Tag Lee A. Fleisher

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…

Deep Vein Thrombosis

Risk Incidence in USA: 170,000–200,000 new cases; 90,000–100,000 recurrent cases. VTE is the third most frequent acute cardiovascular syndrome after MI and CVA. Half of all episodes are associated with recent surgery or hospitalization. VTE is recognized as the leading…

Dandy-Walker Syndrome

Risk Multiple genetic factors; mostly sporadic with limited familial inheritance Range: 1:10,000-30,000 newborns Perioperative Risks Variable phenotypic expression and organ involvement Increased incidence of additional developmental abnormalities Depend upon severity of disease and comorbidities, which may include elevated ICP; craniofacial,…

Cherubism

Risk >250 cases in world literature Cherubs have a 40% chance of having a cherub offspring Perioperative Risks Swelling of lower face causing airway obstruction Displacement of ocular orbit and lower eyelid, causing visual changes Excessive blood loss from curettage…

Chagas Disease

Risk 16–18 million people infected worldwide Rare in southern USA; chronic disease more likely in immigrants from endemic regions (South America, central Brazil prevalence 6–8%) More than 50,000 die each year; mortality estimated at 50% at 4 y secondary to…

Carpenter Syndrome (Acrocephalopolysyndactyly Type II)

Risk Extremely rare; global estimate: 1:1,000,000 births Over 100 reported cases Perioperative Risks Airway obstruction Difficult mask ventilation and/or difficult intubation Increased ICP from craniosynostosis Coexisting congenital anomalies, especially cardiac Overview Manifestation: Craniosynostosis Polysyndactyly Cardiac defects Obesity Maxillary or mandibular…

Bernard-Soulier Syndrome

Risk Estimated to be <1 in 1 million persons, but may be higher due to misdiagnosis and underreporting Rare: Approximately 100 cases reported in literature Perioperative Risks Severe hemorrhage out of proportion to plt count Transfusion reactions Worry About Severe…

Beckwith-Wiedemann Syndrome

Risk 1 per 13,700 individuals. No gender predilection, although with monozygotic twins it is seen more in females than males. Conceptions from IVF have a 3–5 times increased risk of BWS. Perioperative Risks Acute airway obstruction; difficult mask ventilation and…

Autoimmune Diseases, Cold

Risk Rare Autoimmune hemolytic anemias occur in 1 of 80,000 persons; of these, 17.3% are due to cold antibodies. Perioperative Risks Acute hemolysis due to cold Hemoglobinemia Hemoglobinuria Rarely, vascular occlusion Worry About Cooling to 28–31° C will cause hemolysis.…