Mod VI: Intra-abdominal Malformations

Question 1

_{Intra-abdominal Malformations}

Faulty separation of primitive trachea and esophagus (commonly occur together)

Answer 1

Esophageal Atresia & Tracheoesophageal Fistula

Question 2

_{Esophageal Atresia & Tracheoesophageal Fistula}

Incidence of Esophageal Atresia & Tracheoesophageal Fistula:

Answer 2

1:4,000 live births

Question 3

_{Esophageal Atresia & Tracheoesophageal Fistula}

What’s the Most common form of Esophageal Atresia & Tracheoesophageal Fistula?

Answer 3

Type IIIB

Location of fistula variable

Question 4

_{Esophageal Atresia & Tracheoesophageal Fistula}

Clinical presentation of Esophageal Atresia & Tracheoesophageal Fistula

Answer 4

Classic triad

Coughing, choking, cyanosis

Drooling

Regurgitation/Aspiration

Respiratory distress

Unable to pass NGT into stomach

Abdominal distention/gas

No abdominal gas: EA w/o fistula

Increased incidence pneumonia H-type

Question 5

_{Esophageal Atresia & Tracheoesophageal Fistula}

Anomalies associated with Esophageal Atresia & Tracheoesophageal Fistula: VACTERL

Answer 5

V: Vertebral (6 lumbar vertebrae, 13 pair ribs)

A: Anal atresia (imperforated anus)

C: Cardiac

T: TracheoEsophageal Fistula

E: Esophageal atresia

R: Renal agenesis

L: Limb defects

Question 6

_{Esophageal Atresia & Tracheoesophageal Fistula}

Picture showing the different types of Esophageal Atresia & Tracheoesophageal Fistula

Answer 6

_{See picture}

Note Type IIIB, the most common presentation

Question 7

_{Esophageal Atresia & Tracheoesophageal Fistula}

Treatment of Esophageal Atresia & Tracheoesophageal Fistula:

Answer 7

Dependent on stability of infant

Surgery*

^{Delay surgery if pneumonia present until lungs improved (antibiotics, O2)}

Gastrostomy tube placed under local

Reduce aspiration

^{NPO - NGT to LS - ↑ HOB - Intubate and MV if severe}

Question 8

_{Esophageal Atresia & Tracheoesophageal Fistula}

Primary surgery for Esophageal Atresia & Tracheoesophageal Fistula involves:

Answer 8

Ligation of fistula with esophageal anastomosis

Question 9

_{Esophageal Atresia & Tracheoesophageal Fistula}

Staged surgery for Esophageal Atresia & Tracheoesophageal Fistula involves:

Answer 9

Gastrostomy with fistula diversion,

_{and later}

Repair of esophagus

Question 10

_{Esophageal Atresia & Tracheoesophageal Fistula}

Anesthetic considerations/management during Induction w/ Esophageal Atresia & Tracheoesophageal Fistula

Answer 10

Prevention aspiration critical

Maintain upright position

Awake suction of proximal pouch prior to induction

Place gastrostomy to water seal if present

AFOI or inhalation induction

^{Maintains SV/avoids need for PPV}

^{Avoid muscle relaxation and PPV with bag/mask}

Question 11

_{Esophageal Atresia & Tracheoesophageal Fistula}

Anesthetic considerations/management during ET tube placement w/ Esophageal Atresia & Tracheoesophageal Fistula

Answer 11

Difficult if TEF present

Goal: Below fistula and above carina

1st = mainstem right bronchus

2nd= withdrawal ET slowly until BBS heard over L thorax

Question 12

_{Esophageal Atresia & Tracheoesophageal Fistula}

Anesthetic considerations/management during Maintenance w/ Esophageal Atresia & Tracheoesophageal Fistula

Answer 12

Inhalation anesthetic with SV until gastrostomy performed

Monitor inspiratory pressures: Avoid High!

Correct F/E disturbances/cont’d resuscitation efforts

Question 13

_{Esophageal Atresia & Tracheoesophageal Fistula}

ET tube placement in Esophageal Atresia & Tracheoesophageal Fistula

Answer 13

See picture

Question 14

_{Intra-abdominal Malformations}

Intra-abdominal malformation characterrized by failed migration of intestine into abdomen & failed closure of abdominal wall @ 6-8 weeks gestation; typically occurs at base of umbilicus and is known as:

Answer 14

Omphalocele

Viscera outside abdominal wall

Intact membrane (amnion)

Question 15

_{Intra-abdominal Malformations - Omphalocele}

Incidence of Omphalocele:

Answer 15

1:6,000

Question 16

_{Intra-abdominal Malformations - Omphalocele}

Associated congenital anomalies w/ Omphalocele

Answer 16

Cardiac lesions (20%)

Exstrophy bladder

Beckwith-Wiedemann syndrome

^{(mental retardation, hypoglycemia, congenital heart dx, large tongue)}

Question 17

_{Intra-abdominal Malformations - Omphalocele}

What does Omphalocele look like at birth?

