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Crouzon Syndrome

Risk Represents approximately 4.8% of cases of craniosynostosis at birth. Birth prevalence of 1.6:100,000 births. Estimated prevalence in general population of Europe is 0.9:100,000. No race predilection. Perioperative Risks Difficult BMV, difficult intubation, massive blood loss, arterial gas embolism Worry About Difficult airway Intraop blood loss Inadvertent dural sinus injury Postextubation subglottic edema External facial fixation devices Overview Crouzon syndrome is an autosomal dominant disorder characterized…

Croup (Laryngotracheobronchitis)

Risk Children between 6 mo-6 y are at risk (6 mo–3 y at greatest risk). Children with underlying airway abnormalities (e.g., subglottic stenosis) or difficult intubations (e.g., micrognathia) and symptoms are at increased risk and require particular planning. Perioperative Risks Difficulty with intubation because of very narrowed subglottic region Obstruction of the small tracheal tube because of airway secretions. Worry About Risk of rebound tracheal edema…

Crohn Disease

Risk Incidence of 3.1–14.6 cases per 100,000 person-years; prevalence of 26–201 cases per 100,000 persons. Incidence and prevalence are increasing worldwide; incidence is highest in North America and Northern Europe. Race: White >African American >Hispanic and Asian populations for risk. 3–4 times more common in ethnic Jews than non-Jewish whites. More likely to occur in those with a strong family history. Affects males and females equally.…

Cri Du Chat Syndrome (5p− Syndrome)

Risk 1:15,000-50,000 live births Most common human chromosomal syndrome Perioperative Risks Difficult airway management due to anatomic abnormalities of the airways. 30% can present with congenital heart disease. Aspiration risk. May require temp control. Cardiac events (pts may have cardiac abnormalities). Worry About Difficult mask ventilation; airway obstruction secondary to hypotonia Difficult intubation Temperature regulation Overview Microcephaly with profound mental retardation and hypotonia. Characteristic facies with…

Creutzfeldt-Jakob Disease

Risk Iatrogenic transmission (actual and theoretical) Blood products: blood transfusion (3 reported cases). Greater risk with pooled products (since 1999 UK has imported blood fractions) or massive blood transfusion. Risk mitigated by deferral of recipients as donors (initiated: 1998 in France, 2005 in UK) and appropriate blood transfusion policies Organ donation Tissue grafts: Human dura mater (168 reported cases) or corneal grafts (3 reported) Human pituitary…

CREST Syndrome

Risk Pts with Hx of exposure to silica dust or PVC. Usual age group is 30–50 y. Fourfold to ninefold higher incidence in women than men; seen in all races. In USA systemic sclerosis has an estimated incidence of 19 cases per million and prevalence of 240 cases per million (range: 138–286). Perioperative Risk Pts more likely to have compromised renal function at baseline Hypoxia from…

Craniosynostosis

Risk Occurs in 1:2000-2500 live births. May be spontaneous, syndromic, or familial, but it most commonly presents as an isolated abnormality; however, it can present as a component of a syndrome or genetic disorder in 15–40% of pts. Familiarity with associated head shapes can allow bedside diagnosis and differentiation from positional plagiocephaly. Rarely a CT scan will be required to differentiate plagiocephaly from synostosis. Craniosynostosis is…

Craniofacial Clefts

Risk Cleft lip and palates are the most common birth defects in USA. Cleft lip and/or palate incidence in USA is 1:600 newborns. Craniofacial clefts are rare, with incidence of 1:1000 births worldwide. Increased prevalence with Asians, Latinos, and Native Americans. Increased prevalence with exposure to radiation, infections (toxoplasmosis, rubella, and CMV), maternal age, maternal smoking exposure, and vitamin deficiencies. Perioperative Risks Increased risk of airway…

Coronary Artery Spasm

Risk Disease affecting mostly middle- and old-aged men and postmenopausal women Gender difference: Higher incidence in women Periop CAS: Prevalent in elderly male pts with coronary risk factors Teenagers and young adults with illicit substance abuse, primarily cocaine Occurs in 1–5% of percutaneous coronary interventions Ethnic differences: Higher frequency in eastern populations Type A behavior pattern, severe anxiety, and panic disorder Age, smoking, and high sensitivity…

Coronary Artery Disease (Left Main and Non–Left Main Disease)

Risk Incidence in USA: 15.5 million. Approximately 735,000 pts per y with CAD will have an acute MI and approximately 15% of these will die. CAD is responsible for approximately 1 of every 7 deaths in USA. Male predominance in pts <55 y old, but M = F in >55 y old. Risk factors include Htn, diabetes, smoking, familial incidence, hyperlipidemia, and high cholesterol. Perioperative Risks…

