Preoperative Assessment of Newborns and Children

Question 1

When is indicated prophylaxis for endocarditis?

Answer 1

• Prosthetic valve material
• Previous endocarditis
• Some cases of congenital heart diseas
• Heart transplant with valvulopathy

In case of invasive dental procedures, respiratory procedures with manipulation of mucosa, procedure on infected tissues

Question 2

Brief description and anesthetic implications of Arthrogruposis multiplex

Answer 2

Description: multiple congenital contractures. congenital heart disease

Anesthetic implications: possible airway problems due to limitations of mandibular movements

Question 3

Brief description and anesthetic implications of Beckwith syndrome

Answer 3

Description: Birth weight > 4000 g; macroglossia, visceromegaly

Anesthetic implications: Airway problems due to large tongue; hypoglycemia common

Question 4

Brief description and anesthetic implications of Asplenia syndrome

Answer 4

Description: asplenia. Complex congenital heart disease. Malrotation of abdominal organs

Anesthetic implications: Cyanotic congenital heart disease is common. Preoperative echocardiography is needed

Question 5

Brief description and anesthetic implications of Cherubism

Answer 5

Description: asplenia. Fibrous dysplasia of the mandible and maxilla

Anesthetic implications: Intubation may be extremely difficult; tracheostomy may be the only way to secure the airway

Question 6

Brief description and anesthetic implications of Congenital hypothyroidism

Answer 6

Description: Goiter; large tongue; respiratory depression; hypoglycemia; hypotension

Anesthetic implications: Airway obstruction secondary to large tongue, particularly in supine position; slow to awaken at the completion of surgery

Question 7

Brief description and anesthetic implications of Crouzon disease

Answer 7

Description: Craniosynostosis, hypertelorism, hypoplastic mandible

Anesthetic implications: Intubation may be difficult

Question 8

Brief description and anesthetic implications of Dandy-Walker syndrome

Answer 8

Description: Hydrocephalus

Anesthetic implications: Increased intracranial pressure rare in the newborn period; head may be enormously enlarged

Question 9

Brief description and anesthetic implications of DiGeorge syndrome

Answer 9

Description: Thymus and parathyroids absent; hypocalcemia, immune deficiency; aortic arch abnormalities

Anesthetic implications: Irradiate all blood products to prevent graft-versus-host disease; stridor may be due to hypocalcemia

Question 10

Brief description and anesthetic implications of Down syndrome (trisomy 21)

Answer 10

Description: Large tongue, unstable cervical spine, small mouth; high incidence of congenital heart disease, particularly atrioventricular canal; intestinal obstruction

Anesthetic implications: Intubation may be difficult; ? inline traction during intubation, ? cervical spine films prior to intubation in older children; echocardiography required
prior to surgery in the newborn

Question 11

Brief description and anesthetic implications of Ehlers-Danlos

Answer 11

Description: Collagen abnormality-hyperelasticity and fragile tissue; dissecting aneurysm of aorta; bleeding diathesis; heart, lung, GI problems

Anesthetic implications: Poor tissue and clotting defects may lead to hemorrhage; spontaneous pneumothorax

Question 12

Brief description and anesthetic implications of Ellis–van Creveld syndrome

Answer 12

Description: Ectodermal and skeletal defects; congenital heart disease; cleft lip and palate, mandibular hypoplasia; hepatosplenomegaly

Anesthetic implications: Airway problems, intubation may be difficult; chest wall anomalies cause poor lung function

Question 13

Brief description and anesthetic implications of Epidermolysis bullosa

Answer 13

Description: Skin cleavage at dermal-epidermal junction, minor trauma denudes skin

Anesthetic implications: Do not use adhesive tape of any sort; avoid instrumentation of the airway if possible; use a well-padded mask or apply ointment to rim; secure IV,
monitoring devices with Kerlix; sterile technique

Question 14

Brief description and anesthetic implications of Familial dysautonomia (Riley-Day syndrome)

Answer 14

Description: Poor suck and swallow; hyper- and hypotension; insensitivity to pain; absent sweating and lacrimation

Anesthetic implications: Recurrent aspiration and pneumonia; respiratory center insensitive to CO2; labile intraoperative blood pressure

Question 15

Brief description and anesthetic implications of Fetal alcohol syndrome

Answer 15

Description: Growth retardation; microcephaly, craniofacial abnormalities; congenital heart disease; renal abnormalities

Anesthetic implications: Intubation is usually not difficult; ventricular septal defects are common and require prophylaxis for subacute bacterial endocarditis

Question 16

Brief description and anesthetic implications of Glucose-6-phosphate deficiency

Answer 16

Description: Hemolytic anemia caused by drugs and infection

Anesthetic implications: Aspirin, sulfa, methylene blue cause anemia

Question 17

Brief description and anesthetic implications of Goldenhar syndrome

Answer 17

Description: Hemifacial microsomia, congenital heart disease

Anesthetic implications: Very difficult intubation, vertebral instability

Question 18

Brief description and anesthetic implications of Hemangioma with thrombocytopenia (Kasabach–Merritt syndrome)

Answer 18

Description: May involve the airway; bleeding, anemia

Anesthetic implications: Airway involvement may require radiation therapy; transfuse components as necessary

Question 19

Brief description and anesthetic implications of Jeune syndrome (asphyxiating thoracic dystrophy)

Answer 19

Description: Severe thoracic malformations, renal failure

Anesthetic implications: Respiratory failure, prolonged mechanical ventilation; care with drugs excreted by kidneys