Answer 17

See picture

Question 18

_{Intra-abdominal Malformations}

A defect of abdominal wall on right lateral aspect of umbilicus w/ failed closure @ 12-18 weeks gestation is known as:

Answer 18

Gastroschisis

Lacks peritoneal coverage, exposed bowel

Highly susceptible to ECF loss and infection

Usually lateral to umbilicus

Question 19

_{Intra-abdominal Malformations - Gastroschisis}

Incidence of Gastroschisis:

Answer 19

1:30,000

Question 20

_{Intra-abdominal Malformations - Gastroschisis}

Why is Gastroschisis more urgent of a surgery?

Answer 20

Risk of fluid loss!!!

Lacks peritoneal coverage, exposed bowel

Highly susceptible to ECF loss and infection

Question 21

_{Intra-abdominal Malformations - Gastroschisis}

T/F: Gastroschisis is a/w Less incidence of concurrent anomalies

Answer 21

True

Although it is associated with prematurity

Question 22

_{Intra-abdominal Malformations - Gastroschisis}

What does Gastroschisis look like?

Answer 22

See picture

Question 23

_{Omphalocele & Gastroschisis}

Goals of Medical stabilization w/ Omphalocele & Gastroschisis include:

Answer 23

Protect defect

Minimize fluid & heat loss

IV hydration

^{Requires large amounts (150 ml/kg/day) of full-strength BSS plus colloids}

Protection of viscera before surgical repair

NGT drainage

Question 24

_{Omphalocele & Gastroschisis}

How should the viscera of Omphalocele be protected before surgical repair in order to prevent increased heat loss?

Answer 24

Cover sac with sterile, warm, saline soaked gauze

Question 25

_{Omphalocele & Gastroschisis}

How should the viscera of Gastroschisis be protected before surgical repair in order to prevent Increased heat & ECF loss, and risk of infection?

Answer 25

Apply warm

Saline soaked gauze to exposed viscera

Wrap infant in warm

Sterile towels

Question 26

_{Omphalocele & Gastroschisis}

For Surgical repair of Omphalocele & Gastroschisis, hat are 2 major concerns with Primary closure?

Answer 26

Ventilation

Circulation

Question 27

_{Omphalocele & Gastroschisis}

For Surgical repair of Omphalocele & Gastroschisis and after primary closure, when is reopening and staged procedure indicated?

Answer 27

Intragastric pressures > 20 mmHg

24 hrs after primary closure

Question 28

_{Omphalocele & Gastroschisis}

For Surgical repair of Omphalocele & Gastroschisis, when is Staged procedure indicated? Describe it!

Answer 28

Staged with Silastic “silo” with larger defects

Silo size reduced every 2-3 days

Spontaneous ventilation maintained with intubation

Monitor O2 saturation, pulses, & BP to determine appropriate reduction size that allows adequate ventilation and circulation

Ketamine 0.5 – 1mg/kg common

Question 29

_{Omphalocele & Gastroschisis}

Silo size reduced every 2-3 days, pushing bowel back inside the abdomen

Answer 29

See picture

Question 30

_{Omphalocele & Gastroschisis}

Silo size reduced every 2-3 days, pushing bowel back inside the abdomen

Answer 30

See picture

Question 31

_{Omphalocele & Gastroschisis}

Silo size reduced every 2-3 days, pushing bowel back inside the abdomen

Abdominal pressure will be decreased prior to the fascia closure

Answer 31

See picture

Question 32

_{Omphalocele & Gastroschisis}

Anesthetic considerations/management w/ Omphalocele & Gastroschisis:

Answer 32

Aspiration risk

^{Induce/intubate in semi-upright position}

^{Decompress stomach before}

^{AFOI indicated}

↑ inspiratory pressures may be necessary d/t inc abd cavivity pressure

^{Consider cuffed ETT}

^{Maintain high suspicion for pneumothorax}

^{SaO2/BP/ETC02}

Muscle relaxation usually required

^{Work w/ surgeon to figure out what the true intraabdominal pressure is}

Avoid N2O

^{To prevent any bowel distension}

Correct F/E disturbance/Hypovolemic shock

^{Monitor CVP and U/O for adequacy of volume resuscitation}

Thermoregulatory instability = warming measures

Question 33

_{Omphalocele & Gastroschisis}

Summary table

Answer 33

See picture

Question 34

_{Intra-abdominal Malformations}

Hypertrophy of pyloric smooth muscle → gradual obstruction of gastric outlet. This condition is also known as:

Answer 34

Pyloric Stenosis

Question 35

_{Intra-abdominal Malformations - Pyloric Stenosis}

When does Pyloric Stenosis present?

