk increases with severity of underlying disease, presence of malignancy (especially GI), and advancing age (in older [>75 y] hip fracture pts baseline on presentation is 30%). Hospitalized pts lose an average of 5% body weight over the course of admission. Perioperative Risks Postop…

Malignant Hyperthermia and Other Anesthetic-Induced Myodystrophies

Acknowledgement The authors are grateful to Henry Rosenberg, MD, who authored the previous edition of this chapter. It has served as a framework for this updated edition. Risk Incidence of MH impossible to know because of lack of reporting mechanisms; Malignant Hyperthermia Association of the US hears of approximately 1–2 cases per week in North America. More common in males (approximately 2:1). Family Hx of MH…

Lysosomal Storage Disorders

Risk Individually rare, but as a group the incidence is approximately 1:8000. Perioperative Risks Difficult airway management Cardiac or respiratory failure Hemorrhage Worry About Difficult or failed intubation Coexisting cardiac or respiratory disease Difficult vascular access Neurologic involvement and pt cooperation Overview Lysosomal storage disorders are rare, inherited, metabolic connective tissue disorders (often autosomal recessive) with variable anesthetic risk. Caused by a variety of mutations in…

Lymphomas

Risk 5.3% of all cancers; 55.6% of all blood cancers (USA National Institutes of Health Registry) Per annum risk 2.9:100,000 population (European Age-Standardized Incidence UK, 1993–2012) Perioperative Risks Tumor: Mechanical obstruction, compression effect on organs systems (e.g., airway or bowel obstruction) Systemic effects: Myelosuppression including immunosuppression, anemia, thrombocytopenia, paraneoplastic syndromes Cachexia Overview Group of blood cell tumors developed from lymphatic tissues WHO classification: Prognosis depends upon…

Lyme Disease

Risk Approximately 30,000 new cases are reported to the CDC each year; however, this number is likely underestimated. The CDC is currently conducting research, and preliminary results suggest the number of new cases to be around 300,000. Lyme disease is the most commonly reported vector-borne illness in USA. In 2013 95% of confirmed Lyme disease cases were reported from 14 states: Connecticut, Delaware, Maine, Maryland, Massachusetts,…

Ludwig Angina

Risk Main risk factor is odontogenic infection, especially of the second and third molars (90% of all cases). Other factors include dental and gingival disorders, bacterial infection of the floor of the mouth, peritonsillar abscess, IV drug abuse, mandible fracture, tongue piercing, sialadenitis, and puncture wounds of the floor of mouth. Infrequently encountered with contemporary oral hygiene practices and antibiotics. Often occurs in otherwise healthy pts…

Long QT Syndrome

Risk Prevalence of cLQTS: Approximately 1:5000 live births. Incidence of cLQTS: 1 in 10,000. 60–70% of those diagnosed are females. Males under 10 y of age have the highest mortality. Pts usually present in childhood with a cardiac event. Perioperative Risks Torsades de pointes Sudden cardiac death Worry About Sympathetic stimulation with laryngoscopy, pain, etc. Electrolyte abnormalities: hypokalemia, hypocalcemia, and hypomagnesemia Overview cLQTS is diagnosed when…

Lipidemias

Risk Prevalence in USA: 13.1% in people >20 y. Prevalence highest among Hispanics. Cigarette smoking is a risk factor. Incidence highest among men ≥45 y and women ≥55 y. Htn is a risk factor. Low HDL (<40 mg/dL) is a risk factor. Family Hx of premature CHD in first degree relative (male <55 y or female <65 y) is a risk factor. Perioperative Risks Pancreatitis with…

Liddle Syndrome

Risk Extremely rare but described in a variety of populations True incidence and prevalence unknown Perioperative Risks Chronically untreated Htn Worry About Undiagnosed cerebrovascular, CV, and/or renal disease secondary to chronic Htn Worsening of hypokalemia with hyperventilation and nasogastric suctioning Hypokalemia-induced dysrhythmias and potentiation of NMB Overview Monogenic AD gain-of-function mutation in the ENaC resulting in early onset Htn, hypokalemia, and metabolic alkalosis with suppressed plasma…