Cor Pulmonale

Risk RV failure is the third most common cardiac Dx after age 50 y. Of all CHF admissions, 10–20% have some aspect of right heart failure. Gender predominance is male > female. Perioperative Risks Increased risk for respiratory failure, severe right heart failure (≥10% if cor pulmonale Dx made preop) Risk of prolonged postop ventilatory support Worry About Increased PVR may cause systemic hypotension due to…

Conversion Disorder (Functional Neurologic Symptom Disorder)

Risk Reported prevalence varies widely (11–500”100,000). It may account for unexplained symptoms in as much as 1–14% of general medical/surgical pts and possibly as much as 20% of new outpatient neurology referrals. Possibly higher in rural populations, developing areas, lower socioeconomic groups, those less medically sophisticated, and following physical and sexual abuse and trauma. Perioperative Risks Hx of conversion disorder may not increase periop morbidity or…

Constipation

Risk Median prevalence of constipation in adults is 16% (studies range 0.7–79%); in adults aged ≥60 y, it is 33.5%. Prevalence may be higher in nonwhite and institutionalized populations. Male:female ratio: 1:1.5. Perioperative Risks Increased risk of gastroparesis and N/V Increased intra-abdominal pressure Altered resp mechanics and delayed weaning from mechanical ventilation Poor nutritional status impairing wound healing Worry About Risk of pulm aspiration on induction…

Congestive Heart Failure

Risk Heart failure is a syndrome, not a disease. Incidence in USA: About 5.1 million, with more than 650,000 new cases diagnosed annually. Primary discharge diagnosis made in more than 1 million pts. 1-y and 5-y survival rates are 57% and 25% in men and 64% and 38% in women. Median survival after onset is 1.7 y in men and 3.2 y in women. Perioperative Risks…

Congenital Pulmonary Cystic Lesions/Lobar Emphysema

Risk Cause of cardiorespiratory compromise 10–15% associated with CHD Perioperative Risks May develop worsening of cardiorespiratory status Contamination of unaffected lung by infected material from cyst Worry About Associated congenital anomalies Tension pneumothorax Cardiorespiratory compromise Overview Congenital Pulmonary Cystic Lesions (Three Types) Bronchogenic: Abnormal budding and branching of tracheobronchial tree leading to resp distress, pneumonia, and atelectasis Dermoid: Lined with keratinized, squamous epithelium CPAM: Previously known…

Congenital Methemoglobinemia

Risk Navajo Indians, Alaskan Indians, and people of Puerto Rican and Cuban ancestry Normal life span (except for RCM type II) Perioperative Risks Oxidizing agents may increase MetHb to dangerous levels. Mild respiratory/cardiac depression may adversely affect pts with already limited reserve. Pregnancies not compromised. Worry About Measurement of SpO 2 Oxidant drugs (e.g., prilocaine, benzocaine, nitroglycerin, sulfonamides, phenacetin, and nitric oxide), which are contraindicated Myocardial…

Complement Deficiency

Risk C1 esterase inhibitor–deficiency incidence: 1:50,000-150,000 of the general population. Symptoms onset and diagnosis occur approximately at 20 y, and by 30 y approximately 98% of pts have symptoms. C2 deficiency incidence: <0.1% of the general population. Male versus female ratio: 1:6. Higher incidence (6%) in pts with autoimmune disease (see Immune Suppression). Incidence in pts with Hx of Neisseria meningitis: 15%. C3 and C5–C8 deficiencies…

Cogan Syndrome

Risk Extremely rare: approximately 250 reported cases in the literature Mean presentation 30–40 y; however, cases in children and elderly reported No predilection for gender, race, or ethnicity Possible association with IBD Perioperative Risks Hemorrhage Thrombosis and organ/limb ischemia Adrenal insufficiency and immunosuppression due to chronic treatment Postop N/V with vestibuloauditory dysfunction Worry About Activity state of disease and hemorrhage/extension of pathologic vasculitis Coexisting vasculitis affecting…

Coarctation of the Aorta

Risk Sixth most-common congenital heart defect: 4:10,000 live births Recognized in 5–8% of pts with CHD Perioperative Risks Perioperative mortality: 1% when associated with no other cardiac anomalies in neonates, 10% when associated with a VSD, and 50% when associated with HLHS; children and adults: Less than 0.5% Postop risk of paraplegia: 0.5–1.5% (even lower risk if younger than 1 y of age) Worry About Closure…

Coagulopathy, Factor IX Deficiency

Risk Within USA, approximately 4000 persons are affected (20% of all hemophiliacs): incidence: 1:25,000–30,000 males; 75–100 are people born with the disease in USA each year. No racial prevalence. Highest prevalence overwhelmingly in males. Perioperative Risks Increased risk of hemorrhagic complications from any procedure. Of affected individuals, 60% have severe disease (<1% normal circulating factor IX). Of carrier females, 10% have abnormal hemorrhage risk. Worry About…