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Osteogenesis Imperfecta

Risk Incidence: OI occurs in 1:10,000-20,000 live births. Perioperative Risks Owing to the fragility of bones, caution is needed in positioning or transporting these pts. Excessive neck extension may lead to fracture. Scoliosis may cause difficulty with regional anesthesia. High risk for difficult intubation. Temperature control; tendency toward hyperthermia due to a hypermetabolic state. Cardiac events (pts may have cardiac abnormalities). Consider advanced monitoring in case…

Osteoarthritis

Risk Most common type of arthritis with significant disease burden. Globally, affects 9.6% of men and 18% of women ≥60 y old and is ranked as top 11th cause of disability (global years lived with disability). Common presentations are pain, stiffness, and limitation of movement. Most commonly affected joints are the knees (41%), hips (19%), small hand joints (30%), and facet joints in the spine. Perioperative…

Opitz-Frias Syndrome (The G Syndrome)

Risk Overall incidence not reported Very rare congenital disorder Preoperative Risks Very high risk of recurrent pulm aspiration; hypoplasia of both pulm and vascular components of one lung (pulm hypoplasia) High mortality rate in infancy Worry About NM dysfunction of laryngoesophageal apparatus Laryngotracheoesophageal cleft or fistula Difficult tracheal intubation due to assoc craniofacial deformity Assoc congenital anomalies (Htn, hypospadias, wide eyes, cleft lip, cleft palate, cryptorchidism,…

Occlusive Cerebrovascular Disease

Risk Worldwide, 15 million people suffer a stroke each year. Prevalence of stroke in USA: Approximately 3%. Incidence of stroke in USA: 795,000 annually, including 600,000 new cases and 115,000 recurrences. China has the highest rates of mortality (19.9%), followed by Africa and South America. Perioperative Risks Risks for stroke: Cardiac and carotid surgery: CABG = 1–5%; CEA < 3% Noncardiac surgery: Major general surgery 0.08–0.7%;…

Occipital Encephalocele

Risk Most frequent type of encephalocele in North America and Western Europe Incidence in North America: 1:3000–10,000 live births Worse prognosis compared with frontal encephaloceles Perioperative Risks Seizures Hydrocephalus Cranial nerve deficits Poor feeding Spasticity Blindness Worry About Positioning concerns Coexisting congenital anomalies, especially renal and facial Difficult airway Elevated ICP Body temperature changes IV access Blood loss Hemodynamic disturbances Overview Herniation of brain, meninges, and/or…

Obsessive-Compulsive Disorder

Risk Lifetime prevalence is 1–3%, with bimodal onset in childhood and late adolescence/early adulthood Males and females affected equally Perioperative Risks Drug interactions, notably risk of serotonin syndrome in pts on serotonergic agents such as SSRIs and clomipramine Anxiety/panic attacks OCD-related behavior interfering with preop and postop instructions Worry About Serotonin syndrome Coexistent psychiatric illness Anxiety/panic attacks Behaviors triggered by OCD complicating recovery (e.g., noncompliance with…

Noonan Syndrome

Risk Incidence between 1:1000-2500 live births Incidence consistent worldwide Equal distribution between genders Perioperative Risks Airway Cardiovascular Hematologic Infectious Worry About Difficult airway Cardiovascular complications Bleeding Endocarditis Overview Key features include facial anomalies, neck webbing, short stature, chest deformity, spinal deformity (e.g., scoliosis, atlanto-occipital fusion, cervical fusion), congenital heart disease (e.g., pulmonic stenosis, hypertrophic obstructive cardiomyopathy), bleeding diathesis, mental retardation. Congenital heart disease may include pulmonic…

Niemann-Pick Disease

Risk Incidence in live births: 1:100,000-120,000 Affects equally males and females of all ethnic groups NP-D type A frequent in the Ashkenazi-Jewish population No curative therapy, although several symptomatic manifestations are treatable Associated with a decrease in life expectancy, although many pts survive until late adulthood Perioperative Risks NP-D pts require a multitude of diagnostic and therapeutic procedures (e.g., medical imaging, lumbar puncture, intrathecal chemotherapy injection,…

Neuroleptic Malignant Syndrome

Risk Incidence of 0.01–0.02% Mortality rate of 10% 2000 cases of NMS diagnosed annually in USA hospitals Pharmacologic: Typical/“first generation” antipsychotic Rapid dose titration/switching agents/abrupt medication withdrawal/high cumulative dose IM depot/IV administration Multiple concurrent antipsychotics or antipsychotic with lithium/carbamazepine Demographic/miscellaneous: Advanced age Psychiatric/medical comorbidities Anemia Dehydration/malnutrition Pt history of NMS Hot climate/high ambient temperature Perioperative Risks Pulm aspiration Cardiovascular lability Rhabdomyolysis Worry About Potentially life-threatening if…

Neurofibromatosis

Risk NF-1 birth incidence: 1:3000 NF-2 birth incidence: 1:25,000 Schwannomatosis incidence: 1:40,000 Perioperative Risks Depend on tumor extent and location Worry About Difficult intubation Intraop Htn Intraop tachycardia Overview NF-1, formerly known as von Recklinghausen disease, is a relatively common, neurologic genetic disorder with variable clinical presentation. It involves multiple organs, such as the skin and peripheral nervous system, which serve as sites for tumors and…