Leukemia

Risk Estimated 318,389 individuals living with leukemia or in remission in USA Estimated 54,270 new cases and 24,450 deaths of leukemia in 2015 Males >females ALL greater in children (median age of diagnosis 14 y) CML, CLL, and AML common in adults and diagnosed in sixth and seventh decades Perioperative Risks Immunocompromised pt, tumor lysis syndrome (metabolic derangement), tumor compression of organs (anterior mediastinal mass), neutropenia…

Lesch-Nyhan Syndrome

Risk X-linked recessive disorder due to deficiency on the enzyme HGPRT, resulting in the buildup of uric acid Incidence ∼5.2 per million male births (where symptoms appear) Perioperative Risks Hyperuricemia and hyperuricosuria (gout) Airway problems secondary to scarification from self-mutilation (lip and finger biting) Involuntary writhing Repetitive movement of arms and legs Impairment of renal function due to obstructive uropathy Worry About Aspiration pneumonia (poor muscle…

Latex Allergy

Risk Myelomeningocele (25–50%) Congenital urologic anomalies (25–50%) Healthcare workers (3–17%) Atopic individuals (6–11%) General population (0–6%) Perioperative Risks Anaphylactic reaction leading to hypotension, bronchospasm, and CV collapse You’re Reading a Preview Become a Clinical Tree membership for Full access and enjoy Unlimited articles Become membership If you are a member. Log in here

Landouzy-Dejerine Dystrophy (Facioscapulohumeral Muscular Dystrophy)

Risk Prevalence estimated to be 1:20,000 Affects equally males and females Third most common familial myopathy after myotonic dystrophy and Duchenne muscular dystrophy Perioperative Risks No greater risk of malignant hyperthermia than normal population. Possible risk of acute rhabdomyolysis if succinylcholine and/or a halogenated agent is used. However, the dystrophin-glycoprotein complex is not involved in Landouzy-Dejerine dystrophy, and no case of anesthesia-induced rhabdomyolysis has been reported…

Lambert-Eaton Myasthenic Syndrome

Risk Unknown true incidence. Few studies of very specific regions outside USA report prevalence between 0.48 and 3.42 per million. 60–84% of LEMS patients have SCLC. LEMS mostly affects middle-aged adults, with rare occurrence in children. Perioperative Risks Increased risk for fall when ambulating, due to proximal lower extremities weakness Hypotension due to autonomic dysfunction Prolonged emergence secondary to persistent muscle weakness Respiratory compromise or collapse…

Klippel-Feil Syndrome

Risk Incidence estimated at 1:40,000 live births (but milder cases go unrecognized). Slight female predilection (63%). Perioperative Risks Cervical spine instability and cardiopulmonary complications. Often occurs in association with other clinical syndromes (e.g., fetal alcohol, Goldenhar). Worry About Exacerbation of cervical spine instability during airway maneuvers, endotracheal intubation, and subsequent positioning. Overview Congenital abnormality consisting of the following triad of findings: Fusion of two or more…

Kawasaki Disease

Risk Incidence in USA: 20 hospitalizations per 100,000 children Most commonly in pts <3 y of age Asian and Pacific Islanders have a higher rate, implying an unknown genetic effect Infants <6 mo and children >5 y have a higher risk of developing coronary artery lesions Perioperative Risks Risk of myocarditis, valvular disease, pericardial effusions, and arrhythmias during the acute phase, defined as within 2 wk…

Kartagener Syndrome

Risk KS, first described in 1933, is part of a larger family of diseases classified as PCD. The triad of KS consists of bronchiectasis, chronic sinusitis, and situs inversus; it has an incidence estimated at 1:15,000–40,000 births. The disease is likely underdiagnosed because a limited amount of centers have resources to provide an accurate diagnosis. No known predilection for race or gender. Symptoms more prevalent in…