Answer 35

2nd or 3rd week of life

Question 36

_{Intra-abdominal Malformations - Pyloric Stenosis}

Clinical presentation of Pyloric Stenosis

Answer 36

Persistent, projectile, nonbilious vomiting

Metabolic disturbance?

^{Hyponatremic, Hypokalemic hypochloremic metabolic alkalosis with compensatory respiratory acidosis}

Intolerance to feeding

Palpable abdominal mass (“olive”)

Gastric dilation via X-ray

Question 37

_{Intra-abdominal Malformations - Pyloric Stenosis}

GI assessment of Pyloric Stenosis

Answer 37

Palpable abdominal mass (“olive”)

Gastric dilation via X-ray

Question 38

_{Intra-abdominal Malformations - Pyloric Stenosis}

Metabolic disturbance a/w Pyloric Stenosis include:

Answer 38

Hyponatremic

Hypokalemic hypochloremic metabolic alkalosis

with compensatory respiratory acidosis

Question 39

_{Intra-abdominal Malformations - Pyloric Stenosis}

Confirmation of Pyloric Stenosis diagnosis

Answer 39

US

Barium swallow

Question 40

_{Intra-abdominal Malformations - Pyloric Stenosis}

What does Pyloric Stenosis look like?

Answer 40

See picture

Note enlarge pylorus causing the stenosis

Question 41

_{Intra-abdominal Malformations - Pyloric Stenosis}

T/F: Pyloric Stenosis is a surgical emergency

Answer 41

False

Pyloric Stenosis is a Medical Emergency = NOT Surgical

Question 42

_{Intra-abdominal Malformations - Pyloric Stenosis}

Correction of F/E deficits in the medical management of Pyloric Stenosis involves:

Answer 42

Resuscitate with full-strength BSS (NS)

Add K+ after infant begins to urinate

Question 43

_{Intra-abdominal Malformations - Pyloric Stenosis}

Which lab values indicate that the infant is adequately prepared and medically optimized for the OR?

Answer 43

Plasma Na+ > 130 mEq/L

Plasma K+ > 3.0 mEq/L

Plasma Cl- > 85 mEq/L

U/O = 1-2 ml/kg/hr

Normal skin turgor/tear production

Question 44

_{Intra-abdominal Malformations - Pyloric Stenosis}

Surgical repair of Pyloric Stenosis inculde:

Answer 44

Pyloromyotomy

Short procedure/small incision

Question 45

_{Intra-abdominal Malformations - Pyloric Stenosis}

Anesthetic considerations/management to address ↑Risk for aspiration w/ Pyloric Stenosis include:

Answer 45

Awake OG/NGT suction in supine & left lateral position

RSI or AFOI

Question 46

_{Intra-abdominal Malformations - Pyloric Stenosis}

Muscle relaxation required at 2 points during surgery to correct Pyloric Stenosis. What are those two points?

Answer 46

Delivery of pylorus at beginning

Return of pylorus at end of surgical procedure

Question 47

_{Intra-abdominal Malformations - Pyloric Stenosis}

Anesthsia technique: Controlled ventilation or Caudal?

Answer 47

Caudal provides needed muscle relaxation,

reduces anesthetic requirements, &

provides postoperative analgesia

Question 48

_{Intra-abdominal Malformations - Pyloric Stenosis}

T/F: Deep extubation is recommended for infants after surgery to correct Pyloric Stenosis

Answer 48

False

Infant should be fully awake and breathing adequately before removing ETT

Question 49

_{Intra-abdominal Malformations}

The developmental abnormality a/w spontaneous abnormal rotation of midgut around mesentery, which could cause complete or partial duodenal obstruction is also known as:

Answer 49

Intestinal Malrotation & Volvulus

Question 50

_{Intra-abdominal Malformations - Intestinal Malrotation & Volvulus}

Incidence of Intra-abdominal Malformations:

Answer 50

1:500 live births

Question 51

_{Intra-abdominal Malformations - Intestinal Malrotation & Volvulus}

What’s the only definitive treatment for Intestinal Malrotation & Volvulus?