Nelson Syndrome

Risk Incidence: Reported in 8–44% of pts following bilateral adrenalectomy for Cushing disease More likely in pts with younger age and pregnancy Perioperative Risks Lyte imbalances DI Vision loss Challenges specific to type of surgery Worry About Panhypopituitarism Volume status imbalance Steroid supplementation Overview The first case of Nelson syndrome was reported in 1958 by Dr. Del Nelson, who named this condition. It is also known…

Necrotizing Fasciitis

Risk Incidence in USA: Approximately 9–11.5 cases of invasive streptococcal infections, from which 1–1.8 die each year STSS and NF, each comprising an average of 6–7% of these invasive cases, with an associated mortality of 35–50% for STSS and 29% for NF Predisposing risk factors: Diabetes, peripheral vascular disease, alcoholism, IV drug abuse, immunosuppression, obesity, or malnourishment Perioperative Risks Shock, hypoperfusion, organ dysfunction, and hyperglycemia or…

Necrotizing Enterocolitis

Risk Most common life-threatening intestinal surgical emergency in the newborn. Occurs predominantly in premature infants, with 75% in infants weighing <1500 g. Increasing incidence in term and near-term neonates as well. Perioperative Risks CV instability, acidosis, shock, bowel ischemia, bacteremia, patent ductus arteriosus, polycythemia Worry About Persistent metabolic acidosis and intestinal perforation are ominous signs. You’re Reading a Preview Become a Clinical Tree membership for Full…

Narcolepsy

Risk Prevalence approximately 1:2000 Women and men equally affected Prevalence higher in Japan (1:600) Perioperative Risks Potential for masking or mimicking periop complications Potential deleterious interactions between drugs for narcolepsy and drugs administered periop Worry About There is little evidence that pts with narcolepsy actually have an increased periop risk. Theoretical concerns are: Potential drug interactions with anesthetics leading to Hemodynamic changes. Altered anesthetic requirements. Increased…

Myxoma

Risk Although primary cardiac tumors are rare (<0.01%), myxoma is the most common type (50%). 75% develop in LA, with most attached to the interatrial septum. Rarely develop in ventricles. More common in females (70%). Perioperative Risks May be friable and may embolize (30–40% of pts) LV- or RV-inflow obstruction with resultant hypotension May simulate pulm Htn and/or constrictive pericarditis physiology Worry About Hypotension due to…

Myotonia Dystrophica (Myotonic Dystrophy, Steinert Disease)

Risk Incidence of approximately 1:8000. Incidence of the congenital form is higher, with an incidence of 1:100,000 compared with adult-onset form. Perioperative Risks Operative/anesthetic and postop morbidity and mortality are increased and not proportional to severity of disease. High incidence of cardiopulmonary complications, including sudden death, cardiac failure, and cardiomyopathy. Worry About Increasing frequency of symptoms Signs of respiratory or cardiac decompensation Overview Degenerative disease of…

Myoclonic Epilepsy With Ragged Red Fibers

Risk Prevalence: 1:400,000 Maternal inheritance; less commonly a spontaneous mitochondrial gene mutation in those without family Hx Perioperative Risks Lactic acidosis Cardiac/respiratory insufficiency/failure Delayed emergence Worry About Respiratory failure following sedation. Consider aspiration risk. Lactic acidosis. Seizures. Overview Mitochondrial myopathy most commonly characterized by cerebellar ataxia, myoclonus, epilepsy, lactic acidosis, hearing loss, peripheral neuropathy, and short stature. Excess lactic acid load leads to nausea, vomiting, abdominal…

Myocarditis

Risk Incidence of idiopathic or viral myocarditis in the general population is unknown. Infectious and noninfectious causes; viruses are the most common. Pts with autoimmune diseases are at increased risk. Perioperative Risks If pt develops DCM: EF <35% increased risk of MACE. Atrial arrhythmia if significant mitral regurgitation is present. Postop respiratory failure secondary to pulm edema. Worry About Acute cardiovascular decompensation. New-onset atrial or ventricular…

Myocardial Ischemia

Risk Incidence in USA: 1.5 million/y develop acute MI; about 50% are silent (without enough symptoms to cause a medical visit); decreased rate of death in the United States balanced by increased population has kept MI numbers constant since 1970 despite increased population; worldwide, the incidence of MI is 9 million Some 12 million individuals in USA have narrowing of 70% or more of one or…

Myocardial Contusion (Blunt Cardiac Injury)

Risk Incidence unknown, in part due to absence of clear diagnostic criteria/test 2 million motor vehicle accidents/y, with ∼40% involving closed chest injury 20–70% incidence by clinical criteria 16–20% incidence by autopsy Motor vehicle > falls > crush injuries Males >females (5:1) Commotio cordis a rare form of BCI due to low impact chest injury (sports) causing sudden death Perioperative Risks Abnormal ECG Nonspecific ST-T wave…