Question 20

Brief description and anesthetic implications of Klippel-Feil syndrome

Answer 20

Description: Hemi or fused vertebra

Anesthetic implications: Intubation may be difficult

Question 21

Brief description and anesthetic implications of Maple syrup urine disease

Answer 21

Description: Inability to metabolize leucine, isoleucine, and valine

Anesthetic implications:Acid-base imbalance; avoid preoperative fasting, start glucose early and check frequently

Question 22

Brief description and anesthetic implications of Mucopolysaccharidoses (Hurler, Hunter, Morquio syndrome)

Answer 22

Description: Bony abnormalities, dwarfism, kyphoscoliosis, abnormal facies, congenital heart disease

Anesthetic implications: Very difficult intubations, unstable necks, respiratory failure perioperatively

Question 23

Brief description and anesthetic implications of Myasthenia congenita

Answer 23

Description: muscle weakness and fatigue

Anesthetic implications: Avoid muscle relaxants and narcotics

Question 24

Brief description and anesthetic implications of Osteogenesis imperfecta

Answer 24

Description: Pathologic fractures; abnormal platelets, vascular fragility

Anesthetic implications: Extreme caution when positioning and during intubation; blood pressure cuff may cause fractures

Question 25

Brief description and anesthetic implications of Pierre Robin syndrome

Answer 25

Description: Cleft palate, micrognathia, glossoptosis, congenital heart disease

Anesthetic implications: Very difficult intubation, may require tongue suture or awake tracheostomy; best nursed in prone position

Question 26

Brief description and anesthetic implications of Prader-Willi syndrome

Answer 26

Description: Hypotonia, obesity

Anesthetic implications: Hypoglycemia common; assisted ventilation may be required postoperatively

Question 27

Brief description and anesthetic implications of Prune-belly syndrome

Answer 27

Description: Agenesis of the abdominal musculature, renal failure

Anesthetic implications: Respiratory failure common, postoperative ventilation, avoid respiratory depressants; avoid drugs excreted by the kidneys

Question 28

Brief description and anesthetic implications of Treacher Collins syndrome

Answer 28

Description: Micrognathia, midface hypoplasia, congenital heart disease

Anesthetic implications: Very difficult intubation, may require tongue suture or awake tracheostomy; best nursed in prone position

Question 29

Brief description and anesthetic implications of Thrombocytopenia with absent radius syndrome

Answer 29

Description: Episodic thrombocytopenia precipitated by stress, infection, surgery; congenital heart disease

Anesthetic implications: Platelet transfusions prior to surgery; prophylaxis for subacute bacterial endocarditis

Question 30

Brief description and anesthetic implications of Trisomy 18 (Edwards syndrome)

Answer 30

Description: Congenital heart disease; micrognathia; renal malformations; most die in infancy

Anesthetic implications: Ethical considerations concerning surgery in a patient with a fatal anomaly;
assess cardiac status carefully

Question 31

Brief description and anesthetic implications of VATER syndrome

Answer 31

Description: Vertebral, anal, tracheal esophageal fistula, renal, cardiac

Anesthetic implications: Examine carefully for associated anomalies

Question 32

Epiglottis difrerences between infanta and adults

Answer 32

In infants the epiglottis tends to be relatively larger, longer, curvier (omega shape) and floppier

Question 33

Describe the 1-2-2/7-8-9 rule

Answer 33

Patients weighing 1, 2 and 3 Kg Will generally need their endotracheal tube secured 7, 8 and 9 cm at the lips

Question 34

Full-term and premature neonates demonstrate depressed respiratory CO2 response curves and secondary apnea in response to hypoxia

T or F

Answer 34

T

Question 35

Acidosis, hypoxia and hypercarbia increase pulmonary resistence, potencially reversing transitonal circulation and restoring fetal patterns, a condition known as …

Answer 35

Persisten Fetal Circulation

or

Persisten Pulmonary Hypertension of the Newborn

Question 36

How can hypoxia induce bradycardia in newborns?

Answer 36

Chemoreceptors located in the aortic arch and carotid bodies are stimulated by acute hypoxia and drecreased cardiac output normally inducing combined sympathetic and vagal responde, causing tachypnea, bradycardia and peripheral vasoconstriction.

In premature infants, a biphasic response with secondary respiratory depression, potencially to the poitn of apnea and often associated with exatreme bradycardia, may occur

Question 37

Cerebral palsy and Duchene muscular dystrophy are contraindindications for succinylcholine

T or F

Answer 37

F

Succinylcholine is not contraindicated in Cerebral Palsy

Question 38

Name 3 conditions that increase sensitivity to nondepolarizing agents

Answer 38

Myasthenia gravis
Duchene muscular dystrophy
Spinel muscle atrophy

Question 39

Name 3 conditions that decreased the sensitivity to nondepolarizing agents

Answer 39

Burns
Myotonic dystophy
Spastic cerebral palsy

Question 40

Which gene coding mutation is associated with malignant hyperthermia in patients with some types of neuromuscular diseases?

Answer 40

RYR1 - gene coding for ryanodine receptor

Question 41

Why are neonates, particularly premature infants, highly sensitive to supplemental fluid and readily become hypervolemic with overly generous ressuscitation?

Answer 41

Becouse they have relatively low glomerular filtration rate and decreased urine-concentrating capacity

Question 42

How is hypoglicemia in neonates defined?

Answer 42

Glicemia < 45 mg/dL in symptomatic neonates
Glicemia < 35 mg/dL in assymptomatic neonates

Question 43

Neonates that are anesthetized with volatile agentes are at great risk of developing hypothermia because this agentes …

Answer 43

Decreased that brown fat metabolism

Question 44

Name the risk factors for emergence delirium in children

Answer 44

• Toddler age
• Male sex
• Use of volatile agents
• Rapid emergence
• Certain types of surgery (particularly oropharingeal and ofthalmologic surgery)