Answer 51

Surgery provides only definitive treatment

Midgut volvulus is a Surgical Emergency

Question 52

_{Intra-abdominal Malformations - Intestinal Malrotation & Volvulus}

Anesthetic Considerations w/ Intestinal Malrotation & Volvulus:

Answer 52

Increased risk for aspiration

^{Decompress stomach}

Keep hydrated

Invasive lines may be necessary

Question 53

_{Intra-abdominal Malformations}

T/F: Necrotizing Enterocolitis may appear in the absence of functional or anatomical lesions

Answer 53

True

Question 54

_{Intra-abdominal Malformations - Necrotizing Enterocolitis}

Incidence of Necrotizing Enterocolitis - Age group most affected:

Answer 54

5-15 % of infants

Predominantly in premature infants (90%)

Question 55

_{Intra-abdominal Malformations - Necrotizing Enterocolitis}

Mortality rate in Necrotizing Enterocolitis:

Answer 55

40%

Question 56

_{Intra-abdominal Malformations - Necrotizing Enterocolitis}

Pathogenesis of Necrotizing Enterocolitis:

Answer 56

Immature intestine has ↓ability to absorb substrates→ stasis

→ bacterial infection→ ischemia→ necrosis of intestinal mucosa

→ perforation, gangrene, fluid loss, peritonitis, septicemia, DIC

Question 57

_{Intra-abdominal Malformations - Necrotizing Enterocolitis}

Clinical features of Necrotizing Enterocolitis:

Answer 57

Abdominal distention/ileus

↑gastric aspirate

Bloody stools

Temperature instability

Cardiorespiratory instability

Hypovolemia/oliguria

Metabolic acidosis

Question 58

_{Intra-abdominal Malformations - Necrotizing Enterocolitis}

How does Necrotizing Enterocolitis look like?

Answer 58

See picture

Question 59

_{Intra-abdominal Malformations - Necrotizing Enterocolitis}

Treatment for Necrotizing Enterocolitis is primarily medical and includes:

Answer 59

Cessation of oral intake (NPO for 10-14 days)

Decompression of stomach

F/E therapy

^{Full-strength BSS - Correct metabolic acidosis}

Peritoneal drain

Intubation/ventilation

Question 60

_{Intra-abdominal Malformations - Necrotizing Enterocolitis}

How is Necrotizing Enterocolitis Nonresponsive to medical treatment managed?

Answer 60

Exploratory laparotomy to remove gangrenous bowel

Question 61

_{Intra-abdominal Malformations - Necrotizing Enterocolitis}

Anesthetic considerations/management w/ Necrotizing Enterocolitis

Answer 61

Most challenging cases in pediatric anesthesia

Infants are critically ill, usually already intubated on mechanical ventilation

Continue resuscitative measures

Provide muscle relaxation and careful titration of anesthetic drugs*

Infuse full-strength BSS to maintain BP and U/O

Transfuse blood if HCT <30%

Question 62

_{Intra-abdominal Malformations - Necrotizing Enterocolitis}

Anesthetic agents used in the Tx of Necrotizing Enterocolitis include:

Answer 62

Ketamine 0.5-1ug/kg every 20-30” to start

Fentanyl may be added if condition improves

Small doses of volatile agent if dramatic improvement

Question 63

_{Intra-abdominal Malformations - Necrotizing Enterocolitis}

Which Anesthetic agent must be avoided in the Tx of Necrotizing Enterocolitis include:

Answer 63

N₂O

Nitrous oxide

Question 64

_{Intra-abdominal Malformations - Intestinal Obstruction}

The congenital absence or complete closure of a portion of the lumen of the duodenum that causes increased levels of amniotic fluid during pregnancy (polyhydramnios) and intestinal obstruction in newborn babies is also known as:

Answer 64

Duodenal atresia

Question 65

_{Upper GI tract obstruction - Duodenal atresia}

When does Duodenal atresia become evident?

Answer 65

Within first 24 hrs of life when feeding is initiated

Question 66

_{Upper GI tract obstruction - Duodenal atresia}

Clinical features of Duodenal atresia include:

Answer 66

Bile-stained emesis

Upper abdominal distention

Increased volume of gastric aspirate

Associated with trisomy 21 and other intestinal malformations

Question 67

_{Upper GI tract obstruction - Duodenal atresia}

Anesthetic considerations/management w/ Duodenal atresia

Answer 67

Risk of aspiration: RSI

Ensure adequate relaxation for exploration

Preferred technique: combined GETA with Caudal

Extubation at end of surgery can be anticipated

When in doubt, leave ET in with PEEP

Question 68

_{Upper GI tract obstruction - Duodenal atresia}

What does Duodenal atresia look like?

