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Saethre-Chotzen Syndrome

Risk Incidence: 1:25,000–50,000 live births. Perioperative Risks Difficult airway. Seizures. Oculocardiac reflex. The severity of cardiac lesion may affect the hemodynamic stability of the pt during anesthesia. Overview A type of acro-cephalo-syndactyly syndrome, characterized by premature fusion of the coronal sutures, facial dysmorphism, syndactyly, skeletal deformity, and congenital heart malformations. Named after two physicians who independently reported it in the early 1930s—Haakon Saethre, a Norwegian psychiatrist,…

Rubella and Congenital Rubella Syndrome

Risk A rubella epidemic in USA in 1964–1965 resulted in 12.5 million cases of rubella infection and about 20,000 newborns with CRS. The number of reported cases of rubella in USA remains low, with a median of 11 cases annually in 2005–2011 because of vaccination. The overall burden of CRS is still high in developing countries. There were 66 cases reported in Bangladesh, 26 in Romania,…

Rocky Mountain Spotted Fever

Risk Incidence in USA: In most states, most commonly in the southeastern and south central states, there are ∼250-2200 cases per y. Exposure to tick-infested terrain or dogs. Severe infection; very young (<4 y), males and those with G6PD deficiency are at risk for death. Mortality is 23% when untreated, 0.3–4.0% even with early treatment (within first 5 d). Mortality increases with delay in Dx, older…

Riley-Day Syndrome (Familial Dysautonomia, Hereditary and Sensory Autonomic Neuropathy Type III)

Risk Incidence: 1:3700 live births among American and Israeli Jews. Since original description in 1949, more than 600 pts have been identified and registered with the Dysautonomia Center at New York University. Perioperative Risks Prior to 1960 there was a 50% probability of death before age 5 y; currently a newborn with FD has 50% probability of reaching age 40 y, although many require multiple surgical…

Rheumatoid Arthritis

Risk Internationally the prevalence of RA is believed to range from 0.4% to 1.3%. In 2005, an estimated 1.5 million (0.6%) of USA adults >18 y had RA. Male-female ratio: 1:2. Perioperative Risks Risk of neurologic injury is increased due to possible occult damage to the cervical spine. Associated cardiac disease may be present but not clinically apparent. Pulm complications arise secondary to possible pulm fibrosis…

Rheumatic Fever (Acute) and Rheumatic Heart Disease

Risk Common illness among children and young adults. Primary chronic sequelae is RHD. Worldwide estimate is over 15 million cases of RHD, with 282,000 new cases and 233,000 deaths annually. Up to 1% of all school-age children in Africa, Asia, Latin America, and the eastern Mediterranean show signs of the disease. Perioperative Risks In ARF with acute cardiac manifestations (including first-degree heart block and pericarditis), medications…

Reye Syndrome

Risk Incidence prior to 1990: 0.3–0.6:100,000. From 1987 to 1993: 0.03-0.06:100,000; 2 cases/y have been reported since 1994. During early 1980s, an association between aspirin and Reye syndrome was recognized; thereafter, incidence declined dramatically. In 1986, a warning label on all aspirin-containing products was mandated in USA. Perioperative Risks Surgery (all but life-or-death emergencies) contraindicated during Reye syndrome. Following recovery, LFTs must be repeated. Worry About…

Rett Syndrome

Risk Occurs almost exclusively in females. Incidence is 0.4–0.7:10,000. Perioperative Risks Abnormal control of ventilation, with periods of apnea and hyperventilation May have GE reflux Multiple orthopedic and motor movement disorders Worry About Risk of periop apnea not known Risk of succinylcholine-induced hyperkalemia not known Aspiration due to GE reflux and swallowing disorder Cardiac: Prolonged QTc, abnormal autonomic regulation, increased incidence of sudden death Difficult intraop…

Renal Failure, Chronic

Risk Incidence in USA and worldwide: >100 cases of ESRD per million population. Between NHANES of 1988–1994 and that of 2003–2006, the prevalence of CKD in people 60 y and older jumped from 18.8% to 24.5%. Racial prevalence: African Americans, ∼200 cases per million; Hispanics, ∼100 per million; Caucasians, ∼50 per million. Perioperative Risks Overall periop mortality of pts with ESRD: 4%. Overall periop morbidity of…

Renal Failure, Acute

Acknowledgment The author would like to sincerely thank Dr. Robert Sladen for his work on this chapter in the previous edition. Risk Incidence in USA: 1% of all hospital admissions (community-acquired); 5% of all general hospital pts (hospital-acquired); 10–30% of all ICU pts. Acute tubular necrosis (45%) is most common cause in hospitalized pts. Population with highest prevalence: Elderly (>65 y). Two most common definitions: RIFLE…

Reflex Sympathetic Dystrophy (Complex Regional Pain Syndrome)

