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in biliary atresia liver function is down. thus, the pre-op evaluation and correction of what is important?

coagulolpathy -vit k may be given 1-2 days before the scheduled procedure


Congenital lobar emphysema is the postnatal over distention of an otherwise normal lobe of the lung that compresses

adjacent normal lung units and leads to atelectasis


in congenital diseases that lead to air trapping, what element of anesthesia management is avoided in their management?

positive pressure would cause barotrauma and collapse of alveoli


o The recommended induction technique for infants with either congenital lobar emphysema or congenital cystic adenomatoid malformations is

inhalation with sevo and oxygen -w/o, if possible, any positive pressure


for infants with either congenital lobar emphysema or congenital cystic adenomatoid malformations maintenance anesthesia plan includes:

spontaneous breathing with minimal pos. airway pressures


for infants with either congenital lobar emphysema or congenital cystic adenomatoid malformations at induction WHO should be present ?

surgeon should be at induction in the event sudden decompensation warrants urgent thoracotomy


Meticulous attention must be paid to avoidance of nerve and other compression injuries during positioning *** (usually lateral decubitus with the infant's ipsilateral arm placed directly over the head) for infants with what disease processes (2);

for infants with either congenital lobar emphysema or congenital cystic adenomatoid malformations


most common type of cerebral palsy is

spastic cerebral palsy


Administration and dosing of muscle relaxants should be done with caution, because these patients generally have prolonged recovery from neuromuscular blockade. this is true for what patients?

those with CP


CP is commonly associated with

seizure d/o -some children may be given antiepileptic medications even in the absence of seizures for tx of spasticity and behavior


what medication is an important adjunct in managing pain r/t muscle spasm?



hydrocephalus results from

overproduction impaired circulation **underabsorption of CSF


The most common cause of congenital hydrocephalus is

impaired circulation or obstruction of flow caused by structural abnormalities such as stenosis of the aqueduct of Sylvius, tumors, malformations (Chiari's malformation, Dandy-Walker malformation), and trauma-related defects


although this remains controversial in children with congenital hydrocephalus, Medical therapy mainly consists of

diuretic treatment (furosemide and acetazolamide decrease CSF production),


Volatile agents are potent cerebral vasodilators and increase ICP by

increasing cerebral blood flow *** (Isoflurane does it the least)


in patients with hydrocephalus what should our anesthesia mgmt plan consider (in regards to ICP)?

-KETAMINE is contraindicated -Volatiles are potent cerebral vasodilators and increase ICP by increasing CBF. -Succ may be used (transient and attenuated by "defasciulating" dose)


for the patient with spina bifida, a comprehensive preoperative assessment is needed to identify specific anesthetic risks, because myelomeningoceles are often associated with

other congenital anomalies ***


In the case of spina bifida, Tracheal extubation must take place only after the patient has

regained adequate spontaneous respiratory effort and ventilation ***


Craniosynostosis is defined as premature closure of

one or more cranial sutures


Four major sutures separate these bone plates: ***

(1) metopic suture separates the frontal bones (2) sagittal suture separates the parietal bones (3) coronal suture separates the frontal from the parietal bones (4) lambdoid suture separates the parietal bones from the occipital bone


in the management of craniosynostosis, should N2O be used or avoided?



for crainiosynostosis what should be available, in the OR, before skin incision

blood products


feeding difficulty is universal with this anomaly

cleft lip & palate


Cleft lip repair is typically performed between 6 and 12 weeks of age, whereas cleft palate repair is done at

a later age, between 9 and 14 months ***


A universal feature in congenital mandibular hypoplasia is airway compromise resulting from the constricted

mandibular space with resultant glossoptosis (posterior prolapse of the tongue) ***


Pierre Robin , consists of three congenital orofacial abnormalities:

1. micrognathia (small mandible) or retrognathia (posterior displacement of mandible), 2. glossoptosis, 3. cleft palate (present in almost all cases)


Hemifacial microsomia is the one of the most common congenital facial anomalies (second only to cleft lip and palate) It is a facial asymmetry disorder that _____ affects bone, muscle, and soft tissue structures



Treacher Collins syndrome is a rare craniofacial anomaly with an autosomal dominant disorder mode of inheritance and variable expression There is _____and ______ underdevelopment of structures deriving from the first and second pharyngeal arch, groove, and pouch leading to hypoplasia of the supraorbital rims, zygoma, midfacial bones, and mandible as well as ear deformities and cleft palate

bilateral and symmetric


Patients with mandibular hypoplasia, especially in association with Treacher Collins syndrome and hemifacial microsomia, not only are difficult to intubate but may be nearly impossible

to mask ventilate ***


The classic presentation of acute epiglottitis is that of a toxic-appearing, agitated child with a high fever and the so-called 4 Ds:

dysphagia, dysphonia, dyspnea, and drooling