Answer 68

See picture

Note “Double bubble effect”

Question 69

_{Upper GI tract obstruction - Duodenal atresia}

Another look of what Duodenal atresia looks like:

Answer 69

See picture

Question 70

_{Intestinal Obstruction}

Possible Lower GI tract obstructions include:

Answer 70

Terminal ileum

Imperforate anus

Question 71

_{Intestinal Obstruction - Lower GI tract obstructions}

When do Lower GI tract obstructions become evident?

Answer 71

Between 2 & 7 days of age

Question 72

_{Intestinal Obstruction - Lower GI tract obstructions}

Clinical features of Lower GI tract obstructions include:

Answer 72

Progressive distention

Little or no stool passed

Vomiting

Fluid/electrolyte abnormalities

Enormous amount fluids sequestered into intestinal tract

Question 73

_{Intestinal Obstruction - Lower GI tract obstructions}

Anesthetic considerations/management w/ Lower GI tract obstructions include:

Answer 73

Correct F/E deficits

^{Na+ : >130 - U/O: 1-2 ml/kg/hr}

Avoid N2O

Provide adequate muscle relaxation

^{Accomplished with various anesthetic techniques}

Extubation criteria same as upper GI obstruction

Question 74

_{Pediatric condition that can cause indubation issues}

Small/ underdeveloped mandible (micrognathia or mandibular hypoplasia - can be used interchangeably), a tongue that falls back and downwards (glossoptosis) when supine, a/w Cleft palate, neonate w/ this condition often requires intubation for airway protection. The condition is also known as:

Answer 74

Pierre-Robin Syndrome

Question 75

_{Pediatric condition that can cause indubation issues}

Condition characterized by Small mouth, Small/ underdeveloped mandible, Nasal airway is blocked by tissue (choanal atresia), Ocular and auricular anomalies. This condition is also known as:

Answer 75

Treacher-Collins Syndrome

Question 76

_{Pediatric condition that can cause indubation issues}

Condition characterized by Small mouth, Large tongue, Atlantoaxial instability, Small subglottic diameter (subglottic stenosis). This condition is also known as:

Answer 76

Down Syndrome or Trisomy 21

Question 77

_{Down Syndrome or Trisomy 21}

Airway characteristics in Down Syndrome or Trisomy 21

Answer 77

Small mouth

Large tongue

Palate is narrow with a high arch

Midface hypoplasia

Atlantoaxial instability

C1 & C2 subluxation

^{Use glidescope}

Avoid neck flexion during laryngoscopy

Child should receive cervical x-ray screening between 3-5 years of age

Subglottic stenosis

Increased risk of postintubation croup

Use a smaller ETT

Obstructive sleep apnea on extubation

Chronic pulmonary infection

Question 78

_{Down Syndrome or Trisomy 21}

CV issues a/w Down Syndrome or Trisomy 21

Answer 78

Co-existing congenital heart disease is common

Most common = AV septal defect (endocardial cushion defect)

Second most common = VSD

Bradycardia is very common during sevoflurane induction (tx = anticholinergic)

Low levels of circulating catecholamines

Question 79

_{Down Syndrome or Trisomy 21}

What’s the Most common CV issues a/w Down Syndrome or Trisomy 21

Answer 79

AV septal defect

(endocardial cushion defect)

Question 80

_{Down Syndrome or Trisomy 21}

Other physiologic issues a/w Down Syndrome or Trisomy 21

Answer 80

Intellectual disability

Epilepsy

Strabismus

Low muscle tone (hypotonia)

Hyperflexible joints (careful with positioning)

GERD

Thyroid disease

Increased incidence of leukemia

Question 81

_{Pediatric condition that can cause indubation issues}

Condition characterized by, Small/ underdeveloped mandible, Cervical spine abnormality

Answer 81

Goldenhar syndrome

Start w/ glidescope to minimize amount of neck mobility

Question 82

_{Pediatric condition that can cause indubation issues}

Condition characterized by large tongue

Answer 82

Beckwith Syndrome

Question 83

_{Pediatric condition that can cause indubation issues}

Condition charaterized by Small/ underdeveloped mandible, Laryngomalacia, Strider

Answer 83

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