Acknowledgment The author wishes to acknowledge the contribution to the previous edition of this chapter by Dr. David Bandola. Risk Incidence of 5.5:100,000 person years at risk (50,000 new cases per y in USA). Prevalence of 21:100,000 person years at risk. Female:male ratio is 2–4:1. Mean peak age is 37–50 y. Incidence of CRPS I is 1–2% after fractures, 12% after brain lesions; and 5% after…

Raynaud Phenomenon

Risk Prevelance: 3-5% of population (based on population-based surveys of various ethnicities) Five times more prominent in women than men; commonly diagnosed in second, third, and fourth decades of life. Perioperative Risks Rare morbidity of ischemia, resulting in necrosis and gangrene Worry About Associated systemic disorders Arterial thrombosis secondary to prolonged vasospasm and/or ischemic attacks, which can lead to ulcerations and/or gangrene in affected areas Hypothermia…

Q Fever

Risk Greatest after direct or indirect exposure to infected cattle, sheep, or goats; particularly at parturition Less from a variety of other animals, rarely from blood products Abattoir workers, veterinarians, and other animal workers at greatest risk Pts with immune impairment are at a higher risk (e.g., HIV, steroids) Mortality 2.4% overall; chronic infection ∼16%. Perioperative Risks Decreased respiratory reserve secondary to pneumonia Decreased myocardial reserve…

Pyloric Stenosis

Risk Incidence: 1:300-1000 live births. Incidence is 3–5% higher among children of affected parents. More common in males. Perioperative Risks Similar to other abd procedures in pts of same age. Some association with GU anomalies. Some pts have elevated unconjugated bilirubin related to decreased glucuronyl transferase activity; this returns to normal after correction of stenosis. Worry About Full stomach. Recurrent emesis leads to dehydration, electrolyte imbalance,…

Purpura, Thrombotic Thrombocytopenic

Risk TTP rare (1:1,000,000). Adult, pregnancy may be a predisposing factor. Survival 80% at 6 mo; 90% mortality if untreated. Periop risks Rarely reported in pregnancy or postsurgically (case reports point to cardiac/urologic complications). Refer pt for splenectomy if medical therapy fails. Risks include MAHA with variable neurologic deficits and renal dysfunction combined with thrombocytopenia. Worry About Preop drugs, therapies (plasma exchange, steroids, rituximab) CNS/renal dysfunction…

Purpura, Immune Thrombocytopenic

Risk Rare (100:1 million) Children: Male > female Adults: Female > male (2–4:1). Pregnancy: 1:1000 deliveries; 5% of thrombocytopenia in pregnancy, especially if present in first trimester. Perioperative Risks Hemorrhage (case reports put mortality for splenectomy at 1%, one-third of which is related to bleeding). Infection and thrombocytosis post-splenectomy Retrospective data from Taiwan point to a higher risk of postop mortality (OR 1.89), complications (1.47), increased…

Pulmonary Hypertension

Risk Relatively uncommon disease process, with an estimated incidence of 1–5:100,000. Frequently identified as a contributing cause of death in USA, resulting in 6.5:100,000 deaths (2010). Left heart disease underlies 60–85% of pHTN cases. Primary pulmonary disease (e.g., COPD/OSA) is the second most common etiology. Chronic thromboembolic disease causes pHTN in 2–4% of pts after acute PE. Primary PAH is rare but most amenable to medical…

Pulmonary Fibrosis, Idiopathic

Risk Present in ∼42.7–63:100,000 and incidence is ∼16.3:100,000 in USA; more common with increasing age, with majority >55 y. Occurrence higher in males than females; risk factors include smoking, exposures, increasing age, family history, chronic reflux, environmental, viral, and genetics. Perioperative Risks Dependent on degree of underlying lung disease and associated comorbidities. Pulm Htn, H/O PE, OSA, CKD, CAD, and NYHA class 2 or above are…

Pulmonary Embolism

Risk Incidence of pulmonary embolism in USA: 600,000/y No racial predilection Risk factors same as those for DVT Perioperative Risks Presents a risk for hypoxemia and right heart failure. Periop mortality of 50–90% for acute thromboendarterectomy and of ∼10% for chronic thromboendarterectomy. Postop pulm embolism in up to 1% of surgical pts. Pulm embolism accounts for 20–30% of deaths associated with pregnancy. Worry About Recurrent pulm…

Pulmonary Atresia

Risk PA/IVS occurs in 3% of all CHD and has a prevalence of 0.07:1000 live births. PA/VSD occurs in 3.4% of all CHD. 20% of all cases of TOF are physiologically similar to PA/VSD due to extreme pulm artery stenosis. Males are affected more than females. Perioperative Risks RV failure (due to volume overload, pressure overload or both) Hypoxemia (leading to metabolic acidosis) Myocardial ischemia secondary…